Sex cord stromal tumors
Neoplasms containing epithelial elements of sex cord origin (Sertoli and granulosa cells) admixed with elements of mesenchymal origin (Leydig and theca-lutein cells) in varying combinations and degrees of differentiation
4% of testicular tumors; almost all are immunoreactive for alpha inhibin except fibromas, myxomas and sclerosing stromal tumors
References: Hum Path 1998;29:840
Classification:
Fibromas
Granulosa cell tumor (adult, juvenile)
Leydig (interstitial) cell tumor
Mixed or Unclassified Gonadal-Stromal Tumors
Sertoli cell tumors
Tumors of Adrenogenital Syndrome Type
Rare, benign behavior
Ages 21-74 years, painless testicular masses
Two types – resembling ovarian fibroma and resembling solitary fibrous tumor of pleura
Ovarian fibroma-like: probably derived from testicular stromal cells
Solitary fibrous tumor-like: may occur in relation to tunica albuginea or paratestis, case report at Archives 1992;116:277
Case report with focal sex cord component (inhibin+, CD99+), Archives 1999;123:391
Gross: circumscribed, whorled white nodules, arise from tunica albuginea, may extend into testis or paratestis, 1-4 cm
Micro: moderately cellular, bland spindle cells without atypia, stroma is myxoid, vascular or collagenous; ovarian-type have storiform pattern and hyaline fibrous plaques; solitary fibrous type have random spindle cells
Positive stains: vimentin, CD34 in solitary fibrous-like tumors
Negative stains: S100, keratin, desmin, actin
DD: nodular and profuse fibrous proliferation (less cellular, more inflammatory cells)
References: AJSP 1997;21:296
Resembles analogous ovarian tumor
Anti-mullerian hormone immunostaining is specific for Sertoli and granulosa cells and gonadoblastomas (vs. Leydig cells, other tumor types), although may be positive in only a few cells, Hum Path 2000;31:1202
Adult form
Rare, age 20-53 years
Usually non functional; may be associated with gynecomastia
Usually benign; metastases in 10-20% (associated with size >7 cm, hemorrhage, necrosis, angiolymphatic invasion)
Gross: yellow, homogenous, well circumscribed
Gross/micro images: image1
Micro: microfollicular pattern with Call-Exner bodies (pseudorosettes, pseudotubules); also diffuse, trabecular, insular, macrofollicular, gyriform, solid, cystic or gyriform patterns; uniform round cells with scant cytoplasm, angular grooved nuclei; focal atypia, rare mitoses
Micro images: image1, image2, image3, image4
Positive stains: CK 8/18, vimentin, SMA (smooth muscle actin), inhibin, CD99, S100
Negative stains: CD45/LCA, EMA, keratin, mucicarmine
DD: carcinoid tumor
References: Archives 2000;124:1525, Hum Path 1993;24:1120, Mod Path 1997;10:693
Juvenile form
Most common neonatal testicular tumor; 6% of childhood testicular tumors
Average age of onset is less than 1 month; may be congenital
Associated with trisomy 12
Associated with sex chromosome mosaicism if abnormal external genitalia, AJSP 1994;18:316, AJSP 1986;10:577, AJSP 1985;9:737
No association with endocrine manifestations
No metastases, no local recurrences (after orchiectomy)
Gross: mostly cystic, partially solid
Micro: variable sized follicles and amphophilic cystic fluid; spindled smooth muscle and theca cells, polygonal granulosa cells that may appear luteinized; cells have eosinophilic cytoplasm, hyperchromatic nuclei, no grooves; tumor may infiltrate, be densely cellular, have numerous mitoses; cystic fluid is mucicarmine positive
Positive stains: vimentin, low molecular weight cytokeratin, actin, desmin, CD99
EM: granulosa cells with continuous basal lamina, cytoplasmic filaments with evenly distributed dense bodies resembling smooth muscle, AJSP 1996;20:72
References: AJSP 1985;9:87, Archives 1988;112:1129
1-3% of testicular tumors, 3% are bilateral
Any age, mostly 20-60 years old
Often secrete androgens, estrogens or corticosteroids; patients may present with gynecomastia or other feminizing symptoms (Hum Path 1977;8:621), or with precocious puberty without spermatocytic maturation
10% in adults have malignant behavior with metastases to lymph nodes, lung, liver; usually are large (> 5 cm) with necrosis, vascular invasion, nuclear atypia, numerous mitoses, atypical mitotic figures, infiltrative margins, aneuploid, higher MIB-1 activity (AJSP 1998;22:1361)
Case reports: Case of Week #122, 43 year old man with 2 primary malignant Leydig cell tumors (Hum Path 1997;28:1318), metastases to perirenal fat 17 years later (Archives 1999;123:1104)
Treatment: orchiectomy, lymph node dissection if malignant, possibly testis-sparing surgery for young men (Int J Clin Pract 2003;57:912)
Gross: solid, well circumscribed nodules 5 cm or less, distinct golden-brown homogenous cut surface; 10% have extratesticular extension
Micro: sheets, nests, ribbons or cords of large, round/polygonal cells with defined cell borders, eosinophilic cytoplasm and round central nuclei; also vacuoles, lipofuscin or Reinke crystals (35%); may have endocrine atypia; occasionally adipose differentiation, which should not be confused with extratesticular extension (AJSP 2002;26:1424); no/rare mitotic activity
Unusual features are spindle cells, pseudoglandular structures, microcystic change, small cells with scanty cytoplasm, myxoid degeneration, calcification and ossification
Micro image: various images; image #1; #2; #3; Reinke crystals #1; #2; inhibin; cytokeratin; metastases to perirenal fat - #1; #2; #3
case of week - #1; #2; #3; #4; inhibin
contributed by Dr. Mowafak Hamodat, Eastern Health of Newfoundland and Labrador, St.
John’s, Canada- #1; #2; #3; pan-keratin
#1; #2;
calretinin
#1; #2;
inhibin,
synaptophysin,
vimentin
contributed by Dr. Kristine Cornejo, University of Massachusetts Medical Center - #1; #2; #3; #4; #5; inhibin; Mart1; Melan-A; pan-keratin; PLAP
Cytology images: #1;
#2;
#3
Positive stains: steroid hormones, vimentin, inhibin, calretinin,
MelanA, keratin (variable)
Negative stains: anti-mullerian hormone, S100 (usually)
EM: abundant smooth endoplasmic reticulum, mitochondria with tubulovesicular cristae, Reinke’s crystals (long tapered crystals)
DD: nodular Leydig cell hyperplasia (associated with cryptorchidism, usually 1 cm or less, multifocal, does not destroy surrounding tubules), large cell calcifying Sertoli cell tumor (usually multifocal, bilateral, more stroma, calcifications, intratubular growth, no Reinke’s crystals, slightly different immunostaining pattern, Pathol Int 2005;55:366), testicular tumors of adrenogenital syndrome (bilateral, multifocal, clinical symptoms, laboratory findings, shrink after corticosteroid therapy)
References: AJSP 1985;9:177, Archives 2007;131:311, eMedicine
Gonadoblastoma
Almost always associated with pure or mixed gonadal dysgenesis or male pseudohermaphroditism (intersex syndrome)
80% are phenotypic females
20% are phenotypic males, present with gynecomastia, hypospadias, cryptorchidism; usually 46 XY or 45X/46XY
1/3 bilateral
Considered by some an in situ form of malignant germ cell tumor, which may progress to seminoma or embryonal carcinoma
Treatment: bilateral gonadectomy, curative if no invasive component
Gross: tan-yellow with diffuse gritty calcifications
Micro: hyaline bodies surrounded by seminoma-like cells, sex cord cells and calcifications; 2/3 have aggregates of Leydig-like cells
Positive stains: anti-mullerian hormone
EM: some cells show Charcot-Bottcher filaments of Sertoli cells
References: Hum Path 1986;17:531
Not gonadoblastoma
Rare, adults ages 30-69 years
Not associated with gonadal dysgenesis or male pseudohermaphroditism (intersex syndrome)
No metastases
Treatment: orchiectomy
Gross: gray-white, solid/cystic
Micro: seminoma-like cells, sex-cord like cells resemble Sertoli or granulosa cells; no degenerative changes of gonadoblastoma (i.e. no calcifications)
DD: sex cord stromal tumors with entrapped germ cells (AJSP 2000;24:535, germ cells usually at periphery and in clusters, resemble spermatogonia with round nuclei, uniform dusty chromatin, indistinct nucleoli, negative stains for PLAP, inhibin, glutathione-S-transferase
Mixed sex cord stromal elements or undifferentiated features
15% associated with gynecomastia
50% in children
Benign behavior in children; malignant behavior in 20% of older patients
May resemble granulosa cell tumors
Poor prognostic factors: large size, invasive growth pattern, angiolymphatic invasion, nuclear atypia, mitotically active, necrosis
Micro: usually relatively short spindle cells with prominent nuclear grooves and intermixed epithelioid cells, located adjacent to rete testes; may resemble smooth muscle; reticulin surrounds aggregates of cells but not individual cells
Micro images: image1
Positive stains: S100, smooth muscle actin
EM: desmosomes, numerous thin filaments, focal dense-bodies
DD: sex cord stromal tumors with entrapped germ cells (germ cells usually at periphery and in clusters, resemble spermatogonia with round nuclei, uniform dusty chromatin, indistinct nucleoli, negative stains for PLAP, inhibin, glutathione-S-transferase)
References: AJSP 2000;24:535, Mod Path 1997;10:693
Sertoli cell only or mixture with granulosa cell tumors
Anti-mullerian hormone immunostaining specific for Sertoli and granulosa cells and gonadoblastomas (vs. Leydig cells, other tumor types), although may be positive in only a few cells, Hum Path 2000;31:1202
Case reports of feminizing tumors (gynecomastia, rapid growth, advanced bone age) in 2 boys with Peutz-Jeghers syndrome, AJSP 1995;19:50
Case report with extensive heterologous sarcomatous component, Archives 1998;122:907
Seen in 50% with cryptorchid testes, 20% of autopsy testes
Micro: small sclerotic tubules with interstitial fibrosis; tubules with only Sertoli cells are called “Picks” adenoma, but are not neoplastic
Sertoli cell proliferations of infantile testis
Strongly associated with Peutz-Jeghers syndrome (67%), gynecomastia (80%)
Often bilateral
1 of 6 cases associated with large cell calcifying Sertoli cell tumor
May represent proliferative lesions with neoplastic potential or the intraepithelial stage of some Sertoli cell tumors, AJSP 2001;25:1237
Micro: tubules with large and proliferative Sertoli cells replacing germ cells, but limited by basement membrane; basement membranes often thickened and invaginated; no mitoses or atypia
Positive stains: inhibin
Associated with testicular feminization/androgen insensitivity syndrome
Resembles sex cord stromal tumors with annular tubules seen in Peutz-Jeghers syndrome
Benign
Micro: elongated seminiferous tubules lined by Sertoli like cells
Also called androblastoma
1/3 present with gynecomastia without virilism (in contrast to childhood Leydig cell tumors which present with gynecomastia AND virilism)
10% malignant (to local lymph nodes); prognostic factors for malignancy include nuclear pleomorphism, large size (> 5 cm), mitoses, necrosis, nuclear atypia, angiolymphatic invasion
Treatment: orchiectomy (radiation and chemotherapy have little effect)
Gross: firm small nodules, well circumscribed, homogenous gray-white to yellow, focally cystic or hemorrhagic
Micro: trabeculae or cords resembling immature seminiferous tubules lined by Sertoli-like cells; solid pattern resembles seminoma; may have prominent lymphoid aggregates; may have acellular or vascular fibrous stroma; tumor cells with moderate pale to lightly eosinophilic cytoplasm, often large cytoplasmic vacuoles, usually minimal nuclear atypia, minimal mitotic activity
Positive stains: vimentin, cytokeratin AE1/AE3, alpha-1-antitrypsin, neuron specific enolase, inhibin (variable), EMA, S100 (weak if present)
Negative stains: PLAP
EM: cells interconnected by desmosomes; abundant smooth ER, lipid droplets, Charcot-Bottcher filaments
DD: seminoma with tubular pattern (intratubular germ cell neoplasia present, PLAP+, inhibin-, larger and more pleomorphic nuclei, more mitotic activity, AJSP 2002;26:541), spermatocytic seminoma, androgen insensitivity syndrome (hamartomatous nodules of small tubules lined by Sertoli cells, Archives 1999;123:225
References: AJSP 1998;22:709
Very rare, adults (mean age 30-35 years, range 18-80 years)
Painless testicular mass, no hormonal manifestations
Indolent (no metastases)
Not associated with Peutz-Jeghers syndrome
Gross: small (< 2 cm), well circumscribed, hard, yellow-white-tan
Micro: tubules, cords, nests of Sertoli like cells in hypocellular, markedly fibrous stroma; tumor cells have pale cytoplasm with occasional lipid vacuoles; may have mitoses, atypia
Positive stains: keratin, vimentin, SMA
Negative stains: PLAP
DD: adenomatoid tumor, metastatic carcinoma
Reference: AJSP 1991;15:829
Patients usually under age 20
Part of Carney syndrome with testicular Leydig cell tumors, pituitary tumors, pigmented nodular hyperplasia of adrenal cortex, myxomas of skin, soft tissue, heart and breast; spotty skin pigmentation (Peutz-Jeghers syndrome)
Gynecomastia is common clinical presentation
Usually benign
Calcification may be related to strong S100 staining (S100 binds calcium), Hum Path 2002;33:285
Features associated with malignancy: age >35, size > 4 cm, no association with a syndrome, unilateral, unifocal, extratesticular spread, 4+ mitoses/10 HPF, significant atypia, necrosis, angiolymphatic invasion (1 feature is suspicious for malignancy, 2 features suggests malignant behavior is likely)
Gross: 25% bilateral and multifocal; well circumscribed, white-tan cut surface, <2 cm
Gross images: image1, image2, image3
Micro: sheets, nests, cords and solid tubules of cells with abundant eosinophilic cytoplasm separated by fibrous tissue with marked calcification; usually marked neutrophilic infiltration
Micro images: image1, image2, image3, image4, image5, image6, S100
Positive stains: S100 (strong and diffuse), vimentin
Negative stains: keratin (usually), EMA, AFP, hCG, SMA
EM: Charcot-Bottcher crystals
References: AJCP 1991;96:717, AJSP 1997;21:1271
Rare, 6 cases identified
2 of 6 had gynecomastia, one clinically malignant
Gross: yellow, solid, lobulated
Micro: definite Sertoli cell pattern, neoplastic Leydig cells, sex cord patterns of ovarian Sertoli-Leydig cell tumor
Positive stains: CD99 (Mod Path 1998;11:769)
May be hyperplasia of ectopic adrenal cells, not a neoplasm
Palpable bilateral masses in testes of young adults
2/3 have salt-wasting adrenogenital syndrome
Gross: hilar, bilateral, well circumscribed brown-green masses, separated into lobules by dense fibrous bands
Gross/micro images: image1
Micro: sheets, nests and cords of cells with abundant eosinophilic cytoplasm, often with lipochrome pigment; no Reinke’s crystals
Case report: similar tumors in 36 year old woman with congenital adrenal hyperplasia due to 21-OHase deficiency, who received corticosteroids since birth, developed virilizing symptoms and had bilateral ovarian tumors, AJSP 2001;25:1443
References: Archives 2000;124:785
Other tumors not specific to testis
Radiosensitive
Perineural invasion common
Anaplastic large cell lymphoma
Case report in 56 year old Saudi, Mod Path 1996;9:812
Micro: anaplastic large cells resembling Reed-Sternberg cells with horseshoe, wreath-like or multiple nuclei, multiple nucleoli; may have diffuse neutrophilic infiltration
Positive stains: CD30/Ki-1, UCHL-1
Case report of 23 year old man with mature teratoma and retroperitoneal metastasis with mature teratoma and angiosarcoma, Archives 2003;127:360
Micro images: image1
Case report of malignant Brenner tumor of testis and epididymis in 62 year old man, Archives 1991;115:524
Micro: nests of transitional epithelium with focal mucinous differentiation, surrounded by dense fibrous tissue
Case report of 2.5 cm testicular tumor in 24 year old man with metastases to retroperitoneal lymph nodes and i(12p), AJSP 1993;17:738
Granulocytic sarcoma
Tumorous masses of myeloid leukemic infiltrates
Rare; case reports of men ages 48 and 71 years, Mod Path 1997;10:320
Gross: cream-colored to yellow-tan, rubbery-to-firm testicular tumors with extensive paratesticular spread
Micro: primitive cells with scant cytoplasm; or cells with eosinophilic, occasionally granular cytoplasm; prominent myelocytes with round, eccentric nuclei and moderately abundant cytoplasm resembling plasma cells
Positive stains: chloroacetate esterase, myeloperoxidase, lysozyme,CD45/LCA, CD43
Negative stains: CD20, CD3
DD: lymphoma, plasmacytoma
Case report of epithelioid (histiocytic) hemangioma in 29 year old man, AJSP 1990;14:584
Micro: small tubules, some with red blood cells, lined by mesothelial-like cells with uniform, vesicular nuclei
Positive stains: vimentin, factor 8
Negative stains: cytokeratin, EMA
DD: adenomatoid tumor
Extremely rare tumor that usually arises in lymph nodes, AJSP 1999;23:1141
Gross: light tan, solid, may replace entire testis
Micro: whorls and fascicles of spindle cells mixed with small lymphocytes
Positive stains: S100 (strong), vimentin (strong), CD68 (focal), CD4 (focal)
Negative stains: CD1a, CD3, CD20, CD21, CD23, CD34, CD35, CD45, actin, desmin, HMB45, cytokeratin, PLAP
EM: complex interdigitating cytoplasmic dendritic processes, abundant rough endoplasmic reticulum, abundant mitochondria
DD: mesenchymal sarcoma, spindle cell carcinoma, follicular dendritic cell tumor, nodular and diffuse fibrous proliferation
Leukemic involvement of testis common with ALL (8% clinically, 20% microscopically), also AML
Bilateral involvement common
Testis may be first site of relapse
Treatment: radiation therapy, although bone marrow relapse is common
Micro: monomorphic interstitial infiltrate
DD: large cell lymphoma (need clinical history), orchitis (heterogeneous cell population), seminoma (has intratubular germ cell neoplasia, PLAP+)
5% of testicular malignancies
50% of testicular neoplasms in men age 60+, 20% are bilateral (may still be clonal)
Occur at any age (range 16 to 91 years, mean 56 years), 50% with bilateral tumors have lymphoma
Usually disseminated at presentation
Almost always diffuse large B cell subtype, rarely anaplastic lymphoma, Burkitt’s lymphoma, Hodgkin’s lymphoma
Better prognosis if tumor is a primary (5 year survival 60% vs. 17% for disseminated disease/other stages) and unilateral
May progress in Waldeyer’s ring
Case report: follicular lymphoma, large cell type in 6 year old boy, but tumor negative for bcl-2 and t(14;18), Archives 2001;125:551
Treatment: orchiectomy and radiation
Gross: white-tan-pink, fleshy, resembles seminoma, often extratesticular involvement
Micro: splaying apart but relative sparing of tubules by lymphoma cells; vascular invasion in 60%, significant sclerosis in 30%; noncohesive cells with large irregular nuclei, prominent nucleoli, pleomorphism; no intratubular germ cell neoplasia
Micro images: follicular lymphoma
Positive stains: CD20, LCA/CD45
Negative stains: PLAP
DD: spermatocytic seminoma, classic seminoma, chronic orchitis (patchy heterogenous infiltrate)
References: AJSP 1994;18:376, Hum Path 1993;24:675
Benign or malignant
Benign papillary mesothelioma
Case Report: 69 year old man with scrotal swelling, Archives 2000;124:143
Gross: 1.5 cm polypoid pedunculated nodule near head of epididymis
Micro: papillae lined by cuboidal cells with complex branching pattern but no atypia; lymphoplasmacytic infiltrate in stroma
Micro images: image1, image2, image3, image4, image5
Positive stains: vimentin, CAM 5.2, p53 (although benign)
Malignant mesothelioma of testis
Originates from tunica vaginalis (image), which derives from evagination of peritoneum into scrotum
Epidemiology: rare, mean age 55 years, but varies from children to elderly (AJSP 1995;19:815)
Clinical: usually associated with asbestos exposure (Orphanet J Rare Dis 2008 Dec 19;3:34)
Initial symptom often a hydrocele
Often fatal, even in patients with negative resection margins; mean disease specific survival of 29 months (Urology 2005;66:397)
Case reports: 59 year old man (Case of Week #148), no history of asbestos exposure (Cases J 2008 Nov 14;1:310)
Treatment: radial orchiectomy, some recommend retroperitoneal lymph node dissection
Gross: multifocal, friable, papillary tumor within hydrocele sac, often extends into adjacent structures
Gross images: Case of Week - #1; #2
Micro: usually purely epithelioid (not spindled), papillary or tubulopapillary pattern with single layer of atypical mesothelium overlying fibrovascular core; stromal invasion present; variable psammoma bodies
Micro images: Case of Week - low power; high power; calretinin #1; #2; WT1
Positive stains: calretinin, EMA, thrombomodulin, CK7, CK5/6 (variable) (Am J Surg Pathol 2006;30:1)
Negative stains: CK20, CEA
Differential diagnosis:
• adenocarcinoma - more common in the epididymis than in tunica vaginalis, back to back glands or a poorly differentiated pattern, often necrosis and mitotic figures, CK20+, BerEp4+, CEA+, calretinin-, thrombomodulin-, no long thin microvilli on EM
• well differentiated papillary mesothelioma - very rare, papillary but not tubular, papillae are lined by cuboidal cells with a complex branching pattern but no atypia and no invasion; stroma has lymphoplasmacytic infiltrate
• mesothelial hyperplasia - no mass, no complex arborizing papillae, not invasive
Rarely is the first clinical sign of disease
Mean age 57 years, older than germ cell tumors
Lung, prostate, skin (Merkel cell tumors, AJCP 1990;94:384, melanoma) are usual primary sites
Metastases from prostate are usually incidental
20% are bilateral
Gross: multinodular involvement
Gross images: prostate #1, #2
Micro: interstitial pattern, often angiolymphatic invasion, no intratubular germ cell neoplasia
Micro images: prostate #1, #2, #3, prostate (PSA)
Positive stains: mucin (teratomatous elements and yolk sac tumors may also be positive), EMA
Negative stains: PLAP (usually)
DD: Leydig or other sex cord stromal tumor, embryonal carcinoma (particularly if testis is first clinical site of disease)
Case report of 59 year old man with bilateral tumor, Archives 1992;116:1360
Gross: well demarcated, multiple cavities, may involve epididymis
Micro: mucus cells and epithelial cells with basal hyperchromatic nuclei
Case report of extramedullary myeloid tumor (CMML) in 66 year old man with myelodysplasia, Archives 1996;120:389
Micro: large, polygonal cells with pale blue-pink cytoplasm
Positive stains: CD45, CD43, myeloperoxidase, lysozyme, chloroacetate esterase
Case report, Archives 1999;123:244
69 year old man with incidental 7.5 cm solid/cystic tumor completely replacing testis, no extension to tunica albuginea or epididymis; no evidence of disease 3 years after orchiectomy
Micro: bland mucinous epithelium, rare atypia; also mature bone, fibrous stroma with cholesterol clefts, multinucleated giant cells, lymphocytes, foamy macrophages
Case reports, Archives 1981;105:38, Hum Path 1990;21:932
Resembles skin lesions of same name
Case report, Archives 1995;119:96
Considered a monodermal teratomatous tumor of follicular differentiation
Myeloma present in almost all cases either before or after the testicular plasmacytoma
Often men age 40+
Gross: soft, fleshy, tan-gray-white, focally hemorrhagic
Micro: central effacement of tubules by atypical plasma cells (bi- or multinucleated); anaplastic cells may obliterate parenchyma or invade between seminiferous or epididymal tubules; tubules often spared at periphery; no globular differentiation
Micro images: image1, image2, image3, image4
Positive stains: light chain restriction (i.e. staining by kappa OR lambda but not both), CD45 (LCA)
Negative stains: CD3, CD20, CD30, PLAP
References: AJSP 1997;21:590
Miscellaneous
Pathologic staging (pT), requires histologic examination
Primary tumor (T)
pTX: Primary tumor cannot be assessed
pT0: No evidence of primary tumor (i.e. scar but no tumor in testis)
pTis: Intratubular germ cell neoplasia (“carcinoma in situ”, although not an epithelial lesion)
pT1: Tumor limited to the testis and epididymis without vascular/lymphatic invasion; tumor may invade into the tunica albuginea but not the tunica vaginalis
pT2: Tumor limited to the testis and epididymis with vascular/lymphatic invasion, or tumor extending through the tunica albuginea with involvement of the tunica vaginalis
pT3: Tumor invades the spermatic cord with or without vascular/lymphatic invasion
pT4: Tumor invades the scrotum with or without vascular/lymphatic invasion
Regional lymph nodes (N)
pNX: Regional lymph nodes cannot be assessed
pN0: No regional lymph node metastasis
pN1: Metastasis with a lymph node mass 2 cm or less in greatest dimension and 5 or fewer positive lymph nodes, none more than 2 cm in greatest dimension
pN2: Metastasis with a lymph node mass more than 2 cm but not more than 5 cm in greatest dimension; or 6 or more lymph nodes positive, none more than 5 cm in greatest dimension; or evidence of extranodal extension of tumor
pN3: Metastasis with a lymph node mass more than 5 cm in greatest dimension
Distant metastasis (M)
M0: No distant metastasis
M1: Distant metastasis
M1a: Nonregional nodal or pulmonary metastasis
M1b: Distant metastasis other than to nonregional lymph nodes and lungs
S classification (serum tumor markers)
SX: Marker studies not available or not performed
S0: Marker studies within normal limits
S1: LDH < 1.5 x upper limit of normal AND hCG < 5000 mIu/ml AND AFP < 1000 ng/ml
S2: LDH 1.5-10 x upper limit of normal OR hCG 5,000-50,000 mIu/ml OR AFP 1000-10,000 ng/ml
S3: LDH > 10 x upper limit of normal OR hCG > 50,000 mIu/ml OR AFP >10,000 ng/ml
Notes:
Serum tumor markers are measured prior to orchiectomy for assignment of S category, except for Stage IS, in which persistent elevation of serum tumor markers following orchiectomy is required
Reference for LDH: AJCP 1982;78:178
Anatomic Stage / Prognostic groups
TNM and S (serum tumor markers)
0: pTis N0 M0 S0 (Stage 0 is no longer included in FIGO)
I: pT1-4 N0 M0 SX
IA: pT1 N0 M0 S0
IB: pT2-4 N0 M0 S0
IS: any pT/TX N0 M0 S1-3 (measured post-orchiectomy)
II: any pT/TX N1-3 M0 SX
IIA: any pT/TX N1 M0 S0-1
IIB: any pT/TX N2 M0 S0-1
IIC: any pT/TX N3 M0 S0-1
III: any pT/TX any N M1 SX
IIIA: any pT/TX any N M1a S0-1
IIIB: any pT/TX N1-3 M0 S2 OR any pT/TX any N M1a S2
IIIC: any pT/TX N1-3 M0 S3 OR any pT/TX any N M1a S3 OR any T any N M1b any S
Tumor size
Histological subtypes present
% of various types (should state if embryonal component > 50%)
Penetration of tunica albuginea, other structures
Involvement of spermatic cord
Angiolymphatic invasion
Intratubular germ cell neoplasia
Margin of spermatic cord
Mitotic figures
Necrosis
Presence of Leydig cell hyperplasia, hemosiderin-laden macrophages, intratubular calcification, testicular atrophy, testicular development abnormalities
For lymph nodes, diameter of largest node, presence of extranodal extension, number of involved nodes
Spermatic cord margin (sample before cut into tumor since contamination is a common problem, Mod Path 1996;9:762)
Tumor (1 section per cm, representing all grossly different features)
Tumor and tunica
Tumor and hilum (site of extratesticular extension in >90% , AJCP 1999;111:534)
Tumor and normal testis
Normal testis
Epididymis
Paratesticular tumors
Occur in young men (mean 28 years, range 17-37 years)
Present with scrotal mass
Aggressive, with nodal and pulmonary metastases, AJSP 1997;21:219
Gross: 3-4 cm gray-white firm mass, often near epididymis
Micro: nests and cords of small blue cells with scanty cytoplasm in desmoplastic stroma, sometimes with tubules and pseudorosettes; mitotically active; similar to abdominal tumor of same name
Positive stains: keratin, vimentin, desmin, NSE
Negative stains: S100
Mean age 62 (range 34-86 years)
After 4 year follow up, 30% recurred, 30% had metastases (lymph nodes, lungs, liver), 30% died (all grade 3 tumor patients, AJSP 2001;25:1143)
Rare myxoid variant (AJSP
2000;24:927)
Case report: 45 year old man with rapidly enlarging, painless testicular swelling (Case of Week #254)
Gross: usually involves tunica, spermatic cord; rarely scrotal dartos muscle or subcutaneous tissue; mean 5 cm
Gross images: image1
Micro: intersecting bundles of smooth muscle cells with atypia and mitotic figures; may have focal cytoplasmic vacuoles indenting blunt-ended nuclei; may have epithelioid, inflammatory areas
Positive stains: muscle specific actin, smooth muscle actin, desmin; CD34 (33%); occasionally focal cytokeratin and S100
Rare; mean 63 years, range 41 to 87 years
Usually involves spermatic cord; also testicular tunica; rarely epididymis
Usually well differentiated or dedifferentiated liposarcomas
Well differentiated tend to recur, often late; dedifferentiated usually don’t recur; metastases are uncommon
May have areas of low grade leiomyosarcomatous differentiation, which doesn’t affect prognosis, AJSP 2002;26:742
Report of well differentiated inflammatory liposarcoma at many sites including paratesticular, AJSP 1997;21:518
Report of dedifferentiated liposarcomas, AJSP 1994;18:1213
Treatment: radical orchiectomy
Gross: mean 12 cm (range 3 to 30 cm)
Micro: atypical cells with large, hyperchromatic nuclei, within fibrous septa or fat; marked variation in adipocytes size; usually lipoblasts
Micro images: image1, image2, image3
DD: extension from primary retroperitoneal sarcoma, well differentiated resemble benign fatty tumors (no atypical cells), inflammatory liposarcoma resembles lymphoma (monoclonal, usually B not T cell) and inflammatory fibrous pseudotumor (spindle cells usually bland), sclerosing liposarcomas resemble fibromatosis (more cellular, no atypia, denser collagen, CD34 negative)
References: AJSP 2003;27:40
Rare to involve paratesticular regions without testicular involvement
Case report (2 cases) of 35 and 61 year old men with diffuse large cell lymphoma, Archives 2001;125:428
Micro images: image1
Ages 11-68 (mean 47)
Due to mullerian metaplasia of mesothelium or embryonic mesothelial inclusions
Often serous borderline tumors (see below), Brenner tumor; other types also
Treatment: radical orchiectomy
Gross: exophytic papillary lesions involving testicular parenchyma, tunica vaginalis, paratesticular tissue
Micro: serous borderline tumors have arborizing pattern of epithelium overlying fibrovascular cores with detached epithelial fragments; invasive tumors have destructive stromal invasion
Positive stains (papillary serous tumors): B72.3, PLAP, Leu-M1 (CD15), CA125, variable CEA
DD: mesothelioma (narrower papillae, less budding, less stratification, less psammoma bodies)
Reference: AJCP 1986;86:146
Case report of unilateral multicystic mass in connective tissue adjacent to the vas deferens in 46-year-old man., AJCP 1994;101:543
May represent cystic hyperplasia of vestigial Wolffian duct remnants
Gross: distinct from epididymis and vas deferens; multicystic
Micro: simple, ciliated, and cuboidal to columnar epithelium; no sperm present; cysts surrounded by smooth muscle connective tissue collars
Case report in tunica vaginalis of 19 year old, Mod Path 1997;10:608
Micro: proliferation of elongated or round cells with distinct cross striations surrounded by connective tissue
Common non-germ cell tumor of scrotal contents in children/teens
Classified into embryonal, alveolar and pleomorphic types; embryonal classified as spindle cell (see below) or botryoid
Usually invades testis at presentation
Often (40%) metastases to retroperitoneal lymph nodes
May represent overgrowth of sarcomatous component of a teratoma, particularly in young patients
Pleomorphic variant is aggressive, Mod Path 2001;14:595
Treatment: surgery, chemotherapy, radiation therapy may cure many patients
Micro: usually embryonal subtype (small blue cells with myxoid stroma); spindle cell subtype has fasciculated or storiform growth pattern of elongated spindle cells with collagen between tumor cells
Micro images: image1, image2, image3, image4, image5
Spindle cell variant of embryonal rhabdomyosarcoma - Testis chapter
First described in 1992 (AJSP 1992;16:229)
See also Soft Tissue Tumor chapter
Most commonly found in paratesticular region of young boys (Am J Surg Pathol 1992;16:229), but also in adults (Am J Surg Pathol 2005:29:1106, Virchows Arch 2006;449:554)
Case reports: 15 year old boy (Case of Week #145)
Treatment: excision; good prognosis in children (AJSP 1993;17:221), aggressive in adults
Gross: firm, fibrous, whorled cut surface resembling leiomyoma
Micro: uniform proliferation of relatively bland, elongated spindle cells (at least 50% of tumor cells) with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; nuclei are often elongated and vesicular; also scattered spindled or polygonal rhabdomyoblasts with brightly eosinophilic cytoplasm and pleomorphic nuclei
Cytology: numerous spindle cells and large fragments of cytoplasmic processes with cross-striations (Acta Cytol 2005;49:331)
Micro images: #1; #2; #3; #4; myogenin; desmin; SMA; AE1/AE3
Positive stains: desmin, myogenin, vimentin, myoD1, smooth muscle actin (Arch Pathol Lab Med 2006;130:1454)
Negative stains: S100, keratin and caldesmon
Spindle cell variant of embryonal rhabdomyosarcoma (continued)
Differential diagnosis:
● fibrosarcoma - herringbone pattern, may have similar morphology but no rhabdomyoblasts, negative for skeletal muscle markers
● infantile fibromatosis - deep location, fascicles of spindle cells, no cross striations, no undifferentiated cells
● leiomyosarcoma - usually high grade, cigar shaped nuclei, no rhabdomyoblasts, often positive for caldesmon, negative for myoglobin
● neuromuscular hamartoma of soft tissue - usually age < 2 years, affects brachial plexus or sciatic nerve, multinodular growth with connective tissue separating nodules, no rhabdomyoblasts, muscular component is positive for desmin and muscle specific actin, neural component is positive for S100
● rhabdomyoma - benign tumor of skeletal muscle differentiation, no rhabdomyoblasts, no pleomorphism, no necrosis
Borderline tumors also called low malignant potential
Rare; presents as testicular mass, mean age 56 years (range 14-77 years)
Arises from tunica albuginea or intratesticular
Treatment: orchiectomy
Gross: 1-6 cm, cystic
Micro: resembles ovarian counterpart, broad intracystic papillae lined by stratified epithelial cells with mild atypia; may have psammoma bodies
Micro images: image1, image2, image3, image4
Note: paratesticular serous carcinomas can have a borderline component
Positive stains: CK 7, estrogen receptor, progesterone receptor, CD15, MOC-31
Negative stains: CK20, CEA, HER2, calretinin
DD: well differentiated mesothelioma (papillae lined by cuboidal mesothelial cells without stratification, calretinin positive, negative for ER, PgR, CD15, MOC-31)
Reference: AJSP 2001;25:373
Mean age 31 years, range 16 to 42 years
Gross: solid, white-tan, poorly circumscribed, often gritty masses involving soft tissue between testis and epididymis, paratesticular soft tissue or visceral tunica vaginalis
Micro: invasive, well-formed papillae lined by serous cuboidal or columnar cells with eosinophilic cytoplasm and marked atypia, abundant psammoma bodies; areas of borderline serous tumor often present
Positive stains: AE1/AE3, S100, EMA, Ber-EP4, LeuM1 (CD15), B72.3, variable CEA, PLAP
EM: gland formation with delicate luminal microvilli and cilia
References: AJSP 1995;19:1359, Hum Path 1992;23:75
Benign cause of intrascrotal mass
Due to nonneoplastic excess of native smooth muscle in paratestis or spermatic cord between or around vessels or efferent ducts
Mean age 63 years (range 46 to 81 years)
Gross: mean 2.5 cm, range 6 mm to 7 cm
Micro: fascicles of smooth muscle in periductal, perivascular, interstitial, or mixed pattern; no cohesive, interlacing growth pattern of leiomyoma
Gross/micro images: image1
References: AJSP 1999;23:903, Archives 2003;127:E111
Epididymis
Connects efferent ductules to vas deferens
Has head, body and tail
Composed of columnar cells (tall, ciliated with PAS+ nuclear inclusions), clear cells, basal contractile cells (actin positive)
May have “monster” cells similar to seminal vesicle (no significance), AJSP 1981;5:483
Tubules have thick muscular coat
Micro images: image1, image2, image3, image4, image5, image6, image7
Positive stains: CD10
References: AJSP 2003;27:469
Nonpathologic morphologic variations:
Intranuclear eosinophilic inclusions: 72%, usually older patients
Lipofuscin pigment: 33%, usually in efferent ducts and associated with obstructive changes
Cribriform hyperplasia: 42%, usually NOT in normal testis
Paneth cell-like metaplasia: 8%, with hyalin-like globules that are positive for PAS with and without diastase digestion, associated with obstructive changes
Nuclear atypia: 14%, similar to that in seminal vesicles, associated with older age
Note: rarely present within hernia sacs, AJSP 1999;23:880
References: AJSP 1998;22:990
Primary cause of epididymal obstruction
Usually related to cystitis, prostatitis, urethritis that spreads through vas deferens or lymphatics
May cause testicular ischemia and necrosis, later scarring and infertility with preservation of Leydig cells and preserved sexual activity
Acute disease: epididymis enlarged, covered with fibrin, may contain pus and rupture
Brucellosis: affects testis and epididymis in 20% of cases; has granulomatous appearance
Gonorrhea: affects epididymis before testis
Tuberculosis: may cause confluent caseation that spreads into testis and simulates malignancy; may cause scrotal fistula; should culture to rule out M. kansasii and M. avium-intracellulare
Children: usually gram negative rods, congenital genitourinary abnormality
Sexually active men age 35 or less: Chlamydia trachomatis, Neisseria gonorrhoeae
Men age 35+: E. coli, Pseudomonas, other urinary tract infection organisms
Micro images: contributed by Professor Venna Maheshwar, Drs. Kiran Alam and Anshu Jain, J. N. Medical College, India - 25 year old man with epididymal mass - filiarial epididymitis #1; #2; #3; #4
Epididymis - non neoplastic lesions
Resembles cribriform DCIS of breast but not pre-malignant or malignant
May be present in 50% of specimens
Micro: complex arcades and cellular bridges spanning dilated epididymal lumina; hyperchromatic nuclei but no atypia, no mitotic activity
References: Archives 1994;118:1020
Rare, also called cholesteatomas
Usually affects head of epididymis
May be due to ischemia with secondary granulomatous reaction and scarring
Micro: zone of necrosis involving efferent ducts and interstitial connective tissue, with adjacent lymphocytes and macrophages; macrophages form large clusters with cholesterol crystals and foreign body type giant cells in duct lumen; also intratubular epithelial regeneration and proliferation of small ducts showing epithelial regeneration and numerous spermatozoa in their lumen; associated with ceroid granuloma, spermatic granuloma and epidermoid metaplasia of the efferent ducts
References: AJSP 1997;21:951
Hernia sac entrapped epididymis
Resembles epididymis-like inclusions (closely arranged clusters of numerous tubules lined by low cuboidal epithelium, with occasional ovoid cells with clear cytoplasm resembling "peg cells", may be embryonal remnants)
May be difficult to differentiate CD10+ epididymis from those inclusions that are also CD10+ (some inclusions are CD10-), AJSP 2003;27:469
Note: reactive mesothelial hyperplasia in a hernia sac may mimic mesothelioma, micro image1, image2
References: Hum Path 1990;21:339, AJSP 2003;27:469
Resemble those seen in adenomatous hyperplasia of rete testis
Isolated or associated with systemic disease
Spermatic granuloma (epididymitis nodosa)
Resembles vasitis nodosa
Cause: inflammation or trauma damage epithelium or basement membrane, causing spillage of spermatozoa into interstitium (similar to vasitis nodosa) and resulting granulomatous inflammation
Gross: nodule up to 3 cm in head of epididymis
Micro: non-caseating granulomas around spermatozoa
DD: Histoplasma capsulatum
Reference: Archives 1986;110:61
Cystic dilation of efferent ducts lined by ciliated columnar cells with thin connective tissue wall, no smooth muscle
Cysts usually translucent
Lumen contains spermatozoa and proteinaceous fluid
Associated with foreign body giant cell reaction, cholesterol clefts
Very rare; patients present with scrotal masses, including small hydrocele
Often poor prognosis
Gross: centered in epididymis, 2-7 cm, gray-white, firm, hemorrhage and necrosis present, may invade adjacent soft tissue or testis
Micro: tubules, complex tubulopapillary formations lined by cuboidal or columnar predominantly clear cells; may have large cysts with complex papillary or confluent, back-to-back glands lined by columnar cells with clear, lightly amphophilic or eosinophilic cytoplasm; necrosis common; may have invasive margins; no cilia
Positive stains: PAS (glycogen), cytokeratin (AE1/AE3)
DD: metastatic adenocarcinoma, malignant mesothelioma, carcinoma of rete testis
References: AJSP 1997;21:1474
Most common tumor of epididymis (excluding spermatic cord lipomas), usually age 20-39 years, often painful
Similar tumor in spermatic cord, ejaculatory duct, fallopian tube and uterus
Mesothelial origin
May be peculiar form of nodular mesothelial hyperplasia instead of a neoplasm
Benign, even if it extends into testis
Treatment: resection is curative
Gross: circumscribed firm gray-white mass up to 5 cm, may be cystic
Gross images: image1
Micro: unencapsulated, cuboidal to flat cells form cords that are either epithelial like or form channels with dilated lumina simulating vessels; cells have cytoplasmic vacuoles; intervening stroma may have smooth muscle and elastic fibers, desmoplastic quality and inflammatory cells
Micro images: image1, image2, image3, AE1/AE3
Positive stains: hyaluronidase, cytokeratin, EMA
Negative stains: CEA, factor VIII, lipids, mucin, Ulex europaeus I lectin
EM: prominent microvilli, desmosomes, tonofilaments
DD: epithelioid hemangioma (positive for factor 8, Ulex europaeus I lectin, CD34; negative for keratin; no intervening tumor cells between tubular structures), large cell calcifying Sertoli cell tumors (centered in testis, frequent calcifications, intratubular growth, weak/negative cytokeratin)
References: Hum Path 1981;12:360
Similar to melanotic progonoma in jaw
Rarely in epididymis or testis
Usually young infants (mean 7 months, range 3 months to 8 years)
Good prognosis although rare case has metastasized
Appears to recapitulate the retina at 5 weeks of gestation
Treatment: excision
Gross: well circumscribed, brown-black nodule in head of epididymis
Positive stains: NSE, synaptophysin, HMB45, keratin, vimentin
Negative stains: AFP, S100, CEA
EM: melanosomes with varied maturation, cells with neurosecretory granules and cytoplasmic processes suggestive of neuroblasts
References: AJSP 1991;15:233, Hum Path 1985;16:416
Arises from tunica vaginalis
Mean age 54 years, range 12-76 years
Usually malignant and aggressive, similar to peritoneal cavity tumor, AJSP 1995;19:815
Occasional association with asbestos exposure, AJSP 2001;25:1304
Usually fibrous type
Gross: multiple nodules within a hydrocele sac, frequently associated with mass infiltrating spermatic cord or testis
Micro: epithelial or biphasic mesothelioma patterns; papillary, tubular or solid epithelial cells; spindle cells with scanty stroma in fascicles
Positive stains: AE1/AE3, EMA, vimentin
Negative stains: CEA, B72.3, Leu-M1 (CD15), Ber-EP4
DD: adenocarcinoma of rete testis
Endometriosis-like lesion
In men treated with estrogens
Ovarian-like tumor of epithelial type
Serous, mucinous, endometrioid, clear cell, Brenner
Usually benign
Pseudotumor of epididymis
Reactive myofibroblasts
Usually in children after testicular torsion
Secondary tumor of epididymis
Usually extension from testicular lesions
Familial, unilateral or bilateral (40%)
Mean age 36 years
Associated with von Hippel-Lindau (VHL) disease; 65% with bilateral tumors have VHL vs. 18% with unilateral lesions
High levels of vascular endothelial growth factor mRNA by in situ hybridization in one patient with bilateral tumors, Hum Path 1998;29:1322
Benign
Gross: well circumscribed, 1-5 cm, papillary fronds of gray-brown-yellow tissue project into cystic space
Micro: papillary infoldings project into cystic spaces covered by cuboidal/columnar cells; tubules common, colloid type cystic material common; tumor cells have lightly eosinophilic to clear cytoplasm; may see dilated efferent ductules; may have clear cells resembling metastastic renal cell carcinoma
Positive stains: AE1/AE3, alpha-1-antitrypsin, alpha-1-antichymotrypsin, Cam5.2, EMA, vimentin, CEA variable, vascular endothelial growth factor
DD: metastatic renal cell carcinoma, serous borderline tumors (atypia, mitotic activity, stratification, detached cell clusters)
References: Hum Path 1995;26:1341, Archives 1990;114:672, Hum Path 1998;29:1322
Case report in 20 year old man, Archives 2000;124:1518
More common in vulva of young women
3 cases reported in men, one from prostate, one from testis, one from epididymis
Gross/micro images: image1
An anastomosing network of delicate channels at testicular hilum, connects testicular tubules to 12-15 ciliated efferent ducts, which merge into a single duct, the epididymis at its head
Complex tubular architecture may resemble teratoma
Lined by flattened squamous to columnar epithelium with numerous microvilli
Efferent duct lumina are narrower than epididymis, lined by ciliated columnar cells with microvilli
Chordae retis: epithelium-covered strands and columns connect opposite walls of channel
Uncommon benign lesion; usually an incidental finding
May be similar to papillary adenoma of rete testis
Mean age 59 years, range 30-74 years
Gross: may be solid/cystic mass in testicular hilus; usually small, incidental microscopic finding
Micro: tubulopapillary or cribriform proliferations of bland epithelium that projects into dilated channels of rete testis; associated with hyaline globules (represent proteins absorbed from lumen by epithelial lining cells)
Micro images (papillary adenoma): image1, image2, image3
Positive stains: keratin, EMA
Negative stains: vimentin, actin, desmin, S100
DD: yolk sac tumor
References: AJSP 1991;15:350, AJSP 1991;15:66
Also called nodular proliferation of calcifying connective tissue
May represent dystrophic changes; no clinical significance
Micro: nodular sessile or pedunculated protrusions into the rete testis channels, consisting of connective tissue covered by a flattened epithelium, with variable fibroblasts, fibrin-like material, small to large calcium deposits; no inflammatory infiltrates
References: Hum Path 1989;20:58
Cystic dilation (transformation)
Due to obstruction of epididymis or intratesticular excretory ducts
Also after hemodialysis, and associated with calcium oxalate deposits in lumen of rete testis, efferent ducts and adjacent connective tissue, Hum Path 1996;27:336,
Identified in 1% of autopsy / surgical specimens in Spain, AJSP 1996;20:1231
Usually bilateral
Causes: compression of epididymis by tumor of epididymis or spermatic cord, chronic epididymitis, ischemia, cirrhosis, cryptorchidism
Micro: bilateral efferent duct atrophy, nodular proliferation of calcifying connective tissue, columnar transformation of rete testis epithelium, occasional papillary proliferations; no atypia, no mitotic figures
References: Hum Path 1989;20:1065
Infants and children, presents as testicular mass
Cystic dilation of rete testis with compression/atrophy of seminiferous tubules
Developmental anomaly associated with ipsilateral renal agenesis
Underdeveloped rete testis lined by columnar or large cuboidal cells in postpubertal patients with cryptorchidism
Some patients have viable spermatozoa even though there is an obstruction to sperm-conducting mechanism
References: Archives 1997;121:1259
Rete testis - neoplasms
Extremely rare (4 cases reported)
Age 34-62 years, presents as unilateral painless testicular mass
Benign; excision and follow up is adequate
Gross: 1-3 cm, single, well circumscribed, cystic or nodular tan-white mass confined to rete testis
Micro: non-invasive tubular or solid pattern within rete testis, monotonous and bland Sertoli-like cells that fill/distend rete testes; no mitoses
Micro images: various images
DD: Sertoli cell tumor (in testis, often invasive, cells with lipid droplets or vacuolated cytoplasm, variable mitoses)
Very rare
Resembles mesothelioma of tunica vaginalis
Usually age 60+, range 31-91 years, described only in Caucasians
Poor prognosis, often lymphatic metastases
Case report with sertoliform differentiation, Archives 1989;113:1169
Gross: centered in hilum, lack of direct extension through tunica; white-yellow-gray
Micro: transitions from tumor to rete testis, no evidence of teratoma, no other primary tumor; solid, tubular or papillary patterns with clear-cut stromal invasion; slit-like spaces in solid nests of tumor are common; intra-rete growth is common
May have intratubular invasion of testis; may have spindle cell component or sertoliform differentiation
DD: malignant mesothelioma, metastatic carcinomas
References: AJSP 1988;12:492, AJSP 1984;8:625, Archives 1990;114:84, Hum Path 1977;8:219
Spermatic cord
Vas deferens:
Also called ductus deferens
30-40 cm long tubular structure from tail of epididymis to prostatic urethra at level of verumontanum; distal vas deferens joins seminal vesicle to form ejaculatory duct
Rarely present within hernia sacs, AJSP 1999;23:880
Should see complete transection in vasectomy specimens
Drawings: image1, image2, image3, image4
Micro: ciliated, pseudostratified epithelium with prominent nuclear inclusions resting on basal cell layer; markedly thickened muscle coat of 3 layers (inner longitudinal, outer longitudinal, middle circular layer); may contain psammoma bodies
Micro images: image1, image2, image3, image4, image5, image6, image7
Positive stains: CD10
Reference: AJSP 2003;27:469
Vas deferens or epididymis may become entrapped in hernia sacs and subject to inadvertent transection, particularly in children
One study of 7314 boys with herniorrhaphy demonstrated 0.23% with vas deferens, 0.30% with epididymis, AJSP 1999;23:880
Vas deferens resembles inclusions in hernia sacs (single or separate gland-like structures lined by pseudostratified columnar epithelium with luminal cilia and an encircling stromal coat, may be embryonic remnants)
Vas deferens is CD10+; stromal coat > 1.0 mm (even in prepubertal boys), histologically similar inclusions are CD10 negative, stromal coat < 0.8 mm but may be CD10+, AJSP 2003;27:469
References: Hum Path 1990;21:339
May cause testicular infarct if not treated quickly
Usually occurs in first year of life, also towards puberty due to trauma or violent movement
Associated with incomplete testicular descent, absent scrotal ligaments, absent gubernaculum testis or testicular atrophy causing testis to be abnormally mobile
In 2/3 of cases, occurs in intravaginal portion of spermatic cord (i.e. within tunica vaginalis); extravaginal torsion associated with infancy
Thick walled arteries remain patent, so get vascular engorgement and venous infarction (enlarged and painful testis)
Torsion must last at least 6-24 hours to cause an infarct
Torsion without an infarct causes infertility proportionate to duration of torsion
Treatment: untwist and fix testis to dartos muscle, or orchiectomy
Note: opposite testis should be fixed to dartos muscle as preventive measure
Gross images: testicular torsion and infarction
Micro: up to 6 hours – venous congestion and interstitial hemorrhage; later neutrophils in capillaries; at 4 days see hemorrhagic infarction and coagulative necrosis; later granulation tissue; years later may see fibrosis and calcification
DD: (a) torsion of testicular appendage, 90% of time involves appendix testis (dramatic pain out of proportion to minute size of appendage), 10% of time involves epididymis
(b) testicular pain due to Henoch-Schonlein purpura (small vessel vasculitis)
Granulomatous condition of vas deferens that resembles spermatic granuloma of epididymis
Usually post vasectomy or herniorrhaphy
Occasionally associated with recanalization
Micro: proliferating ductules and dilated tubules containing spermatozoa in wall of vas deferens; also associated with small bundles of hyperplastic smooth muscle; vas deferens may show irregular thickening
Ductules may connect with vas deferens (like diverticula or salpingitis isthmica nodosa but with inflammation and less smooth muscle hypertrophy)
May see perineural or vascular invasion by proliferating ductules
Micro images: image1, image2, image3, image4, image5, image6
DD: adenocarcinoma (no history of prior surgery, no intraluminal spermatozoa)
Pseudosarcomatous myofibroblastic proliferation of spermatic cord
Usually incidental at herniorrhaphy
Ages 52-76 years; may be due to ischemia or torsion
Resembles nodular fasciitis
May be due to ischemia/ torsion
Similar lesions arise in epididymis
References: AJSP 1992;16:448
Benign tumors are usually lipomas; fat collections around hernia sac are not true lipomas
Contamination by testicular tumors is a problem, particularly with seminomas, Mod Path 1996;9:762
In adults, most common tumors are MFH, liposarcoma, leiomyosarcoma, fibrosarcoma
Treatment is orchiectomy with high ligation of cord and radiation therapy
Non-neoplastic masses are mesothelial cysts and dermoid cysts
Locally aggressive but nonmetastasizing soft tissue tumor of pelvic soft tissue and perineum, almost always in adult women
Case report of tumors in 4 men at AJSP 1992;16:1059
Local recurrence common, but don’t metastasize
Gross: myxoid, nonencapsulated
Micro: infiltrative tumors composed of fibromyxoid matrix with occasional bland spindle cells with delicate cytoplasmic processes in background of scattered vessels of variable size, some with hypertrophy or hyalinization of vessel wall
Positive stains: vimentin, variable muscle specific actin, alpha smooth muscle actin, desmin
Negative stains: S100
Benign soft tissue tumor
Gross: well circumscribed
Micro: alternating hypocellular and hypercellular zones, capillary sized vessels
DD: aggressive angiomyxoma
Embryonal rhabdomyosarcoma
Most common childhood malignant tumor of spermatic cord
Peak age is 9 years
80% overall survival
Gross: fleshy gray-white to pink-tan mass, 4-6 cm, may be mucoid
Micro: small cells with hyperchromatic nuclei, minimal cytoplasm as well as cells with rims of eosinophilic cytoplasm and spindle cells with cytoplasmic tails and variable cross striations; myxoid or collagenous stroma
Positive stains: desmin, muscle specific actin, myoglobin
Case report of 31 year old man with 7 cm tumor; resembles fibromatosis at other sites, AJCP 1995;104:403
May recur locally
DD: proliferative funiculitis, nodular and diffuse fibrous proliferation
Case report in 32 year old man, Archives 2002;126:357
Gross: circumscribed mass
Common, usually small
Well differentiated tumor with myxoid MFH-area reported at AJSP 1999;23:1480
Well differentiated tumor (“atypical lipomatous tumor”) with smooth muscle reported at AJSP 1990;14:714
Malignant fibrous histiocytoma
Rare (<50 cases reported)
Usually age 50+; 2 cases reported in men 65 and 70 years old, Mod Path 2002;15:59
Gross: 1-20 cm, may have associated satellite nodules
Micro: similar to tumor at other sites
Micro images: image1, image2, image3, image4
Case report at Archives 1990;114:899
Positive stains: polygonal cells - NSE, chromogranin, synaptophysin; sustentacular cells - S100
Case report in 32 year man, AJSP 1996;20:1145
Gross: encapsulated, rubbery tumor with beige-yellow gelatinous cut surface
Micro: adipose tissue with extensive areas of myxoid change and abundant thin and thick-walled blood vessels
