Fallopian tubes & broad ligament
Fallopian tube mixed epithelial & mesenchymal tumors
Adenosarcoma
Board of reviewers: C. Blake Gilks, M.D.
Deputy Editor-in-Chief: Gulisa Turashvili, M.D., Ph.D.
Last author update: 12 December 2024
Last staff update: 12 December 2024
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PubMed search: Adenosarcoma
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Li JJX, Ip PPC. Adenosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/fallopiantubesadenosarcoma.html. Accessed December 21st, 2024.
Definition / general
- Biphasic neoplasm consisting of a malignant stromal component and a benign epithelial component
Essential features
- Uncommon gynecological neoplasm that is exceedingly rare as a primary tumor in the fallopian tubes
- Biphasic neoplasm with benign Müllerian epithelium surrounded by neoplastic stromal cells
- Diagnosis is based on histological appearance and recognition of at times subtle, stromal atypia
Terminology
- Müllerian adenosarcoma
Epidemiology
- Extrauterine adenosarcomas are rare neoplasms
- Fallopian tube adenosarcoma in the published literature is limited to isolated case reports (Gynecol Oncol 1995;59:412, Int J Gynecol Pathol 2022;41:82)
Sites
- Fallopian tubes
Pathophysiology
- Adenosarcomas are believed to arise from Müllerian mesenchymal cells with mutations restricted to the mesenchymal component (J Pathol 2016;238:381)
- Extrauterine adenosarcoma has been reported to arise from endometriosis (Ann Coloproctol 2014;30:232, Fertil Steril 2004;81:1142)
Etiology
- Currently uncertain
Diagnosis
- Diagnosed based on histological appearance
- No specific immunohistochemical marker or molecular feature for diagnosis
Laboratory
- No specific laboratory findings
Radiology description
- Heterogenous mass on ultrasound (Int J Gynecol Pathol 2022;41:82)
- Hyperintense on T1 magnetic resonance imaging (MRI) (Tunis Med 2024;102:116)
Prognostic factors
- TP53 mutation, high grade histology and sarcomatous overgrowth are associated with poor prognosis (J Pathol 2015;235:37)
- Complete surgical excision is more difficult in fallopian tube / extrauterine adenosarcomas (Int J Gynecol Pathol 2022;41:82)
- Case of low grade adenosarcoma reported to be disease free with salvage surgery and adjuvant chemotherapy after peritoneal recurrence (Gynecol Oncol 1995;59:412)
Case reports
- 26 year old woman with acute abdomen (Gynecol Oncol 1995;59:412)
- 35 year old woman with menorrhagia (Tunis Med 2024;102:116)
- 50 year old woman with catamenial rectal pain and deep dyspareunia (Fertil Steril 2004;81:1142)
- 55 year old woman with abdominal pain and bloating (Int J Gynecol Pathol 2022;41:82)
Treatment
- Surgical excision is the mainstay of treatment (Int J Gynecol Pathol 2022;41:82)
- Chemotherapy considered for advanced or recurrent disease (Ther Adv Med Oncol 2023;15:17588359231157645)
- Endocrine therapy used only for low grade adenosarcoma (Ther Adv Med Oncol 2023;15:17588359231157645)
Gross description
- Nodular, polypoid mass or papillary projections into tubal lumen
Microscopic (histologic) description
- Biphasic tumor with glands among a stromal component that forms thin papillae or broad polypoid fronds that project into mucosal surface
- Epithelial component composed of endometrioid, ciliated, hobnail, mucinous or metaplastic squamous epithelium
- Mesenchymal component is neoplastic
- Periglandular condensation (cuffing) around epithelium
- Low grade stroma commonly resembles endometrial type stroma
- Distinguished from normal endometrial stroma by cellular periglandular cuffs and presence of mitotic figures (usually ≥ 4 mitoses per 10 high power fields in majority of cases)
- High grade stroma is associated with greater nuclear atypia, more frequent mitosis, necrosis and hemorrhage, although stromal cells may be deceptively bland and sparsely cellular in the areas away from the glands
- Sarcomatous overgrowth (> 25% of tumor composed of stromal component only) is more common in high grade adenosarcoma (71% in high grade versus 10% in low grade) (Mod Pathol 2022;35:1684)
- Sarcomatous overgrowth was present in 1 reported case of fallopian tube adenosarcoma (Int J Gynecol Pathol 2022;41:82)
- Sarcomatous overgrowth (> 25% of tumor composed of stromal component only) is more common in high grade adenosarcoma (71% in high grade versus 10% in low grade) (Mod Pathol 2022;35:1684)
- Heterologous (5 - 26%) and sex cord-like differentiation (5%) can be seen (Mod Pathol 2022;35:1684, Diagn Pathol 2024;19:56)
- Can be associated with endometriosis
Microscopic (histologic) images
Contributed by Joshua J.X. Li, M.B.Ch.B. and Philip P. C. Ip, M.B.Ch.B.
Adnexal tumor nodule
Biphasic pattern
Bland epithelial component
Periglandular condensation
Stromal mitosis
Stromal atypia
Malignant stroma
Variable stromal atypia
Serosal tumor nodule
Smooth muscle actin
Positive stains
- Epithelial component
- Stromal component (expression of the following may be absent in high grade tumors)
- Hormone receptors (androgen, estrogen and progesterone receptors) (Diagn Pathol 2020;15:119)
- CD10
- WT1
- Smooth muscle actin
- Vimentin
- Abnormal p53 in sarcomatous overgrowth (Int J Gynecol Cancer 1999;9:37)
Negative stains
- Epithelial component
- Stromal component
- Reference: Am J Surg Pathol 2008;32:1013
Sample pathology report
- Fallopian tube, salpingectomy:
- High grade adenosarcoma (see comment)
- Comment: Sections show a biphasic tumor consisting of bland ciliated epithelium arranged in compressed glands and periglandular cuff of atypical stromal cells. The latter are spindled and occasionally epithelioid, displaying moderate to marked nuclear enlargement, hyperchromasia and pleomorphism with frequent mitotic figures. Necrosis is identified. The features are those of a high grade adenosarcoma.
Differential diagnosis
- Adenomyoma:
- Majority of stroma composed of smooth muscle
- Lacks periglandular cuff of stromal cells
- Endometriosis:
- Lacks prominent periglandular cuff of stromal cells
- Presence of small arterioles similar to those in the proliferative phase endometrium
- Carcinosarcoma:
- Epithelial component neoplastic with malignant histological features
- Stromal component typically high grade
- Endometrial stromal sarcoma (ovary):
- Lacks epithelial component
- Specific molecular aberrations (low grade: PHF1, SUZ12; high grade: BCOR, YWHAE)
- Fibrosarcoma (ovary):
- Marked atypia with numerous and often abnormal mitoses
- Lacks epithelial component
Board review style question #1
Which of the following is useful in differentiating adenosarcoma from carcinosarcoma of the uterine adnexa?
- Heterologous sarcomatous differentiation
- Involvement of the uterine corpus
- Periglandular cuffing of atypical stromal cells surrounding bland epithelial component
- Stromal hypercellularity and atypia
- TP53 sequencing for detection of TP53 mutation
Board review style answer #1
C. Periglandular cuffing of atypical stromal cells surrounding bland epithelial component. The primary differentiation between adenosarcoma and carcinosarcoma is based on histologic features including architecture. Answer A is incorrect because heterologous sarcomatous differentiation can be observed in both entities. Answer E is incorrect because TP53 mutation can be present in both entities. Answer B is incorrect because involvement of the uterine corpus does not favor adenosarcoma over carcinosarcoma or otherwise. Answer D is incorrect because hypercellularity and atypia can be seen in both entities.
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Reference: Adenosarcoma
Comment Here
Reference: Adenosarcoma
