Cervix

• Müllerian adenosarcoma can occur in multiple sites:
  • Uterine corpus > cervix > ovary / pelvis
  • Extrauterine adenosarcoma may show associated endometriosis
  • 10% occur in the cervix (Gynecol Oncol 2016;143:636)
• Patients with cervical primaries are younger whereas corpus and ovarian primaries typically affect postmenopausal patients (Gynecol Oncol 2016;143:636)

• Hysterectomy with bilateral salpingectomy oophorectomy is the standard of treatment and is curative in most cases
• Radiation therapy is considered in patients with advanced stage (FIGO stage II or more) or after recurrence
• Fertility sparing surgery (FSS) via cervical conization may be an option for a subset of patients:
  • Recent data show no decrease in disease free or overall survival after FSS for FIGO stage IA tumors (Front Oncol 2019;9:237)
  • Older reports do not support this finding; however, patients from these groups were of higher clinical stage (e.g., FIGO IB)

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• Biphasic (malignant stromal and benign glandular components)
• Glandular component is bland and evenly dispersed
• Epithelial metaplasia can be appreciated but atypia or frank malignant features are absent
• Most glands have narrow lumens, usually compressed by the underlying mesenchymal growth giving a leaf-like appearance
• Cystic dilation with rigid contours is common
• Periglandular cuffing:
  • Stroma around the glands is usually more cellular and atypical; in these cellular areas, mitotic activity is increased, usually ≥ 4 mitoses/10 high power fields
  • Stroma in this region is sometimes referred to as the cambium layer
• Diagnosis of adenosarcoma relies on the identification of the following features:
  • Intraglandular projections and leaf-like (phyllodes-like) architecture
  • Marked stromal cytologic atypia
  • Periglandular stromal condensation (cuffing)
  • Rigid cystic dilation
  • Mitotic activity ≥ 2 mitoses/10 high power fields
• Diagnosis of adenosarcoma is favored if ≥ 2 of the above features are diffusely present
• Uterine polyps that are morphologically worrisome for (but not diagnostic of) Müllerian adenosarcoma have recently been shown to follow a benign clinical course, requiring only conservative management (Mod Pathol 2022;35:106)
  • Tumors with up to 3 of the above changes, when focal, fall under this category
  • The term "atypical uterine polyp" has been proposed for such cases
• High grade sarcoma is defined as pleomorphic sarcoma cells that are identifiable at low power magnification; nuclei are enlarged, hyperchromatic and contain prominent nucleoli
  • Rhabdomyosarcomatous differentiation is frequent
  • Sarcomatous component may have sex cord differentiation (Int J Gynecol Pathol 2016;35:153)
• Adenosarcoma with sarcomatous overgrowth:
  • Stromal overgrowth is defined as pure sarcoma representing ≥ 25% of the tumor
  • Sarcoma can be homologous or heterologous and frequently displays high grade cytologic features
  • Aggressive variant (Am J Surg Pathol 1989;13:28)
  • Seen in approximately 10% of cases

Contributed by Carlos Parra-Herran, M.D. and AFIP images



Contributed by Ayse Ayhan, M.D., Ph.D.

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• CD10, WT1, muscle specific actin, desmin, ER, PR (Gynecol Oncol 2004;93:680, Int J Gynecol Cancer 2004;14:1118)
• High grade adenosarcoma and adenosarcoma with sarcomatous overgrowth frequently show abnormal p53 staining (overexpressed or null)

• Müllerian adenosarcoma harbors a number of somatic gene alterations that are exclusive to the mesenchymal component; this supports the hypothesis that this lesion is primarily a mesenchymal neoplasm (J Pathol 2016;238:381)
• Amplification of MDM2 and CDK4 is seen in approximately 25% of cases
• Adenosarcomas with sarcomatous overgrowth have a higher number of copy number variations, MYBL1 amplification, ATRX mutations, global chromosomal instability and chromothripsis (up to thousands of clustered chromosomal rearrangements occur in a single event in localized and confined genomic regions in one or a few chromosomes) (Mod Pathol 2016;29:1070, J Pathol 2015;235:37, Am J Surg Pathol 2017;41:1513)

• Adenofibroma:
  • Benign glands within fibrotic stroma
  • < 2 mitoses/10 high power fields
  • Less stromal cellularity without periglandular cuffing or atypia
  • This entity is no longer recognized by the WHO; there is growing consensus that this lesion does not exist in the uterus
• Carcinosarcoma:
  • Epithelial component is malignant (benign in adenosarcoma) (Am J Surg Pathol 2021;45:374)
• Endocervical / endometrial polyp:
  • Glands lack leaf-like architecture or rigid cystic dilation
  • Lack of periglandular stromal condensation
  • Lack of stromal atypia
• Endometrial stromal sarcoma:
  • Absence of epithelial elements
  • Normal epithelial elements can be entrapped by the mesenchymal proliferation, mimicking adenosarcoma; however, this usually happens only at the periphery of the lesion and on the endometrial surface; moreover, leaf-like growth and periglandular condensation are absent
• Rhabdomyosarcoma:
  • Differential in cases of high grade adenosarcoma with heterologous differentiation
  • Pure rhabdomyosarcoma lacks benign epithelial elements admixed within the tumor

A 32 year old woman presents with abnormal vaginal bleeding and is found to have a 4.5 cm polypoid lesion protruding from the cervical os. Histologic evaluation shows that the lesion arises from the cervix and has bland epithelium with leaf-like architecture. There is periglandular cuffing by markedly atypical stromal cells with a mitotic index of 8 per 10 high power fields. The stromal component comprises 75% of the lesion. Which of the following features defines this as a high grade sarcoma?

1. ≥ 25% of the lesion is the stromal component
2. Mitotic index > 2 per 10 high power fields
3. Periglandular cuffing by stromal cells
4. Pleomorphic tumor cells visible at low power

D. Pleomorphic tumor cells visible at low power categorize this lesion as a high grade adenosarcoma, which is associated with metastasis, recurrence and overall poor prognosis. The presence of sarcomatous overgrowth (≤ 25% stromal component) is frequently associated with high grade cytologic features.

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Reference: Cervix - Adenosarcoma