Cervix
Sarcoma / lymphoma / other
Adenosarcoma

Author: Carlos Parra-Herran, M.D. (see Authors page)

Revised: 2 May 2017, last major update May 2017

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Adenosarcoma cervix

Related topics: With sarcomatous overgrowth
Cite this page: Adenosarcoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/cervixadenosarcoma.html. Accessed July 24th, 2017.
Definition / general
  • Mixed lesion with malignant mesenchymal and benign glandular components
  • Also called Müllerian adenosarcoma
  • More common in the endometrium
  • Most patients are young with median age of 37 - 39 years at presentation
  • Good prognosis if no myometrial invasion, bland histology and no sarcomatous overgrowth (Oncol Rep 1998;5:939)
  • Median survival is 4 years; 40% recur
  • Adverse prognostic factors are:
    • Myometrial and vascular space invasion: myometrial invasion is seen in 14% of cases and is associated with adverse outcome (Hum Pathol 1990;21:363, Int J Gynecol Pathol 1992;11:75)
    • Stromal overgrowth: highly associated with extrauterine spread at presentation; high rates of recurrence and death (Am J Surg Pathol 1989;13:28)
    • High grade sarcomatous component: highly associated with extrauterine spread, recurrences and metastases; usually associated with overgrowth; however, the high grade component may be minor
Case reports
Treatment
  • Hysterectomy 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
Gross description
  • The lesion is frequently interpreted as an endometrial or endocervical polyp on clinical and radiologic evaluation
  • Broad based or sessile polypoid mass on gross examination
Microscopic (histologic) description
  • Biphasic (malignant stromal and benign glandular components)
  • The 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
  • The stroma around the glands is typically more cellular and atypical; in these cellular areas, mitotic activity is increased, usually 4 or more mitoses/10 HPFs
  • The 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
    • Rigid cystic dilation
    • Mitotic activity ≥ 2 mitoses/10 HPFs
  • The diagnosis of adenosarcoma is favored if 2 or more of the above features are diffusely present
  • It has been recently shown that tumors with up to 3 of the above changes, when focal, behave in a benign fashion with no recurrences or adverse outcome
  • High grade sarcoma is defined as pleomorphic sarcoma cells identifiable at low power magnification; nuclei are enlarged, hyperchromatic and contain prominent nucleoli
Microscopic (histologic) images

Images hosted on Pathout server:
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Contributed by Carlos Parra-Herran, M.D.

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Phyllodes tumor-like
pattern
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Phyllodes tumor-like
pattern (more cellular
than adenofibroma)
Positive stains
Negative stains
Electron microscopy description
  • Stromal cells resemble endometrial stromal cells
Molecular features
  • Müllerian adenosarcoma harbors a number of somatic gene alterations that are exclusive to the mesenchymal component, supporting the theory 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 localised and confined genomic regions in one or a few chromosomes, Mod Pathol 2016;29:1070, J Pathol 2015;235:37)
Differential diagnosis
  • Carcinosarcoma: epithelial component is malignant (benign in adenosarcoma)
  • 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
  • Rhabdomyosarcoma: differential in cases of high grade adenosarcoma with heterologous differentiation
    • Pure rhabdomyosarcoma lacks benign epithelial elements admixed within the tumor
Additional references