Mesenchymal / mixed epithelial & mesenchymal tumors


Editorial Board Member: David B. Chapel, M.D.
Deputy Editor-in-Chief: Jennifer A. Bennett, M.D.
Adam Lechner, B.M.
Carlos Parra-Herran, M.D.

Last author update: 20 May 2022
Last staff update: 5 October 2022

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed Search: Adenosarcoma cervix

Adam Lechner, B.M.
Carlos Parra-Herran, M.D.
Page views in 2023: 21,051
Page views in 2024 to date: 10,525
Cite this page: Lechner A, Parra-Herran C. Adenosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cervixadenosarcoma.html. Accessed June 16th, 2024.
Definition / general
  • Rare, mixed lesion with malignant mesenchymal and benign glandular components
Essential features
  • Leaf-like glands composed of bland epithelium and condensed periglandular stroma with atypia and mitotic activity
  • Most are of low malignant potential with good probability of disease free and overall survival
  • 3 most important prognostic factors are: (1) presence of sarcomatous overgrowth, (2) histologic grade and (3) depth of myometrial invasion
  • Stromal cells may lose CD10 and PR when sarcomatous overgrowth is present; other markers may be gained in cases of heterologous differentiation
  • Recurrence may consist solely of sarcomatous component
  • Also called Müllerian adenosarcoma
ICD coding
  • ICD-O: 8933/3 - adenosarcoma
  • ICD-10: C53.9 - malignant neoplasm of cervix uteri, unspecified
  • 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)
  • Multiple small series have implicated hyperestrogenism (e.g., in the setting of tamoxifen therapy or ovarian thecoma) as a risk factor for uterine sarcomas including adenosarcoma (Int J Gynecol Pathol 1996;15:222, Gynecol Oncol 1985;21:135)
    • Due to a small population, these associations may be coincidental
  • Prior pelvic radiation therapy may increase risk
Clinical features
  • Common presenting features include (Adv Anat Pathol 2010;17:122):
    • Abnormal vaginal bleeding (most common)
    • Pelvic pain
    • Abdominal mass
    • Vaginal discharge
  • Lesion is frequently interpreted as an endometrial or endocervical polyp on clinical and radiologic evaluation
  • Recurrence is usually composed of solely sarcomatous component
Prognostic factors
  • Most uterine adenosarcomas are of low malignant potential with favorable prognosis:
    • 83% are FIGO stage I at time of diagnosis with 63 - 84% 5 year overall survival (Gynecol Oncol 2010;119:305)
    • Low grade histology, absence of myometrial invasion or sarcomatous overgrowth all confer good prognosis (Oncol Rep 1998;5:939)
    • Presence of a tumor stalk is an independent protective factor for both disease free and overall survival (Front Oncol 2019;9:237)
  • Cervical primary is associated with improved disease free survival compared to uterine corpus primary (Front Oncol 2019;9:237)
  • Adverse prognostic factors are:
  • Recurrence of uterine adenosarcoma (up to 46%) with mean time to recurrence of 18.3 months (Gynecol Oncol 2014;135:455)
Case reports
  • 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)
Gross description
  • Broad based or sessile polypoid mass on gross examination
  • Cut surface displays predominantly solid tumor with numerous cysts
  • Reference: Adv Anat Pathol 2010;17:122
Gross images

Images hosted on other servers:

Endocervical polypoid lesion

Microscopic (histologic) description
  • 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
  • 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
Microscopic (histologic) images

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

High grade adenosarcoma

Missing Image

Adenosarcoma with sarcomatous overgrowth

Missing Image Missing Image

Low grade adenosarcoma

Missing Image Missing Image

Phyllodes tumor-like pattern

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

Biphasic tumor

Missing Image Missing Image Missing Image Missing Image

Periglandular cuff

Missing Image Missing Image Missing Image

Intraglandular papillae

Missing Image

Stromal mitoses

Missing Image

Squamous metaplasia

Missing Image Missing Image Missing Image

Sarcomatous overgrowth

Missing Image

Heterologous elements

Virtual slides

Images hosted on other servers:

Uterine adenosarcoma

Positive stains
Negative stains
Electron microscopy description
  • Stromal cells resemble endometrial stromal cells
Molecular / cytogenetics description
  • 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)
Sample pathology report
  • Uterus, total hysterectomy:
    • Müllerian adenosarcoma, high grade, with sarcomatous overgrowth and heterologous rhabdomyoblastic differentiation (3.1 cm); lesion involves cervix and lower uterine segment
    • Myometrial / cervical stromal invasion is present (> 50% of the wall)
    • Lymphovascular invasion is not identified
    • Margins are negative
    • AJCC stage pT1c Nx Mx (FIGO stage Ic)

  • Cervix, polyp, polypectomy:
    • Müllerian adenosarcoma, low grade (2.5 cm) (see comment)
    • Comment: Tumor cells are positive for ER and PR (strong staining in > 90% of cells) as well as CD10.
Differential diagnosis
  • 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:
  • 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
Board review style question #1

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
Board review style answer #1
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.

Comment Here

Reference: Cervix - Adenosarcoma
Back to top
Image 01 Image 02