Cervix

Mesenchymal / mixed epithelial & mesenchymal tumors

Adenosarcoma


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

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

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PubMed Search: Adenosarcoma cervix

Adam Lechner
Carlos Parra-Herran, M.D.
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Cite this page: Lechner A, Parra-Herran C. Adenosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cervixadenosarcoma.html. Accessed August 10th, 2022.
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
Terminology
  • Also called Müllerian adenosarcoma
ICD coding
  • ICD-O: 8933/3 - adenosarcoma
  • ICD-10: C53.9 - malignant neoplasm of cervix uteri, unspecified
Epidemiology
Sites
  • 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)
Etiology
  • 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
Treatment
  • 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
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High grade adenosarcoma

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Adenosarcoma with sarcomatous overgrowth

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Low grade adenosarcoma

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Phyllodes tumor-like pattern




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

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Periglandular cuff


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Intraglandular papillae

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Stromal mitoses

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Squamous metaplasia


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Sarcomatous overgrowth

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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.

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Reference: Cervix - Adenosarcoma
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