Ovary

Sex cord stromal tumors

Sclerosing stromal tumor


Editorial Board Member: Gulisa Turashvili, M.D., Ph.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Jennifer A. Bennett, M.D.

Last author update: 24 May 2022
Last staff update: 24 May 2022

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PubMed search: Ovarian sclerosing stromal tumor

Jennifer A. Bennett, M.D.
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Cite this page: Bennett J. Sclerosing stromal tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorsclerosingstroma.html. Accessed February 6th, 2023.
Definition / general
  • Sclerosing stromal tumor is a benign stromal tumor that has a pseudolobular appearance resulting from alternating cellular and hypocellular areas
Essential features
  • Alternating cellular and hypocellular areas impart a pseudolobular appearance
  • Pseudolobules contain a haphazard arrangement of epithelioid (lutein) and spindled cells
  • Hemangiopericytoma-like vessels are conspicuous in both components
  • Positive for sex cord markers but negative for EMA and cytokeratin
ICD coding
  • ICD-10: D27.9 - benign neoplasm of unspecified ovary
  • ICD-11: 2F32 - benign neoplasm of ovary
Epidemiology
Sites
  • Ovary
Pathophysiology
Etiology
  • Unknown
Clinical features
Diagnosis
  • Mass is often observed on imaging
  • Diagnosis is made by histological examination
Laboratory
Radiology description
  • CT:
    • Plain: nonhomogeneous density
    • Contrast: peripheral ring enhancement; may see enhancement of central patch or internal septa
    • Venous phase: enhancement decreases but increases with centripetal progression; no enhancement of cystic component (Oncol Lett 2016;11:3817)
  • MRI:
    • T1 weighted: slight hyperintense periphery, irregular hypointense center
    • T2 weighted: hyperintense cystic components or heterogeneous solid mass with intermediate to high intensity
    • T1 with contrast: early peripheral enhancement with centripetal progression (AJR Am J Roentgenol 2005;185:207)
Radiology images

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CT ovarian mass

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MRI ovarian mass

Prognostic factors
Case reports
Treatment
  • Complete surgical excision (oophorectomy)
Clinical images

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Laparoscopy

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Large mass

Gross description
  • Typically unilateral and well circumscribed, ranging from 1.0 - 23 cm (mean: 8.4 cm) (Histopathology 2022;80:360)
  • White-yellow variegated solid mass, often edema and cysts
  • Hemorrhage, calcifications and rarely necrosis may be seen
Gross images

Contributed by Jennifer Bennett, M.D.
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Yellow-white mass

Frozen section description
  • Alternating cellular and hypocellular areas that impart a pseudolobular appearance may be seen in Krukenberg tumors; carefully examine for incipient metastases
Frozen section images

Contributed by Jennifer Bennett, M.D.
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Alternating cellularity

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Hypercellular pseudolobule

Microscopic (histologic) description
  • Alternating cellular and hypocellular areas impart a pseudolobular appearance
  • Hypocellular foci may be ill defined in pregnancy due to expansion of the pseudolobules by lutein cells (Int J Gynecol Pathol 2015;34:357)
  • Thin, dilated and branching hemangiopericytoma-like vasculature is often conspicuous in both components
  • Pseudolobules comprised of a jumbled admixture of epithelioid (lutein) and spindled cells with minimal atypia
    • Epithelioid cells: round nuclei with prominent nucleoli, vesicular chromatin and clear to vacuolated cytoplasm
    • Spindled cells: elongated nuclei with indistinct nucleoli, bland chromatin and scant eosinophilic cytoplasm
      • Typically round to ovoid but may show angulation if edema is striking
  • Hypocellular areas can be edematous, collagenous (variably keloid-like) or myxoid
  • Mitoses are often inconspicuous but rarely can be up to 12/10 high power fields, no atypical forms (Int J Gynecol Pathol 2016;35:549)
  • Infarct type necrosis and calcification infrequent
  • References: Cancer 1973;31:664, Histopathology 2022;80:360
Microscopic (histologic) images

Contributed by Jennifer Bennett, M.D.
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Alternating cellularity

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Pseudolobule

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Collagenous background

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Abundant eosinophilic cytoplasm

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Inhibin

Virtual slides

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Sclerosing stromal tumor

Positive stains
Negative stains
Electron microscopy description
  • 3 cell types (Int J Gynecol Pathol 1988;7:280):
    • Epithelioid cells: membrane bound cytoplasmic lipid, well developed Golgi
    • Spindled cells: fibroblast-like, prominent rough endoplasmic reticulum
    • Undifferentiated primitive mesenchymal cells (in hypocellular areas): few organelles, rare cilia and basal lamina
  • Smooth muscle differentiation supported by aggregates of cytoplasmic filaments with interspersed dense bodies, pinocytotic vesicles and basal lamina (Ultrastruct Pathol 1992;16:363)
Molecular / cytogenetics description
Sample pathology report
  • Right ovary, oophorectomy:
    • Sclerosing stromal tumor (5.0 cm)
Differential diagnosis
Board review style question #1
A 30 year old woman presented with pelvic pain; ultrasound detected a 10 cm mass in the left ovary. She underwent an oophorectomy. You receive the intact specimen with a 10 cm well circumscribed yellow-white edematous lesion with multiple cysts that replaces the entire ovary. Histologic examination shows cellular areas that alternate with hypocellular edematous foci. Staghorn vessels are prominent. What is the likely diagnosis?

  1. Pregnancy luteoma
  2. Sclerosing stromal tumor
  3. Solitary fibrous tumor
  4. Steroid cell tumor
  5. Thecoma
Board review style answer #1
B. Sclerosing stromal tumor

Comment Here

Reference: Sclerosing stromal tumor
Board review style question #2

Which of the following is true regarding the ovarian lesion pictured above?

  1. It is characterized by a recurring mutation
  2. It is only seen in pregnancy
  3. It is typically white, firm and uniform on cut surface
  4. Marked cytologic atypia, tumor cell necrosis and atypical mitoses are common
  5. The cellular foci are composed of lutein and spindled cells
Board review style answer #2
E. The cellular foci are composed of lutein and spindled cells

Comment Here

Reference: Sclerosing stromal tumor
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