Ovary
Sex cord stromal tumors
Sclerosing stromal tumor
Author: Jennifer A. Bennett, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Topic Completed: 17 December 2019
Minor changes: 19 October 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed search: Ovarian sclerosing stromal tumor (free full text[sb])
Table of Contents
Definition / general | Essential features | ICD coding | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Laboratory | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Frozen section description | Frozen section images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Bennett J. Sclerosing stromal tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorsclerosingstroma.html. Accessed January 23rd, 2021.
Definition / general
- Sclerosing stromal tumor (SST) is a benign stromal tumor with 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 conspicuous in both components
- Positive for sex cord markers but negative for EMA and cytokeratin
ICD coding
- ICD10: D27.9 - benign neoplasm of unspecified ovary
- ICD-0 : 8590/1 C56 - sex cord gonadal stromal tumor, NOS
Epidemiology
- Most present at < 30 years (range: 7 months to 80 years)
Sites
- Ovary
Pathophysiology
- Unknown
Clinical features
- Most present with menorrhagia or abdominopelvic pain
- Typically nonfunctioning but virilization or precious puberty may occur
- Rare associations with Meigs syndrome (Eur J Gynaecol Oncol 2004;25:528, J Obstet Gynaecol 2010;30:747)
Diagnosis
- Mass is observed on imaging
- Diagnosis is made by histological examination of an ovarian resection specimen
Laboratory
- CA-125 often within normal limits but elevated levels have been reported (Eur J Gynaecol Oncol 2017;38:326)
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
Images hosted on other servers:
CT ovarian mass
MRI ovarian mass
Prognostic factors
- Benign prognosis
Case reports
- 14 year old girl with primary amenorrhea (Case Rep Radiol 2015;2015:271394)
- 18 year old woman with irregular menses (Int J Surg Case Rep 2015;9:72)
- 20 year old woman with pelvic pain (BMJ Case Rep 2016;2016:bcr2015214101)
- 24 year old woman with menstrual irregularities and lower abdominal pain (Iran J Pathol 2017;12:402)
- 80 year old woman with incidental pelvis mass discovered on ultrasound (J Menopausal Med 2014;20:80)
Treatment
- Surgical excision (oophorectomy)
Clinical images
Images hosted on other servers:
Laparoscopy
Large mass
Gross description
- Typically unilateral and well circumscribed, ranging from 1.5 to 17 (mean 11) cm
- Gray-white-yellow solid mass often with a single large cyst or multiple small cysts
- Texture ranges from firm and rubbery to edematous and myxoid / mucoid
- Hemorrhage, calcifications and rarely necrosis may be seen (Cancer 1973;31:664)
Gross images
Contributed by Jennifer Bennett, M.D.
Yellow-white mass
Frozen section description
- Mass with notable cellular and hypocellular regions, pseudolobules, staghorn vessels and minimal cytologic atypia are helpful in excluding primary epithelial ovarian malignancies
- Caveat cellular and hypocellular regions with pseudolobules may be seen in Krukenberg tumors, so carefully examine for incipient metastases
Frozen section images
Contributed by Jennifer Bennett, M.D.
Alternating cellularity
Cellular pseudolobule
Microscopic (histologic) description
- Alternating cellular and hypocellular areas impart a pseudolobular appearance (Cancer 1973;31:664)
- 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 are often conspicuous in both components
- Pseudolobules comprised of a jumbled admixture of epithelioid (lutein) and spindled cells with minimal atypia (Cancer 1973;31:664)
- Epithelioid cells: round nuclei with prominent nucleoli, vesicular chromatin and clear to vacuolated cytoplasm
- Abundant eosinophilic cytoplasm often seen in pregnancy (Int J Gynecol Pathol 2015;34:357)
- Occasionally have a signet ring-like appearance
- 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
- Epithelioid cells: round nuclei with prominent nucleoli, vesicular chromatin and clear to vacuolated cytoplasm
- Hypocellular areas can be edematous, collagenous (+/- keloid-like) or myxoid
- Mitoses often inconspicuous but rarely can number up to 12/10 high power fields, no atypical forms (Int J Gynecol Pathol 2016;35:549)
- Infarct type necrosis and calcification infrequent
Microscopic (histologic) images
Contributed by Jennifer Bennett, M.D.
Alternating cellularity
Pseudolobule
Collagenous background
Abundant eosinophilic cytoplasm
Inhibin
Positive stains
- Sex cord markers inhibin, calretinin, SF1, FOXL2
- ER, PR, CD10, Vimentin, smooth muscle actin
- Diffuse TFE3 expression in a subset of tumors (Anticancer Res 2017;37:5441)
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
- Trisomy 12 has been reported in a subpopulation of cells (Am J Surg Pathol 1998;22:83, Eur J Gynaecol Oncol 2004;25:257, Pediatr Dev Pathol 2008;11:300)
Sample pathology report
- Right ovary, oophorectomy:
- Sclerosing stromal tumor (5.0 cm)
Differential diagnosis
- Thecoma (Am J Surg Pathol 2014;38:1023)
- Typically postmenopausal
- Estrogenic manifestations in 50%
- Staghorn vessels absent
- No admixture of cells
- Steroid cell tumor
- Hormonal manifestations in 50%
- Staghorn vessels absent
- No admixture of cells
- Fibroma
- Peri- or postmenopausal
- Often have a uniform white cut surface
- Staghorn vessels absent
- No pseudolobules
- Metastatic signet ring cell carcinoma (Krukenberg tumor) (Am J Surg Pathol 2006;30:277)
- Bilateral in > 50%, often with extraovarian disease at presentation
- Staghorn vessels absent
- Admixed with glands, nests or cords of malignant cells (may be very focal)
- Cytokeratin strongly and diffusely positive
- Pregnancy luteoma
- Often bilateral and multifocal
- Brown and hemorrhagic cut surface
- No admixture of cells
- Solitary fibrous tumor (Histopathology 2018;72:749)
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 oophorectomy and you receive an 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?
- Pregnancy luteoma
- Sclerosing stromal tumor
- Solitary fibrous tumor
- Steroid cell tumor
- Thecoma
Board review style answer #1
Board review style question #2
- Which of the following is true regarding the ovarian lesion pictured above?
- It is characterized by a recurring fusion
- It is only seen in pregnancy
- It is typically white, firm and uniform on cut surface
- Marked cytologic atypia, tumor cell necrosis and atypical mitoses are common
- 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. This is an ovarian sclerosing stromal tumor.
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Reference: Sclerosing stromal tumor
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