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 April 16th, 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)
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)
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)
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
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
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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Reference: Sclerosing stromal tumor