Definition/General
Introduction:
Sertoli cell tumor is a rare sex cord-stromal tumor composed purely of Sertoli cells
Accounts for <1% of ovarian tumors
Typically benign with excellent prognosis
Shows tubular architecture without Leydig cells.
Origin:
Originates from sex cord elements with pure Sertoli cell differentiation
Shows testicular-type differentiation
Contains tubular structures
Usually non-functioning hormonally.
Classification:
Pure Sertoli cell tumor (most common)
Sertoli cell tumor with lipid storage
Sclerosing Sertoli cell tumor
Large cell calcifying Sertoli tumor
Malignant Sertoli cell tumor (extremely rare).
Epidemiology:
Peak incidence in young adults (2nd-3rd decade)
Mean age 25-30 years
Unilateral in >95% cases
Benign behavior in majority
Rare association with Peutz-Jeghers syndrome.
Clinical Features
Presentation:
Asymptomatic pelvic mass (most common)
Abdominal/pelvic pain
No virilization (unlike Sertoli-Leydig tumors)
Abdominal distension
Normal menstrual cycles.
Symptoms:
Pelvic mass (70-80%)
Abdominal pain (40-50%)
No hormonal effects typically
Menstrual regularity maintained
Asymptomatic (30-40%).
Risk Factors:
Young age (reproductive years)
Peutz-Jeghers syndrome (rare association)
No established genetic factors
No environmental associations
Family history rare.
Screening:
Pelvic ultrasound (solid mass)
Tumor markers usually normal
Hormonal assessment (usually normal)
CT/MRI for characterization
No specific tumor markers.
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Gross Description
Appearance:
Solid, well-circumscribed mass
Yellow to tan-gray cut surface
Size ranges from 2-12 cm (average 5 cm)
Lobulated appearance
Firm consistency.
Characteristics:
Unilateral in >95% cases
Smooth capsule
Cut surface shows solid homogeneous areas
Yellow-tan color
Firm consistency
No hemorrhage or necrosis.
Size Location:
Variable size (1-15 cm)
Average size 4-8 cm
No specific ovarian location preference
Smaller than Sertoli-Leydig tumors
Slow growth pattern.
Multifocality:
Usually unifocal
Bilateral involvement rare (<5%)
No metastatic potential (benign cases)
Associated with other tumors in Peutz-Jeghers syndrome.
Microscopic Description
Histological Features:
Tubular structures lined by uniform cells
Sertoli cells with oval nuclei
Eosinophilic to clear cytoplasm
Basement membrane present
No Leydig cells in stroma.
Cellular Characteristics:
Columnar to cuboidal cells
Oval nuclei with fine chromatin
Inconspicuous nucleoli
Eosinophilic cytoplasm
Low mitotic activity.
Architectural Patterns:
Tubular pattern (classic)
Solid nests (focal)
Trabecular pattern
Pseudopapillary areas
Hyalinized stroma may be present.
Grading Criteria:
Benign: No significant atypia, <2 mitoses/10 HPF
Atypical: Moderate atypia, focal
Malignant: Severe atypia, >2 mitoses/10 HPF, necrosis (extremely rare).
Immunohistochemistry
Positive Markers:
Inhibin-alpha (90-95%)
Calretinin (80-90%)
SF-1 (steroidogenic factor)
Vimentin
CD99 (variable)
Smooth muscle actin (focal).
Negative Markers:
Cytokeratin (usually negative)
EMA (negative)
S-100 (negative)
Chromogranin (negative)
AFP (negative)
Beta-hCG (negative).
Diagnostic Utility:
Inhibin positivity confirms sex cord origin
Calretinin supports diagnosis
Distinguish from other sex cord tumors
SF-1 confirms stromal origin.
Molecular Subtypes:
STK11/LKB1 mutations (Peutz-Jeghers cases)
Low mutation burden
Similar to testicular Sertoli tumors
Chromosomal stability.
Molecular/Genetic
Genetic Mutations:
STK11/LKB1 mutations (Peutz-Jeghers syndrome)
Sporadic cases - low mutation rate
Chromosomal stability
DICER1 mutations (rare).
Molecular Markers:
Inhibin overexpression
Sex cord gene expression
Low Ki-67 proliferation index
p53 wild-type.
Prognostic Significance:
Excellent prognosis (benign cases)
Complete excision curative
No recurrence if completely excised
Malignant potential extremely rare.
Therapeutic Targets:
Surgical excision curative
Enucleation possible
Fertility-sparing surgery
No adjuvant therapy needed.
Differential Diagnosis
Similar Entities:
Sertoli-Leydig cell tumor (contains Leydig cells)
Granulosa cell tumor (different morphology)
Endometrioid carcinoma (epithelial markers)
Carcinoid tumor (neuroendocrine markers).
Distinguishing Features:
Sertoli cell: Pure Sertoli cells
No Leydig cells
Sertoli-Leydig: Contains Leydig cells
Virilization
Granulosa: Coffee-bean nuclei
Call-Exner bodies
Endometrioid: CK7 positive
Epithelial features.
Diagnostic Challenges:
Pure Sertoli vs mixed tumors
Benign vs malignant assessment
Complete sampling important
Peutz-Jeghers association.
Rare Variants:
Large cell calcifying Sertoli tumor
Sclerosing Sertoli cell tumor
Sertoli cell tumor with lipid
Malignant Sertoli cell tumor.
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
Ovarian mass, [side], measuring [X x Y x Z] cm
Diagnosis
Sertoli Cell Tumor
Classification
WHO Classification: Sex Cord-Stromal Tumor, Sertoli Cell Tumor
Histological Features
Shows tubular structures lined by uniform Sertoli cells without Leydig cell component
Special Studies
IHC: Inhibin-alpha [positive], Calretinin [positive], SF-1 [positive]
Final Diagnosis
Ovarian Sertoli Cell Tumor, [Side]