Definition/General

Introduction:
-Thecoma is a benign sex cord-stromal tumor composed of theca cells
-Accounts for 1-2% of ovarian tumors
-Produces estrogen causing hormonal effects
-Contains lipid-rich spindle cells.
Origin:
-Originates from ovarian stromal cells with thecal differentiation
-Shows steroidogenic activity
-Contains lipid-laden cells
-Produces predominantly estrogen.
Classification:
-Typical thecoma (90%)
-Luteinized thecoma (with lutein cells)
-Sclerosing thecoma (with fibrosis)
-Fibrothecoma (mixed with fibroma)
-Malignant thecoma (extremely rare).
Epidemiology:
-Peak incidence in postmenopausal women (6th-7th decade)
-Mean age 59 years
-Unilateral in 95% cases
-Estrogen effects in 60-70%
-More common than granulosa cell tumors.

Clinical Features

Presentation:
-Estrogenic effects (postmenopausal bleeding, endometrial hyperplasia)
-Pelvic mass
-Abdominal distension
-Breast tenderness
-Vaginal discharge.
Symptoms:
-Postmenopausal bleeding (60-70%)
-Pelvic/abdominal mass (80%)
-Abdominal pain (40%)
-Breast enlargement
-Vulvar itching
-Weight gain.
Risk Factors:
-Postmenopausal age
-Previous ovarian disorders
-Family history (rare)
-No established hormonal factors
-Environmental associations unclear.
Screening:
-Serum estrogen levels (elevated)
-Inhibin B (elevated)
-FSH/LH (suppressed)
-Endometrial assessment (hyperplasia screening)
-Pelvic ultrasound
-Endometrial biopsy.

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Gross Description

Appearance:
-Solid, well-circumscribed mass
-Yellow to orange cut surface (lipid content)
-Size ranges from 2-20 cm (average 8 cm)
-Lobulated appearance
-Soft consistency.
Characteristics:
-Unilateral in 95% cases
-Smooth capsule
-Cut surface shows yellow-orange tissue
-Lobulated pattern
-Soft to firm consistency
-No hemorrhage or necrosis.
Size Location:
-Variable size (1-25 cm)
-Average size 6-10 cm
-No specific ovarian location preference
-Bilateral involvement rare
-Slow growth pattern.
Multifocality:
-Usually unifocal
-Bilateral involvement in <5%
-No metastatic potential
-May coexist with fibroma (fibrothecoma).

Microscopic Description

Histological Features:
-Spindle to polygonal cells with lipid-rich cytoplasm
-Abundant pale cytoplasm
-Oval nuclei with fine chromatin
-Intervening collagen
-Hyalinization may be present.
Cellular Characteristics:
-Plump spindle cells with abundant cytoplasm
-Pale, vacuolated cytoplasm (lipid)
-Oval nuclei with smooth contours
-Inconspicuous nucleoli
-Low mitotic activity.
Architectural Patterns:
-Sheets and fascicles
-Storiform pattern
-Hyalinized areas
-Edematous regions
-Calcifications may be present.
Grading Criteria:
-Benign thecoma: No significant atypia, <4 mitoses/10 HPF
-Atypical thecoma: Moderate atypia
-Malignant thecoma: Severe atypia, >4 mitoses/10 HPF (extremely rare).

Immunohistochemistry

Positive Markers:
-Inhibin-alpha (95-100%)
-Calretinin (90-95%)
-SF-1 (steroidogenic factor)
-Vimentin (100%)
-Smooth muscle actin (focal)
-CD99 (variable).
Negative Markers:
-Cytokeratin (negative)
-EMA (negative)
-S-100 (negative)
-Desmin (negative)
-CD34 (negative)
-Chromogranin (negative).
Diagnostic Utility:
-Inhibin positivity diagnostic for sex cord-stromal tumor
-Calretinin supports diagnosis
-Distinguish from fibroma (inhibin negative)
-SF-1 confirms steroidogenic function.
Molecular Subtypes:
-Similar to other sex cord tumors
-Low mutation burden
-Steroidogenic gene expression
-Estrogen pathway activation.

Molecular/Genetic

Genetic Mutations:
-Low mutation rate
-Chromosomal stability
-Different from epithelial tumors
-Steroidogenic pathway genes expressed.
Molecular Markers:
-Inhibin overexpression
-Steroidogenic enzymes (aromatase, 17β-HSD)
-Estrogen receptor expression
-Low Ki-67 proliferation index.
Prognostic Significance:
-Excellent prognosis
-Benign behavior
-No malignant potential (typical cases)
-Complete excision curative.
Therapeutic Targets:
-Surgical excision curative
-Enucleation possible
-Estrogen effects resolve after removal
-No hormonal therapy needed.

Differential Diagnosis

Similar Entities:
-Fibroma (inhibin negative, less lipid)
-Granulosa cell tumor (different morphology)
-Steroid cell tumor (polygonal cells)
-Luteinized thecoma
-Fibrothecoma.
Distinguishing Features:
-Thecoma: Lipid-rich cells
-Inhibin positive
-Estrogen production
-Fibroma: Dense collagen
-Inhibin negative
-Granulosa: Coffee-bean nuclei
-Call-Exner bodies
-Steroid cell: Polygonal cells
-Crystalloids.
Diagnostic Challenges:
-Thecoma vs fibroma distinction
-Fibrothecoma diagnosis
-Lipid demonstration
-Hormonal correlation
-Sampling adequacy.
Rare Variants:
-Luteinized thecoma
-Sclerosing thecoma
-Fibrothecoma
-Thecoma with minor granulosa elements
-Malignant thecoma (extremely rare).

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

Thecoma

Classification

WHO Classification: Sex Cord-Stromal Tumor, Thecoma

Histological Features

Shows spindle cells with abundant pale, lipid-rich cytoplasm

Hormonal Effects

Associated with [estrogenic effects/no hormonal effects]

Special Studies

IHC: Inhibin-alpha [positive], Calretinin [positive], SF-1 [positive]

Estrogen levels: [elevated/normal]

Final Diagnosis

Ovarian Thecoma, [Side]