Henry Moon Lecture I have nothing to disclose Ovarian tumors in the - - PowerPoint PPT Presentation

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Henry Moon Lecture

Ovarian tumors in the young Robert H. Young MD I have nothing to disclose

• R. E. Scully
• Described

– Juvenile granulosa cell tumor – Retiform Sertoli-Leydig cell tumor – Small cell carcinoma, hypercalcemic type – Sclerosing stromal tumor – Sex cord tumor with annular tubules – Luteinized thecomas with sclerosing peritonitis

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OVARIAN TUMORS IN THE FIRST THREE DECADES Germ cell (1/3 primitive) 75% Surface epithelial 10% Sex cord-stromal 10% Others including 5% lymphoma- leukemia tumor-like lesions THE FIRST DECADE

• Primitive germ cell tumors

uncommon

• Dermoids rare before 2 years
• Surface epithelial tumors rare
• All sex cord tumors seen
• Follicle cysts common (esp. neonatal)
• Small cell carcinoma rare

THE SECOND DECADE

• Primitive germ cell tumors progressively more

frequent (peak 18-20 years)

• Surface epithelial tumors (esp. mucinous) seen

with increased frequency

• Retiform Sertoli-Leydig cell tumors peaks
• Follicle cysts common (perimenarchal)
• Small cell carcinoma accelerates
• Metastases rare but seen

THE THIRD DECADE

• Primitive germ cell tumors still

“common”

• Surface epithelial tumors and metastases

each progressively more common

• Sertoli-Leydig cell tumors peak
• Small cell carcinoma peaks
• Sclerosing stromal tumor peaks
• Pregnancy-related issues most common

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ISSUES RELATED TO OVARIAN MASSES DURING PREGNANCY 1. Non-specific changes 2. Ovarian pregnancy and trophoblastic lesions 3. Specific tumor-like lesions 4. Alterations in morphology of sex cord tumors

• 5. Function due to stromal

luteinization

PREGNANCY-RELATED TUMOR-LIKE LESIONS

1. Pregnancy luteoma 2. Hyperreactio luteinalis 3. Large solitary follicle cyst 4. Granulosa cell tumorlets

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Ovarian Tumors with Functioning Stroma

Tumors During pregnancy Trophoblast -Containing Tumors Idiopathic- Primary, Metastatic

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Tumors in the young

• 1. Primitive germ cell tumors
• 2. But also dermoids!
• 3. Sex cord-stromal tumors
• a. Sertoli Leydig/Juvenile granulosa
• b. Some pure stromal tumors
• 4. Steroid cell tumors
• 5. Do not forget metastases, sadly
• 6. Selected specific beasts!
• a. Small cell carcinoma
• b. Desmoplastic small round cell tumor

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Yolk Sac Tumor

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IMMATURE TERATOMAS

• Account for 20% of malignant germ cell

tumors

• Account for 15% of ovarian cancers in

first two decades

• Median age, 18 years
• 25% have ipsilateral and 10%

contralateral dermoid cyst

• Rarely have elevated hCG and AFP

levels

Gross Features a Major Clue in Distinction Between Immature Teratoma and Dermoid Cyst

• 1. Average size of immature teratoma 16 cm.
• 2. Average size of dermoid cyst 8 cm.
• 3. Most immature teratomas solid and cystic, more the former

and with worrisome appearance.

• 4. Dermoid cyst generally dominantly cystic and solid foci

lack hemorrhage and necrosis.

• 5. A grossly typical dermoid is likely to be that after

microscopic examination in vast likelihood.

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Juvenile Granulosa cell tumor

0 - 67 (Av 13) yrs. 97% <30 yrs.

Retiform Sertoli-Leydig cell tumor

2 - 39 (Av 15) yrs.

Small Cell Carcinoma hypercalcemic type

1 - 44 (Av 23) yrs.

32 Cases (16 years or less)

26 JGCT 3 AGCT 3 Cystic, Nos

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CARDINAL FEATURES OF JUVENILE GRANULOSA CELL TUMOR

• Follicles that are typically variable in

size and shape

• Luteinized cells
• Mitotically active
• Pleomorphic cells in 10 – 15% of cases
• Generally inconspicuous stroma

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DIFFERENTIAL DIAGNOSIS – JUVENILE GRANULOSA CELL TUMOR

• Adult granulosa cell tumor
• Small cell carcinoma
• Thecoma
• Primitive germ cell tumors
• Undifferentiated carcinoma
• Transitional cell carcinoma
• Clear cell carcinoma
• Pregnancy luteoma
• Large solitary follicle cyst

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Luteinized Thecoma(tosis) Associated with Sclerosing Peritonitis (LTSP)

• Age 10 mo to 85 y (median

27)

• Abdominal distention, pain,

ascites

• Bilateral (25/27)
• Sclerosing peritonitis

(25/27)

• 2-31 cm (mean 10 cm),

round or lobulated

• Soft, often markedly

edematous, hemorrhagic

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Sclerosing Stromal Tumor

• Young women -

80% under 30

• Rarely hormone-

producing

• Unilateral, solid

to cystic

• Benign

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Type of Sertoli- Leydig cell tumor Well differentiated 15% 34 50% Intermediate 70% 25 45% Poorly differentiated 15% 25 53% Heterologous 20% 23 39% Retiform 15% 15 23%

A N D F R E A G E

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CLINICAL PRESENTATION

Age range 1 to 44 (mean 23) y Abdominal pain and swelling Elevated serum Ca++ in 62% Rare familial cases 99% unilateral Small cell carcinoma of hypercalcemic type

Ovarian Tumors with Paraendocrine Hypercalcemia

Small cell CA 59% Clear cell CA 18% Serous CA 6% Squamous cell CA in dermoid cyst 6% Dysgerminoma 6% Mucinous CA 3% Mixed clear cell/endometrioid CA 1% Steroid cell tumor 1%

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