1) Clear Cell Adenocarcinoma

CASE 51 year old Nulligravid Epidemiology RULE IN (CONSISTENT WITH THE CASE) Patients with clear cell adenocarcinoma are usually in the fifth or sixth decade (Ackermann) Two-thirds of women with clear carcinoma are nulliparous (Berek et al.) RULE OUT (INCONSISTENT WITH THE CASE) DECISION

Clinical Manifestations Ultrasound Findings

1 year history of on and off abdominal pain and palpable mass 19x16 cm unilocular mass of the left ovary yellow-graying mass with smooth and cystic areas cut section is hemorrhagic and rich in blood vessels

Incidence of bilaterality is less than 10% (Ackermann) Grossly spongy, often cystic appearance (Ackermann); Contain mucinous, serous or hemorrhagic fluid Pelvic mass (up to 30 cm)- partially cystic with yellow, gray and hemorrhagic areas

DDX

Gross Features

 areas of hemorrhage areas of necrosis clusters of cells with pale and scant cytoplasm having large nuclei, prominent nucleoli, and abundant mitotic figures thrombus?? vacuoles/signet ring cells??

- Characterized by large epithelial cells with abundant clear cytoplasm (Robbins) - Microscopically can grow in tubularcystic, papillary, and solid-sheet fashions. The cores of the papillae often exhibit prominent hyalinization (Ackermann) - Most common cell types are clear, hobnail and flat - Clear cells are typically polyhedral, have distinct cell membranes and contain abundant cytoplasm and eccentric nuclei (Sternberg et - Hobnail cells are characterized by bulbous nuclei that protrude into the lumina of tubules and cysts and line papillae (Sternberg et al.) - Tumor cells show anaplastic high-grade nuclei with abundant clear cytoplasm and show a distinctive hobnailed pattern with cells that protrude into the gland lumens like upholsterers nails

al.)

Histological Features

2) DYSGERMINOMA CASE 51 year old Nulligravid RULE IN (CONSISTENT WITH THE CASE) RULE OUT (INCONSISTENT WITH THE CASE) - Occurs mainly in children and young women - 75% occur in the second and third decades - Average age is 22 years and 90% of patients are less than 30 years DECISION RULED OUT

Epidemiology

DDX

Clinical Manifestations Ultrasound Findings Gross Features

1 year history of on and off abdominal pain and palpable mass 19x16 cm unilocular mass of the left ovary yellow-graying mass with smooth and cystic areas cut section is hemorrhagic and rich in blood vessels

Most common clinical presentation is with abdominal distention, and abdominal mass or abdominal pain Somewhat more common on the right side and is bilateral in 15% of cases Areas of cystic degeneration, necrosis and hemorrhage are occasionally present - On cut surface, they have a yellow-white to gray-pink appearance and are often soft and fleshy - Generally solid tumors with a median diameter of 15cm - The serosal surface is smooth or bosselated - Sectioned surfaces are typically soft, fleshy, lobulated

and cream-colored, gray, pink or tan

areas of hemorrhage areas of necrosis clusters of cells with pale and scant cytoplasm having large nuclei, prominent nucleoli, and abundant mitotic figures thrombus?? vacuoles/signet ring cells?? Histological Features

- Mitotic figures are usually numerous - The nuclei are round, medium sized and relatively uniform with vesicular chromatin and prominent nucleoli

- Composed of large vesicular cells having a clear cytoplasm, well-defined cell boundaries, and centrally placed regular nuclei - Consists of polygonal cells with abundant granular eosinophilic or clear cytoplasm - Cells grow in sheets, nests or trabeculae that are separated by fibrous septa; septa vary from thin wisps of connective tissue to thick fibrous bands - Dysgerminoma cells are dispersed in sheets or cords separated by scant fibrous stroma - The fibrous stroma is infiltrated with mature lymphocytes and occasional granulomas - Tumor cells usually group themselves in well-defined nests separated by fibrous strands infiltrated by lymphocytes - Occasionally, a pseudotubular, alveolar, or cordlike arrangement may be seen (the latter particularly at the tumor periphery), which may be quite confusing - Focal necrosis, hyaline changes in vessels, germinal centers, and granulomatous foci may be present - The individual tumor cells are uniform and have large squared-off nuclei, one or more prominent elongated

nucleoli, and abundant clear to finely granular cytoplasm that contains glycogen and sometimes fine droplets of fat. The cell membrane is prominent - Epithelioid cells and multinucleated Langhans-type giant cells are present in many dysgerminomas and often coalesce to form sarcoid-like granulomas

3) SCLEROSING-STROMAL TUMOR

CASE Epidemiology Clinical Manifestations DDX Ultrasound Findings Gross Features 51 year old Nulligravid 1 year history of on and off abdominal pain and palpable mass 19x16 cm unilocular mass of the left ovary yellow-graying mass with smooth and cystic areas cut section is hemorrhagic and rich in blood vessels

RULE IN (CONSISTENT WITH THE CASE)

RULE OUT (INCONSISTENT WITH THE CASE) Occurs in a younger age group 80% occur in women < 30 years old

DECISION RULED OUT

Vast majority of tumors are unilateral, wellcircumscribed, with a solid or solid-cystic and white to yellow cut surface Less homogeneous gross appearance

 areas of hemorrhage areas of necrosis clusters of cells with pale and scant cytoplasm having large nuclei, prominent nucleoli, and abundant mitotic figures thrombus?? vacuoles/signet ring cells?? Histological Features

- Characterized microscopically by a lobular pattern of growth, interlobular fibrosis, marked vascularity, and the presence of a dual cell population: collagen-producing spindle cells and lipid-containing round or oval cells; some of the latter may have a signet ring appearance and thus simulate a Krukenberg tumor - Composed of cellular pseudolobules separated by hypocellular areas containing edematous or collagenous stroma - The cellular areas contain a mixture of spindle cells resembling fibroblasts as well as rounded lutein cells - Lutein cells have ovoid to round nuclei, prominent nucleoli and abundant eosinophilic to clear vacuolated cytoplasm - Occasional signet ring-like cells may also be seen; however, these signet ring-like cells does not contain mucin or express epithelial markers but instead contain cytoplasmic lipid - Presence of dilated thin-walled vessels

4) FIBROMA CASE Epidemiology DDX Clinical Manifestations Ultrasound Findings Gross Features 51 year old Nulligravid 1 year history of on and off abdominal pain and palpable mass 19x16 cm unilocular mass of the left ovary yellow-graying mass with smooth and cystic areas cut section is hemorrhagic and rich in blood vessels RULE IN (CONSISTENT WITH THE CASE) Occurs at an average age of 48 years RULE OUT (INCONSISTENT WITH THE CASE) occur almost invariably after puberty DECISION RULED OUT

Average 6 cm in diameter Hemorrhage, necrosis and calcification and cyst formation may be seen Typically solid, lobulated, firm, uniformly white, and usually not accompanied by adhesions; a few are soft and edematous

 areas of hemorrhage areas of necrosis clusters of cells with pale and scant cytoplasm having large nuclei, prominent nucleoli, and abundant mitotic figures thrombus?? vacuoles/signet ring cells??

- Composed of closely packed spindle stromal cells arranged in a feather-stitched or storiform pattern - Hyaline bands, edema, and hyaline globules may be present - Cytologic atypia and mitotic activity are usually absent or minor in extent

Histological Features

5) THECOMA CASE Epidemiology Clinical Manifestations Ultrasound Findings 51 year old Nulligravid 1 year history of on and off abdominal pain and palpable mass 19x16 cm unilocular mass of the left ovary yellow-graying mass with smooth and cystic areas cut section is hemorrhagic and rich in blood vessels RULE IN (CONSISTENT WITH THE CASE) Occur postmenopausal; mean age of 59 years RULE OUT (INCONSISTENT WITH THE CASE) DECISION RULED OUT

Average about 7 cm in diameter Occasionally, cystic change, hemorrhage, necrosis and calcifications may be seen Typically solid yellow masses; predominantly white and only focally yellow

Gross Features DDX

Histological Features

areas of hemorrhage areas of necrosis clusters of cells with pale and scant cytoplasm having large nuclei, prominent nucleoli, and abundant mitotic figures thrombus?? vacuoles/signet ring cells??

- Large, rounded, ill-defined cells with moderate to abundant pale, sometimes vacuolated cytoplasm, usually containing lipid, are typically present - Composed of sheets to confluent nodules, of ovoid to to spindle cells with round to ovoid shape nuclei, inconspicuous nucleoli, ample pale or vauolated, lipidrich cytoplasm and indistinct cellular borders - Minimal cytolic atypia and mitotic activity - Composed of fascicles of spindle cells with centrally placed nuclei and a moderate amount of pale cytoplasm - The intervening tissue may show considerable collagen deposition and focal hyaline plaque formation - Degree of cellularity varies considerably

REFERENCES Jonathan S. Berek & Neville F. Hacker. Berek & Hacker's Gynecologic Oncology Stephen S. Sternberg, Stacey E. Mills & Darryl Carter. Sternberg's Diagnostic Surgical Pathology, 4e Robert F. Ozols. Ovarian Cancer: American Cancer Society Atlas of Clinical Oncology Robert A. Soslow & Carmen Tornos. Diagnostic Pathology of Ovarian Tumors