Ovary and Fallopian Tube Pathology 4

Question 1

What are some important features of germ cell tumors?

Answer 1

• 20% of ovarian tumors; resemble germ cell tumors in testis
• Usually children and young adults
• Usually benign cystic teratomas
• 8% are mixed
• Survival: 95% disease free survival due to chemotherapy with bleomycin, etoposide and cisplatin

Question 2

What are the subtypes of germ cell tumors?

Answer 2

• Teratoma
  
  • mature cystic
  • immature teratoma
  • somatic carcinoma
• Dysgerminoma
• Embryonal
  
  • Endodermal sinous tumor
  • Embryonal
  • Choriocarcinoma
  • Trophoblastic tumors
• •

Question 3

What are the major features of mature type teratomas?

Answer 3

• Mature if only contains adult tissues
• Excellent prognosis, even if peritoneal implants are present
• May rupture into peritoneal cavity causing foreign body reaction that simulates metastatic carcinoma or miliary tuberculosis
• Tumors arise from a single germ cell after first meiotic division
• Affects young-aged females (<20, risk for immature teratoma)
• Cystic tumor with all 3 germ layers contributing to tissue formation.
• 46,XX
• 10% to 15% bilateral
• 1% exhibit malignant transformation (most often, squamous cell carcinoma)

Question 4

What are the major histologic features of mature type teratomas?

Answer 4

• Opened cystic teratoma (top) showing the “Rokitansky protuberance” that typically harbors various teratomatous elements.
• Microscopic section of a “dermoid” cyst showing epidermal (skin) components.
  
  • about 1% undergo malignant transformation
  • most commonly it is squamous cell carcinoma
• Struma ovarii - grossly visible thyroid tissue
• Struma carcinoid - derived from intestinal epithelium
  
  • may produce 5-hydroxytryptamine (and the carcinoid syndrome)

Question 5

What are the major histological features of immature teratomas?

Answer 5

• Bulky, rapidly growing tumors in adolescents and young-aged females
• Usually solid (unlike mature teratomas!)
• Grading is based on the amount of immature neuroepithelium (arrows) as this component predicts risk for extra-ovarian spread.
• Differential diagnosis
  
  • fetal tissue: mature
  • embryonic tissue: immature

Question 6

What are the major features of dysgerminomas?

Answer 6

• Less than 1% of ovarian malignancies
• Counterpart of testicular seminoma
• Usually young patients (81% under age 30)
• Metastasize to opposite ovary, retroperitoneal nodes and peritoneal cavity
• Survival: 95%
• Mixture with choriocarcinoma, yolk sac or embryonal carcinoma worsens prognosis
• Hemisected oophorectomy specimen shows nodular tumor separated by fibrous septae. Tumor section shows tan and fleshy cut surface
• Radiosensitive

Question 7

What are the major histological features of dysgerminomas?

Answer 7

• Solid sheets of dysgerminoma cells
• Separated by thin vascularized fibrous septae
• Harboring lymphocytes.
• Occasion syncytial trophoblasts (giant cells)
• High-power magnification shows cells with thin cytoplasmic membranes, cleared cytoplasm, and large, angulated nuclei.
• “Fried -egg” appearance

Question 8

What are the major features of the yolk sac tumor?

Answer 8

• Also called endodermal sinus tumor
• May be derived from embryonal carcinoma
• Usually children or young adults (median age 19 years) with abdominal pain and rapidly growing mass, increasing alpha fetoprotein (AFP) and alpha-1-antitrypsin serum levels; negative hCG
• Fatal without chemotherapy since most have subclinical metastases at presentation

Question 9

What are the major histological findings of the yolk sac tumor?

Answer 9

• Characteristic Schiller-Duval body with tumor cells mantling a vessel
• Reticular pattern (anastomosing network of cells & gland-like spaces)
• Intra- and extracellular hyaline droplets
• AFP+++

Question 10

What are the major histological findings for choriocarcinomas?

Answer 10

• Key features
  
  • elaborate high serum levels of β-hCG
  • most exist in combination with other germ cell tumors
  • microscopy:
    
    • syncytiotrophoblasts
    • cytotrophoblasts and intermediate trophoblasts
    • hemorrhage
• Aggressive tumors that exhibit hematogenous metastases
• Does not respond to chemotherapy

Question 11

What are the major classifications of sex-cord stromal tumors?

Answer 11

• Granulosa cell tumors
  
  • adult (>90%)
  • juvenile (<10%)
• Fibrothecoma
  
  • fibroma
  • thecoma
  • mixed
• Sertoli-Leydig cell tumors
  
  • Sertoli cell tumor
  • Leydig cell tumors
  • mixed
  • others
• •

Question 12

What are the major histologic features of adult granulosa cell tumros?

Answer 12

• Commonly seen in post-menopausal women.
  
  • juvenile form gives rise to precocious puberty
• High estrogen production (associated with endometrial hyperplasia/carcinoma)
• Usually indolent course, but may recur years after initial excision (5% to 25% malignant)—Low malignant potential
• Typical histology:
  
  • solid nests and trabecular of small cells (bottom left).
  • Call-Exner bodies (arrows) are gland-like structures with eosinophilic material.
  • note characteristic nuclear grooves (bottom)
• •

Question 13

What are the major histologic findings of fibromas/thecomas?

Answer 13

• Presents in middle-aged females
• Fibromas associated with Meig’s syndrome (fibroma + ascites + R-sided hydrothorax) and the Basal-cell nevus syndrome (autosomal dominant disorder characterized by multiple basal cell carcinoma, odontogenic keratinocyst of jawbones, and CNS tumors)
• Pure thecomas are rare and may secrete estrogen
• Benign course, but must differentiate from fibrosarcoma, a malignant neoplasm showing cytological atypia and high mitotic counts.
• Histology: fibroblasts (fibroma), plump, lipid-containing spindle cells (thecoma), or mixture of the two cell types

Question 14

What are the major histological functions of Sertoli-Leydig cell tumros?

Answer 14

• Primary affects females in 2nd and 3rd decades of life.
• Pure Sertoli cell tumor may produce estrogens; Sertoli-Leydig cell tumors more commonly virilizing.
• Moderate to poorly differentiated tumors or those with heterologous components (eg., mucinous glands or mesenchymal elements) have worse prognosis.
• Nests and solid tubules composed of cuboidal Sertoli cells. Note characteristic hollow tubules of Sertoli cells (arrows).

Question 15

What are the major findings of metastatic carcinoma of the ovary?

Answer 15

• Uterus/cervix
  
  • endometrioid and serous ca from endometrium
  • adenocarcinoma from cervix
• Breast
  
  • lobular and ductal ca
• Gastro-intestinal
  
  • signet ring cell ca from stomach (Krukenberg’s tumor)
  • mucinous adenocarcinoma from appendix and colon

Question 16

What are the major histologic features of metastatic carcinoma of the ovaries?

Answer 16

• History of non-ovarian cancer
• Bilateral involvement
• Usually small
• Diffuse involvement

Question 17

What is pseudomyxoma peritonei?

Answer 17

• Mucous neoplasm involving peritoneal surface with extensive mucinous ascites (jelly-belly)
• More often appendiceal than ovarian in origin
• Prognostically important to microscopically evaluate mucinous deposits for amount of cytological appearance of neoplastic epithelium

Question 18

What are the correlation of tumors with tumor markers?

Answer 18