Female Genital tract - B/86

Question 1

Metastatic ovarian tumors - Frequency, most common primary tumor, characteristics.

Answer 1

5% frequency. The primary tumor is called a “Krückenberg tumor” and it normally is located in the GI tract. These tumors are usually solid, containing anaplastic cells and glands. May be mucin secreting.

Question 2

Pathogenesis of ovarian tumors - Hereditary and Sporadic forms

Answer 2

Hereditary cases are associated with genetic mutations in the BRCA genes - BRCA1 and BRCA2. BRCA1 mutations carry a higher risk.
Sporadic cases are associated with overexpression of the HER2/NEU and KRAS proteins and mutations in p53.

Question 3

The three cell types which can develop carcinoma in the ovary.

Answer 3

Surface epithelial cells, germ cells, stromal cells

Question 4

Classification of the tumors of surface epithelial cells of the ovary

Answer 4

Serous (serous cyst adenoma, serous borderline and serous cystadenocarcenoma), Mucinous (mucinous cystadenoma, borderline and cystadenoma), Endometrioid and Brenner tumors.

Question 5

Serous tumors of the surface epithelium of the ovary

Answer 5

Serous cyst adenoma - Benign, 25% are bilateral. Smooth glistening cell wall with a cystic space filled with serous fluid. Papillary tumors have papillary projections on the outer surface, or they may be projecting into the cystic cavity. Single layer of columnar cells, within the tips of the papillae we can potentially find psamoma bodies.

Serous borderline - Seen in younger women, often pregnant. Can potentially invade the peritoneum and behave as a carcinoma.

Serous cystadenocarcinoma - 65% are bilateral. Nodular irregularity, solid, necrotic and hemorrhagic. Anaplasia of lining cells. Invades the surroundings. Frequent loss of heterozygosity of p53 and BRCA1

Question 6

Mucinous tumors of surface epithelium of the ovary

Answer 6

Generally: the epithelium consists of mucin secreting cells which form more and larger cysts. The cysts are filled with sticky, gelatinous fluid rich in glycoproteins. Papillary bodies are less frequent - no psamoma bodies. They are also more often unilateral.

Mucinous cystadenoma - Endocervical type epithelium mostly. Intestinal type epithelium (resembling GI) is also possible.

Mucinous border-line tumor - Intestinal type epithelium

Mucinous cystadenocarcinoma - 77% are bilateral (metastatic) while 23% are unilateral (primary). Stromal invasion distinguishes these tumors from border-line tumors. Smooth capsule, cystic and solid areas of tumor evenly distributed throughout the ovary. Without discrete nodularity. Stromal invasion, involves more atypia of the cells and stratification.

Question 7

Endometrioid tumors of the ovary

Answer 7

Can be solid or cystic, may appear as a projection from an endometric cyst. H: tubular glands within the lining of the cystic spaces. Usually malignant, associated with mutation in PTEN tumor suppressor gene.

Question 8

Brenner tumor of the ovary

Answer 8

Solid, usually unilateral. Stroma which contains nests of transitional-like epithelium. Nests may be cystic and lined by mucous secreting cells. Encapsulated, gray-white in color.

Question 9

Classification of the germ cell tumors of the ovary

Answer 9

Teratoma, dysgerminoma, Choriocarcinoma

Question 10

Teratoma of the ovary

Answer 10

Benign (mature) cystic teratoma - differentiation of totipotent cells into mature tissues representing all three germ layers (ectoderm, endoderm, mesoderm). Usually the cyst is lined by epidermis - thus it is called a dermoid cyst. May contain hair, sebaceous glands, teeth, cartilage, bone. Either one of these tissues can become malignant. 90% unilateral. Most often on the right side.

Immature malignant teratoma - often solid with areas of necrosis. Contain a variety of immature areas of differentiation toward various tissues. This includes neuroepithelia - which tend to be quite aggressive and gives metastasis.

Question 11

Dysgerminoma of the ovary

Answer 11

Counterpart of seminoma in males. 80-90% are unilateral. Solid tumors containing sheets, or chords of clear cells which are then separated by fibrous strands. The stroma may contain lymphocytes and granulomas. All of them are malignant. Sensitive to radiotherapy.

Question 12

Choriocarcinoma of the ovary

Answer 12

Always unilateral. Identical to placental tumors as it also contains two types of epithelium (cytotrophoblasts and syncytiotrophoblasts). Hemorrhagic foci may be seen. Give metastasis early - IN CONTRAST TO PLACENTAL TUMORS; THIS TUMOR IS RESISTANT TO CHEMOTHERAPY.

Question 13

Classification of stromal tumors of the ovary

Answer 13

Granulosathecal cell tumors, Thecomafibroma, Sertoli-Leydig cell tumors

Question 14

Granulosathecal cell tumors of the ovary

Answer 14

Occur in any age. Unilateral. Gray-yellow, contain cystic spaces, ovarian follicles may be seen. Theca cells may release estrogen which can lead to endometrial and/or breast carcinoma.

Question 15

Thecomafibroma of the ovary

Answer 15

Unilateral. Solid gray in appearance. Contains fibrous or theca cells. They are rarely malignant, and usually hormonally inactive. May cause ascites and hydrothorax.

Question 16

Sertoli-Leydig cell

Answer 16

Unilateral. Solid, small, shows development of testis an tubules or cords of Sertoli cells. Rarely malignant. Masculinization of defeminization.

Question 17

Clinical correlation of all the ovarian tumors

Answer 17

No symtoms until they are in the advanced stage. Non functional neoplasms lead to lower abdominal pain, abdominal enlargement, and increased pressure on adjacent organs, ascites. CA-125 is a molecular marker which is good for monitoring response to treatment.