Path - Ovary

Question 1

what is autoimmune oophoritis and what is almost always associated with? what can it lead to?

Answer 1

failed self tolerance of endocrine organ - ovary (histo shows CD4 and CD8 T cell and plasma cell infiltration)
Addison’s disease
infertility

Question 2

presentation of polycystic ovary syndrome

what is a key lab finding? what are the outcomes of this?

Answer 2

obesity, hirsutism, type II DM, premature atherosclerosis, infertility (obviously)
increased LH:FSH - increased LH induces excess androgen production –> hirsutism and androgen converted to estrone –> increased risk of endometrial carcinoma

Question 3

benign vs borderline vs malignant serous/mucinous ovarian surface epithelial tumors

Answer 3

benign: simple flat lining in PREmenopausal women (30-40)
borderline: better prognosis than malignant, but still carry metastatic potential
malignant: thick shaggy lining, most common in POSTmenopausal women (60-70)

Question 4

genetics associated with increased risk of malignant surface epithelial ovarian tumors

Answer 4

BRCA1 have increased risk of serous carcinoma

Question 5

type I vs type II pathways of epithelial tumors

Answer 5

I - progress from benign tumors to borderline to low grade malignant
II - arise from INCLUSION CYSTS/serous intraepithelial carcinoma and are usually high grade serous carcinomas

Question 6

how to definitively differentiate/diagnose malignant from benign epithelial tumors?

Answer 6

destructive stromal invasion

Question 7

what mutation is commonly associated with mucinous epithelial tumors?

Answer 7

KRAS

Question 8

what are Brenner tumors? what is the typical presentation? gross appearance?

Answer 8

surface epithelial ovarian tumors composed of bladder like epithelium (transitional cells)
90% unilateral
solid tan/yellow and firm

Question 9

benign vs malignant epithelial tumor clinical features

Answer 9

all present with lower ab pain and ab enlargement, urinary frequency, dysuria pelvic pressure
malignant lesions can have progressive weakness, cachexia and weight loss, massive ascites, OMENTAL CAKE (from local spread to peritoneum)

Question 10

who usually get germ cell tumors?

Answer 10

children and young adults (15-30)

Question 11

what is a cystic teratoma? what makes it malignant?

Answer 11

tumor composed of fetal tissue derived from two or three embryologic layers
presence of IMMATURE tissue (primitive NEUROEPITHELIUM) or somatic malignancy in the tissue (SCC of skin)

Question 12

gross appearance of mature vs immature teratoma

Answer 12

mature: hair and sticky sebaceous debris, teeth common (looks greenish)
immature: fleshy grey tan color with lots of hemorrhaging

Question 13

what is the most common benign germ cell tumor vs malignant?

Answer 13

cystic teratoma (most common overall)
dysgerminoma is most common malignant

Question 14

genetic mutation associated with dysgerminoma, gross appearance, prognosis, possible lab elevation

Answer 14

express KIT and 1/3 have activating mutation in KIT gene
looks yellow/white to pinkish - soft and fleshy
96% cure rate if confined to ovary, 80% if spread
serum LDH may be elevated

Question 15

endodermal sinus tumor presentation, key lab, gross appearance, key histological finding

Answer 15

malignant, most common germ cell tumor in CHILDREN
serum a-fetoprotein is elevated
similar to immature teratoma - very bloody
Schiller-duval bodies - central vessel surrounded by epithelium in a space lined by epithelium (GLOMERULUS-LIKE)

Question 16

ovarian choriocarcinoma

Answer 16

composed of cytotrophoblasts and syncytiotrophoblasts (placental tissue) but NO villi
small and hemorrhagic - EARLY hematogenous spread
labs show high B-hCG (produced by syncytiotrophoblasts)
CHEMORESISTANT (placental version is responsive)

Question 17

presentation of granulosa sex cord stromal tumors, key histological finding

Answer 17

often produces estrogen - presents with signs of estrogen excess (precocious puberty if young, menorrhagia, endometrial hyperplasia if postmenopausal)
Call-Exner bodies - gland like structures with central acidophilic material

Question 18

what biomarker is used to monitor treatment in granulosa tumors? what mutation is present in adults but not juveniles?

Answer 18

inhibin

FOXL2

Question 19

what are fibromas (sex cord stromal tumors) associated with in terms of clinical presentation?

Answer 19

pleural effusions, ascites = Meigs syndrome

also associated with basal cell nevus syndrome (Children)

Question 20

Sertoli-Leydig cell tumors

Answer 20

hormonally active - can produce masculinization/defeminization
over 50% have DICER1 mutation - dysregulation of micro-RNAs
histology shows REINKE CRYSTALS (pink cells with crystals)

Question 21

what does a pure Leydig cell tumor look like?

Answer 21

egg - yellow

Question 22

Krukenberg tumor

Answer 22

metastatic mucinous tumor that involves both ovaries (BILATERAL)
signet ring carcinoma (gastric origin)

Question 23

what would a mucinous tumor of the appendix with metastasis to the ovary present with?

Answer 23

pseudomyxoma peritonei - massive amounts of mucus in peritoneum