Term
| how does turner's syndrome affect the ovaries? |
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Definition
| turner's syndrome (45,X, short stature, coarctation of the aorta, infertility, amenorrhea) pts usually have atrophic streak ovaries (leads to endocrine abnormalities) |
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Term
| what does ovarian torsion cause? what does it mimic? |
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Definition
| causes: ovarian pain/enlargement, mimics: cystic disease/tumor |
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Term
| what are follicular cysts? |
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Definition
| ovarian follicular cysts originate in *unruptured graafian follicles or in follicles that have ruptured and *immediately sealed. these cysts are usually multiple, occasionally larger than 2 cm and filled w/serous fluid. the outer thecal cells can be conspicuous and may have a large cytoplasm. granulosa cells can be identified. these cystic follicles are so common to almost be physiologic. |
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Term
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Definition
| luteal cysts are normally present in the ovary, and are *lined by a rim of bright yellow luteal tissue containing *luteinized granulosa cells. these may rupture and cause a *peritoneal irritation/reaction. |
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Term
| what is polycystic ovary disease (PCOD or stein leventhal syndrome)? |
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Definition
| polycystic ovary disease is a central pathologic abnormality with *numerous follicle cysts, often associated with *oligomenorrhea. this affects 3-6% of women at reproductive age. |
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Term
| what are common clinical presentations of PCOD? |
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Definition
| anovulation, obesity, hirsutism, virulism (more masculine features) |
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Term
| how do ovaries affected by PCOD appear grossly? histologically? |
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Definition
| grossly: the ovaries may be 2x normal size. histologically: PCOD appears as multiple subcortical cysts with a thickened superficial cortex and *hyperplasia of the theca interna |
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Term
| how does PCOD manifest its hormonal symptoms? |
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Definition
| the increased theca interna cells lining the cysts lead to an increase in luteinizing hormone. increased estrogen is due to conversion of androgens, however the enzymes involved in androgen biosynthesis are poorly regulated, so this leads to some virilizing features (some androgens may also be produced). |
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Term
| what is stromal hyperthecosis? |
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Definition
| stromal hyperthecosis is characterized by *hypercellular, luteinized stroma* cells histologically and uniform enlargement of the ovary (up to 7 cm) with a tan white appearance. this is usually bilateral and is seen in *postmenopausal women. in younger women, this may *blend w/PCOD. the clinical effects are similar to PCOD, but the virilization may be more striking. |
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Term
| what characterizes the incidence ovarian CA generally? |
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Definition
| ovarian CA is the 5th most common cause of CA in women and accounts for a disproportionate number of fatal CA (mainly b/c no good screening tests). approx 80% of tumors are benign, mostly occurring in women 25-45. however, in older women (40-65), malignant tumors are more common. |
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Term
| what are risk factors for ovarian tumors? |
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Definition
| nulliparity, family hx, heritable mutations, gonadal dysgenesis, and BRCA-1/2 |
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Term
| what is the estimated risk of developing ovarian CA if the pt has BRCA-1/2? |
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Definition
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Term
| what % of ovarian CA is p53 found in? |
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Definition
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Term
| what is the incidence of ovarian adenocarcinomas expressing the Her2/neu oncogene? what is the prognosis? |
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Definition
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Term
| how do OCPs affect ovarian CA risk? why? |
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Definition
| OCPs lower ovarian CA risk b/c most ovarian tumors arise from the surface epithelium, which is linked to the repeated trauma of ovulation. since OCPs prevent ovulation, CA arising from the associated trauma is less likely. |
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Term
| what makes up the majority of ovarian CA? |
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Definition
| serous cystadenocarcinomas |
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Term
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Definition
| monodermal germ cell teratomas which have a lot of thyroid tissue |
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Term
| what are the broad categories for ovarian CA? |
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Definition
| surface epithelial-stromal cell tumors, germ cell tumors, sex cord-stroma tumors, and metastasis to the ovaries. |
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Term
| what are the surface-epithelial ovarian tumors? |
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Definition
| serous, mucinous, endometrioid, clear cell, and brenner tumor |
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Term
| what characterizes the surface epithelial-stromal cell tumors? |
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Definition
| these are the *most common ovarian tumors (2/3) and the most likely to be malignant (90%). they usually affect pts 20+. |
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Term
| are there markers for surface epithelium tumors? |
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Definition
| there are no significant serum screening tools available. CA-125 is present in the serum of 80%+ of pts w/serous and endometrioid CA, but this can be elevated in any peritoneal irritation. osteopontin is a new biomarker which has been found to be higher in ovarian CA pts, but this is still in development. |
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Term
| what is associated with a significant reduction in surface epithelium tumor risk? |
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Definition
| fallopian tube ligation and OCPs |
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Term
| what grades are surface epithelial tumors divided into? |
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Definition
| benign, borderline, and malignant |
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Term
| what characterizes the serous subtype of surface epithelial tumors? |
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Definition
| these appear as cystic spaces w/papillary formations (glandular lining) filled with *clear fluid. they can be benign, borderline, or malignant and make up 25-30% of all ovarian tumors. serous adenocarcinomas are the most common malignant ovarian tumor, w/benign+borderline tumors more common between 20-45 and serous carcinoma occurring later in life. *30-50% may be bilateral (serous subtype -> most likely to be bilateral).* |
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Term
| what risk factors are associated specifically with surface epithelial serous tumors? |
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Definition
| nulliparity. high grade tumors are associated with BRCA1/2 mutations and p53 mutations. low grade tumors arising from borderline tumors often have KRAS and BRAF oncogene mutations. |
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Term
| how do the benign serous tumors appear? |
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Definition
| as a smooth glistening cyst wall w/no epithelial thickening or w/small papillary projections, lined by columnar cells w/cilia (benign, well differentiated). |
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Term
| how do the borderline serous tumors appear? prognosis? |
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Definition
| there is an *increased number of papillary projections w/complex stromal papillae, stratification of the epithelium and mild nuclear stratification of the epithelium and mild nuclear atypia *w/o destructive infiltrative growth into the stroma. prognosis if confined to the ovary: 100% 5 yr survival, if spreads to the peritoneum: 90% |
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Term
| how do the malignant serous tumors appear? how are the graded? what happens if they spread to the peritoneum? prognosis? |
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Definition
| there are larger amounts of *solid or papillary masses, irregularity of tumor, nodularity of capsule, marked nuclear atypia, pleomorphism mitotic figures, and multinucleation. they may be graded high/low. if these spread to the peritoneum = ascites. prognosis if confined to the ovary: 70% 5 yr survival, if spreads to the peritoneum: 25% |
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Term
| what characterizes the mucinous subtype of surface epithelial tumors? |
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Definition
| most mucinous tumors appear as larger multiloculated (honeycomb) cysts filled with turbid fluid (gelatinous glycoproteins). these make up 15-25% of ovarian tumors and 10-20% of bilateral ovarian tumors (less than stroma). these are usually seen in middle adult life (rare before puberty OR after menopause) and they will grow larger than serous tumors. 80% are borderline/benign. |
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Term
| what is pseudomyxoma peritonei? |
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Definition
| the implant of surface epithelial mucinous tumors on peritoneal structures (may accidentally happen during surgical removal) |
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Term
| what are risk factors for surface epithelial mucinous tumors? |
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Definition
| smoking as well as mutations of the KRAS proto-oncogene |
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Term
| how do benign surface epithelial mucinous tumors appear? |
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Definition
| they are lined by well differentiated tall columnar epithelium *w/apical mucin and absence of cilia (which would be seen in serous type) |
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Term
| how do borderline surface epithelial mucinous tumors appear? |
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Definition
| they may have cells w/more papillary growth, nuclear atypia, and nuclear stratification |
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Term
| how do malignant surface epithelial mucinous tumors (mucinous cystadenocarcinomas) appear? |
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Definition
| these have more solid growth patterns w/obvious nuclear atypia and stratification. need to look for stromal invasion. |
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Term
| what is the prognosis for surface epithelial mucinous tumors? |
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Definition
| noninvasive 10 yr survival is 95% and 90% for invasive. mucinous CA that has spread outside the ovary is usually fatal. |
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Term
| why is dx for some ovarian tumors so difficult? |
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Definition
| b/c sometimes the only signs/symptoms are increased abdominal girth (tumor volume+ascites) and there are no effects on other organ structures (bladder, ureters, etc) |
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Term
| what characterizes the endometrioid subtype of surface epithelial tumors? prognosis? |
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Definition
| these resemble endometrial adenocarcinoma (some of which are associated with exactly that) and appear as a large cystic mass w/solid areas (not multiloculated). the benign and borderline grades of this type are less common, but the ovarian endometrioid CA type is more common and accounts for ~20% of all tumors. most are small, well differentiated and have a *good prognosis. |
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Term
| are the endometrioid subtype of surface epithelial tumors associated with endometriosis? |
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Definition
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Term
| what genetics are associated with the endometrioid subtype of surface epithelial tumors? |
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Definition
| the *PTEN tumor suppressor gene mutation*, KRAS and beta-catenin oncogenes, and p53 mutations (common in poorly differentiated tumors). |
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Term
| what characterizes the clear cell subtype of surface epithelial tumors? |
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Definition
| these are thought to be a variant of endometrioid adenocarcinoma (just w/more clear cells) and may occur in association w/endometriosis or endometrioid CA of the ovary and *resembles the clear cell CA of the endometrium. they have a cystic or solid appearance and a prominent clear cytoplasm. they are seen in pts 40-50 yrs old (pre-perimenopausal). |
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Term
| how does the clear cell subtype of surface epithelial tumors appear histologically? |
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Definition
| as solid tumors where the clear cells are arranged in sheets or tubules. neoplastic cells line the cysts and large tumor cells with clear cytoplasm are are characterized as having "hobnail" cytoplasm (protrudes outward). |
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Term
| what is the prognosis for the clear cell subtype of surface epithelial tumors? |
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Definition
| 65% 5 yr survival if confined to the ovaries, however if the tumor is more aggressive, it can often spread beyond the ovary. |
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Term
| what is a cystadenofibroma? |
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Definition
| a benign tumor with a pronounced proliferation of *fibrous stroma under the columnar lining epithelium. cystadenofibromas appear small and multilocular w/simple papillary processes and may have mucinous, serous, endometrioid and transitional epithelium. borderline lesions are uncommon. |
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Term
| what characterizes the brenner tumor subtype of surface epithelial tumors? |
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Definition
| these are basically adenofibromas in which the epithelial component consists of a *nest of transitional-type epithelial cells. grossly they appear unilateral, firm, white and solid and histologically they appear as solid and or cystic nests of cells resembling transitional cells *w/grooved nuclei (coffee bean-like). most are benign (but some borderline/malignant have been reported), mucinous glands may be present and they can vary in size from small to large. |
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Term
| what categorizes the germ cell tumors? |
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Definition
| these are the second most common tumor/malignancy in the ovary and tend to occur in younger pts. there are 4 types: teratomas, dysgerminomas, endodermal sinus/yolk sac tumors, and choriocarcinomas |
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Term
| what are the 3 kinds of teratomas? |
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Definition
| mature (*most common*, adult tissue, completely benign), immature (more aggressive, primitive), and monodermal (one predominant cell type, like struma ovarii: 100% mature thyroid tissue). |
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Term
| what characterizes the mature teratomas? |
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Definition
| mature teratomas are usually seen in younger women, appear cystic (aka dermoid cyst) and are derived from totipotent cells (reason for heterogenous tissue composition). these may be b/l in 10-20%. the cyst wall may appear wrinkled and gray and contain tissue resembling what might be seen in an adult: hair, skin (squamous cells), bone, cartilage, teeth, thyroid, brain - each of which may contain a CA of its own (1% incidence). |
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Term
| what is the pathogenesis for mature teratomas? |
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Definition
| mature teratomas may arise from the ovum after the first meiotic division, and are typically 46xx. |
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Term
| what characterizes the monodermal teratomas? |
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Definition
| these are teratomas w/one predominant cell type and the 3 kinds are: *struma ovarii (composed of thyroid tissue, can cause hyperthyroidism), *carcinoid (arise from intestinal epithelium, can produce carcinoid syndrome), and *strumal carcinoid (both thyroid and carcinoid elements are present). |
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Term
| what characterizes the immature teratomas? |
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Definition
| in these, primitive, embryonic tissue is seen, such as immature hair, bone, cartilage, glands, nerve tissue. grossly immature teratomas appear bulky w/a smooth external surface and a solid cut surface of necrosis and hemorrhage. these are mostly seen in prepubertal adolescents/young women and prognosis is related to grade+stage. |
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Term
| what characterizes the dysgerminomas? |
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Definition
| these are the ovarian counterpart to seminomas in the testes. dysgerminomas appear solid, soft, fleshy yellow white grossly. histologically: large vesicular cells w/clear cytoplasm, well defined borders, centrally located nuclei, *lymphocytic infiltrate, and *prominent nuclear membranes. these comprise 50% of *malignant germ cell tumors and are seen in the 2nd/3rd decade of life. they tend to be unilateral and *may produce elevated levels of chorionic gonadotropin. by definition *all are malignant, but like seminomas they have a good prognosis. they may present asymptomatically. |
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Term
| what characterizes the yolk sac tumor/endodermal sinus tumor? |
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Definition
| these are rare, but are the 2nd most common malignant germ cell tumor. they are associated with high levels of *AFP, *alpha 1 antitrypsin, and *schiller duval bodies (primitive glomerular structures formed by germ cells around a central blood vessel). these are more rapidly growing, and therefore are more malignant/symptomatic. yolk sac tumor/endodermal sinus tumors may present as abdominal pain w/a large, rapidly growing lesion in children/young women. |
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Term
| what characterizes choriocarcinomas? |
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Definition
| choriocarcinomas are more commonly of placental origin and are an example of extraembryonic differentiation of malignant germ cells. in the ovary, usually they exist in combination w/other germ cell tumors (pure choriocarcinomas are rare). these appear soft, yellow, fleshy, and necrotic and tend to be very aggressive w/early metastasis to lungs, liver, and bone. |
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Term
| what categorizes the sex cord-stroma cell tumors? what are the sex cord-stroma cell tumors? |
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Definition
| these are more rare and affect all ages equally. these may have some endocrine involvement (particularly estrogen). they include the granulosa-theca cell tumors, fibroma-thecomas, and sertoli-leydig cell tumors |
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Term
| what are the granulosa-theca cell tumors? |
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Definition
| these contain varying proportions of granulosa and theca cell differentiation (may be composed of entirely granulosa cells, or combination of both). 2/3 occur in *postmenopausal women. grossly: yellow surface, unilateral small foci to larger solid/cystic masses. histologically: cells growing in cords, sheets, nests (thecoma component) and *call-exner bodies (neoplastic cells in gland-like structures)*. granulosa-theca cell tumors *produce large amounts of estrogen and can stimulate *endometrial hyperplasia/CA/breast changes. *inhibin is found to be in higher levels in these tumors and may serve as a useful marker. along w/brenner's tumor, these will also have the grooved coffee bean nuclei. |
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Term
| what are the fibroma-thecoma tumors? |
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Definition
| fibroma/thecoma tumors are usually seen in combination w/varying ratios of cell type. grossly: unilateral, solid, lobulated, hard, gray-white and covered by intact serosa. histologically: fibroma potion - fibroblasts, cylindrical cigar-like cells, thecomas - plump spindle cells w/lipid droplets, *may be secreting estrogen*. these may be associated w/basal cell nevus syndrome and **meige's syndrome: ovarian tumors (usually fibromas) which are associated w/ascites and hydrothorax (usually pleural effusion on R side) = increased abdominal girth.** |
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Term
| what are the sertoli-leydig cell tumors? |
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Definition
| these tumors consist of recapitulation of the testes cells, usually causing masculinization/defeminization - but sometimes these androgens are converted to estrogens = possible estrogenic effect. these are seen in the 2nd/3rd decade and appear unilateral, solid, gray and golden brown. histologically, tubules composed of sertoli/leydig cells are seen (looks like testes) along w/glands in cord or nest formation. SCAT (sex cord tumor with annular tubules) may also be seen. |
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Term
| what characterizes tumors that metastasize to the ovaries? |
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Definition
| possible mets to the ovary are from the stomach, colon, contralateral ovary, uterus, and fallopian tubes. |
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Term
| what characterizes gastric CA mets to the ovary? |
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Definition
| diffuse gastric CA mets to the ovary = the krukenberg tumor, which is large, lobular, often bilateral and tends to be solid (but can be cystic/necrotic). histologically, *signet ring cells are seen diffusely: abundant intracellular mucin pushing the nucleus to the periphery and possibly some spindle cells. pts may present w/vaginal bleeding and/or palpable bilateral enlarged ovaries. |
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Term
| how would colon CA met to the ovary appear? |
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Definition
| glandular epithelium would likely be seen in the ovary if the met was well-differentiated, but the cells lining the gland would still be abnormal (high N:C ratios), papillary structures pushing out and hemorrhage would also likely be seen. GI symptoms would usually also be seen: diarrhea, constipation, blood (due to primary lesions). |
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Term
| what characterizes small cell CA mets to the ovary? |
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Definition
| small cell CA may met to the ovary from the lungs etc and appear histologically as predominant crowding small cells w/small cytoplasms, dark homogeneous nuclei formed in cords, trabeculae and nests. |
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Term
| what characterizes burkitt's lymphoma mets to the ovary? |
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Definition
| this would give the starry sky appearance w/prominent lymphocytes being the dark "sky" and macrophages engulfing the apoptotic lymphocytes being the "stars". this, like most metastatic tumors is more likely to be found in elderly pts. |
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