Ovarian Tumors


Ovarian Tumors




Ovarian Tumors

1)   Categories: Epithelial, Sex cord stromal tumors, Germ cell tumors
a.     Epithelial (surface-derived)
                                               i.     Features
1.     Most common, 65-70% of ovarian tumors
2.     Most likely to seed the omentum
                                              ii.     Types
1.     Serous
a.     Types
                                                                                                     i.     Serous cystadenoma
1.     Benign
2.     Most common benign ovarian tumor
                                                                                                    ii.     Serous cystadenocarcinoma
1.     Malignant
2.     Bilateral
3.     Most common malignant bilateral ovarian tumor
4.     CA-125 tumor marker
b.     Appearance
                                                                                                     i.     Cysts lined by ciliated cells (similar to fallopian tube)
                                                                                                    ii.     Psammoma bodies
1.     Round collections of calcium
c.      Features
                                                                                                     i.     Most common primary benign and malignant tumors
d.     Low-Grade Serous Ovarian Cancer (LGSOC)
                                                                                                     i.     Younger than HGSOC
                                                                                                    ii.     Longer overall survival vs HGSOC, however slower growing cells are less responsive to chemotherapy
                                                                                                  iii.     Treatment is often surgical (aggressively)
e.     Borderline Ovarian Tumors
                                                                                                     i.     Rare
                                                                                                    ii.     Atypical epithelial proliferation without stromal invasion
1.     25% will have invasive disease on final pathology
2.     Excellent survival
2.     Mucinous
a.     Types
                                                                                                     i.     Mucinous cystadenoma
1.     Benign
2.     Grow very rapidly
                                                                                                    ii.     Mucinous cystadenocarcinoma      
1.     Malignant
b.     Appearance
                                                                                                     i.     Similar to endocervical cells
                                                                                                    ii.     Cysts lined by mucus-secreting cells
                                                                                                  iii.     Large, multiloculated tumors
c.      Features
                                                                                                     i.     Seeding produces pseudomyxoma peritonei
1.     Gelatinous ascites
2.     Compresses other organs causing obstruction or cachexia
                                                                                                    ii.     Staging surgery includes removal of the appendix
1.     Is believed to differentiate appendiceal cancer metastasizing to the ovary
3.     Endometrioid
a.     Resembles Endometrial cells
b.     Malignant
c.      Commonly bilateral
4.     Transitional-Cell
a.     Resembles transitional-like epithelium or urothelium
b.     Usually malignant
c.      Often has a stromal component, in which case it is a Brenner tumor (which itself can be benign or malignant)
5.     Clear Cell
a.     Resemble renal cells
b.     Most often malignant
c.      Poor prognosis
b.     Germ cell tumors
                                               i.     Features
1.     15-20% of ovarian tumors
2.     Small number are malignant
                                              ii.     Types
1.     Teratoma
a.     >99% benign
b.     Most common benign germ cell tumor
c.      Congenital neoplasms that differentiate into different cell populations
                                                                                                     i.     Ectoderm, endoderm, mesoderm
d.     Types
                                                                                                     i.     Immature (malignant)
1.     <1% of teratomas
2.     All 3 germ cell layers in haphazard manner
3.     Often with neuroepithelium
                                                                                                    ii.     Mature (benign)
1.     Dermoid Cyst
a.     Contain all 3 cell populations
b.     May contain Rokitansky nodule, a thickened portion of the cyst wall
c.      Compared to immature teratomas, have greater cystic component
                                                                                                  iii.     Monodermal/Highly Specialized
1.     Struma Ovarii
a.     Mature thyroid tissue
b.     Secretes thyroid hormone (tumor marker)
2.     Carcinoid Neoplasm
a.     Secrete bioactive polypeptides and amines, may develop carcinoid syndrome
                                                                                                                                                           i.     Flushing/Diarrhea
b.     Differs from appendiceal carcinoid tumors which undergo first-pass by liver
2.     Dysgerminoma
a.     Most common malignant germ cell tumor
b.     Tumor Marker - Increased LDH
                                                                                                     i.     ‘Lysol Kills Germs’
c.      Similar to seminoma of testis
d.     A/w streak gonads of Turner Syndrome (XO)
3.     Yolk Sac (endodermal sinus tumor)
a.     Malignant
b.     Most common in toddlers <4yo
c.      Schiller-Duval bodies
                                                                                                     i.     Central vessel surrounded by tumor cells contained in a cystic space lined by flattened tumor cells.
                                                                                                    ii.     Represents an attempt to form yolk sacs
d.     Increased a-FP
                                                                                                     i.     ‘Grade A Eggs’
e.     Hyaline bodies
4.     Embryonal carcinoma
a.     Teenagers
b.     Resemble early embryos
                                                                                                     i.     Contain amniotic cavity, thick germ discs that may include primitive gut, and voluminous yolk sac cavity
c.      Tumor marker – Increased b-HCG
                                                                                                     i.     “Teenagers have sex and make embryos”
5.     Polyembryoma
a.     Rare, very aggressive
b.     Is a combination of multiple germ cell tumors
                                                                                                     i.     For instance, may possess embryoid bodies and yolk sac features
c.      A/w Klinefelter’s Syndrome (XXY)
c.      Sex cord stromal tumors
                                               i.     Features
1.     Least common, 3-5% of ovarian tumors
2.     May have hormonal activity
3.     Majority are benign
                                              ii.     Types
1.     Thecoma-Fibroma
a.     Benign
b.     A/w Meig’s Syndrome
                                                                                                     i.     Triad of Pleural Effusion, Ascites and Ovarian Tumor
1.     Ascites and effusion resolve after removal of tumor
c.      Commonly calcified
2.     Granulosa Cell
a.     Low-grade malignant tumor
b.     Tumor Marker – Inhibin B
                                                                                                     i.     ‘Insulin If Fails GCT’
c.      Call-Exner bodies
                                                                                                     i.     Eosinophilic fluid-filled spaces between granulosa cells
3.     Sertoli-Leydig
a.     Very rare
b.     Benign
c.      Produces androgens and can virilize female genitalia
d.     May have crystals of Reinke
                                                                                                     i.     Rod-shaped crystals
4.     Gonadoblastoma
a.     Malignant
b.     Mixture of germ cell tumor and sex-cord stromal tumor
c.      A/w abnormal chromosomal karyotype, gonadal dysgenesis, or presence of Y chromosome
d.     Commonly have microcalcifications
2)   Metastases to Ovary
a.     Features
                                               i.     Traditionally, 5% of ovarian tumors viewed as metastatic from other location (however recent evidence suggests serous ovarian cancer represents metastases from the fallopian tube, which would change this number)
1.     Reason we do prophylactic salpingectomies or fimbriectomies.
                                              ii.     Of non-gynecologic mets, Breast is most common, followed by Stomach
b.     Types
                                               i.     Krukenberg Tumor
1.     Stomach primary; may affect both ovaries
2.     Hematogenous spread
3.     Signet-ring cells
3)   Ovarian Cancer Staging (and Fallopian Tube and Peritoneal)
0: No evidence of primary tumor
I: Confined to ovaries or tubes
IA: One ovary or tube, capsule intact, no tumor on ovarian surface, no cells in ascites or peritoneal washings
IB: Both ovaries or tubes, capsules intact, no tumor on ovarian surfaces, no cells in ascites or peritoneal washings
IC: One or both ovaries
                                    IC1: Surgical spill
IC2: Capsule rupture pre-op or tumor on ovarian surface
                                    IC3: Malginant cells in ascites or peritoneal washings
II: Pelvic extension
                        IIA: Extension to uterus/tubes
                        IIB: Extension to other pelvic organ
III: Peritoneal Metastasis or Nodes
IIIA: Microscopic peritoneal metastasis beyond pelvis or retroperitoneal
                                    IIIA1: Positive retroperitoneal nodes only
IIIA1(i): Mets <10mm
                                                IIIA1(ii): Mets >10mm
                                    IIIA2: Microscopic extrapelvic peritoneal involvement
IIIB: Macroscopic peritoneal metastasis beyond pelvis <2cm in greatest dimension
IIIC: Peritoneal metastasis beyond pelvis >2cm in greatest dimension or lymph node metastasis
IV: Spread beyond peritoneal cavity or metastases
                        IVA: Pleural effusion with positive ascites
IVB: Hepatic/splenic parenchyma mets, metastasis beyond abdomen
4)   Ovarian Cancer Presentation and Clinical Course
a.     Ovarian cancer is often diagnosed at advanced stages.
                                               i.     Most common presentation is bloating, abdominal pain, nausea/emesis
1.     Imaging may find ascites, carcinomatosis, pelvic mass
                                              ii.     The ascites compresses their GI tract, making it difficult to take in nutrition. Increasing tumor burden also compresses the gut, and can result in adhesions and obstructions.
1.     Patients typically die from obstruction and malnutrition, and may receive a palliative PEG tube placement


Image from ObGynKey.com

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