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OVARIAN TUMORS, image, image, image, image, image, image, image, image,…

- - - - SEROUS CYSTADENOMA, ADENOFIBROMA AND SURFACE PAPILLOMA
        
        Definition: benign serous tumors composed of cells resembling fallopian tube epithelium
        
        Macroscopic:
        1) Wide variation in size
        2) Cystadenomas have smooth outer and inner surfaces; they may be septated and filled with watery fluid
        3) Cysts <1 cm are designated cortical inclusion cysts
        4) Cystadenofibromas contain cysts surrounded by a variable amount of solid areas
        5) Adenofibromas are typically solid and punctated with small cysts
        6) Surface papillomas are exophytic
        
        Microsocpic:
        1) Epithelial lining consists of non-stratified cuboidal or columnar cells (resembling tubal secretory or ciliated cells) in varying proportions
        2) In some tumors, the epithelial lining is flattenered and non-descript
        2) Serous cystadenomas are predominantly cystic
        3) Serous adenofibromas are composed of small glands and cysts in a prominent fibrous stroma
        4) Serous cystadenofibromas have cysts and broad and simple papillae embedded in prominent fibromatous stroma
        5) Surface papillomas are characterized by small simple papillae
        6) If <10% of the the total tumor volume (except for surface involvement) shows epithelial prolifeation within the cysts that would otherwise qualify as serous borderline tumor, the tumor is designated as serous cystadenoma with focal epithelial proliferation
    - - SEROUS BORDERLINE TUMOR (SBT)
        
        Definition: non-invasive, low-grade, proliferative serous epithelial neoplasm
        
        Epidemiology: median age is 50 years
        
        Pathogenesis: somatic mutations of KRAS or BRAF are most common in these tumors, although BRAF mutations are less frequent in advanced stage than stage I tumors
        
        Macroscopic:
        1) Tumors are generally >5 cm and may be intracystic (and lined by excresences) and/or exophytic with surface involvement
        2) Approximately 1/3 are bilateral
        
        Microscopic:
        1) Hierarchical branching papillae with variable amounts of stroma in the cores
        2) The epithelium is stratified, with tufting and cell detachment
        3) Admixture of cell types and the nuclei are heterogeneous
        4) There are variable numbers of eosinophilic cells containing moderate amounts of cytoplasm are often seen as budding/free-floating clusters and single cells
        5) Subtypes: Serous borderline tumor, micropapillary/cribriform serous borderline tumor
        6) Micropapillary/cribriform subtype has elongated micropapillae without stromal cores that directly emanate from the large papillae (AKA caput medusa) and/or cribriform spaces. The cells have high N:C and the nuclei are small and uniform, with small nucleoli
        
        Cytology:
        1) Papillary structures with relatively uniform small cells
        2) Mild to moderate pleomorphism
        3) Increased N:C
        4) Psammoma bodies frequently seen
        5) On the basis of morphological features alone, SBT cannot be distinguished from LGSC
    - - LOW-GRADE SEROUS CARCINOMA (LGSC)
        
        Definition: low-grade serous carcinoma is an invasive serous neoplasm with low-grade malignant features
        
        Epidemiology: median age 43 but wide range; a small subset have a Hx of SBT
        
        Pathogenesis:
        1) May exhibit KRAS, NRAS, BRAF, USP9X and EIF1AX*
        2) High-stage tumors are less frequently associated with BRAF mutations
        3) KRAS mutation is associated with tumor recurrence
        4) Loss of 9p and homozygous deletions of the CDKN2A/2B locus have also been reported
        
        Microscopic:
        1) Variety of patterns including small nests, glands, papillae, micropapillae or inverted macropapillae - frequently free-floating within unlined clear spaces
        2) Often, several patterns of invasion are present in an individual tumor
        3) The nuclei exhibit mild to moderate atypia, with < 3 -fold variation in nuclear size
        4) Occasionally have a central nucleolus
        5) Usually 1-2 mitoses/mm^2
        7) Psammoma bodies often present
        8) Necrosis is rare
        
        Immunohistochemistry:
        1) Diffusely positive: PAX-8, CK7, ER, WT1
        2) Patchy positive: p16
        3) Wildtype: p53
        
        Cytology:
        1) Cannot be distinguished from SBT on basis of morphology alone
      - HIGH-GRADE SEROUS CARCINOMA (HGSC)
        
        Definition: high-grade epithelial neoplasm demonstrating serous differentiation
        
        Epidemiology: wide age range but median is 65 years; HGSC is the most common ovariant carcinoma (70%); higher incidence in Caucasians; FHx of of breast/ovarian cancer and infertility are risk factors
        
        Pathogenesis:
        1) Tumors arise from tubal-type epithelium
        2) Nearly every tumor harbours a TP53 mutation
        3) About 15% cases due to BRCA1 or 2
        3) ~1% due to germline mutations in RAD51C/D and BRIP1
        
        Macroscopic:
        1) Usually bilateral, large and exophytic
        2) Fluid-filled cysts
        3) Solid areas are tan to white and frequently display extensive necrosis
        4) Commonly extensive extraovarian involvement
        
        Microscopic:
        1) Solid, papillary, labyrinthine (with slit-like spaces), glandular or cribriform architecture
        2) Nuclei are large and markedly atypical (size variability > 3-fold)
        3) High mitotic activty >5 per/mm^2, including atypical mitoses
        4) Necrosis and multinucleated cells are common
        5) Unusual morphologies: microcystic architecture with intraluminal and intracytoplasmic mucin, cytoplasmic clearing and (varey rarely) signet ring cells; follicular pattern; spindle cells
        6) Homologus recombination-deficient tumors frequently display variant architecture, including SET (solid, endometrial-like, transitional) patterns
        
        Immunohistochemistry:
        1) Diffuse positive: WT1
        2) Abnormal pattern: p53 can be overexpressed, not expressed or (rarely) diffuse cytoplasmic staining with weak nuclear staining
        3) Positive: CK7, CA125, PAX8, ER, p16
        4) Negative: CK20, napsin A, CEA, HNF-1beta
        5) Retained: PTEN and ARID1A
        
        Cytology:
        1) 3D clusters of pleomorphic cells with numerous variably oversized cytoplasmic vacuoles
        2) Marked nuclear atypia
        3) High N:C
        4) Cells can be in papillary structures, as non-descript cohesive groups or single cells
        5) Psammoma bodies commonly seen
  - - - MUCINOUS CYSTADENOMA AND ADENOFIBROMA
        
        Definition: benign tumors with gastrointestinal or Mullerian-type mucinous epithelium
        
        Epidemiology: wide age range (median age = 50); benign mucinous neoplasms account for about 80% of all primary ovarian mucinous tumors. Mucinous cystadenomas are much more common than adenofibromas
        
        Pathogenesis: association of some with a dermoid cyst suggests a germ cell origin. Some arise in association with Brenner tumors. KRAS mutations are found in a significant proportion of tumors
        
        Macroscopic:
        1) Unilateral
        2) Multilocular with a smooth outer surface
        3) Size range from few cms to >30 cm (mean = 10 cm)
        4) Adenofibromas are usually smaller, solid and punctuated with small cysts
        
        Microscopic:
        1) Composed of multiple cysts and glands lined by simple non-stratified mucinous epithelium resembling Mullerian, gastric foveolar-type, or intestinal epithelium containing goblet cells and sometimes neuroendocrine or Paneth cells
        2) Rare simple papillae may be seen
        3) Adenofibromas have small cysts embedded within a prominent fibromatous stroma
        4) Nuclei small and basally located without cytologic atypia
        5) Absent to minimal mitoses and apoptotic bodies
        6) A component of dermoid cyst or Brenner tumor may be found
        7) Stromal luteinization: stromal cells adjacent to the mucinous epithelium show abundant granular eosinophilic or pale cytoplasm and round nuclei
    - - MUCINOUS BORDERLINE TUMOR (MBT)
        
        Definition: architecturally complex non-invasive mucinous neoplasm with gastrointestinal-type differentiation
        
        Epidemiology: wide age range includes pediatric patients (mean age = 45)
        
        Pathogenesis: arise from mucinous cystadenomas and have similar associations with Brenner tumors and dermoid cysts. KRAS mutations in 30-75% of the tumors
        
        Macroscopic:
        1) Mean tumor size 20 cm (some as large as 50 cm)
        2) Nearly always unilateral
        3) Smooth external surface and multiloculated with smooth walls and mucinous contents
        4) Solid areas can be seen and should be sampled - may represent areas of invasion or mural nodules
        
        Microscopic:
        1) Similar appearance to benign counterpart
        2) Mild cytologic atypia
        3) Mitoses generally predominantly in crypts and less prominent on luminal surface
        3) Epithelial proliferation must account for >10% of the epithelial volume to qualify as an MBT
    - - MUCINOUS CARCINOMA
        
        Definition: invasive mucinous neoplasm composed of gastrointestinal-type cells
        
        Epidemiology: median age 55; 3-4% of all primary ovarian carcinomas
        
        Pathogenesis:
        1) Many develop from MBT
        2) Some may arise from a mature cystic teratoma or Brenner tumor
        3) KRAS and TP53 mutations in 64%
        
        Macroscopic:
        1) Large
        2) Unilateral
        3) Solid
        4) Cystic
        5) Intact and smooth outer surface and mucoid contents
        6) Some tumors may rupture and be associated with adhesions
        
        Microscopic:
        1) Often a continuum of architectural and cytological atypia that includes benign, borderline and carcinomatous areas
        2) Characterized by 2 different patterns of invasion:
        
        Expansile/confluent: displays marked glandular crowding with little/absent intervening stroma, creating a labyrinthine appearance
        
        Infiltrative/destructive: irregular glands, nests and single cells with malignant cytological features, often in a desmoplastic stroma
        3) An infiltrative pattern, particularly in the setting of bilateral ovarian involvement, should raise suspicion for metastatic mucinous carcinoma, and prompt evaluation for an extraovarian source
        4) There is currently no standardized grading system for primary mucinous carcinoma
        
        Immunohistochemistry:
        1) Diffusely positive: CK7, CA 19-9, SATB2 (in mucinous tumors associated with mature teratomas)
        2) Variably positive: CK20, CEA and CDX2, PAX 8
        3) Negative: CA125, WT1, napsin A, vimentin, ER and PR