Seromucinous Tumor of the Ovary MingChieh Lin Ovarian

Seromucinous Tumor of the Ovary Ming-Chieh Lin 林明杰

Ovarian Mucinous Tumors l Definition ¡Taditional: l. Ovarian tumors some or all of whose epithelial cells contain intracytoplasmic mucin • Endocervix (endocervical-like, Mullerian type) • Gastrointestinal ¡ 2014 WHO l. Mucinous tumor • Gastrointestinal epithelium l. Seromucinous tumor • Endocervical-type mixed with serous or other Mullerian cell types

Ovarian Seromucinous Tumors l Classification: ICD-O codes ¡Benign l Seromucinous cyatadenoma l Seromucinous adenofibroma 8474/0 9014/0 ¡Borderline l Seromucinous borderline tumor 8474/1 ¡Malignant l Seromucinous carcinoma 8474/3

Benign Seromucinous Tumors l Definition: ¡A benign ovarian tumor with endocervicaltype mucinous and tubal-type (serous) cells or more Mullerian cell types, all accounting for at least 10% of the epithelium. l Synonym ¡Seromucinous cystadenoma (adenofibroma) ¡Mullerian cystadenoma of mixed cell types

Benign Seromucinous Tumors l Epidemiology ¡ 1% of benign epithelial neoplasms ¡Peak age: late reproductive age l Clinical features ¡Non-specific ¡Usually diagnosed incidentally

Benign Seromucinous Tumors l Macroscopy ¡Unilocular cyst ¡Serous or mucoid fluid l Microscopy ¡Variable admixtures of serous and mucinous (endocervical-type) ¡Endometrioid, transitional, squamous

Benign Seromucinous Tumors l Histogenesis ¡Likely from endometriosis l Prognosis and predictive factors ¡Benign

Seromucinous Borderline Tumors l Definition ¡A non-invasive, proliferative epithelial tumor composed of more than one epithelial cell type, most often serous and endocervical-type mucinous; however, endometrioid, and less often, clear cell, transitional or squamous may be seen l Synonyms ¡Endocervical-type mucinous borderline tumor ¡Mullerian mucinous borderline tumor ¡Atypical proliferative Mullerian tumor

Seromucinous Borderline Tumors l Epidemiology ¡ 15% of all mucinous borderline tumor l In the past, these tumors have been considered a subset of mucinous tumor (endocervical type) l Clinical features ¡Average age: 34 -44 y/o ¡Non-specific S/S ¡Most are FIGO stage I ¡Rare advanced stage l Implants and/or lymph node involvement

Seromucinous Borderline Tumors l Macroscopy ¡Mean size: 8 -10 cm ¡Typically unilocular, viscid fluid ¡Papillary projections ¡Often associated with endometriosis ¡Bilateral: 40%

Seromucinous Borderline Tumors l Microscopy ¡Architectural features similar to serous borderline tumors l. Complex papillary architecture • branching in a hierarchical manner into progressively smaller papillae • Terminating in small detached epithelial tufts l. Larger papillae • Edematous stroma with neutrophils

Seromucinous Borderline Tumors l Microscopoy ¡Epithelial lining l Stratified l Low grade nuclear atypia l Most are endocervical-type mucinous or serous cells l Endometrioid, squamous, transitional, clear cells l Goblet cells (-) ¡ Often associated with endometriosis l Immunohistochemistry ¡ CK 7(+)/CK 20(-)/CDX-2(-) ¡ ER(+)/PR(+)

Serous borderline tumor-100 X

Resembling a serous borderline tumor with bulbous papillae The papillae are lined by stratified endocervical-like mucinous cells The papillae are line by stratified eosinophilic cells, with numerous PMN in the papillae and mucin

Seromucinous Borderline Tumors l Histogenesis ¡Data is limited ¡Associated with endometriosis, at least 1/3 cases l. Mucinous metaplasia ¡ER (+) l. Similar to endometrioid tumor ¡ARID 1 A mutation (loss of expression): 1/3 l. Similar to endometrioid and clear cell carcinoma

Seromucinous Borderline Tumors l Prognosis and predictive factors ¡Good outcome even with peritoneal implants

Endometriosis-associated Tumor l Endometrioid tumor l Clear cell tumor l Seromucinous tumor

Seromucinous Carcinoma l Definition ¡A carcinoma composed predominantly of serous and endocervical-type mucinous epithelium. ¡Foci of clear , endometrioid and squamous cells l Synonyms ¡Endocervical-type mucinous carcinoma ¡Mixed epithelial carcinoma of Mullerian type

Seromucinous Carcinoma l Epidemiology ¡Quite uncommon ¡Data not available l Clinical features ¡Mean age: 45 y/o

Seromucinous Carcinoma l Macroscopy ¡Mean tumor size: 12 cm ¡Bilateral: >50% ¡Unilocular or multilocular with papillary projections and solid areas

Seromucinous Carcinoma l Microscopy ¡Lack of consensus on diagnostic criteria l Immunohistochemically ¡Similar to seromucinous borderline tumor

Seromucinous Carcinoma l Prognosis and predictive factors ¡Data limited ¡In one series of 54 cases (Am J Surg Pathol. 2002, 26(12): 1529 -1541) l. Stage I: outcome is favorable l. Advanced stage: 50% die of disease

聯醫 1589521 l 40 -year-old female l. Right ovarian cyst l. Laparoscopic oophorectomy l Unilocular cyst, 7 x 5 cm l Multiple small papillary projections

FS-100 x-Cyst with papillary projections

PMN FS-200 X Endocervical epithelium Epithelial proliferation

FS-200 X-epithelial proliferation

聯醫 1589521 -Frozen Section l Right ovary, frozen section: ¡Mucinous borderline tumor, endocervical-like type (Seromucinous borderline tumor)

40 X-Cyst with papillary projections

40 X-Cyst with papillary projections

200 X-endocervical epithelium

400 X-ciliated cells

200 X-epithelial proliferation

200 X-epithelial proliferation

400 X-Inflammatory cells

200 X-Endometriosis CD 10

400 X-Atypical endometriosis

CK 7 -100 X CK 7 -200 X

CK 20 -100 X

ER-200 X

聯醫 1589521 l Ovary, right, laparoscopic oophorectomy ¡ Mucinous borderline tumor, endocervical type (Seromucinous borderline tumor), associated with endometriosis