case report Title: uterine mass Master: Dr.Mahzooni Resident: Dr.Soleimani 92/7/6

Patient charactristics A 56years old female presented with history of abnormal vaginal bleeding, wiehgt loss, abdominal distention Paraclinic data: Pap smear : normal Sonography : myomatous uterous with normal size uterine,thickness of endometrium was 4mm CT scan : multiple peritoneal seeding and para- aortic LAP and massive ascitis

Patient charactristics she underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy On gross examination of the specimen, cut section of the uterus showed a ulcerative and necrotic grey brown to yellow mass that occupy endometrial cavity

Microscopic examination

DDX of AUB Atrophic endometrium Ovarian mass (Granolosa cell tumor,techoma) Endometrial tumor Metastasis(RCC,ovarian clear cell carcinoma,..)

Undifferentiated carcinoma probably the end of the line for endometrial carcinomas of endornetrioid, serous, and other microscopic types It grows in the form of solid sheets of epithelial cells without any signs of differentiation Some of the reported cases of undifferentiated carcinoma have occurred in young women and have pursued a fulminant clinical course

IHC CA 125 : negative CK7 : negative CK20 : negative CEA: negative CD10 : positive WT1 : positive Vimentin : positive

Undifferentiated carcinoma Compared to typical endometrioid adenocarcinomas, the undifferentiated components tend to show relative loss of cytokeratin, ER, and PR expression EMA expression, however, appears to be retained in most examples

Endometrial carcinoma ( clear cell type) usually express pan-cytokeratins, EMA, CA125, Ber- EP4, B72.3, CK7, and vimentin Usually negative for CK20 and WT1, strong cytoplasmic expression of CEA.

Endometrial carcinoma (Papillary serous carcinoma ) commonly express pan-cytokeratins, EMA, CA125, Ber- EP4, B72.3, CK7,and vimentin usually negative for CK20 strong cytoplasmic expression of CEA WT-1 is uncommonly positive

Metastasis Renal clear cell: PAX2+, RCC+, CAM5.2+, vimentin+,CD10+, CK7 (rare to negative), inhibin–, A103–, HepPar-1– Ovarian clear cell: CK7+, ER+ (patchy), PAX2+ (~1/3),WT1–/+, RCC (rare), CD10–, inhibin–, A103–, HepPar-1–

Dx: Endometrial stromal sarcoma (ESS) Tumors composed of endometrial stroma tend to occur in middleaged women (average age, 45 years) and often present with vaginal Bleeding. Traditionally designated endolymphatic stromal myosis, infiltrate the myometrium and have a particular tendency to permeate lymph vessels

Endometrial stromal sarcoma ● CD10 positive ● Vimentin positive ● Reticulin positive surrounding individual cells ● Cytokeratin negative, except in sex cord elements ● SMA, desmin mostly negative except for stromal myoma ● Epithelial membrane antigen (EMA) negative

Endometrial stromal sarcoma Wilms tumor gene (WT1) is a tumor- suppressor gene located at chromosome 11p13 that is involved in Wilms tumor development, and the WT1 gene product has been found to be expressed during the development of the urogenital system These antibodies may stain both the nuclei and the cytoplasm of cells, but from the standpoint of using this antibody in diagnostic pathology, it is important to evaluate only the presence or absence of nuclear immunoreactivity (similar to estrogen and progesterone receptor antibodies, where any cytoplasmic reactivity should be ignored).

Endometrial stromal sarcoma At ProPath, we have also observed nuclear reactivity with WT1 in a number of other female genital tract tumors and tissues, including normal endometrial stroma and endometrial stromal sarcomas, normal uterine smooth muscle cells and uterine leiomyosarcomas, granulosa cell tumors, thecomas, and mixed mullerian tumors (in both the epithelial and stromal elements)

تشخيص Endometrial stromal sarcoma (ESS)