Mu’ath M.A. Rjoub Supervised by: Dr. Huda Zahawi, FRCPath. King Abdullah University Hospital )KAUH(

Clinical History A 56 year old single female, presented complaining of postmenopausal bleeding. She underwent Total Abdominal Hysterectomy. Gross Description : We recieved a previously opened and distorted TAH specimen from a peripheral hospital, the specimen measuring 16.5x10x6.5 cm and weighing 215 gm, the uterine cavity was totally replaced by papillary like necrotic and friable mass measuring 13.5x12x6 cm. The cervix measuring 4.5 x 1 cm.The tumor is invading the myometrium and less than 1 cm away from serosa and totally replacing the endometrial and endocervical cavity.

Microscopy

H&E

Differntial Diagnosis 1. Undifferentiated carcinoma. 2. High grade serous carcinoma. 3. Cervical lymphoepithelioma like carcinoma. 4. Endometrial lymphoepithelioma like carcinoma. 5. Lymphoma. 6. Sarcoma.

Cam 5.2

EMA

Differential Diagnosis 1. Undifferentiated carcinoma. 2. High grade serous carcinoma. 3. Cervical lymphoepithelioma like carcinoma. 4. Endometrial lymphoepithelioma like carcinoma. 5. Lymphoma. 6. Sarcoma.

P53

Differential Diagnosis 1. Undifferentiated carcinoma. 2. High grade serous carcinoma. 3. Cervical lymphoepithelioma like carcinoma. 4. Endometrial lymphoepithelioma like carcinoma. 5. Lymphoma. 6. Sarcoma.

LCA

SynaptophysinChromogranin A

Immunohistochemistry Tumor cells were also negative for WT-1, ER, CD10, Actin and Desmin. This leaves us with the diagnosis of lymphoepithelioma like carcinoma.

EBV

Diagnosis Endometrial lymphoepithelioma like carcinoma.

Discussion Lymphoepithelial carcinoma is a distinct neoplasm of the nasopharynx with consistent etiologic association with Epstein–Barr virus (EBV). Carcinomas with morphologic features of Lymphoepithelial carcinoma, which occur outside of the nasopharynx, are called lymphoepithelioma like carcinoma (LELC).

Discussion cont. Regarding the lower female genital tract, LELC has been described in the vulva, vagina and cervix. To the best of our knowledge, only four cases have been previously reported in the uterine corpus. In one of the reported cases, there was uterine and cervical involvement, but the main tumoral mass was located in the endometrial cavity inducing the authors to conclude an endometrial origin.

Discussion cont. Microscopically, a diffuse and lymphoid rich stroma, the absence of glands and papillary architecture, together with cytologic features excluded the diagnosis of endometrioid and serous type carcinoma. Among the other rare types of nonendometrioid carcinoma, the tumor most similar to this diagnosis was undifferentiated, large cell-type carcinoma, but the presence of an intense inflammatory component was consistent with endometrial LELC.

Discussion cont. The tumor cell immunoprofile in our case was similar to those of the reported cases that were cytokeratin and epithelial membrane antigen positive. Leucocyte common antigen, estrogen and progesterone receptors, neuron specific enolase, chromogranin, synaptophysin, and p53 were negative. They did not find evidence of Epstein–Barr virus (EBV) infection using immunohistochemistry nor polymerase chain reaction (PCR).

Discussion cont. Further studies on large series of cases are warranted to determine the relationship of this unusual type of endometrial carcinoma with endometrioid and nonendometrioid carcinoma, type I and type II, respectively. In one of the reported cases, the uninvolved endometrium was atrophic rather than hyperplastic, and the immunostaining for p53 was negative.

Prognosis Unfortunately, our patient was transferred to another hospital for treatment and we lost her follow up. The limited number of endometrial LELC does not allow to determine their prognosis. In one of the patients, a follow-up of 24 months did not reveal relapse and/or distant metastasis, but one of the cases described by Vargas et al had an aggressive course.

Thank You

References Endometrial lymphoepithelioma like carcinoma: absence of Epstein–Barr virus genomes.S. RAHIMI*, A. LENAy & G. VITTORIy,Departments of *Pathology and Gynaecology, Ospedale San Carlo-IDI IRCCS, Rome, Italy. Vargas MP, Merino MJ. Lymphoepithelioma-like carcinoma: an unusual variant of endometrial cancer, a report of two cases. Int J Gynecol Pathol 1998;17:272–6. Axelsen SM, Stamp IM. Lymphoepithelioma-like carcinoma of the vulvar region. Histopathology 1995;27:281–3. Dietl J, Horny HP, Kaiserling E. Lymphoepithelioma-like carcinoma of the vagina: a case report with special reference to the immunophenotype of the tumor cells and tumor infiltrating lymphoreticular cells. Int J Gynecol Pathol 1994;13:186–9. Mills SE, Austin MB, Randal ME. Lymphoepithelioma-like carcinoma of the uterine cervix. A distinctive undifferentiated carcinoma with inflammatory stroma. Am J Surg Pathol 1985;9:883–9.