Primary extranodal CD8 positive epitheliotropic T-cell lymphoma arising in a leiomyoma of the uterus

Abstract

A 38 year old woman suffered from dysmenorrhoea and abnormal vaginal bleeding. Hysteroscopic examination showed a well-circumscribed submucosal tumour of the uterine cavity, which was thought to be a submucosal leiomyoma. It was enucleated and fractional curettage from the cervix and corpus was performed.Macroscopically, the tumour measured 5 Â 3 Â 2.5 cm and showed a soft, white-yellowish cut surface, resembling that of a uterine leiomyoma. On histology, the mucosal glands were loosely infiltrated by small-to medium-sized lymphocytes with irregular nuclear contours. The submucosa and remnants of leiomyoma-like masses were heavily infiltrated by a dense heterogeneous population of cells (Fig. 1). The predominantly lymphoid infiltrate was intermingled with a variable number of plasma cells, histiocytes and epithelioid cells.The differential diagnosis included a leiomyoma containing a heavy lymphocytic infiltrate 1 , a pseudolymphoma or lymphoma-like lesion 2 -4 , an inflammatory pseudotumour 5 and a true malignant lymphoma of the uterus.The lymphatic cells consisted of morphologically atypical small-to medium-sized cells mixed with a variable number of blasts. The nuclei were irregular in size and shape, and some showed indentations (Fig. 2). The nucleoli were usually small; however, the blasts showed single or multiple medium-sized nucleoli. The tumour cells showed moderate epitheliotropism, and some lymphoepithelial lesions were found (Fig. 2). The neoplastic lymphocytes infiltrated blood vessels, resulting in moderate angiocentricity (Fig. 3). In addition, small remnants of B-cell follicles, suggestive of an inflammatory pseudotumour, were found. Immunohistochemically, the majority of the atypical lymphatic cells expressed the T-cell antigens CD3, CD8 (Fig. 3) and hF1. NK cell markers (CD16 and CD56) were negative; however, the cytotoxic molecule TIA1 and granyzyme were found. Molecular analysis revealed a monoclonal rearrangement of the T-cell receptor -g chain locus (Fig. 4), confirming the diagnosis of a monoclonal T-cell process. Molecular DNA analysis for Chlamydia trachomatis was negative.The woman underwent computed tomography of her neck, thorax and abdomen, cystoscopy, proctoscopy, bone marrow aspiration and full haematological investigations. No abnormality was found. The stage of her disease was IE, according to the Ann Arbor classification. Hysterectomy and pelvic lymphadenectomy was carried out. There was no evidence of disseminated disease. A small scar was observed at the base of the enucleated tumour at the cervico-endometrial transitional zone. The woman's postoperative recovery was uneventful. After 40 months follow up, there has been no evidence of recurrence.

Primary lymphomas of the cervix and uterus: The University of Pennsylvania's experience and a review of the literature

“…Other B-cell lineages have been reported including pre-cursor B-Cell lymphoblastic lymphoma, MZL, and intra-vascular DLBCL [11,15 -18]. Non-B cell primary lymphomas of the cervix and uterus including Natural Killer and T-Cell lymphomas have also been reported [9,19,20]. A case of post-transplant lymphoproliferative disorder isolated to the cervix has been reported as well [21].…”

Sonographic appearance of uterine lymphoma: Case report and review of the literature

“…The most common subtype of malignant lymphoma in the uterus is diffuse large B‐cell lymphoma, followed by follicular lymphoma . T‐cell lymphoma arising in the uterus had been described in very few cases . T‐cell lymphoma is considered to be very aggressive, which might explain the rapid changes in uterine size in our patient.…”

“…1,3 T-cell lymphoma arising in the uterus had been described in very few cases. [21][22][23] T-cell lymphoma is considered to be very aggressive, 24 which might explain the rapid changes in uterine size in our patient. This is one of a few case reports describing the sonographic features of uterine lymphoma.…”