Small intestine perforation due to metastatic uterine cervix interdigitating dendritic cell sarcoma: a rare manifestation of a rare disease

Abstract

Interdigitating Dendritic Cell Sarcoma (IDCS) is an infrequent dendritic cell tumor which mainly affects the lymphatic system. Intestinal metastasis from uterine IDCS is extremely rare. Here we report a case of a 76-year-old female presenting with vaginal bleeding and acute abdomen. The final diagnosis revealed a small bowel perforation due to metastatic involvement from uterine cervix IDCS. In this paper, we report the clinical manifestation, computed tomography and histopathological findings helpful for the accurate diagnosis of this rare tumor.

Keywords: dendritic cell neoplasm; interdigitatin dendritic cell sarcoma; small intestine; uterine cervix..

Figure 1

Axial contrast-enhanced computed tomography (CT) images through upper abdomen A, B) and coronal multiplanar reconstruction CT image C) show a round soft tissue mass (arrow) in close proximity to small bowel loop (jejunum) consistent with metastatic involvement from interdigitating dendritic cell sarcoma (IDCS) of the uterine cervix

Figure 2

A) axial, B) coronal and C) sagittal contrast-enhanced computed-tomography images through lower abdomen and pelvis demonstrate a large solid mass at the uterine cervix and vagina (M). There is no evidence of ascites, intraperitoneal dissemination or lymphadenopathy. R denotes rectum; UB denotes urinary bladder; U denotes uterus.

Figure 3

A) Macroscopic picture shows the gross appearance of the small intestinal tumor. The mass is composed of homogeneous white tissue that infiltrates the bowel wall and forms two nodular masses within the mesentery; B) Histological photomicrograph of the tumor showing spindle and epithelioid cells with variable degrees of atypia and polymorphism (Hematoxylin and eosin stain; x600); C) Photomicrograph shows tumor cells with strong and diffuse cytoplasmic and nuclear reactivity for S-100 protein immunostain; D) Photomicrograph shows tumor cells with cytoplasmic reactivity for CD4 immunostain.