Intra-Abdominal Desmoplastic Small Round Cell Tumor: A Diagnostic Dilemma In Resource-Constrained Settings – A Case Report & Literature Review

Abstract

Introduction. Desmoplastic small round-cell tumours (DSRCT) are primarily intra-abdominal soft tissue sarcomas that are extremely rare and aggressive with a rapidly deteriorating clinical course and poor outcome despite therapy. It belongs to the family of small round blue-cell tumours, symptoms of the disease are non-specific and metastasis is often present at diagnosis. Objective. This report presents a typical case of an intra-abdominal DSRCT while highlighting how challenging the diagnosis can be in resource-poor settings and best-practice in the management of its attendant complications. Case presentation. This is a case 10-year-old boy who presented with a two-month history of weight loss and a two-week history of colicky abdominal pain, abdominal distension and vomiting and loss of appetite. He was pale, had left mandibular lymphadenopathy with a distended abdomen and ascites. A palpable mass was occluding ~80% of his rectal lumen. CT scan showed intra-abdominal masses in the rectovesical pouch and hepatic flexure with abdominopelvic lymphadenopathy Patient was worked up for exploratory laparotomy, colostomy placement and tumor biopsy, with the subsequent histology and immunohistochemistry a diagnosis of DSRCT was made. The patient developed moderate anaemia, acute kidney injury, hypertension and anarsarca while on admission; these were successfully managed with blood transfusions, intravenous fluids and drugs subsequently. He had a re-exploration for fecal disimpaction and adhesiolysis due to persistent intestinal obstruction. Patient was discharged 15 days after admissionin a stable clinical state. Conclusion. Desmoplastic round-cell tumour (DSRCT) primarily occurr within the abdomen, it is capable of mimicking the symptomatology of other intra-abdominal malignancies and a high index of suspicion is requisite to make a diagnosis especially in resource-poor settings with swift implementation of treatment modalities necessary to halt disease progress.