Desmoplastic small round-cell tumour of the peritoneal cavity: case report and literature review

• Published in: Annals of the Royal College of Surgeons of England Vol. 102; no. 4; pp. e77 - e81
• Main Authors: Tsoukalas, N, Kiakou, M, Nakos, G, Tolia, M, Galanopoulos, M, Tsapakidis, K, Kamposioras, K, Christofyllakis, C, Dimitrakopoulos, G, Sambaziotis, D
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01-04-2020; Royal College of Surgeons
• Subjects: Abdomen; Adult; Antigens; Ascites; Chemoradiotherapy, Adjuvant; Chemotherapy; Constipation; Cytokeratin; Cytoreduction Surgical Procedures; Desmoplastic Small Round Cell Tumor - diagnosis; Desmoplastic Small Round Cell Tumor - pathology; Desmoplastic Small Round Cell Tumor - therapy; Fatal Outcome; Growth factors; Humans; Male; Medical prognosis; Mesothelioma; Metastasis; Online Case Report; Patients; Peritoneal Cavity - diagnostic imaging; Peritoneal Cavity - pathology; Peritoneal Cavity - surgery; Peritoneal Neoplasms - diagnosis; Peritoneal Neoplasms - therapy; Sarcoma; Tomography, X-Ray Computed; Tuberculosis; Tumors; Prognosis; Young adults; Adolescents; Rare tumour; Abdominal peritoneum; Desmoplastic small round-cell tumour
• ISSN: 0035-8843; 1478-7083
• DOI: 10.1308/rcsann.2019.0180

Desmoplastic small round-cell tumour is a very rare neoplasm, which usually arises from the abdominal or pelvic peritoneum of adolescents and young adults. Early diagnosis is difficult, because most tumours present with non-specific gastrointestinal symptoms after a long asymptomatic period. It is generally a very aggressive tumour, which grows rapidly with poor prognosis and an overall five-year survival rate of 15% despite multimodal treatment. Despite multiple treatment strategies, the management of desmoplastic small round-cell tumour still remains a clinical challenge and no consensus about a therapeutic protocol has been established. A 35-year-old man presented with mild abdominal pain, constipation and weight gain, and was eventually diagnosed with desmoplastic small round-cell tumour, which was shown to be limited to the abdomen. After incomplete debulking surgery, radiotherapy and chemotherapy, he developed multiple metastatic nodular foci in chest and the pleura and, unfortunately, he died due to disease progression.