Intra-abdominal desmoplastic small round cell tumour in a 56-year-old female: Case report of a very rare presentation of an unusual tumour

Abstract

Introduction: This case report discusses the rare diagnosis of intra-abdominal desmoplastic small round cell tumour (DSRCT) in a 56-year-old female.

Presentation of case: An incidental intra-abdominal lesion was found during investigation of joint pain. Ultrasound-guided biopsy suggested desmoid tumour, after undergoing laparotomy and en-bloc excision of the tumour due to concerning radiological progression, the final histology was desmoplastic small round cell tumour. At six-week follow-up imaging, no recurrence or metastatic disease was noted. She declined chemotherapy and specialist follow-up, electing to have routine follow up with her General Practitioner only.

Discussion: Intra-abdominal DSRCT is rare and mainly seen in young males. To our knowledge, this is the only reported case of DSRCT in a female over the age of 50.

Conclusion: There should be timely discussion between different surgical units to provide efficient care. Any disparity between radiological and histological appearance should prompt further review and investigation in order to ensure misdiagnosis is avoided and appropriate treatment is provided. Despite cytoreductive surgery, survival is dismal due to the aggressive nature of the tumour, and its low numbers limiting adequate study into post diagnosis care.

Keywords: Case report; Desmoplastic small round cell tumour; Intra-abdominal tumour; Surgical oncology.