D N Church1, J Bailey, J Hughes, C J Williams. 1. Department of Medical Oncology, Bristol Haematology and Oncology Centre, Horfield Road, Bristol, BS2 8ED, UK. david.church@ubht.swest.nhs.uk
Abstract
BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare sarcoma primarily affecting young men. We report two cases in young women mimicking gynaecological malignancy. CASES: A 23-year-old woman underwent caesarean section for obstructed labour. At surgery, multiple tumour deposits were found throughout abdomen and pelvis. Histology and PCR confirmed DSRCT. Despite chemotherapy, the patient relapsed and died 27 months after diagnosis. A 29-year-old woman presented with abdominal distension and elevated Ca125. Imaging demonstrated widespread tumour within abdomen and pelvis. Histology confirmed DSRCT. Although attaining a complete response to chemotherapy, she relapsed within 2 months and died 11 months after diagnosis. CONCLUSION: DSRCT should be considered in the differential diagnosis of young women presenting with abdominal distension and multiple masses on imaging.
BACKGROUND:Desmoplastic small round cell tumour (DSRCT) is a rare sarcoma primarily affecting young men. We report two cases in young women mimicking gynaecological malignancy. CASES: A 23-year-old woman underwent caesarean section for obstructed labour. At surgery, multiple tumour deposits were found throughout abdomen and pelvis. Histology and PCR confirmed DSRCT. Despite chemotherapy, the patient relapsed and died 27 months after diagnosis. A 29-year-old woman presented with abdominal distension and elevated Ca125. Imaging demonstrated widespread tumour within abdomen and pelvis. Histology confirmed DSRCT. Although attaining a complete response to chemotherapy, she relapsed within 2 months and died 11 months after diagnosis. CONCLUSION: DSRCT should be considered in the differential diagnosis of young women presenting with abdominal distension and multiple masses on imaging.