Imaging of desmoplastic small round cell tumour in adults

Abstract

Desmoplastic small round cell tumour (DSRCT) belongs to the histological descriptive category of small round blue cell tumours. DSRCT primarily occurs in adolescents and young adults between the ages of 15 and 25 years and has a male predominance. DSRCT is an aggressive disease with a poor prognosis; timely diagnosis is therefore critical to the management of these patients. Although their radiographic appearances overlap with other aggressive malignancies, there are certain imaging features that can suggest the diagnosis and expedite the initiation of appropriate therapy. The aim of our pictorial review is to describe the imaging findings of primary and metastatic DSRCT in adults.

Extensive Abdominopelvic Desmoplastic Small Round Cell Tumor — Case Report and Review of the Literature

“…[3][4][5][6][7] Metastases appear especially in the liver, with or without ascites, in the lungs, lymph nodes (groin, neck, mediastinum, abdomen) and bones. 3,4 This rapidly growing tumor presents with abdominal discomfort, distention or pain, weight loss, hepatomegaly with ascites, bowel, bile or urinary tract obstruction due to compression, and other rare signs and symptoms according to its localization.…”

“…8 MRI delineates the extent of the disease, so it can be helpful in planning the surgical intervention. DSRCT shows an intense uptake of the fluorodeoxyglucose tracer (FDG) on PET/CT; this may have a role in staging and follow-up 5 . On gross examination, the tumor is large with a bosselated surface, with gray-tan cut-surfaces and areas of necrosis.…”

Rare peritoneal tumour presenting as uterine fibroid

“…Haemorrhage and necrosis can be identified using varying signal intensities inside the mass. 3 In patients with fibroid uterus who underwent uterine artery embolisastion, haemorrhagic necrosis can be found. But the loss of Junctional zone between endometrium and myometrium and heterogeneity point towards malignancy.…”

FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up

“…Diagnosis and staging of DSRCT has relied heavily upon anatomic imaging [8]. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful for identifying sites of disease, but they are limited in their ability to show the viability and metabolic activity of tumors.. 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) combined with CT imaging has been well defined as a useful imaging modality for identifying primary tumors of unknown origin and metastases and for monitoring tumor response to therapy as well as recurrence in several other pediatric malignancies, including sarcomas [9–11].…”

“…Computed tomography (CT) and magnetic resonance imaging (MRI) are useful for identifying sites of disease, but they are limited in their ability to show the viability and metabolic activity of tumors.. 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) combined with CT imaging has been well defined as a useful imaging modality for identifying primary tumors of unknown origin and metastases and for monitoring tumor response to therapy as well as recurrence in several other pediatric malignancies, including sarcomas [9–11]. Although FDG PET/CT has been used to assess DSRCT [8, 12–17], a definitive role for functional imaging in this disease has not yet been well established. In previously reported cases of DSRCT, FDG PET/CT has been shown to be a valuable tool for evaluation of disease.…”