Objective:

To investigate the clinical

imaging and pathological features of desmoplastic small round cell tumor (DSRCT).

Methods:

The clinical data

imaging and pathological features of 30 DSRCT patients confirmed by pathology in Fudan University Cancer Hospital were retrospectively analyzed

and their manifestations were characterized.

Results:

Among the 30 cases

22 were male and 8 were female

Age 17-49 years

and the median age was 32 years. The first clinical symptom was abdominal mass in 11 cases (36.7%). Of the 30 patients

20 were first-time patients

9 cases were postoperative recurrence

and 1 case was not followed up after mass resection. Of the 29 cases

because one patient took positron emission tomography (PET) as the firstdiagnosis. Therefore

28 patients with computed tomography (CT) or magnetic resonance imaging (MRI) imaging data were selected for evaluation. CT and MRI showed that the mass was located in the greater omentum in 22 cases (78.6%)

the lesser omentum in 12 cases (42.9%)

and the mesenteric space in 13 cases (46.4%). Among the involved organ envelopes

17 cases (60.7%) were subhepatic

6 cases (21.4%) were subsplenic

and 6 cases (21.4%) were other organ envelopes. 25 of 28 patients underwent CT enhancement

liquefaction necrosis in 13 cases (52.0%)

calcification in 6 patients (24.0%). The CT value of the liquefied and necrotic area of the mass was -4.9-40.5 HU (average 17.8 HU)

and the CT values of the solid component before and after enhancement were 17.8-41.6 HU (average 38.6 HU) and 41.6-105.4 HU (average 73.5 HU). Six patients underwent MRI examination

and the abdominal and pelvic cavity showed no obvious organ origin

multiple nodules and mass shadows

mostly the mesentery. T1- weighted imaging (T1WI) showed equal low signal; T2-weighted imaging (T2WI) showed mixed high signal; liquefied necrosis showed high signal; diffusion-weighted imaging (DWI) showed high signal; dynamic enhancement showed mil

d to moderate heterogeneous enhancement. Diffuse strong positive expression of broad-spectrum cytokeratin AE1/AE3 by immunohistochemistry (76.7%)

Vimentin VIM (56.7%) and Desmin (76.7%). Immunohistochemical diffuse strong positive expression AE1/AE39(76.7%)

vimentin (56.7%) and junction protein (76.7%).

Conclusion:

DSRCT usually occurs in young men. Most of the images show multiple diffuse masses in the mesangial or organ space of the abdominal omentum

bladder and rectum space mass

no definite organ origin

cystic necrosis

calcification

enhanced scanning light and moderate uneven delayed enhancement. The combination of clinical

imaging and pathology could help to improve the understanding of DSRCT.