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Desmoplastic Small Round Cell Tumor of the Abdomen: Radiologic-Histopathologic Correlation

¹ Mallinckrodt Institute of Radiology (P.J.P., A.J.F., D.M.B.)
² Lauren V. Ackerman Laboratory of Surgical Pathology (L.P.D., P.C.H.), Washington University School of Medicine, 510 S Kingshighway Blvd, St Louis, MO 63110.

View larger version (146K): [in a new window]   Figure 1a. Vague pelvic pain and a palpable mass in a 31-year-old woman. (a) Sagittal pelvic sonogram shows a well-defined, 12 x 7-cm, hypoechoic mass (cursors) posterior to the bladder (B). Multiple smaller peritoneal masses were present (not shown). (b) Contrast material–enhanced axial CT scan shows the paravesical mass (arrowheads), which was separable from the uterus and adnexa at both US and CT. B = bladder. (c) Photograph of the dominant mass after resection shows a lobulated, rubbery, yellow-tan tumor. Multiple smaller tumors were removed from the omentum (not shown). (d) Photomicrograph shows sharply demarcated, cohesive nests of uniform tumor cells (arrowheads) surrounded by thick bands of fibrous stroma. (Hematoxylin-eosin stain; original magnification, x230.)

View larger version (135K): [in a new window]   Figure 1b. Vague pelvic pain and a palpable mass in a 31-year-old woman. (a) Sagittal pelvic sonogram shows a well-defined, 12 x 7-cm, hypoechoic mass (cursors) posterior to the bladder (B). Multiple smaller peritoneal masses were present (not shown). (b) Contrast material–enhanced axial CT scan shows the paravesical mass (arrowheads), which was separable from the uterus and adnexa at both US and CT. B = bladder. (c) Photograph of the dominant mass after resection shows a lobulated, rubbery, yellow-tan tumor. Multiple smaller tumors were removed from the omentum (not shown). (d) Photomicrograph shows sharply demarcated, cohesive nests of uniform tumor cells (arrowheads) surrounded by thick bands of fibrous stroma. (Hematoxylin-eosin stain; original magnification, x230.)

View larger version (113K): [in a new window]   Figure 1c. Vague pelvic pain and a palpable mass in a 31-year-old woman. (a) Sagittal pelvic sonogram shows a well-defined, 12 x 7-cm, hypoechoic mass (cursors) posterior to the bladder (B). Multiple smaller peritoneal masses were present (not shown). (b) Contrast material–enhanced axial CT scan shows the paravesical mass (arrowheads), which was separable from the uterus and adnexa at both US and CT. B = bladder. (c) Photograph of the dominant mass after resection shows a lobulated, rubbery, yellow-tan tumor. Multiple smaller tumors were removed from the omentum (not shown). (d) Photomicrograph shows sharply demarcated, cohesive nests of uniform tumor cells (arrowheads) surrounded by thick bands of fibrous stroma. (Hematoxylin-eosin stain; original magnification, x230.)

View larger version (175K): [in a new window]   Figure 1d. Vague pelvic pain and a palpable mass in a 31-year-old woman. (a) Sagittal pelvic sonogram shows a well-defined, 12 x 7-cm, hypoechoic mass (cursors) posterior to the bladder (B). Multiple smaller peritoneal masses were present (not shown). (b) Contrast material–enhanced axial CT scan shows the paravesical mass (arrowheads), which was separable from the uterus and adnexa at both US and CT. B = bladder. (c) Photograph of the dominant mass after resection shows a lobulated, rubbery, yellow-tan tumor. Multiple smaller tumors were removed from the omentum (not shown). (d) Photomicrograph shows sharply demarcated, cohesive nests of uniform tumor cells (arrowheads) surrounded by thick bands of fibrous stroma. (Hematoxylin-eosin stain; original magnification, x230.)

View larger version (151K): [in a new window]   Figure 2a. Increasing abdominal girth and palpable masses in a 35-year-old man. (a, b) Contrast-enhanced CT scans show bulky, lobulated soft-tissue masses (arrowheads) throughout the peritoneum. Areas of low attenuation are present (straight arrow in b). Note the minimal perihepatic ascites (curved arrow in a). Desmoplastic small round cell tumor was diagnosed with US-guided biopsy.

View larger version (135K): [in a new window]   Figure 2b. Increasing abdominal girth and palpable masses in a 35-year-old man. (a, b) Contrast-enhanced CT scans show bulky, lobulated soft-tissue masses (arrowheads) throughout the peritoneum. Areas of low attenuation are present (straight arrow in b). Note the minimal perihepatic ascites (curved arrow in a). Desmoplastic small round cell tumor was diagnosed with US-guided biopsy.

View larger version (129K): [in a new window]   Figure 3a. Persistent, nonspecific abdominal symptoms in a 23-year-old woman. (a) Initial contrast-enhanced CT scan shows an omental soft-tissue mass (arrowheads) and ascites (arrows). No other masses were present. Desmoplastic small round cell tumor was diagnosed with CT-guided biopsy, and the patient underwent omentectomy and chemotherapy. (b) Contrast-enhanced CT scan obtained 8 months after a shows interval development of diffuse, nodular peritoneal thickening (arrowheads) and mesenteric soft-tissue infiltration (arrow), findings indicative of tumor progression. (c) Contrast-enhanced CT scan obtained 1 month after b shows scalloping of the hepatic capsule from serosal tumor spread (arrowheads) and hematogenous hepatic metastases (straight arrows). A right pleural effusion (curved arrow) is also present.

View larger version (138K): [in a new window]   Figure 3b. Persistent, nonspecific abdominal symptoms in a 23-year-old woman. (a) Initial contrast-enhanced CT scan shows an omental soft-tissue mass (arrowheads) and ascites (arrows). No other masses were present. Desmoplastic small round cell tumor was diagnosed with CT-guided biopsy, and the patient underwent omentectomy and chemotherapy. (b) Contrast-enhanced CT scan obtained 8 months after a shows interval development of diffuse, nodular peritoneal thickening (arrowheads) and mesenteric soft-tissue infiltration (arrow), findings indicative of tumor progression. (c) Contrast-enhanced CT scan obtained 1 month after b shows scalloping of the hepatic capsule from serosal tumor spread (arrowheads) and hematogenous hepatic metastases (straight arrows). A right pleural effusion (curved arrow) is also present.

View larger version (124K): [in a new window]   Figure 3c. Persistent, nonspecific abdominal symptoms in a 23-year-old woman. (a) Initial contrast-enhanced CT scan shows an omental soft-tissue mass (arrowheads) and ascites (arrows). No other masses were present. Desmoplastic small round cell tumor was diagnosed with CT-guided biopsy, and the patient underwent omentectomy and chemotherapy. (b) Contrast-enhanced CT scan obtained 8 months after a shows interval development of diffuse, nodular peritoneal thickening (arrowheads) and mesenteric soft-tissue infiltration (arrow), findings indicative of tumor progression. (c) Contrast-enhanced CT scan obtained 1 month after b shows scalloping of the hepatic capsule from serosal tumor spread (arrowheads) and hematogenous hepatic metastases (straight arrows). A right pleural effusion (curved arrow) is also present.

View larger version (126K): [in a new window]   Figure 4a. Progressive abdominal pain and distention in a 12-year-old girl. (a) Contrast-enhanced CT scan shows hepatomegaly and innumerable hepatic metastases (arrows). Also note the hypoattenuating portal lymphadenopathy (arrowheads). Desmoplastic small round cell tumor was diagnosed with US-guided hepatic biopsy. (b) More caudal section through the pelvis shows ascites (short arrow) and a heterogeneous retrovesical mass (arrowheads) with areas of low attenuation and foci of punctate calcification (long arrow). Two additional peritoneal masses were present at higher levels. Areas of calcification became densely confluent on CT scans obtained after chemotherapy (not shown).

View larger version (114K): [in a new window]   Figure 4b. Progressive abdominal pain and distention in a 12-year-old girl. (a) Contrast-enhanced CT scan shows hepatomegaly and innumerable hepatic metastases (arrows). Also note the hypoattenuating portal lymphadenopathy (arrowheads). Desmoplastic small round cell tumor was diagnosed with US-guided hepatic biopsy. (b) More caudal section through the pelvis shows ascites (short arrow) and a heterogeneous retrovesical mass (arrowheads) with areas of low attenuation and foci of punctate calcification (long arrow). Two additional peritoneal masses were present at higher levels. Areas of calcification became densely confluent on CT scans obtained after chemotherapy (not shown).

View larger version (122K): [in a new window]   Figure 5. Vague abdominal symptoms in a 36-year-old man. Contrast-enhanced CT scan shows a lobulated soft-tissue mass (arrowheads) within the retroperitoneum that causes partial obstruction of the left kidney with a delayed nephrogram. Desmoplastic small round cell tumor was diagnosed with CT-guided biopsy.