RESUMO
Objective:To investigate the magnetic resonance imaging (MRI) features of desmoplastic small round cell tumor (DSRCT) of the abdomen and pelvis.Method:The retrospec-tive and descriptive study was conducted. The clinicopathological data of 8 patients with DSRCT of the abdomen and pelvis, including 3 cases admitted in Yueqing People's Hospital and 5 cases admitted in Wenzhou People's Hospital, from January 2008 to June 2022 were collected. There were 5 males and 3 females, aged (43±5)years. All patients underwent MRI plain and enhanced scanning. Observa-tion indicators: (1) imaging features of DSRCT of the abdomen and pelvis; (2) treatment and pathological examination characteristics of DSRCT of the abdomen and pelvis; (3) follow-up. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distri-bution were represented as M(range). Count data were described as absolute numbers. Results:(1) Imaging features of DSRCT of the abdomen and pelvis. ① Tumor location. Of the 8 patients, there were 6 cases with tumors located respectively at the lower edge of the liver in the right quarter costal region, the medial side of the ileocecal region in the right iliac region, the medial side of the caecum in the right iliac region, the gastro-pancreatic space in the left quarter costal region, the mesenteric space in the left iliac region and the right side of pelvic bladder, and 2 cases with tumors located at retroperitoneal space of left quarter rib region. ② Tumor size. There were 13 lesions in the 8 patients, and the maximum diameter of tumor was 9.1 (range, 3.5?20.0)cm. Of the 8 patients, there were 5 cases with single tumor and 3 cases with multiple tumors. ③ Tumor shape and boundary. Of the 8 patients, there were 4 cases with tumor in expansive growth and 4 cases with tumor in invasive growth. There were 5 cases with tumor of intratumoral necrosis and cystic degene-ration, 4 cases with tumor of intratumoral hemorrhage, 4 cases with tumor of intratumoral spot calcification, 3 cases with tumor of peritumoral tissue exudation. One patient may combined with multiple imaging manifestations. ④ Imaging characteristics of MRI plain scanning. Of the 8 patients, there were 4 cases with tumor of homogeneous hypointensity signal and 4 cases with tumor of hypointensity mixed with speckled hyperintensity (with hemorrhage) in T1 weighted imaging of MRI plain scanning. There were 3 cases with tumor of homogeneous hyperintensity and 5 cases with tumor of high signal at the edge, patchy and spot-shaped in the center in T2 weighted imaging of MRI plain scanning. There were 5 cases with tumor of high, equal and low confounding signals and 3 cases with tumor of high and low signals in T2 weighted imaging fat suppression sequence of MRI plain scanning. There were 3 cases with tumor of uniform high signals and 5 cases with tumor of high, equal and low mixed signals in diffusion weighted imaging of MRI plain scanning. ⑤ Imaging characteristics of MRI enhanced scanning. All 8 patients had tumor of heterogeneous enhancement in MRI enhanced scanning, including 2 cases with significant enhancement in arterial phase, continuous enhancement in portal phase, slightly reduced enhancement in delayed phase, 4 cases with moderate enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase, 2 cases with mild enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase. Of the 8 patients, there were 3 cases with tumor of annular enhancement with intratumoral strip or grid signals and 3 cases with tumor of peritumoral blood vessels increased and thickened signals. ⑥ Tumor invasion and metastasis. Of the 8 patients, there were 4 cases with tumor invaded bowel, 2 cases with tumor invaded surrounding tissues, 1 case with tumor invaded left kidney, spleen and pancreatic tail, 1 case with tumor invaded distal of left ureter. There were 5 cases with abdominal, retroperitoneal and inguinal lymph nodes enlargement, 4 cases with multiple nodular thickening of peritoneum and ascites, 2 cases with tumor liver and lung metastasis and 1 case with tumor rib, femur and sacrum metastasis. One patient may combined with multiple tumor metastasis. (2) Treatment and patholo-gical examination characteristics of DSRCT of the abdomen and pelvis. Of the 8 patients, 3 patients underwent complete resection as clear tumor boundary, 3 patients underwent tumor partial resection as tight adhesion between tumor and surrounding blood vessels, 2 cases underwent tumor tissue pathological examination as extensive metastasis of peritoneum, omentum, mesentery and surrounding intestine. All 8 patients were diagnosed as DSRCT by microscopic examination, electron microscopic examination, immunohistochemical staining and cytogenetic examination. (3) Follow-up. All 8 patients underwent postoperative follow-up and died during the follow-up.Conclusion:MRI features of abdominal and pelvic DSRCT include single or multiple lobulated masses with unclear boundaries, invading the omentum, mesentery, peritoneum and adjacent tissues in most cases, mixed signals and heterogeneous mild to moderate enhancement in enhanced scanning.
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Objective:To investigate the computed tomography (CT) features of primary liver leiomyosarcoma (PHLMS).Methods:The retrospective and descriptive study was conducted. The clinical and imaging data of 10 patients with PHLMS who were admitted to 4 medical centers including 3 cases in Wenzhou Central Hospital, 3 cases in the Second Affiliated Hospital of Wenzhou Medical University, 2 cases in Wenzhou People's Hospital and 2 cases in Yueqing People's Hospital from January 2011 to December 2020 were collected. There were 5 males and 5 females, aged from 41 to 83 years, with a median age of 55 years. All 10 patients underwent abdominal CT examination. Observation indicators: (1) CT features; (2) treatment and pathological examination; (3) follow-up and survival. Follow-up using postoperative outpatient or inpatient examination to detect patient survival was conducted. Patients underwent imaging examination to detect tumor recurrence. Follow-up was up to December 2020. Measurement data with skewed distribution were represented as M (range). Count data were described as absolute numbers. Results:(1) CT features: results of CT examination showed that each of the 10 patients had only one single tumor, including 3 cases with tumor on the left lobe of liver and 7 cases with tumor on the right lobe of liver. The tumor diameter of the 10 patients was 8.0 cm(range, 4.5-13.5 cm). Results of plain CT scan of 10 patients showed that 7 cases had tumor in expansive growth as round or oval, with clear boundaries and the tumor parenchyma showing low-density signals and patchy lower density area can be seen in the center, and 3 cases had tumor in infiltrative growth as patchy, with unclear boundaries and the tumor parenchyma showing iso-density signals. The CT scan value of 10 patients was 40 HU(range, 23-47 HU). Results of enhanced CT scan at arterial phase of the 7 cases with tumor in expansive growth showed that 3 cases undergoing tumor parenchyma with uneven mild enhancement signals, 3 cases undergoing tumor parenchyma with uneven moderate enhancement signals and 1 case undergoing tumor parenchyma with uneven significant enhancement signal. The CT scan value of 7 cases was 68 HU(range, 62-88 HU). Results of enhanced CT scan at arterial phase of the 3 cases with tumor in infiltrative growth showed that the peripheral region had high signal than the central region. The CT scan value of 3 cases was 73 HU(range, 67-90 HU). Results of enhanced CT scan at portal vein phase in the 7 cases with tumor in expansive growth showed that the tumor parenchyma showing continuous and progressive mild and moderate enhancement with uneven density, and the CT scan value was 63 HU(range, 55-78 HU). Of the 7 cases, 3 cases showed the enhancement range of tumor parenchyma with the trend of fusion and filling, and 4 cases showed small patchy or nodular enhancement and grid like enhancement in the center and periphery of the tumor. Results of enhanced CT scan at portal vein phase in the 3 cases with tumor in infiltrative growth showed that the enhancement withdrew, the density was uneven, and the CT scan value was 58 HU(range, 50-62 HU). Results of enhanced CT scan at delayed phase in 10 patients showed that the enhancement in the tumor withdrew slowly, and the CT scan value was 53 HU(range, 50-60 HU). Of the 10 patients, 4 cases showed decreased density of enhanced nodules around the tumor and 6 cases showed partially fused and filled to the center of tumor with no enhancement in the necrotic part. (2) Treatment and pathological examination: all 10 patients underwent completed tumor resection successfully, and no metastasis was found in liver or the hilar region. Results of postoperative pathological examination showed that each of the 10 patients had only one single visible tumor with tumor diameter of 8.0 cm(range, 4.5?13.5 cm). Of the 10 patients, 7 cases had tumor with complete or incomplete pseudocapsule with clear boundary and 3 cases had tumor without pseudocapsule and the boundary was unclear. All 10 patients had tumor with hard parenchyma and the section was mostly gray and fish like. Among them, patchy or punctate necrosis was seen in 7 cases, small patchy or punctate hemorrhage was seen in 3 cases, and small patchy calcification was seen in 2 cases. Microscopically, the tumor tissue was crisscross, the tumor cells were in spindle shaped, the nuclei were in round, oval, blunt at both ends or in thin rod like, with obvious heteromorphism, large and deeply staining, and obviously division. Immunohistochemical staining showed positive staining of smooth muscle actin, desmin and vimentin. (3) Follow-up and survival: all 10 patients were followed up postoperatively for 6 to 130 months, with a median follow-up time of 55 months. The overall survival time of 10 patients were 10 to 120 months, with a median overall survival time of 46 months. Of the 10 patients, 2 cases died of tumor recurrence and distant metastasis 10 and 11 months after operation, respectively and 8 cases survived >12 months.Conclusions:Results of plain CT scan of PHLMS show clear or unclear cysts and solid masses with uneven density. Results of enhancement CT scan of PHLMS show persistent uneven enhancement in tumor parenchyma and the surrounding area.