Objective To investigate the radiological and clinical features of osteoarthrosis in congenital insensitivity to pain with anhidrosis (CIPA).Methods The clinical and radiological features in 8 cases of CIPA diseases were retrospectively analyzed. There were five males and three females,age ranged from 11 to 18 years with a median age of (14±3) years. X-ray plain film findings in eight cases were analyzed for the location, extent and contour of the lesions, surrounding bony sclerosis, as well as joint and soft tissue changes. Results The main clinical presentations were insensitivity to pain, anhydrosis, fever, mental retardation and infection. In these eight cases, a total of 166 pieces of bone were involved, including the plantar and phalanges (n=115),phalanx (n=29).The radiological findings include dissolution of the entire bone or most of the bone (n=113), dissolution of a few small areas of the bone (n=40), dissolution of the bone with only residual punctate areas (n=7), the“pushpin sign”and“pencil tip sign”and“flat cut sign”of the finger osteolysis (n=6), sclerosis of peripheral bone (n=12), pathological fractures (n=2). Joint dislocation (n=3),joint deformity (n=3), and Charcot joint (n=3) were observed.Necrosis of soft tissue(n=4), complete absence of soft tissue in the finger or toe (n=4), dot-like calcification of the soft tissue(n=3) were also observed. Conclusion Osteoarthrosis in patients with CIPA had certain X-ray and clinical characteristic findings, which could facilitate its diagnosis.

Objective To investigate the CT characteristics of primary pulmonary sarcomatoid carcinoma (PSC). Methods CT characteristics of 14 cases with primary pulmonary saromatoid cacinoma confirmed by surgical pathology were analyzed retrospectively. Results Among the 14 cases, the clinical manifestations were chest pain and cough in 6 cases, cough with blood-stained sputum in 5 cases, chest tightness and asthma in 2 cases, and no symptoms in 1 case. All the 14 cases had solitary masses in the lung. Carcinoma of 7 cases located in the upper lobe of the right lung, and carcinoma of 1 case located in the inferior lobe of the right lung. Carcinoma of 3 cases was located in the upper lobe of the left lung, and 3 cases were found in the inferior lobe of the left lung. Eleven cases had peripheral tumors and 3 cases had central tumors. CT plain scan revealed an isodense mass. There were 12 cases with uneven mass density and necrosis, and 2 cases had uniform mass density. The smooth margin was identified in 7 masses, and ill-defined margin and lobulation was shown in 7 masses. Cavity was observed in 1 mass, and speckled calcification was seen in 1 mass. Pleural invasion and thickening was in 5 cases, pneumothorax occurred in 1 case, and there was 1 case of liver metastasis. Enhancement scan showed patchy or annular enhancement around the mass, and the central enhancement of the mass was not obvious or uneven patchy enhancement. Conclusions The CT characteristics of primary pulmonary sarcomatoid carcinoma are solitary masses in the lung, especially if the mass is located in the periphery of the upper lobe of the right lung with uneven density, and enhancement scan shows patchy or annular enhancement around the mass.

Objective:To investigate the computed tomography (CT) features of primary hepatic fibrosarcoma (PHF).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 8 patients with PHF who were admitted to 2 medical centers from January 2010 to December 2019, including 3 cases of Yueqing People's Hospital of Zhejiang and 5 cases of Wenzhou People's Hospital of Zhejiang were collected. There were 5 males and 3 females, aged 50 years(range, 39?60 years). All patients underwent abdominal CT plain and enhanced examination, and surgical procedures were selected based on patients' will and individual situations. Surgical tumor specimens were given pathological examination and immunohistochemical staining. Observation indicators: (1) imaging features of CT examination; (2) treatment and pathological examinations; (3) follow-up. Follow-up was performed by outpatient examination or hospitalization to detect survival of patients and tumor recurrence up to December 2020. Measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers. Results:(1) Imaging features of CT examination: 8 patients received preoperative CT examinations, 3 cases of which were diagnosed as malignancies, 3 cases were diagnosed as hepatic space-occupying lesions, 2 cases were diagnosed as intrahepatic cholangiocarcinoma. ① Tumor location and general status: 8 patients had single tumor, of which 6 cases had tumor located in the right lobe of the liver and 2 cases had tumor located in the left lobe of the liver. The tumor was in oval shape in 5 patients and in circular in 3 patients. The maximum diameter of tumor in 8 patients was 9.3 cm(range, 4.0?15.0 cm). There were 1 case with tumor maximum diameter ≤5.0 cm, 4 cases with tumor maximum diameter as 5.1?10.0 cm, 3 cases with tumor maximum diameter >10.0 cm. ② CT plain scan showed uniform low density in 2 cases and uneven low density in 6 cases and the CT value of 8 cases was 40 HU(range, 29?43 HU). The tumor showed expansive growth with clear boundary in 6 cases, and invasive growth with unclear boundary in 2 cases. Five cases had intratumoral necrosis and cystic transformation, 2 cases had intratumoral strip hemorrhage, and 1 case of intratumoral calcification. There was 1 case with hilar lymph node metastasis. ③ Enhanced CT scan showed 8 cases with uneven enhancement in arterial phase, 8 cases with uneven tumor density in portal vein phase, 8 cases with intratumoral enhancement slowly withdrew in delayed phase and the density lower than liver parenchyma. (2) Treatment and pathological examinations: ① 8 patients under-went surgical resection, without intrahepatic or hilar metastasis. ② The results of postoperative pathological examination showed 2 cases with grade Ⅰ tumor differentiation (low potential malignancy), 5 cases with grade Ⅱ and Ⅲ tumor differentiation (moderate potential malignancy), 1 case with grade Ⅳ tumor differentiation (high potential malignancy). ③ Immuno-histochemical staining showed that 8 cases were positive for vimentin, CD10, p53 and negative for smooth muscle actin, keratin19, epithelial membrane antigen, endotheliocyte 34. All 8 patients were diagnosed as PHF. (3) Follow-up: all 8 patients were followed up for 7 to 128 months, with a median follow-up time of 53 months. The overall survival time of 8 patients was 15 to 122 months, and the median overall survival time was 45 months. One patient died of tumor recurrence and distant metastasis at 17 months after surgery, and the survival time of 7 patients was >28 months.Conclusions:CT plain scan of PHF showed uneven low-density masses. After enhancement, the arterial phase shows the tumor ring or peripheral nodular uneven enhancement, the intratumoral strip-shaped enhanced blood vessels. The portal vein phase shows the tumor-like small patches or grid-like continuous enhancement. The delayed phase is characterized by the slow withdrawal of intratumoral enhancement.

Objective:To study the CT manifestations of malignant hepatic perivascular epithelioid cell carcinoma (PEComa).Methods:Clinical data of nine patients undergoing surgery with histologically confirmed malignant hepatic PEComa in Yueqing People's Hospital, Wenzhou People's Hospital, and Yongjia County People's Hospital from January, 2010 to June, 2022 were retrospectively collected, including two males and seven females with a median age of 47 (43, 56) years old. The CT findings, including tumor size, shape, boundary, density, and enhancement patterns, were analyzed.Results:CT scans showed that all nine tumors were single lesions. Five tumors were located in right liver lobe, three in left lobe, and one in caudate lobe. The median tumor diameter was 5.7 (range, 3.3-16.0 ) cm. In terms of tumor shape, three were round, four were quasi-circular, and two were irregular. Eight tumors had well-defined boundaries, while one was ill-defined. Nine tumors showed uneven densities and were lower than the adjacent liver parenchyma. Four tumors had a false capsule, one contained fatty tissue, and six had necrotic and cystic changes. In the arterial phase of contrast-enhanced CT scanning, two tumors showed moderate enhancement and seven showed significant enhancement. In the portal venous and delayed phases, enhancement decreased significantly in four cases, showing a " fast-in and fast-out" pattern. In four cases, the enhancement persisted, showing a " fast-in and slow-out" pattern. One case showed no enhancement in any phase but had a strip-like enhanced vessel inside the tumor. Five cases had significantly thickened vessels around the tumor.Conclusion:The CT manifestations of PEComa are as follows: round or quasi-circular lesions with well-defined boundaries, uneven low densities, significant enhancement in the arterial phase and rapid washout or persistent enhancement in the portal venous and delayed phases.

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.