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Objective:To investigate the CT, magnetic resonance imaging(MRI) and clinicopathological cha-racteristics of perivascular epithelioid cell tumors (PEComas) in children, and improve the diagnostic accuracy of PEComas.Methods:The CT, MRI and clinicopathological characteristics of 6 children confirmed with PEComas by histopathology caming from Beijing Children′s Hospital, Capital Medical University during March 2019 and April 2022 were retrospectively analyzed.Results:The patients aged from 1.7 to 11.9 years old (with mean age of 7.6 years old). The male to female ratio was 1∶2.Of the 6 solitary tumors identified, 2 cases were found in the kidney, 2 cases in the abdominal cavity, 1 case in the pelvic cavity, and 1 case in the groin.The solitary tumors had well-defined borders.Five solitary tumors were shaped like an ellipse and 1 solitary tumor was multinodular-shaped.Lace-like or nodular changes were observed at tumor margins(3 cases). The maximum diameters of the tumors ranged from 4.8 to 15.9 cm (mean value: 9.3 cm). Five tumors showed peripheral invasion and 2 cases had lymph node metastasis.Two patients were complicated with tuberous sclerosis.One case suffered from tumor recurrence after operation.The tumors (6 cases) were isodensity or low density on the plain CT scan, and gradual heterogeneous enhancement was observed after injection of the contrast agent.A majority of the tumors had tortuous (5 cases) and thickened blood vessels inside, and a few of them (2 cases) underwent gravel-like calcification.Patch-like or nodule-like high signal intensity could be seen in the center of the tumors (3 cases) on MRI T1WI and T2WI, while the peripheral part of the lesions showed iso-signal intensity (equal to muscle). On diffusion weighted imaging(DWI) (b=800 s/mm 2), the lesions exhibited restricted diffusion at the peripheral part with significant enhancement, and iso-signal intensity at the central part with none or slight enhancement.Histological classification results revealed that 1 of 6 cases was a benign PEComa and 5 cases were malignant PEComas.The expression rates of Melan-A and SMA were 100.0% and 83.3%, respectively. Conclusions:The CT, MRI and pathological manifestations of PEComas in children have some distinct features.PEComas are often an oval-like mass with clear borders and nodular margins in the abdominal and pelvic cavity, and tortuous and thickened blood vessels can be seen in the tumor.The periphery of the tumor is the main region to be enhanced by the contrast agent.Local invasion and distant metastasis can be observed.Most of the tumors are malignant in pathology.Both melanocyte and muscle cell markers can be found in the expression of immunohistochemistry.
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Objective@#To evaluate the imaging and clinicopathological characteristics of pancreas perivascular epithelioid cell tumors (PEComa).@*Methods@#The clinical data of patients with pancreatic PEComa admitted in Changhai Hospital of Navy Medical University from Nov 2013 to Nov 2017 were retrospectively analyzed. Related literature on PEComa were searched and the image and clinicopathological characteristics were investigated.@*Results@#Two pancreatic PEComa patients were admitted in Changhai Hospital. Twenty-nine PEComa patients were reported in previous literature. A total of 31 cases were detected, including 3 men and 27 women. The onset age of the patients ranged from 17-74 years old with a mean of 48 years old. The lesions were located at the pancreatic head and neck in 20 cases, and at the pancreatic body and tail in 11 cases. 27 cases had a single lesion, and 4 cases had multiple lesions. Tumor diameter ranged from 1.0-11.5 cm with a mean of 4.1 cm. The cystic solid of the lesions was recorded in 23 patients, and 18 cases had solid mass. Pancreatic duct dilation was reported in 21 patients and 14 of them did not have pancreatic duct dilation. Enhancement mode of the lesions was examined in 21 patients, and the enhancement degree of the lesions were higher than that of the pancreas in 16 cases.@*Conclusions@#PEComa has a certain imaging characteristic, which could help obtain a preoperative diagnosis.
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Objective To investigate the CT feasures of hepatic perivascular epithelioid cell tumors (PEComa),to improve the understanding and diagnosis of this disease.Methods CT findings and clinical data of 13 patients with hepatic PEComa were analyzed retrospectively.Results Among the 1 3 PEComa patients,2 cases were multiple focis and 1 1 cases were single lesion.The maximum diameter of the lesions ranges from 26 mm to 96 mm,with mean diameter (52.77±24.94)mm.The density of 6 cases large lesions was heterogeneous on plain CT image,and the atery phase showed significantly inhomogeneous enhancement and the enhancement decreased in venous phase.7 cases of small lesions was homogeneous in density and showed uniform enhancement in arterial phase.The adjacent organs were compressed in 4 cases,the hepatic artery was located at the margin or inside the tumor in 4 cases,the portal vein or its branch passed through the tumor in 2 cases,and the inferior vena cava was compressed and shifted in 1 case.Conclusion CT manifestations of hepatic PEComa have some characteristics,when the tumor shows homogeneous in density and uniform enhancement in arterial phase,and the enhancement decreases in venous phase,with the thick vessel in the center or at the peripheral,it may prompt diagnosis.
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Objective To investigate the MSCT manifestations of hepatic fat-poor perivascular epithelioid cell tumor (PEComa). Methods CT and pathological findings of 8 patients with hepatic fat-poor PEComa confirmed by surgery were assessed retrospectively.Results 8 cases had solitary lesion,all lesions with round or round-like shape.The largest diameter ranged 20-110 mm.The fat density was not measured by CT scan.6 lesions composed of solid component,and solid part showed obviously enhancement on arterial phase.On portal venous phase and parenchymal phase,the tumors showed equal or low or slightly high density.2 lesions showed cystic necrosis,peripheral enhancement on arterial phase,1 lesion showed continuous enhancement and the enhancement degree increased,and another lesion showed reduced enhancement.All lesions had tortuous vascular in the center of lesions or at the peripheral.Immunohistochemistry examinations showed that HMB45,Melan-A and SMA were positive in all cases,CD31,CD34 and S-100 expressed positive in partial cases. Conclusion The CT findings of hepatic fat-poor PEComa are lack of specificity.When the enhancement and clearance pattern of liver mass is similar to hepatocellular carcinoma or adenoma,the clinical history does not support the diagnosis,may consider the possibility of PEComa when tortuous vascular in the center of lesions or at the peripheral on arterial phase.
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Objective To investigate the imaging features of perivascular epithelioid cell tumor of liver (PEComa),and to improve the diagnostic level of the disease. Methods A retrospective analysis of imaging manifestations was performed on 11 cases of hepatic hemangioma confirmed by surgery and pathology. All patients underwent CT and MRI examination before PET/CT examination ,then received operation. Results Eleven cases were solitary hepatic lesions ,10 of which were located in the right lobe of the liver ,and 1 in the left. The lesions were round or quasi-circular and the diameter ranged from about 13.5cm to about 1.8 cm,with an average of 5.7 cm. CT plain scanning showed that the liver was slightly lower density ,with clear boundary. The smaller lesions were homogeneous density,while the larger ones were not,and the lower density area was visible. Enhancement of peripheral lesions in the arterial phase was enhanced. The lesions in the portal vein were not uniformly enhanced , and visible separation was found in large lesions. In the delay phase ,the lesions were significantly decreased or slightly lower. MRI plain scan showed the lesion T1WI showed moderate signal or slightly low signal ,and large lesion signal was not uniform,and T2WI lesions showed slightly high signal while DWI lesions high signal and the enhanced features were similar to those of CT. PET/CT examination showed that visible radioactive uptake of abnormal concentration or uneven concentration were found in lesions with diameter larger than 3.0 cm,and the maximum SUV value ranged from 12.2 to 3.6,with an average value of 5.7. Lesions with diameter less than 3.0 cm showed a slight concentration of radioactive uptake and the highest SUV value was 4.7,with an average value of 2.9. The only 11.8 cm lesion showed no significant radioactivity ,which was as the same as that of normal liver tissue. Delayed PET/CT imaging(1 hour)showed that SUV values of all lesions were decreased in varying degrees. Conclusion PET/CT imaging of perivascular epithelioid cell tumor of liver has its characteristics. The combina-tion of PET/CT imaging and routine imaging examination will benefit the improvement of diagnosis level obviously.
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Objective To study the clinicopathological characteristics and diagnosis of hepatic perivascular epithelioid cell tumour (PEComa).Methods Retrospective analysis was made on the clinicopathological and immunohistochemical phenotype of the 9 PEComa cases.Results The average age was 39 years,male to female ratio was 1∶ 8.Tumours were grossly well-circumscribed and solid.The maximum diameter were from 1.0 cm to 9.0 cm,with the average of 3.6 cm.The tomours were of nested architecture and composed of uniform epithelioid cells with clear cytoplasm and round nuclei.A subset of tumours was dominated by spindle cells.Thick-wall blood vessels and fat cells were common.Tumour cells were positive for HMB45,MelanA,and SMA (8/8,7/7,4/4).Some of them were positive for S-100 (4/ 8).All of the 9 cases were negitive for hepatocyte (0/9).Ki-67 index of the tumours were below 10％ (9/ 9).All 9 cases were followed up from 3 months to 6 years,all were alive and recurrence free.Conclusion Hepatic PEComa is often seen in adult women with some specific histological patterns and immunophenotypes.Definite diagnosis of PEComa can be made by combining clinicopathological characteristics with a panel of immunohistochemical marks.PEComas grow slowly,often responds to surgical resection and rarely recur.
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Objective:To analyze the clinicopathological characteristics, diagnosis, and differential diagnosis of hepatic perivascu-lar epithelioid cell neoplasm (PEComa). Methods:The clinicopathological features of seven hepatic PEComa patients treated in Shanxi Cancer Hospital from March 2008 to March 2015 were studied with light microscopy and immunohistochemistry combined with litera-ture review. Results:The age range of the seven patients was 41-56 years old (median:51.5 years old). Two were males and five were females. Their histopathological subtypes were classic type (2/7), smooth muscle type (3/7), and fat type (2/7), respectively. Immunohis-tochemical study showed that HMB45 was positive in 7 cases, SMA and VIM were expressed in 6 cases, Melan A was positive in 5 cas-es, and Desmin was expressed in 2 cases. In addition, the mean proliferative index was 1.3%(range:0%-5%), and TFE3 was negative in all cases. Conclusion:Hepatic PEComa is a rare primary liver tumor. The diagnosis and treatment of this disease should be intensi-fied, and long-term close follow-up is necessary. Positive result for HMB45, Melan A, and SMA are the key features of PEComa.
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Clear cell myomelanocytic tumors (CCMTs) of the falciform ligament/ligamentum teres are extremely rare. CCMTs are a variant of perivascular epithelioid cell tumors. We present a case of hepatic CCMT in a 54-year-old woman with abdominal pain. The patient had an 8.8 cm well-demarcated tumor in the right lobe of the liver. Contrast-enhanced computed tomography showed a heterogeneous mass that enhanced significantly in the arterial and portal venous phases, and was less enhanced in the delayed phase. The patient underwent a right hemihepatectomy and cholecystectomy. The tumor cells had clear to slightly eosinophilic cytoplasm, vesicular nuclei, and were positive for HMB-45 and smooth muscle actin. The patient had no recurrence after 36 months follow-up. A review of the literature identified 10 hepatic CCMTs. Hepatic CCMTs are usually benign tumors of young women that present as large masses located in the right lobe of the liver.
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Perivascular epithelioid cell tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Although PEComas have the potential to behave in a malignant fashion, malignant PEComas arising from the retroperitoneum are extremely rare. A 68-year-old woman presented with a painful palpable mass in her left upper abdomen. Computed tomography of the abdomen showed a 9 cm sized heterogeneous mass in left para-aortic space and multiple hypervascular nodules in the liver. 18F-fludeoxyglucose-PET/CT showed multifocal hypermetabolic lesions in retroperitoneum, liver, and skeletal bones. Percutaneous needle biopsies were done on the retroperitoneal and hepatic mass. Both specimens were positive for human melanoma black-45 (HMB-45) on histological and immunohistochemical staining which was compatible with PEComas. Herein, we report a rare case of retroperitoneal PEComa with multiple metastases involving liver and bone at initial diagnosis that exhibited aggressive behavior and resulted in a devastating prognosis.
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Aged , Female , Humans , Bone Neoplasms/pathology , Fluorodeoxyglucose F18 , Liver Neoplasms/pathology , Perivascular Epithelioid Cell Neoplasms/diagnosis , Positron-Emission Tomography , Tomography, X-Ray ComputedABSTRACT
Objective To investigate the clinical featurcs of hepatic perivascular epithelioid cell carcinoma (PEComa) and the experiments in the surgical treatment of PEComa.Methods The clinical data of 16 patients with hepatic PEComa who received surgical treatment at the First Affiliated Hospital of Nanjing Medical University from January 2008 to January 2012 were retrospectively analyzed.The incidence,clinical manifestations,imagiong characteristics,surgical outcomes and pathological manifestations of this disease were analyzed.B sonography,hepatic function test,tumor markers test and epigastric computed tomography (CT) were applied to detect tumor recurrence and metastasis.The follow-up was ended in December 2012.Result Middle aged and female patients took large part of the patients.Of the 16 patients,8 had no subjective symptoms,and other patients had discomfort in the right upper quadrant,pain or tenderness of the liver.All the tumors were solitary,and most of them located at the right liver (11 tumors were in the right liver,4 in the left liver,1 in the caudate lobe).No specific features were detected by preoperative B sonography,while inhomogeneous low density in the tumor region was detected on by CT.All the patients received partial liver resection without morbidity and morality.The results of immunohistochemistry showed that thc expressions of HMB-45,Melan-A and vascular smooth muscle actin were positive.The mean time of follow-up was 27.9 months (range,9.0-46.0 months),no tumor recurrence and death was observed during the follow-up.Conclusions Middle aged females are susceptive to hepatic PEComa,and patients have no specific clinical presentations.Preoperative CT examination is helpful for differential diagnosis of PEComa,and partical hepatectomy can achieve satisfactory short-term clinical outcomes.
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Perivascular epithelioid cell tumor (PEComa) is extremely rare, which originated from mesenchymal cells in the intestine, and composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. We report here on a case of PEComa in the sigmoid colon. A 62-year-old woman presented with hematochzia 10 days ago. Her abdominal computed tomography scan showed a 5 cm sized intraluminal fungating heterogeneously enhanced, high density mass, which infiltrated pericolic tissue surrounding the sigmoid colon. Colonoscopy showed a purple colored polypoid mass with lobulating contour in the sigmoid colon. She underwent laparoscopic anterior resection. On the histologic examination, the tumor consisted of polygonal epithelioid cells with sheet-like growth of nests, which looked like alveolar tissues in lung. The tumor cells were strongly positive stained with human melanoma black-45 (HMB-45). Pathologic examination was compatible with PEComa. Sixteen months after surgery, she did well without tumor recurrence after surgery. We review the literatures concerning PEComa of the intestine focusing on endoscopic findings.
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Female , Humans , Middle Aged , Colonic Neoplasms/diagnosis , Colonoscopy , Gastrointestinal Hemorrhage , Melanoma-Specific Antigens/metabolism , Perivascular Epithelioid Cell Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
A 7-year-old boy presented with hematochezia and abdominal pain. A 3.7-cm-sized mass was identified in the ascending colon by abdominal computed tomography and colonoscopy. The patient underwent surgical resection. Pathological examination revealed a low-grade perivascular epithelioid cell tumor (PEComa). PEComa in the colon is very rare. Only a few cases have been reported so far. An effective treatment method for this rare tumor has not been established yet. The patient received adjuvant interferon-alpha immunotherapy for 1 year. He has been tumor-free for 26 months since the initial diagnosis. This report is the first documented case of the use of interferon-alpha for pediatric PEComa of the colon.
Subject(s)
Child , Humans , Abdominal Pain , Colon , Colon, Ascending , Colonoscopy , Epithelioid Cells , Gastrointestinal Hemorrhage , Immunotherapy , Interferon-alpha , Perivascular Epithelioid Cell NeoplasmsABSTRACT
Objective To investigate the clinicopathological features,the diagnosis and therapy of primary pulmonary perivascular epithelioid cell tumor (PEComa). Methods One case of primary pulmonary perivascular epithelioid cell tumor diagnosed and treated in April 2010 was presented. The clinical symptoms,imaging, pathology features, treatment and follow-up record of the patient were analyzed retrospectively,and relevant literatures were reviewed. Results The patient had not special clinical symptoms and was found through the medical checkup. The imaging methods could easily find the tumor but hard to diagnose it. The final diagnosis depended on the pathologic features and immunohistochemistry techniques. Among present literatures, scarce cases of pulmonary malignant PEComa were reported. Conclusion Primary pulmonary PEComa is a very rare tumor that arises from mesenchymal tissues, especially the malignant cases. The diagnosis of PEComa mainly depends on the pathological features. Because of the uncertain biologic behavior pulmonary PEComa should be followed up closely for a long term.
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Perivascular epithelioid cell tumor (PEComa) is a rare family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis and clear cell 'sugar' tumor of the lung. Although this type of tumor has been described in the literature in organs such as kidney, lung, uterus and urinary bladder, there are few reports of gastrointestinal tract-related tumor. We report here on a case of PEComa arising in the transverse colon. This occurred in a 41-year-old male who had no history of tuberous sclerosis complex. Histopathologically, the tumor consisted of nests or sheets of epithelioid cells with eosinophilic cytoplasm. The tumor cells were positive for HMB-45, vimentin and caldesmon, but they were negative for S-100 protein, cytokeratin and CD117, according to immunohistochemical staining. Careful follow up is warranted because the biological behavior of PEComa has not yet been documented. We present here a case of colonic PEComa that was confirmed by immunohistochemical staining and the histopathologic findings, and we include a review of the literature.