Multi-slice spiral CT manifestations of perivascular epithelioid cell tumors / 中国基层医药

Objective:To investigate CT manifestations of perivascular epithelioid cell tumors.Methods:The multi-slice spiral CT manifestations in 11 patients with perivascular epithelioid cell tumors which were pathologically confirmed in the First Affiliated Hospital of Shantou University Medical College between October 2012 and August 2021 were retrospectively analyzed.Results:Perivascular epithelioid cell tumors were located in the kidney of eight patients, in the right lobe of the liver of two patients, and in the descending colon of one patient. The largest tumor, approximately 20.0 cm × 26.0 cm × 11.0 cm, was located in the kidney. The smallest tumor, 2.4 cm × 2.6 cm × 3.4 cm, was located in the colon. CT plain scans showed uniform density without bleeding, calcification, or fat in two patients, and non-uniform density with fatty change in two patients. Contrast-enhanced CT images showed non-uniform enhancement in the arterial phase in 10 patients and uniform enhancement in one patient. Contrast-enhanced CT images showed decreased degrees of enhancement of the lesions in 11 patients from the portal venous phase to the delayed phase and dilated and tortuous vascular shadows in 11 patients.Conclusion:The multi-slice spiral CT manifestations of perivascular epithelioid cell tumors are characteristic and the multi-slice spiral CT is of value for the diagnosis of perivascular epithelioid cell tumors.

Clinical diagnosis and treatment of liver perivascular epithelioid cell neoplasms / 中华肝胆外科杂志

Objective:To analyze the clinical features and prognosis of liver perivascular epithelioid cell neoplasms (PEComa).Methods:The clinical data of 12 patients with liver PEComa diagnosed by pathology at the First Affiliated Hospital of Xi 'an Jiaotong University from October 2011 to November 2021 were retrospectively analyzed. There were 1 male and 11 females, with a median age of 44 (range 20 to 63) years old. The clinical manifestations, laboratory examinations, imaging features, treatment methods, postoperative pathological features and treatment outcomes of these patients were collected and analysed. Postoperative follow-up by telephone or patient's follow-up records.Results:Among the 12 patients with hepatic PEComa, 8 patients (66.7%) were asymptomatic and 11 patients (91.7%) had a single lesion. All patients underwent surgical treatment, including local tumor resection in 10 patients (83.3%) and extended hemihepatectomy in 2 patients (16.7%). Enhanced CT showed the lesion to be a quasi-round homogeneous low-density mass, enhanced in arterial phase with hepatic artery branches in the lesion, and decrease in enhancement degrees in portal vein phase and delayed phase. Postoperative pathology of the lesions in all the 12 patients was benign. Immunohistochemical results showed that the positive rates of melanoma cell markers HMB45, Melan-A and smooth muscle actin were 100.0%(12/12), 83.3%(10/12) and 91.7%(11/12) respectively. The median follow-up period was 27 months, and no recurrence or metastasis was found.Conclusion:Hepatic PEComa occurred commonly in women with obscure symptoms. The lesion was mainly single and it had no correlation with hepatitis. It is easily confused with primary liver cancer and liver metastasis on medical imagings. PEComa expressed markers of both melanocyte and smooth muscle cell, and radical surgical resection resulted in good results.

CT, magnetic resonance imaging and clinicopathological characteristics of perivascular epithelioid cell tumor in children / 中华实用儿科临床杂志

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.

A clinical study on 17 patients with liver perivascular epithelioid cell tumors / 中华肝胆外科杂志

Objective:To study the clinical manifestations and prognosis of patients with liver perivascular epithelioid cell tumor (PEComa).Methods:The clinical manifestations, imaging findings, pathological features, treatment and prognosis of 17 patients with liver PEComa managed in Shenzhen People's Hospital from September 2002 to January 2020 were retrospectively analysed.Results:The patient age of onset of liver PEComa was 22 to 54 years (average 34.8 years). There were 13 females and 4 males. 82%(14/17) of patients presented with no significant symptoms. 15/17 had solitary tumors. The diagnostic rate using preoperative B-ultrasound, CT and MRI was low. Postoperative pathological studies showed the tumor cells were mainly epithelioid cells with hyaline or eosinophilic cytoplasm. There were abundant blood vessels in the tumor tissues. Adipose tissues were also seen. Immunohistochemistry showed both HMB45 and Melan-A positivities to arrive at the diagnosis. All patients were treated with surgical resection. There were no recurrence, metastasis, or death on follow-up.Conclusion:Hepatic PEComa occurred more frequently in young women and it had no specific clinical manifestations. Except for a small number of tumors with adipose tissue detected on preoperative imagings, this tumor was difficult to diagnose, or even be suspected on preoperative imagings. Pathological examination combined with immunohistochemistry after surgery established the diagnosis. Surgery resulted in good prognosis.

A Review on Placental Tuberculosis

Vein of GALEN aneurysmal malformation (VGAM) is a rare congenital disease caused by arteriovenous shunts between the choroidal arteries and the porencephalic ectatic vein. The diagnosis is often made in utero or during infancy, endovascular treatment remains the most suitable therapeutic means in a well-equipped environment. Here we report here the case of a patient complaining of headache for 1 year, and whose brain CT imaging showed the presence of Galen veinaneurysm with associated non-communicating hydrocephalus. In the absence of the appropriate technical platform, the placement of a ventriculoperitoneal shunt relieved our patient’s symptoms

Tuberculous mastitis: a rare case report

Tuberculous mastitis is a granulomatous mastitis due to infection by mycobacterium tuberculosis. It’s a rare entity witch is often confused with breast carcinoma or pyogenic breast abscess because of its clinical and radiological aspect. The diagnosis is histological with identification of an epithelioid cell granulomas and necrosis. The treatment is based on anti-tuberculous therapy with a good clinical issue. Authors report a case of tubercular mastitis in a post-menopausal female diagnosed on adenectomy.

Imaging characteristics of pancreatic perivascular epithelioid cell tumor: analysis of 2 cases and literature review / 中华胰腺病杂志

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.

Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report

ABSTRACT CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung. Treatment with everolimus was started. The disease stabilized in the third month of treatment, according to the response evaluation criteria in solid tumors. CONCLUSION: PEComas are tumors with unpredictable behavior. Therefore, these patients require long-term follow-up, even in cases of correct diagnosis and benign PEComa.

Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathological analysis of 60 cases / 中华病理学杂志

Objective@#To investigate the clinical manifestations, imaging features, clinicopathologic features, and differential diagnosis of solitary fibrous tumors/anginoblastomas (SFT/HPCs) originating in the central nervous system.@*Methods@#Sixty cases of SFT/HPCs originating in the central nervous system were collected at Nanjing Jinling Hospital, from January 1, 2008 to December 31, 2016. The clinical data, imaging data, histomorphologic changes and immunohistochemical finding were analyzed in the sixty cases.@*Results@#The 60 cases included 26 males and 34 females, aged 14 to 85 (median 49) years. The main clinical manifestations were headache, dizziness with nausea and vomiting. Radiologically, the tumors were large, enhancing, solid and cystic masses attached to the dura. Histopathologically, the neoplasms were composed of spindle cells with oval nuclei, inconspicuous nucleoli and moderate amount of eosinophilic cytoplasm arranged in fascicles with areas of hyalinized stroma, myxoid changes and a staghorn vascular pattern. Immunohistochemically, tumor cells of all cases were positive for vimentin (100.0%, 60/60), STAT6 (98.3%, 59/60), CD34 (61.7%, 37/60), and the tumor cells were typically positive for CD99, bcl-2, EMA and SSTR2 as well.Negative for S-100 protein, SOX10, E-cadherin, GFAP. Ki-67 index ranged from 1% to 50%. Forty cases were followed up for 6 to 82 months with average of 40 months, 30 patients were alive and 10 patients died.@*Conclusions@#Central nervous system SFT/HPCs can be aggressive and relapses may occur several years after diagnosis. STAT6 is highly sensitive and specific for the diagnosis. Complete tumor resection is optional treatment followed by radiotherapy and chemotherapy. There is a correlation between the prognosis and the location of the disease, the histological grade, Ki-67 index, and fusion gene variants.

Malignant perivascular epithelioid cell tumor in kidney: A case report and literature review / 肿瘤

Objective: To investigate the clinicopathological characteristics, diagnosis, and treatment of malignant perivascular epithelioid cell tumor (PEComa) in kidney. Methods: The cinical diagnosis and treatment outcome of a case of PEComa were reported. The morphological characteristics of renal PEComa were analyzed. The PEComa-related literatures were reviewed, and the diagnosis and treatment strategies of PEComa were summarized. Results: A 21-year-old female patient with a solid mass in the left kidney underwent nephrectomy. The pathological examination revealed PEComa. After 2 years, the computed tomography (CT) scan showed several masses in the lung and bone, which were speculated to be metastases from the kidney lesion. The patient received chemotherapy with recombinant human endostatin and Apatinib. Despite active treatment, the tumor was still progressing, and the patient died of respiratory failure 45 months after the original diagnosis. Literature reviews showed that PEComa patients had not typical clinical symptoms, and the positive immunohistochemical results of human melanoma black-45 (HMB45), melanoma antigen (Melan-A), and smooth muscle actin (SMA) were the key features in the diagnosis of PEComa. Conclusion: PEComa is a kind of rare tumor. The diagnosis and treatment of this disease should be intensified, and the long-term close follow-up is necessary.

CT imaging features of hepatic perivascular epithelioid cell tumor / 实用放射学杂志

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.

TFE3-Expressing Perivascular Epithelioid Cell Tumor of the Breast

Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.

MSCT manifestations of hepatic fat-poor perivascular epithelioid cell tumor / 实用放射学杂志

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.

Epithelioid Cell Histiocytoma on the Palm Mimicking Pyogenic Granuloma / 대한피부과학회지

No abstract available.

PEComa (neoplasia de células epiteliodes perivasculares) asociado con cáncer papilar de tiroides bilateral sincrónico / PEComa (Perivascular Epithelioid Cell Neoplasms) associated with synchronous bilateral papillary thyroid cancer

Resumen Introducción: Los tumores de células epitelioides perivasculares (PEComas) son neoplasias poco frecuentes de origen mesenquimal. Estos incluyen el angiomiolipoma epitelioide (EAML), una neoplasia con potencial maligno y cuya presentación simultánea con cáncer de tiroides es bastante rara. Presentación del caso: Hombre de 40 años con masa de crecimiento rápido en relación con polo inferior derecho de tiroides e infiltración de tráquea. La TAC abdominopélvica contrastada muestra múltiples masas renales con masa dominante en polo superior izquierdo. El estudio histopatológico de tiroides evidenció una lesión en lóbulo derecho correspondiente a cáncer papilar de tiroides (PTC) de variante de células altas y una segunda neoplasia en el lóbulo contralateral correspondiente a PTC moderadamente diferenciado de variante clásica. En riñón se encontró una lesión correspondiente a EAML, con células de aspecto epitelioide equivalentes al 40%. Discusión: El presente artículo corresponde al primero en la literatura en describir la presentación simultánea de EAML con PTC bilateral sincrónico (SBiPTC). Se describe una vía molecular común, que corresponde a la vía TSC/mTOR, la prevalencia de SBiPTC y los marcadores inmunohistoquímicos para diagnóstico de EAML.

Abstract Introduction: Perivascular Epithelioid Cell Neoplasms (PEComas) are uncommon tumors of mesenchymal origin. These ones include the epithelioid angiomyolipoma (EAML), a tumor with malignant potential and whose simultaneous presentation with thyroid cancer is quite rare. Presentation of case: Forty years old man with fast-growing mass in relation to lower right thyroid pole and tracheal infiltration. Abdominopelvic contrast-enhanced CT shows multiple renal masses with dominant mass in the left upper pole. Thyroid histopathology showed a lesion in right lobe corresponding to tall cell variant of papillary thyroid cancer (PTC) and a second neoplasm in the contralateral lobe corresponding to classical variant of moderately differentiated PTC. In the kidney was found a tumor corresponding to EAML with epithelioid aspect cells equivalent to 40%. Discussion: This article corresponds to the first in the literature to describe the simultaneous presentation of EAML with synchronous bilateral PTC (SBiPTC) and its possible association. A common molecular pathway corresponding to the TSC/mTOR pathway is described, as well as SBiPTC prevalence and immunohistochemical markers for EAML diagnosis.

Angiomyolipoma of the Glabellar Region

Angiomyolipomas are hamartomatous lesions consisting of smooth muscle bundles, thick-walled blood vessels, and mature adipocytes. They are usually found in the kidneys of tuberous sclerosis patients and more rarely in other organs, such as the liver, the oral cavity, the sinonasal tract, the heart, the large intestines, the lungs, and the skin. Cutaneous angiomyolipoma has shown to be very rare and generally occurs at the ends of the digits, the elbows, the ears, and the nose. Herein, we report the first documented case of angiomyolipoma of the glabellar region.

Epithelioid angiomyolipoma of the liver: a case report

Epithelioid angiomyolipoma (EAML) of liver is a rare neoplasm. Hepatic EAML is often misdiagnosed as other neoplasms such as hepatocellular carcinoma due to non-specific clinical and radiologic features. The morphologic features under microscope and immunohistochemistry staining profile are important in the diagnosis EAML. Here, we report a case of 52-year-old man who found 1.2 cm mass in liver by routine checkup. On the impression of hepatocellular carcinoma, lateral sectionectomy of the liver was done. Microscopically, the tumor is composed of predominant epithelioid cells with vascular component and foamy cells. These cells were positive for HMB45, MelanA, and smooth muscle actin and negative for epithelial membrane antigen. The final diagnosis was hepatic EAML.

Clinicopathological characteristics of hepatic perivascular epithelioid cell tumours / 中华普通外科杂志

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.

Perivascular Epithelioid Cell Tumor in the Stomach

Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST), and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.