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.

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.

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.

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.

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.

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.

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.

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.

A Case of Malignant PEComa of the Uterus Associated with Intramural Leiomyoma and Endometrial Carcinoma

Perivascular epithelioid cell tumors (PEComas) refers to a family of mesenchymal neoplasms composed of angiomyolipomas, clear cell “sugar” tumors of the lung, and lymphangioleiomyomatoses. These tumors have a distinctive and common component of perivascular epithelioid cells that show an association with blood vessel walls and immunohistochemically display myomelanocytic differentiation. The unique neoplasms have been shown to have an expanded range through a variety of case reports, including visceral, intra-abdominal, soft tissue, and bone tumors. The retroperitoneum, abdominopelvic region, and uterus have been reported to be the most common sites. Most PEComas follow a benign course. However, reports of malignant PEComas are increasing. Many papers have described uterine PEComas, but to our knowledge, there have not yet been any reports of a malignant PEComa arising concomitant with another epithelial tumor and mesenchymal tumor. We report herein the case of a 67-year-old woman who experienced a malignant uterine PEComa infiltrating a preexisting intramural leiomyoma with synchronous well differentiated endometrial carcinoma and multiple liver and lung metastases.

Sclerosing Perivascular Epithelioid Cell Tumor of the Lung: A Case Report with Cytologic Findings

Benign perivascular epithelioid cell tumor (PEComa) of the lung is a rare benign neoplasm, a sclerosing variant of which is even rarer. We present a case of 51-year-old man who was diagnosed with benign sclerosing PEComa by percutaneous fine needle aspiration cytology and biopsy. The aspirate revealed a few cell clusters composed of bland-looking polygonal or spindle cells with fine granular or clear cytoplasm. Occasional fine vessel-like structures with surrounding hyalinized materials were seen. The patient later underwent wedge resection of the lung. The histopathological study of the resected specimen revealed sheets of polygonal cells with clear vacuolated cytoplasm, variably sized thin blood vessels, and densely hyalinized stroma. In immunohistochemical studies, reactivity of tumor cells for human melanoma black 45 and Melan-A further supported the diagnosis of benign sclerosing PEComa. To the best of our knowledge, this is the first case of benign sclerosing PEComa described in lung.

Multicentric angiomyolipoma in kidney, liver, and lymph node: case report/review of the literature / Angiomiolipoma multicêntrico em rim, fígado e linfonodo: relato de caso/revisão da literatura

ABSTRACT Introduction: The angiomyolipoma (AML) is constituted by adipose tissue, blood vessels and smooth muscle fiber; follows a benign clinical course, with slow growth and absence of metastasis, occurring in tuberous sclerosis or sporadically. Objective: We intend to describe the clinical, radiological and histological aspects of this tumor. Case report: A-64-year-old woman presented with abdominal pain and dyspepsia. Tomography showed hepatic, renal and mesenteric nodules. Histological evaluation of mesenteric lymph node revealed perivascular epithelioid differentiation neoplasia, compatible with AML/perivascular epithelioid cell tumor (PEComa), positive for anti-S100, anti-human melanoma black-45 (HMB-45) and anti-smooth muscle actin. Conclusion: We described a rare AML with renal, hepatic and lymph node involvement, representing a multicenter version instead of metastasis. .

RESUMO Introdução: O angiomiolipoma (AML) constitui-se de tecido adiposo, vasos sanguíneos e fibras musculares lisas; tem curso clínico benigno, crescimento lento e ausência de metástases, ocorrendo no complexo esclerose tuberosa ou esporadicamente. Objetivos: Objetivamos descrever aspectos clinicorradiológicos e histológicos desse tumor Relato do caso: Paciente do sexo feminino, 64 anos, com dor abdominal e dispepsia. Tomografia mostrou nódulos hepáticos, renais e mesentéricos. Exame histopatológico de linfonodo mesentérico evidenciou neoplasia com diferenciação epitelioide perivascular condizente com AML/neoplasias de células epitelioides perivasculares (PEComa), positiva para anti-S100, anti-human melanoma black-45 (HMB-45) e antiactina de músculo liso. Conclusão: Descrevemos raro envolvimento simultâneo renal, hepático e linfonodal por AML, representando versão multicêntrica, e não doença metastática. .

Malignant Perivascular Epithelioid Cell Tumor (PEComa) Arising in the Omentum with Metastatic Peritoneal Seeding: A Case Report / 대한내과학회지

The World Health Organization (WHO) defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. They are rarely malignant. Indeed, only a few cases have been reported in the English literature, and this is the first case of PEComa reported in Korea. A 64-year-old man presented with an abdominal mass and peritoneal seeding. The patient underwent mass excision with descending colon resection and anastomosis. The primary mass and peritoneal seeding was diagnosed as a PEComa histopathologically. Seven months later, he developed liver metastasis and aggravated peritoneal seeding. Here, we report a case of malignant PEComa of the omentum.

A 6-year-old girl with vaginal spotting who was diagnosed with perivascular epithelioid cell neoplasm after vaginoscopic resection

Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor with unknown malignant potential. We report a case of a 6-year-old child with history of brain tumor (pineoblastoma), who presented with intermittent vaginal spotting for 6 months. A vaginoscopy revealed a 1.5x1.0-cm mass on the vaginal wall. Pathological examination demonstrated that the tumor was composed of clear cells with organoid patterns, which were immunohistochemically positive for HMB-45 and TFE3, and negative for CK, HNF1-B, SOX10, Melan A, and S-100 protein. These findings were consistent with PEComa arising from the vagina. Regular follow-up with magnetic resonance imaging has shown no signs of recurrence. This case shows that early detection of PEComa and subsequent regular follow-ups are important because of the neoplasm's unknown malignant potential.

Pigmented Perivascular Epithelioid Cell Tumor (PEComa) of the Kidney: A Case Report and Review of the Literature

Heavily pigmented perivascular epithelioid cell tumors (PEComa) are rare, only eight cases of which have been reported. Unlike typical epithelioid angiomyolipoma, most of these tumors have been encountered in female patients without tuberous sclerosis. The long-term prognosis thereof is undetermined. Cytological similarity and heavy melanin pigment make it difficult for pigmented PEComa to be differentiated from pigmented clear cell renal cell carcinoma or malignant melanoma. The immunoprofile of tumor cells, such as human melanoma black-45 expression, as well as the absence or presence of other melanocytic or epithelial markers, are helpful in determining a differential diagnosis. Here we report a case of heavily pigmented PEComa of the right kidney and review the literature describing this tumor. In this case, the immunoprofile and clinical features corresponded well to those described in the literature. Since the prognosis of such disease has not yet been established, close follow-up of this patient was recommended.

Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver: A Case Report and Review of the Literature

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor consisting of distinctive perivascular epithelioid cells, and is commonly detected in the uterus. The liver is an uncommon site for primary PEComa. In this study, we report a case of primary hepatic PEComa in a 36-year-old woman. Upon gross examination, the tumor was a well-defined, brownish solid mass, measuring 6.5x5.2x4.5 cm. Microscopically, the tumor consisted largely of epithelioid cells and some spindle cells with a clear to eosinophilic cytoplasm and a rich network of delicate capillaries in the stroma. With the exception of their relatively large size and microscopically sinusoidal infiltrative growth pattern, all other histopathologic features of the tumor were consistent with their being benign. The tumor cells were positive for human melanoma black-45 and smooth muscle actin, and negative for cytokeratin-cocktail and c-kit.