Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the...Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvemen...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.展开更多
Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hep...Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.展开更多
Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological,immunohistochemical and molecular findings.We report a c...Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological,immunohistochemical and molecular findings.We report a case of PEComa arising in the antrum of a 71-year-old female with melena.The tumor,located predominantly in the submucosa as a well delimited nodule,measured 3.0 cm in diameter and was completely resected,with no evidence of the disease elsewhere.Histologically,it was composed predominantly of eosinophilic epithelioid cells arranged in small nests commonly related to variably sized vessels,with abundant extracellular material,moderate nuclear variation and discrete mitotic activity.No necrosis,angiolymphatic invasion or perineural infil-tration was seen.Tumor cells were uniformly positive for vimentin,smooth muscle actin,desmin and melan A.Although unusual,PEComa should be considered in the differential diagnosis of gastric neoplasia with characteristic epithelioid and oncocytic features and prominent vasculature.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.P...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.PEComa originating in the liver is extremely rare,with most cases being benign,and only a few cases are malignant.Good outcomes are achieved with radical surgical resection,but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.CASE SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month.An ultrasoundguided percutaneous liver aspiration biopsy was performed.Postoperative pathological immunohistochemical staining was HMB45,Melan-A,and smooth muscle actin positive.Perivascular epithelioid tumor was diagnosed.The tumor was large and could not be completely resected by surgery.Further digital subtraction angiography revealed a rich tumor blood supply,and interventional embolization followed by surgery was recommended.Finally,the patient underwent transarterial embolization(TAE)combined with sorafenib for four cycles.Angiography reexamination indicated no clear vascular staining of the tumor,and the tumor had shrunk.The patient was followed up for a short period of time,achieved a stable condition,and surgery was recommended.CONCLUSION Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.展开更多
Perivascular epithelioid cell tumors （PEComas） are a family of rare mesenchymal neoplasms. The PEComas, composed of epithelioid and spindle cells, have the same cellular and immtmohistocherrtical features but are fo...Perivascular epithelioid cell tumors （PEComas） are a family of rare mesenchymal neoplasms. The PEComas, composed of epithelioid and spindle cells, have the same cellular and immtmohistocherrtical features but are found in different visceral and soft tissue sites. Here, we report the histological and immunohistochemical features of one case of PEComa restricted in the pelvic visceral peritoneum of a male patient. The patient was treated with radical surgery, and was well and on follow-up visits without tumor recurrence.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell morphology.The tumor is characterized by immunoreactivity for melanocytic and myogenic ma...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell morphology.The tumor is characterized by immunoreactivity for melanocytic and myogenic markers but can be misdiagnosed as more common tumors with similar characteristics,including gastrointestinal stroma tumors or leiomyosarcomas.Recently,a subset of PEComas has been reported to harbor a transcription factor binding to TFE3 fusion.Herein,we report a rare case of TFE3-expressing malignant PEComa arising from the mesentery.CASE SUMMARY A 50-year-old woman presented with abdominal discomfort for 3 months.Results of laboratory tests were all within the normal ranges,and the patient had no notable medical history.Magnetic resonance imaging revealed a large tumor on the right side of the pelvic floor,which was originally suspected to be a primary ovarian tumor.However,during surgery,the tumor was revealed to have originated from the mesentery.Histologically,the tumor was composed of bundles of spindle cells and sheets of epithelioid cells.Extensive coagulative necrosis and numerous mitotic figures were observed.Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin,HMB-45,and TFE3 expression.Tumor involvement of the rectal serosa was identified,leading to a final diagnosis of malignant PEComa of the mesentery.Surgical resection was followed by adjuvant chemotherapy.No recurrence or metastasis was observed over a 6-month follow-up period.CONCLUSION Malignant PEComa of the mesentery is extremely rare and should be distinguished from morphological mimics through differential diagnosis and immunohistochemistry.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cau...BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent one.We describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current literature.CASE SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested intussusception.Endoscopic direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by surgery.Postoperative pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar pattern.The cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was rare.The immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spot<5%).Combined with the results of pathology and immunohistochemistry,we diagnosed the tumor as PEComa.Postoperative recovery was good at the 4 mo follow-up.CONCLUSION The diagnosis of PEComa mainly depends on pathology and immunohistochemistry.Radical resection is the preferred treatment method.展开更多
A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial res...A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.展开更多
A 4-mo history of both epigastralgia and back pain was presented in a 39-year-old male. Computed tomography showed right lung nodule and abdominal mass attached to the gastric wall, measuring approximately 30 mm and 7...A 4-mo history of both epigastralgia and back pain was presented in a 39-year-old male. Computed tomography showed right lung nodule and abdominal mass attached to the gastric wall, measuring approximately 30 mm and 70 mm in diameter. Since biopsy samples from the lung and abdomen revealed poorly differentiated adenocarcinoma and malignant tumor, clinicians first interpreted the abdominal mass as metastatic carcinoma, and a right lower lobectomy with following resection of the mass was performed. Gross examination of both lesions displayed gray-whitish to yellow-whitish cut surfaces with hemorrhagic and necrotic foci, and the mass attached to the serosa of the lesser curvature on the gastric body. On microscopic examination, the lung tumor was composed of a proliferation of highly atypical epithelial cells having abundant eosinophilic cytoplasm, predominantly arranged in an acinar or solid growth pattern with vessel permeation, while the abdominal tumor consisted of sheets or nests with markedly atypical epithelioid cells having pleomorphic nuclei and abundant eosinophilic to clear cytoplasm focally in a radial perivascular or infiltrative growth pattern. Immunohistochemically, the latter cells were positive for HMB45 or α-smooth muscle actin, but the former ones not. Therefore, we finally made a diagnosis of malignant perivascular epithelioid cell tumor(PEComa) arising in the gastric serosa, combined with primary lung adenocarcinoma. Furthermore, small papillary car-cinoma of the thyroid gland was identified. The current case describes the coincidence of malignant PEComa with other carcinomas, posing a challenge in distinction from metastatic tumor disease.展开更多
Perivascular epithelioid cell tumor(PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by ...Perivascular epithelioid cell tumor(PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell's morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically. Pathological examination indicated that PEComa originated from the pancreas, but symptoms related to tuberous sclerosis were absent. Since PEComa is extremely rare in the pancreas, it is likely to be ignored in differential diagnosis. In conclusion, our article highlighted the clinicopathological features of PEComa, and we conducted a literature review focusing on PEComa so as to deepen the understanding of this tumor type.展开更多
"Perivascular epithelioid cutaneous" cell tumors(PEComa) are a family of mesenchymal tumors with shared microscopic and immunohistochemical properties: They exhibit both smooth muscle cell and melanocytic di..."Perivascular epithelioid cutaneous" cell tumors(PEComa) are a family of mesenchymal tumors with shared microscopic and immunohistochemical properties: They exhibit both smooth muscle cell and melanocytic differentiation. Non-neoplastic counterpart of PEComa's cells are unknown, as well as the relationship between extracutaneous PEComa and primary cutaneous ones. We will review the clinical setting, histopathologic features, chromosomal abnormalities, differential diagnosis and treatment options for cutaneous PEComa.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasm that can arise in many different organs with a broad spectrum of biological behavior, from indolent to aggressive progression. Only...BACKGROUND Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasm that can arise in many different organs with a broad spectrum of biological behavior, from indolent to aggressive progression. Only ten cases of gastric PEComas have been reported in the English literature, which were treated with endoscopic, laparoscopic, or open resections. Due to its rarity, the optimal surgical management and prognosis of this tumor are still uncertain.CASE SUMMARY We present a case of robotic wedge resection of a 6.5 cm bleeding lesion of the gastric fundus located 3 cm below the esophago-gastric junction in a 55-year-old man. Biopsy revealed a malignant tumor with epithelioid cells focally positive for muscle markers desmin and smooth muscle actin. In addition, histology revealed that the tumor was positive for HMB-45, melan-A(MART-1), microphthalmia transcription factor and negative for pan-cytokeratin AE1/AE3, CD34, p40, DOG-1, CD117(c-kit), S100, CD3, CD79 a, caldesmon and myogenin. These markers suggested the possibility of a PEComa. The patient underwent a diagnostic laparoscopy via the da Vinci? Si? system and robotic wedge resection. Final pathology confirmed a malignant gastric PEComa with negative margins. At his 11-mo follow-up visit, the patient remained disease-free.CONCLUSION Gastric PEComa can be treated with a robotic R0 resection with acceptable postoperative and short-term oncological outcomes.展开更多
Perivascular epithelioid cell tumors （PEComas） are a group of rare mesenchymal neoplasms which composed of histologically and immunohistochemically distinctive perivascular epithelioid cells （PECs）. In recent year...Perivascular epithelioid cell tumors （PEComas） are a group of rare mesenchymal neoplasms which composed of histologically and immunohistochemically distinctive perivascular epithelioid cells （PECs）. In recent years, PEComas have been manily reported in liver, kidney, lung, uterus and adnexa,1 but rare in small or large intestine.2-6 Here, we describe a case of PEComa restricted in the cecal mucosa, which displayed histological characteristics of epithelioid clear cells.展开更多
A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lu...A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.展开更多
BACKGROUND Melanotic Xp11-associated tumors are rare mesenchymal-derived tumors. So far,most primary melanotic Xp11-associated tumors have been reported in the kidney, and reports of this tumor in the gastrointestinal...BACKGROUND Melanotic Xp11-associated tumors are rare mesenchymal-derived tumors. So far,most primary melanotic Xp11-associated tumors have been reported in the kidney, and reports of this tumor in the gastrointestinal tract are rare.CASE SUMMARY Here we describe the case of a 25-year-old woman who presented with a melanotic Xp11-associated tumor in the sigmoid colon. Colonoscopy revealed a large mucosal bulge in the sigmoid colon, approximately 32 cm inside the anus.The surface was rough with local erosion. The tumor was brittle on biopsy and bled easily. Computed tomography revealed thickening of the rectal wall with edema. Postoperative pathology indicated the likelihood of a perivascular epithelioid cell tumor. Histologically, the tumor comprised plump epithelioid cells with abundant clear to lightly eosinophilic cytoplasm and round nuclei arranged in an alveolar or trabecular pattern. The tumor cells were strongly positive for HMB-45, Melan-A, Cathepsin K, and TFE3 but negative for vimentin,smooth muscle actin, S100 protein, CD10, CK20, and desmin. The tumor cells had a low Ki-67 labeling index(approximately 2%). Fluorescence in situ hybridization revealed TFE3 fracture. Based on these histologic and immunohistochemical features, a diagnosis of melanotic Xp11-associated tumor of the sigmoid colon was made.CONCLUSION In summary, we report the clinicopathological features of a primary tumor that is extremely rare in the sigmoid colon and review the clinicopathological characteristics of melanotic Xp11-associated tumors, compatible with the very rare tumor termed "melanotic Xp11 translocation renal cancer" in all aspects.展开更多
基金Supported by Nantong Municipal Health Commission,No.MSZ2022036.
文摘Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.
基金Supported by Cuiying Scientific and Technological Innovation Program of Lanzhou University Second Hospital,No.CY2022-QN-A13 and No.CY2023-QN-A02Lanzhou Science and Technology Plan Project,No.2023-4-26+1 种基金Gansu Provincial Natural Science Foundation Project,No.23JRRA1630 and No.24JRRA331Gansu Provincial Health Research Program Project,No.GSWSKY-2019-32.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.
基金supported by grants from the Foundation of Jiangsu Collaborative Innovation Center of Biomedical Functional Materials,Basic Research Program-Youth Fund Project of Jiangsu Province(BK20140092)the National Natural Science Foundation of China(81400650,81273261 and 81270583)
文摘Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.
文摘Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological,immunohistochemical and molecular findings.We report a case of PEComa arising in the antrum of a 71-year-old female with melena.The tumor,located predominantly in the submucosa as a well delimited nodule,measured 3.0 cm in diameter and was completely resected,with no evidence of the disease elsewhere.Histologically,it was composed predominantly of eosinophilic epithelioid cells arranged in small nests commonly related to variably sized vessels,with abundant extracellular material,moderate nuclear variation and discrete mitotic activity.No necrosis,angiolymphatic invasion or perineural infil-tration was seen.Tumor cells were uniformly positive for vimentin,smooth muscle actin,desmin and melan A.Although unusual,PEComa should be considered in the differential diagnosis of gastric neoplasia with characteristic epithelioid and oncocytic features and prominent vasculature.
基金Supported by Gansu Provincial Natural Science Foundation,No.21JR7RA417Lanzhou Science and Technology Development guiding Plan Project,No.2019-ZD-72.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells,has a low incidence,and can involve multiple organs.PEComa originating in the liver is extremely rare,with most cases being benign,and only a few cases are malignant.Good outcomes are achieved with radical surgical resection,but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.CASE SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month.An ultrasoundguided percutaneous liver aspiration biopsy was performed.Postoperative pathological immunohistochemical staining was HMB45,Melan-A,and smooth muscle actin positive.Perivascular epithelioid tumor was diagnosed.The tumor was large and could not be completely resected by surgery.Further digital subtraction angiography revealed a rich tumor blood supply,and interventional embolization followed by surgery was recommended.Finally,the patient underwent transarterial embolization(TAE)combined with sorafenib for four cycles.Angiography reexamination indicated no clear vascular staining of the tumor,and the tumor had shrunk.The patient was followed up for a short period of time,achieved a stable condition,and surgery was recommended.CONCLUSION Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.
文摘Perivascular epithelioid cell tumors （PEComas） are a family of rare mesenchymal neoplasms. The PEComas, composed of epithelioid and spindle cells, have the same cellular and immtmohistocherrtical features but are found in different visceral and soft tissue sites. Here, we report the histological and immunohistochemical features of one case of PEComa restricted in the pelvic visceral peritoneum of a male patient. The patient was treated with radical surgery, and was well and on follow-up visits without tumor recurrence.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell morphology.The tumor is characterized by immunoreactivity for melanocytic and myogenic markers but can be misdiagnosed as more common tumors with similar characteristics,including gastrointestinal stroma tumors or leiomyosarcomas.Recently,a subset of PEComas has been reported to harbor a transcription factor binding to TFE3 fusion.Herein,we report a rare case of TFE3-expressing malignant PEComa arising from the mesentery.CASE SUMMARY A 50-year-old woman presented with abdominal discomfort for 3 months.Results of laboratory tests were all within the normal ranges,and the patient had no notable medical history.Magnetic resonance imaging revealed a large tumor on the right side of the pelvic floor,which was originally suspected to be a primary ovarian tumor.However,during surgery,the tumor was revealed to have originated from the mesentery.Histologically,the tumor was composed of bundles of spindle cells and sheets of epithelioid cells.Extensive coagulative necrosis and numerous mitotic figures were observed.Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin,HMB-45,and TFE3 expression.Tumor involvement of the rectal serosa was identified,leading to a final diagnosis of malignant PEComa of the mesentery.Surgical resection was followed by adjuvant chemotherapy.No recurrence or metastasis was observed over a 6-month follow-up period.CONCLUSION Malignant PEComa of the mesentery is extremely rare and should be distinguished from morphological mimics through differential diagnosis and immunohistochemistry.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent one.We describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current literature.CASE SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested intussusception.Endoscopic direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by surgery.Postoperative pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar pattern.The cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was rare.The immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spot<5%).Combined with the results of pathology and immunohistochemistry,we diagnosed the tumor as PEComa.Postoperative recovery was good at the 4 mo follow-up.CONCLUSION The diagnosis of PEComa mainly depends on pathology and immunohistochemistry.Radical resection is the preferred treatment method.
文摘A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.
文摘A 4-mo history of both epigastralgia and back pain was presented in a 39-year-old male. Computed tomography showed right lung nodule and abdominal mass attached to the gastric wall, measuring approximately 30 mm and 70 mm in diameter. Since biopsy samples from the lung and abdomen revealed poorly differentiated adenocarcinoma and malignant tumor, clinicians first interpreted the abdominal mass as metastatic carcinoma, and a right lower lobectomy with following resection of the mass was performed. Gross examination of both lesions displayed gray-whitish to yellow-whitish cut surfaces with hemorrhagic and necrotic foci, and the mass attached to the serosa of the lesser curvature on the gastric body. On microscopic examination, the lung tumor was composed of a proliferation of highly atypical epithelial cells having abundant eosinophilic cytoplasm, predominantly arranged in an acinar or solid growth pattern with vessel permeation, while the abdominal tumor consisted of sheets or nests with markedly atypical epithelioid cells having pleomorphic nuclei and abundant eosinophilic to clear cytoplasm focally in a radial perivascular or infiltrative growth pattern. Immunohistochemically, the latter cells were positive for HMB45 or α-smooth muscle actin, but the former ones not. Therefore, we finally made a diagnosis of malignant perivascular epithelioid cell tumor(PEComa) arising in the gastric serosa, combined with primary lung adenocarcinoma. Furthermore, small papillary car-cinoma of the thyroid gland was identified. The current case describes the coincidence of malignant PEComa with other carcinomas, posing a challenge in distinction from metastatic tumor disease.
文摘Perivascular epithelioid cell tumor(PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell's morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically. Pathological examination indicated that PEComa originated from the pancreas, but symptoms related to tuberous sclerosis were absent. Since PEComa is extremely rare in the pancreas, it is likely to be ignored in differential diagnosis. In conclusion, our article highlighted the clinicopathological features of PEComa, and we conducted a literature review focusing on PEComa so as to deepen the understanding of this tumor type.
文摘"Perivascular epithelioid cutaneous" cell tumors(PEComa) are a family of mesenchymal tumors with shared microscopic and immunohistochemical properties: They exhibit both smooth muscle cell and melanocytic differentiation. Non-neoplastic counterpart of PEComa's cells are unknown, as well as the relationship between extracutaneous PEComa and primary cutaneous ones. We will review the clinical setting, histopathologic features, chromosomal abnormalities, differential diagnosis and treatment options for cutaneous PEComa.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa) is a rare mesenchymal neoplasm that can arise in many different organs with a broad spectrum of biological behavior, from indolent to aggressive progression. Only ten cases of gastric PEComas have been reported in the English literature, which were treated with endoscopic, laparoscopic, or open resections. Due to its rarity, the optimal surgical management and prognosis of this tumor are still uncertain.CASE SUMMARY We present a case of robotic wedge resection of a 6.5 cm bleeding lesion of the gastric fundus located 3 cm below the esophago-gastric junction in a 55-year-old man. Biopsy revealed a malignant tumor with epithelioid cells focally positive for muscle markers desmin and smooth muscle actin. In addition, histology revealed that the tumor was positive for HMB-45, melan-A(MART-1), microphthalmia transcription factor and negative for pan-cytokeratin AE1/AE3, CD34, p40, DOG-1, CD117(c-kit), S100, CD3, CD79 a, caldesmon and myogenin. These markers suggested the possibility of a PEComa. The patient underwent a diagnostic laparoscopy via the da Vinci? Si? system and robotic wedge resection. Final pathology confirmed a malignant gastric PEComa with negative margins. At his 11-mo follow-up visit, the patient remained disease-free.CONCLUSION Gastric PEComa can be treated with a robotic R0 resection with acceptable postoperative and short-term oncological outcomes.
文摘Perivascular epithelioid cell tumors （PEComas） are a group of rare mesenchymal neoplasms which composed of histologically and immunohistochemically distinctive perivascular epithelioid cells （PECs）. In recent years, PEComas have been manily reported in liver, kidney, lung, uterus and adnexa,1 but rare in small or large intestine.2-6 Here, we describe a case of PEComa restricted in the cecal mucosa, which displayed histological characteristics of epithelioid clear cells.
文摘A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.
基金Supported by The Key Research & Development and Transformation Project of Qinghai Province for 2018(No.2018-SF-113)
文摘BACKGROUND Melanotic Xp11-associated tumors are rare mesenchymal-derived tumors. So far,most primary melanotic Xp11-associated tumors have been reported in the kidney, and reports of this tumor in the gastrointestinal tract are rare.CASE SUMMARY Here we describe the case of a 25-year-old woman who presented with a melanotic Xp11-associated tumor in the sigmoid colon. Colonoscopy revealed a large mucosal bulge in the sigmoid colon, approximately 32 cm inside the anus.The surface was rough with local erosion. The tumor was brittle on biopsy and bled easily. Computed tomography revealed thickening of the rectal wall with edema. Postoperative pathology indicated the likelihood of a perivascular epithelioid cell tumor. Histologically, the tumor comprised plump epithelioid cells with abundant clear to lightly eosinophilic cytoplasm and round nuclei arranged in an alveolar or trabecular pattern. The tumor cells were strongly positive for HMB-45, Melan-A, Cathepsin K, and TFE3 but negative for vimentin,smooth muscle actin, S100 protein, CD10, CK20, and desmin. The tumor cells had a low Ki-67 labeling index(approximately 2%). Fluorescence in situ hybridization revealed TFE3 fracture. Based on these histologic and immunohistochemical features, a diagnosis of melanotic Xp11-associated tumor of the sigmoid colon was made.CONCLUSION In summary, we report the clinicopathological features of a primary tumor that is extremely rare in the sigmoid colon and review the clinicopathological characteristics of melanotic Xp11-associated tumors, compatible with the very rare tumor termed "melanotic Xp11 translocation renal cancer" in all aspects.
