Page views in 2021 to date (this page and chapter topics): 576,86 Perivascular epithelioid cell tumor (PEComa), also called clear cell ''sugar'' tumor of the lung, is a rare benign tumor arising from perivascular epithelioid cells (PECs). We report a case of a 15-year-old boy who presented with right lower lobe lesion which turned out to be a clear cell tumor of t
PEComas (tumours showing perivascular epithelioid cell differentiation) are a family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis, clear cell sugar tumour of the lung, and a group of rare, morphologically and immunophenotypically similar lesions arising at a variety of visceral and soft tissue sites PEComas are a group of mesenchymal tumours composed of perivascular epithelioid cells. This family of tumours includes a spectrum of neoplasms in different organs including angiomyolipoma of the kidney and liver, sugar tumour of the lung, lymphangiomatosis, and lymphangiomyoma . To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. Firm diagnostic criteria for malignant pulmonary PEComa need urgently to be established
PEComa is a family of tumours derived from perivascular epithelioid cells (PECs)
PEComa is a family of ubiquitous mesenchymal neoplasms showing dual melanocytic and myogenic differentiation. It is believed to be a proliferation of perivascular epithelioid cells and encompasses several lesions, namely, angiomyolipoma, lymphangioleiomyomatosis (LAM), clear cell sugar tumor of the lung, and PEComa proper Background: Perivascular Epithelioid Cell Tumors (PEComa) is an extraordinarily rare mesenchymal neoplasm especially the malignant type originating from the lung. To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. Firm diagnostic criteria for malignant pulmonary PEComa need urgently to be established
Perivascular Epithelioid Cell Tumour (PEComa) of the Soft Tissue PEComa re-screened from the group of high grade sarcomas without definite differentiation range from pleomorphic to monomorphic cytohistopathological features. Immunostaining for HMB45 of unclassified sarcomas is useful for the classification of these tumours Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms that may show overlapping morphology and immunohistochemistry with uterine smooth muscle tumors. In this study, we evaluated the morphologic, immunohistochemical, and molecular features of 32 PEComas, including 11 with aggress The World Health Organization defines perivascular epithelioid cell tumors (PEComas) as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs) [ 1 ] Her lung lesions resolved, a retroperitoneal mass significantly reduced in size, and response lasted for approximately 10 months. Both patients were initially treated with imatinib without response and both were living three years after development of metastatic disease . 4. Conclusion. PEComa is a rare but increasingly recognized tumor Malignant perivascular epithelioid cell tumour (PEComa) of soft tissue: a unique case. Harris GC(1), McCulloch TA, Perks G, Fisher C. Author information: (1)Canterbury Health Laboratories, Christchurch, New Zealand. Tumours of perivascular epithelioid cells (PEComas) are being increasingly reported at visceral and somatic sites
PEComatous tumors arise from perivascular epithelioid cells and can present in the lung in following forms: 1) Lymphangiomyomatosis; 2) Clear cell sugar tumor of lung; 3) PEComatosis - with overlapping features between lymphangiomyomatosis and PEComa. Other tumors in this family include renal and extrarenal angiomyolipoma, primary extrapulmonary sugar tumor, clear cell myomelanocytic tumor. PEComa (clear cell sugar tumor) of the lung is a rare benign tumor of the lung probably arising from the perivascular epithelioid cells (PECs). We report a case of pulmonary PEComa arising from the periphery of the right lobe of a 64‐year‐old male Epidemiology. PEComas are far more common in females 4.. Associations. tuberous sclerosis complex ; Pathology Histology. All PEComas share similar and distinctive cellular morphology - peripheral epitheloid cells - which have no counterpart in normal tissue 4.These tumors appear as sheets and nests of epithelioid or spindle-shaped cells associated with blood vessel walls The diagnosis of PEComa was made after surgery on the basis of morphological and immunohystochemical criteria. Radiological and intra operative findings suggest that the mass has an hepatic origin.. Finally, the patient was identified as a benign perirenal PEComa coexisting with micro invasive lung adenocarcinoma (T1aN0M0R0). The patient was discharged on the 10th postoperative day. Figure 1 ( A) Chest CT detected a 13×4 mm nodule in the right upper lung; ( B) abdominal MRI revealed a perirenal tumor, 18×28 mm in size, close to the hilum.
Purpose . Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in nonclassic anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history Perivascular epithelioid cell tumours (PEComas) other than angiomyolipoma, clear cell sugar tumour of the lung, and lymphangioleiomyomatosis are very rare mesenchymal tumours. The uterus seems to be the most prevalent site of involvement, but only 13 cases of uterine PEComa have been described. Three of these cases exhibited local aggressive behaviour and only one showed metastasis . Less frequently, PEComas arise in various other locations throughout the body including soft tissue, bone, and visceral organs DDx. eosinophilic granuloma, usual interstitial pneumonia, emphysema. Treatment. medical therapy, lung transplantation. Lymphangioleiomyomatosis, abbreviated LAM, is a rare lung pathology that predominantly afflicits women of childbearing age. It is also known as lymphangiomyomatosis
PEComa of the lung ABSTRACT Perivascular epithelioid cell tumor (PEComa), also called clear cell ''sugar'' tumor of the lung, is a rare benign tumor arising from perivascular epithelioid cells (PECs). We report a case of a 15-year-old boy who presented with right lower lobe lesion which turned out to be a clear cell tumor of the lung CASE PRESENTATION: In the first case, malignant uterine PEComa with vaginal and multiple lung metastasis was misdiagnosed preoperatively as uterine leiomyosarcoma despite a preoperative punch biopsy and immunohistochemical analysis of the metastatic vaginal mass. In the second case, synchronous uterine PEComa showing benign histology with lymph. Both CCT and AML have been identified as a group of neoplasms with perivascular epithelioid cell differentiation (PEComa). We report a case with multiple PEComas of a combination of CCT of the lung and AML of the liver. The patient underwent surgical resection of an abnormal nodule of the lung 5 years after treatment of AML of the liver 1 Introduction. Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor composed of perivascular epithelioid cells with immunohistochemical characteristics of smooth muscle and melanocytic markers.Although the morbidity of this tumor can happen with either sex, it has a predilection for women. Unlike typical angiomyolipoma, PEComa can display an aggressive clinical course with a 47.
Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features A family of mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These cells do not have a normal anatomic homolog. (From Fletcher CDM, et. al., World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, 2002) essive behavior. Two distinct morphologies were observed: classic (n=30) and those with a lymphangioleiomyomatosis appearance (n=2). In the former, patients ranged from 32 to 77 (mean: 51) years and 13% had tuberous sclerosis. Tumors ranged from 0.2 to 17 (mean: 5.5) cm with 77% arising in the corpus. Epithelioid cells were present in 100% and a spindled component was seen in 37%. Nuclear. Perivascular epithelioid cell tumors of the lung are rare, benign neoplasms, usually presenting as a solitary pulmonary nodule on chest roentgenograms. Most lesions are solitary and asymptomatic, and are located within the peripheral lung. This is a case report of a 44-year-old woman who presented with thrombocytosis and solitary pulmonary nodule of the lung, which was removed by a thoracotomy
Bonetti F, Pea M, Martignoni G, et al.: Clear cell (sugar) tumor of the lung is a lesion strictly related to angiomyolipoma-the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology. 1994, 26: 230-236. 10.1080/00313029400169561 PEComas of the lung encompass different lesions including clear cell sugar tumor, angiomyolipoma, benign and malignant PEComa. PEComa affects adults with a wide age range (8-73 years, mean age of 57), showing a predominant incidence in middle-age and elderly people and a slight male predominance 1 21 22. No relationship with smoking. The PEComa family includes angiomyolipoma (AML), clear cell sugar tumor of the lung and extra pulmonary sites, lymphangioleiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Very rarely, PEComas may arise in the skin Pulmonary PEComa (formerly known as clear cell sugar tumor) is a benign localized mass arising in the lung characterized by epithelioid clear cells which express melanocytic markers such as HMB45 and Melan-A [1,2,3].As the new name indicates, it is composed by perivascular epithelioid cells (PEC) which are positive, besides for melanocytic markers, for contractile proteins, mainly alpha. Maria Cecilia Mengoli, Pathology Unit, Azienda Unit Sanitaria Locale/ICCS eggio Emilia, Italy - Tel. 39 0522 296279 - benign PEComa and malignant PEComa has been created; 4. pulmonary myxoid sarcoma with EWSR1-CREB1 irregular outlines and conspicuous nucleoli. The cyto-plasm varies from clear, eosinophilic to basophilic and.
Due to the rarity of cases of PEComa, the criteria for the diagnosis of malignancy have not yet been fully established. The uterus is the most frequently affected organ, but only 13 cases showing malignant clinical features have been reported ().Folpe et al reported 26 cases of PEComa of soft tissue and gynecologic origin in 2005, 6 and proposed provisional criteria that could be used to. PEComa is very rare mesenchymal neoplasm which is formed by perivascular epithelioid cells and is characterized by dual melanocytic and myoid differentiation. Up to now only a very few cases of PEComa of the liver have been described worldwide. We herein present a patient who underwent a right hemihepatectomy for a huge tumor which could not be identified by imaging investigations Pathology of the lung mass 56 confirmed the same. 57 58 Conclusion 59 Pigmented PEComas are rare and difficult to distinguish from malignant melanoma. 60 Findings of atypical spindle cell neoplasm with focal pigment production which is 61 positive for HMB-45 and negative for S-100 stain makes PEcoma more likely The specimen pathology was found to be metastatic PEComa and was sent for additional genetic testing. Fig. 1 Axial T1 weighted post-contrast magnetic resonance imaging (MRI) demonstrating a large heterogeneously enhancing mass arising from the deep surface of the lower rectus abdominis muscle (arrows Lung pathology 1. LUNG PATHOLOGY They have rounded outlines. The one toward the center of the photograph contains Langhans giant cells. Granulomas are composed of transformed macrophages called epithelioid cells along with lymphocytes, occasional PMN's, plasma cells, and fibroblasts..
Tutorial contains images and text for pathology education. At high magnification, the alveoli in this lung are filled with a smooth to slightly floccular pink material characteristic for pulmonary edema. Note also that the capillaries in the alveolar walls are congested with many red blood cells. Congestion and edema of the lungs is common in. Posted on January 12, 2021 January 12, 2021 Author pathologyoutlinesblog Categories Image Quiz Tags Image Quiz, lung, lung pathology, microscopic images, pathology, Pathology Outlines, pathologyoutlines, Pulmonary Leave a comment on 12 January 2021: Image Quiz #47. Website Powered by WordPress.com ported a case of lung hyaline cell sugar tumor, which was very similar to pancreas hyaline cell tumor; conse-quently, we suggested the term PEComa for PEC tumors located in different places and with no connection. Armah et al.  proposed that PEComa tumors, with the exception of AML, LAM, and CCST, should b This review better defines the natural history of PEComa-NOS of various primary sites and outlines the importance of long-term surveillance, given a number of cases of late recurrence. Ideal treatment strategies remain undefined, but an emerging role for mTOR inhibitors raises enthusiasm in the treatment of these rare tumors
TTF-1, a lung adenocarcinoma marker, and p40, a squamous cell carcinoma marker, have emerged as useful markers for non-small cell lung carcinoma 10, 11 and are thus supposed to be expressed in. PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who. CHRONIC VENOUS CONGESTION - LUNG. 'The most common mechanism for CVC lung is left sided heart failure. The Left heart failure many be due to coronary heart disease or long standing hypertension. Gross Morphology: The lungs are heavy and is rusty brown colored on cut section as a result of hemosiderin laden macrophages PEComa is a part of a family of tumors that include other neoplasms such as angiomyolipoma (AML), lymphangioleiomyomatosis, clear cell sugar tumor of the lung, and a group of histologically and immunophenotypically similar tumors arising in other locations. 2-4. PEComa of the GI tract is predominantly found in the colon and small intestine Am J Surg Pathol 2000 Sep;24(9):1239-46 Abstract quote The perivascular epithelioid cell family of tumors (PEComas), defined by their co-expression of melanocytic and muscle markers, includes angiomyolipoma, lymphangioleiomyoma, and clear cell sugar tumors of the lung, pancreas, and uterus
Tutorial contains images and text for pathology education A fungus ball composed of blue-staining hyphal elements of Aspergillus is seen here in a bronchus. Fungus balls may also form when fungi colonize cavitary lesions of tuberculosis Olympus BX50, BX40 or BH2 or AO or Nikon with 15x eyepiece: 0.096 mm2; AO with 10x eyepiece: 0.12 mm2; Nikon or Olympus with 10x eyepiece: 0.16 mm Lung transplantation-related pathology encompasses a spectrum of disorders that include, but are not limited to, indications for lung transplantation (seen in explanted lungs), surgical complications (airway anastomotic and vascular complications), ischemia-reperfusion injury, rejection (acute and chronic), infections, and posttransplantation lymphoproliferative disorders (PTLDs) respiratory cytology cytology in outline format with mouse over histology previews
Understanding Your Pathology Report: Lung Cancer. When your lung was biopsied, the samples taken were studied under the microscope by a specialized doctor with many years of training called a pathologist.The pathologist sends your doctor a report that gives a diagnosis for each sample taken. Information in this report will be used to help manage your care These changes have resulted from advances in molecular testing and radiology as well as greater use of immunohistochemistry in subtyping tumors. The major changes in the 2015 WHO classification of adenocarcinomas of the lung (resected tumors) are: 1) Discontinuing the terms bronchioloalveolar carcinoma and mixed subtype adenocarcinoma; 2.
A sarcoma is a type of cancer that starts in tissues like bone or muscle. Bone and soft tissue sarcomas are the main types of sarcoma. Soft tissue sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can be found in any part of the body Ga Hie Nam, M.D. Assistant Professor, Pathology and Laboratory Medicine. Academic Office Location: Pathology and Laboratory Medicine. UConn Health. 263 Farmington Avenue. Farmington, CT 06030-3985. Website (s): Clinical Laboratory and Pathology Services Lung transplantation-related pathology. This image shows bronchial-associated lymphoid tissue (BALT): A collection of small mature lymphocytes is present which is associated with pigment. Although.
Classifications of Lung Tumors in 1967 and 1981, of Lung and Pleural Tumors in 1999 and Tumors of the Lung, Pleura, Thymus and Heart in 2004.3-6 Due in part to remarkable advances in lung cancer genetics and therapy in the past decade, there are significant changes since the 2004 WHO classifica Pulmonary apical cap, abbreviated PAC, is a rare benign lung pathology that is found at one or both of the apices
Pathology Outlines. Pathology Outlines is a multi-authored online textbook covering a wide range of topics with macroscopic and microscopic pathology images Normal lung. Pathology Pointer Note the outlines of two major lobes, upper and lower, with a well-defined fissure. This indicates that the section is from a left lung. Preparation Fixed, Gough section. View Gross photograph. Specimen Lung Image Credit Colin M. Bloor, M.D. Department of Pathology School of Medicine University of California, San. ournal of Case reports in Pratie ( CrP) 21 CS RPORT PEComa (Pure Epithelioid Angiomyolipoma) of kidney AnilA.K.R. 1, P Aul Augustine 2,thARA somAnAthAn 1, J AyAsRee.K 1 1Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala 2Department of Surgical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala AbstrACt A 60-year-old male patient presented with complaints of. Spindle cell lesions. A spindle cell lesion ( MPNST ). H&E stain. Spindle cell lesions are seen frequent enough that one ought to have a solid approach to 'em. A general introduction to spindle cells is found in the spindle cell article The 2015 World Health Organization (WHO) Classification of Tumors of the Lung, Pleura, Thymus and Heart has just been published (). 1 This follows previous WHO Classifications of Lung Tumors in 1967 and 1981, of Lung and Pleural Tumors in 1999 and Tumors of the Lung, Pleura, Thymus and Heart in 2004.3, 4, 5, 6 Due in part to remarkable advances in lung cancer genetics and therapy in the past.
Pathology, College of Medicine and Veterinary Medicine, Edinburgh University, Edinburgh, UK Wallace W A H (2009) Histopathology 54, 28-42 The challenge of classifying poorly differentiated tumours in the lung The lung is a common site for primary carcinomas as well as a wide range of metastatic carcinomas an The AMPECT trial was conducted at 9 U.S. sites and treated 34 adult patients, with 31 confirmed as PEComa by a central pathology laboratory. PEComa origin sites in these patients included the.
A collaborative expert panel, sponsored by a joint effort of the College of American Pathologists (CAP), the International Association for the Study of Lung Cancer (IASLC), and the Association of Molecular Pathology (AMP) has issued a preliminary guideline document. 2 The initial release of this preliminary document was accompanied by an. Posted on January 12, 2021 January 12, 2021 Author pathologyoutlinesblog Categories Image Quiz Tags Image Quiz, lung, lung pathology, microscopic images, pathology, Pathology Outlines, pathologyoutlines, Pulmonary Leave a comment on 12 January 2021: Image Quiz #47. Website Powered by WordPress.com Some features of other types of interstitial lung disease may be seen to a limited extent, but do not predominate Bronchiolitis obliterans / organizing pneumonia; Desquamative interstitial pneumonia Department of Pathology Stanford University School of Medicine Stanford CA 94305-5342 . Original posting/updates: 11/20/1 PEComa is a family of rare mesenchymal tumours which can occur in any part of the human body. Primary PEComas of the pancreas are extremely rare tumours with uncertain malignant potential. A 17-year-old female was admitted to the hospital due to melena. She required several transfusions. CT scan demonstrated a mass at the head of the pancreas measuring 4.2 cm in maximum diameter
WebPathology is a free educational resource with 11174 high quality pathology images of benign and malignant neoplasms and related entities. Visual survey of surgical pathology with 11174 high-quality images of benign and malignant neoplasms & related entities Regarding gross pathology, the mass appeared to be a 4-cm, gray and white, segmented, circumscribed tumor confined to the lower right lung lobe parenchyma under the pleura ().Histologically, the tumor was composed of epithelioid cells with abdundant clear cell cytoplasm
Abstract. Gestational trophoblastic disease (GTD) is subclassified into hydatidiform mole (HM), Gestational trophoblastic tumours (GTT) and non-neoplastic trophoblastic lesions. Hydatidiform mole, partial and complete, originate from villous trophoblast and are considered as preneoplastic conditions. The risk for development of persistent. Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas. American journal of clinical pathology 2011; 136(5):762-6. 32. Lewis JS, Ritter JH, El-Mofty S. Alternative epithelial markers in sarcomatoid carcinomas of the head and neck, lung, and bladder-p63, MOC-31, and TTF-1. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 2005; 18(11):1471-81. 33 Among them, pure epithelioid PEComa/epithelioid angiomyolipoma is the most challenging due to the overlapping immunohistochemical expression of cathepsin K and melanocytic markers, such as HMB45.
INTRODUCTION. Lung cancer is the most common cancer worldwide and is still responsible for the most cancer deaths according to the World Health Organization, more than double the next two highest cancers of liver and lower gastrointestinal tract . (See Clinical manifestations of lung cancer.). The pathologic features of the major lung malignancies will be reviewed here Pathology and Genetics of Tumours of the Lung, Pleura, Thymus, and Heart. IARC Press: France: Lyon, 2004, pp 344. Google Scholar 4. Cooper WA, O'Toole S, Boyer M et al. What's new in non-small. DISCLAIMER: Transplant Pathology Internet Services makes no warranties that the information contained herein is in every respect accurate or complete, and is not responsible for any errors or omissions or for the results obtained from the use of such. Users of this information are encouraged to confirm the information contained herein with other sources One difficulty in defining the natural history of PEComa-NOS is the large number of patients who had undergone resection of what was felt to be a different entity a number of years earlier and subsequently presented with PEComa. Unless the initial pathology was reviewed and felt to be consistent with primary PEComa-NOS, cases such as this were. The objective of the study was to explore the clinical expression, radiological and pathological features, differential diagnosis, and biological behavior of a clear cell myomelanocytic tumor. In a case involving a clear cell myomelanocytic tumor located in the hepatic falciform ligament, we evaluated clinical expression, radiological characteristics, histopathology, immunohistochemistry, and.
The pathology report is important to the staging of lung cancer. In some cases, the report can provide some or all of the information needed to stage the disease. Usually, additional tests will be needed for staging, including positron emission tomography (PET) and bone scans , to determine if the malignancy has metastasized (spread), and what. telescoping. thermal artifact. vacuolation. glandular molding. mimicks crowding but with tearing of surrounding stroma. pseudopapillary endometrium. lots of strips of endometrium. histiocytes. can have vacuoles and look like signet ring cells