Histology of soft tissue sarcoma in extremities and trunk wall. It may also be found in the legs, trunk, head or neck regions. Diagnostic criteria handbook in histopathology pdf free download. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver.
Soft tissue tumor immunohistochemistry update archives of. Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioidlike features. Most patients will present at a community hospital setting with a mass lesion. Epithelioid sarcoma accounts for less than 1% of soft tissue tumors and usually pre sents as a slow. Characterization of five cases article pdf available in skeletal radiology 427 february 20 with 784 reads how we measure. Epithelial mesothelioma diagnosis epithelioid mesothelioma accounts for the majority of cases. This constellation of morphological and immunohistochemical features strongly supports the diagnosis of epithelioid sarcoma, classic type. Epithelioid sarcoma es is a rare malignancy notorious for its tendency to histologically mimic granuloma annulare and other palisading granulomatous processes. Melanoma amelanotic melanoma will show similar large epithelioid cells but will generally display more pleomorphism and epidermal involvement. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. Kaposis sarcoma ks is a lowgrade, spindlecell neoplasm first described by moritz kaposi, in 1872.
Histopathology showed a large softtissue neoplasm with extensive hemorrhage figure 1. An immunohistochemical analysis evaluating the utility of cytokeratin 56 in distinguishing superficial epithelioid sarcoma from spindled squamous cell carcinoma. Epithelioid sarcoma resembling benign fibrous histiocytoma. Histopathology images of epithelioid hemangioendothelioma by. Epithelioid sarcoma es is rare with a poor prognosis and for which a loss of ini1 expression has been recently reported. Pseudomyogenic hemangioendothelioma pheepithelioid sarcomalike hemangioendothelioma esh is a rare vascular tumor of intermediate malignancy that commonly occurs in soft tissue of distal extremities of young adults.
Epithelioid sarcoma of the extremities akpinar f, dervis e. It commonly presents itself in the distal limbs fingers, hands, forearms, or feet of young adults as a small, soft mass or a series of bumps. Epithelioid sarcoma in children and adolescents springerlink. Epithelioid sarcoma es is a softtissue tumor, composed of large, polygonal cells resembling carcinomas. We report a case of a 65 years old male, with history of abdominal pain and upper gastrointestinal bleeding. It often occurs in the abdominal cavity, mesentery, omentum majus, and affects mostly adults, and men more frequently than women 5.
Epithelioid hemangioendothelioma is a malignant vascular tumour. Epithelioid myofibroblastoma of the breast is an extremely rare morphologic variant of myofibroblastoma composed of epithelioid cells comprising more than 50% of the tumour cell population. Histopathology images of epithelioid hemangioendothelioma poorly defined and infiltrative tumour, characterized by nests and cords of spindle to epithelioid cells embedded in a hyaline, myxoid, chondroid or collagenous stroma. Epithelioid sarcoma is a rare, aggressive keratinpositive sarcoma that coexpresses cd34 in 50% of cases and may mimic an angiosarcoma. The histopathological diagnosis of myxoid liposarcoma, epi.
Sep 23, 2014 epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Epithelioid inflammatory myofibroblastic sarcoma is an aggressive variant of inflammatory myofibroblastic tumor. Bleeding hand mass in an older man mdedge dermatology. Although the exact pathogenesis of ks is not known, infection with hhv8 ksassociated herpes virus, combined with other genetic and environmental factors, has. Epithelioid sarcomas are rare, mesenchymal tumors of unknown histogenesis and display multidirectional differ entiation, which is predominantly epithelial. To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma. Proximaltype epithelioid sarcoma laboratory medicine. Cytologic features of epithelioid hemangioendothelioma.
Pathology of epithelioid sarcoma dr sampurna roy md. Both epithelioid sarcoma and meningioma showed frequent expression of the perineurial markers glut. Epithelioid sarcoma is a rare, slowgrowing type of soft tissue cancer. Proximal epithelioid sarcomaa misnomer, histopathology. Differential diagnosis of epithelioid histiocytoma pathology. Epithelioid sarcomas es are rare, slow growing sarcomas with a protracted clinical course that typically occur in distal extremities of young adults, see soft tissue chapter. Epithelioid angiosarcoma, middle ear, histopathology, immunohistochemistry, surgery introduction epithelioid angiosarcoma eas is a rare and highgrade aggressive malignancy originating from endothelial cells, most commonly arises in the deep soft tissues 1. Proximaltype epithelioid sarcoma is an aggressive variant of epithelioid sarcoma most often occurring in soft tissues of the proximal limbs, characterized by polygonal cells, marked nuclear atypia, and varied rhabdoid features. A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and highgrade angiosarcoma. The who classification of tumours of soft tissue and bone. These tumours are usually present in the deep soft tissues but. The treating surgeon operated completely within the sartorius compartment and did not violate any other compartments, however the. Whereas focal myxoid change does occur, to our knowledge only two cases of es with diffuse myxoid stroma have been reported.
The tumour occupies an intermediate position in the spectrum of epithelioid vascular tumours lying between the benign epithelioid hemangioma and the more aggressive epithelioid angiosarcoma. The final pathology came back as a high grade soft tissue sarcoma. Pseudomyogenic hemangioendothelioma is an extremely rare soft tissue tumor, also named as epithelioid sarcoma like hemangioendothelioma, which occurs more frequently in young adult males. Epithelioid sarcoma is a malignant tumor with an already poor prognosis, aggravated by the commonly delayed diagnosis and resulting inadequate treatment 2,3. It accounts for less than 1% of all soft tissue sarcomas. Soft tissue ehe equally affects both sexes, but liver and. Epithelioid angiosarcoma, immunohistochemistry, histopathology. It is not associated with pax3 rearrangements, a typical finding in alveolar rhabdomyosarcoma and biphenotypic sinonasal sarcoma. The 5year survival rate for epithelioid sarcoma patients is 5070%, and the 10year survival rate is 4255%. August 2017 soft tissue tumor immunohistochemistry updatewei et al 1073. Typical for the epithelioid sarcoma is immunohistochemical. A proximal variant of es, proximaltype epithelioid sarcoma pes, described in 1997 by guillou, demonstrates a more aggressive clinical course. Always think of epithelioid sarcoma when an extremity lesions looks granulomatous keratin positivity is typically seen in both. An immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis markku miettinf.
We report a study of 106 cases with clinical, histologic, and immunohistochemical data, including ini1 expression, and followup data. Classic features of epithelioid sarcoma epithelioid and spindled cells in a granulomatous pattern with central hyalinization, necrobiosis or necrosis, are not commonly seen in pes mrt cells are usually polyhedral and epithelioid with abundant eosinophilic cytoplasm, sometimes with dense accentuation resulting in rhabdoid morphology. Epithelioid sarcoma es, a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. Epithelioid sarcoma sarcoma program at cincinnati childrens. A rare case of pseudomyogenic hemangioendothelioma phe. Dec 28, 2009 epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues, but a variety of primary sites, including the adrenals, thyroid, skin, and bone, are encountered. Curent concepts in pathology of soft tissue sarcoma. Online mendelian inheritance in man omim 601607 mentzel t march 2010. Proximaltype epithelioid sarcoma affects middleaged patients and arises in proximal and deepseated locations, primarily the pelvis, perineum, and genital tract. Histopathological differential diagnoses for epithelioid sarcoma include both benign and malignant conditions such as granuloma annulare, necrobiosis lipoidica, fibrous histiocytoma, nodular fasciitis, melanoma, clearcell sarcoma of the tendon and aponeurosis, metastatic squamous cell carcinoma, synovial sarcoma, epithelioid. The name was given by enzinger in 1970 to a group of soft tissue sarcomas that were confused with a variety of malignant and benign conditions, especially granulomatous process, synovial sarcoma and ulcerating squamous cell carcinoma. Cutaneous epitheiloid vascular tumours are heterogeneous groups of vascular proliferations sharing in common the epithelioid morphology of tumour cells. Selected topics in the pathology of epithelioid soft.
Richard l kempson md robert v rouse md department of pathology stanford university school of medicine stanford ca 943055342. Selected topics in the pathology of epithelioid soft tissue tumors. Read proximal epithelioid sarcomaa misnomer, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. This is one of the reasons epithelial mesothelioma has the best prognosis of the different mesothelioma cancers. Kaposi sarcoma kaposiform hemangioendothelioma leiomyomacutaneous leiomyomadeep soft tissue leiomyomageneral leiomyosarcomageneral lipoblastoma lipoblastomatosis lipofibromatosis lipoma lipoma arborescens lipomatosis lipomatosis of nerve littoral cell hemangioma of spleen low grade fibromyxoid sarcoma low grade myofibroblastic. The cells of classicaltype epithelioid sarcoma are typically small, uniform, and relatively bland d. Soft tissue, lung formerly known as intravascular bronchioalveolar tumour, pleura, liver, peritoneum, bone, lymph nodes and skin. Pathology of epithelioid hemangioendothelioma dr sampurna. It was originally recognized as a variant of epitheloid sarcoma. Endoscopy demonstrated a posterior duodenal wall perforation communicating with a solid retroperitoneal neoformation.
Epithelioid sarcoma american journal of clinical pathology. We report a case of es on the right hand of a 23yearold man that histopathologically resembled a benign fibrous histiocytoma. Histopathological examination of a skin biopsy from a nodule on the palm. Epithelioid morphology, mimicking carcinoma, is a key or defining feature of several soft tissue tumors and may be seen in a wide variety of other tumors. Epithelioid sarcoma surgical pathology criteria stanford. University of groningen diagnostic and prognostic aspects in. Pseudomyogenic epithelioid sarcoma like hemangioendothelioma. The histopathology in synovial sarcoma may be biphasic consisting of spindled and epithelioid cells, monophasic only spindled cells, or undifferentiated difficult to recognise without cytogenetic tests. Epithelioid sarcoma is an uncommon slowgrowing soft tissue malignancy. Magnetic resonance imaging, which was available from all cases, was assessed. Phe typically has a multifocal presentation and can involve several tissue planes, including the dermis, subcutis, muscle and bone. In 2011, according to the national cancer institute, only 10,980 people in the united states will be diagnosed with a sarcoma.
First described in 1970 by enzinger as epithelioid or spindled cells in a multilobular granulomalike pattern with central hyalinization or necrobiosis cancer 1970. Synovial sarcoma has fusion of ss18 with ssx1, ssx2 or ssx4. Fetsch, md epithelioid sarcoma es is a distinctive soft tissue neoplasm with a predilection for the distal extremities of young adults. Pediatric patients also tend to display less lymphatic spread and metastasis. Epithelioid sarcoma es has been a rarely developed malignancy affects soft tissues present in the upper limb. Epithelioid sarcoma es is a distinctive soft tissue neoplasm with a predilection for. Pdf pseudomyogenic hemangioendothelioma epithelioid. Epithelioid sarcoma is a rare soft tissue sarcoma in young adults 2039 year olds involving the upper extremities 60% of the time. The spindle cells are arranged in plump fascicles with hyalinization and distinct lobulation figures 2,3 biphasic tumours have spindle cells resembling synoviocytes and plump epithelial cells forming glands cords figure 4, the epithelioid component is indicated with the arrow. A diagnosis of epithelioid sarcoma was established based on the combination of the histopathological and immunohistochemical findings. Epithelioid sarcoma es is a rare malignant soft tissue tumor, which is. Of the 106 cases, 70 were the conventional subtype and 36 the large cell subtype. Nearly all ess with typical histology 94% were positive for keratin 8 k8. Jul 10, 2008 epithelioid sarcoma es is a softtissue tumor, composed of large, polygonal cells resembling carcinomas.
Therefore it is absolutely necessary to carry out immunohistochemical studies for the correct diagnosis. Cytopathologic features of epithelioid inflammatory. The epithelioid angiosarcoma ea consisted mostly of irregular slitshaped vessels lined by sheets of atypical endothelial cells figure 2. We use cookies to make interactions with our website easy and meaningful, to better understand the use of our services, and to tailor advertising. Proximaltype epithelioid sarcoma of the head and neck hn. Pdf background epithelioid sarcoma is a rare, highgrade malignant tumor of the. The epithelioid variant of imt, known as epithelioid inflammatory myofibroblastic sarcoma eims, is first described by marinoenriquez and is clinically aggressive and has a poor prognosis. Epithelioid sarcoma genetic and rare diseases information. The average age of the five male and one female patient was 10.
Erg and fli1 protein expression in epithelioid sarcoma. Epithelioid inflammatory myofibroblastic sarcoma of. Epithelioid hemangioendothelioma ehe is a vascular tumor of intermediate malignancy that may occur in a variety of sites, including soft tissues, 1,2 bone, 3,4 lung, 5 liver, 6 pleura and peritoneum, 7,8 skin, 2,9 lymph nodes, 10,11 stomach, 12 and brain. Other than the name, classical type epithelioid sarcoma shares only the distinctive immunophenotype of proximal type epithelioid sarcoma.
The current 20 world health organization who classification of tumors of soft tissue and bone 1 was published 11 years after the prior volume. A rare aggressive tumor presenting simultaneously in spine and pelvis abstract abhijeet b kadam, ashok k rathod department of orthopedic surgery, lokmanya tilak medical college and general hospital, sion, mumbai, india a. Oncolex cancer encyclopedia about sarcoma soft tissue sarcoma in extremities and trunk wall histology there are many different histological types of malignant soft tissue tumors sarcomas. In some cases, histogenesis is uncertain, and the designation reflects the architectural pattern eg, alveolar sarcoma of soft parts, epithelioid sarcoma, clear cell sarcoma. Twentyeight patients were treated for a primary epithelioid sarcoma of the hand. Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the royal national orthopaedic hospital. Immunohistochemical study and interphase fluorescence in situ hybridization fish analysis were carried out.
Immunohistochemistry of epithelioid soft tissue sarcomas. In vast description of es attaining as a subcutaneous or dermal tissue mass in distal segments of the upper or lower limbs. Sep 16, 2011 epithelioid sarcoma is a rare cancer that most often occurs in the soft tissue of the fingers, hands and forearms of young adults. The tumour may display varied growth patterns but the main clinical significance of recognition of this variant lies in the fact that it mimics invasive lobular carcinoma of the breast, which has obvious. Evaluation of perineurial differentiation in epithelioid sarcoma.
Twentyseven patients 96% had excisions before evaluation, including 11 39% with multiple prior excisions with varying diagnoses before epithelioid sarcoma, and all had surgical treatment after referral. Case report epithelioid angiosarcoma of the middle ear. The mass is often mistaken for a nonsarcomatous lesion, as sarcomas are fortunately extremely rare. Examples include liposarcoma, synovial sarcoma, leiomyosarcoma, rhabdomyosarcoma rms, fibrosarcoma, and angiosarcoma. Epithelioid hemangioendothelioma ehe, first described by weiss and enzinger in 1982, is a malignant vascular neoplasm with indolent behavior in the majority of cases. Six cases of epithelioid sarcoma were studied by conventional light microscopy and immunohistochemistry. Most cases begin in the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, though it can start in other areas of the body. Ballard 2 1department of pathology, lawrence general hospital, lawrence, ma 01842 2st georges medical school, grenada, west indies abstract. From the department of pathology, the university of alabama at. Epithelioid sarcoma is rare and commonly misdiagnosed.
Eas is character ized by an epithelioid morphologic appearance. Particular clinical findings included the location of the tumours. Based on the who classification epithelioid vascular tumours are classified on the basis of their biological behavior into benign tumours epithelioid haemangioma, epithelioid angiomatous nodule and malignant tumours epithelioid. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. However, there is clearly a need for further analyses, using cytogenetic as well as high resolution genomic techniques, on larger series of morphologically wellcharacterized tumors from patients with adequate followup to establish the spectrum of aberrations occurring within each type of soft tissue sarcoma, as well as to allow proper. It can affect both children and adults, but is most common in young adulthood. Epithelioid sarcoma extremely rare, aggressive malignancy slow growing tumor with a high rate of recurrence and metastasis etiology of es is obscure inactivation of the smarcb1ini1 tumor suppressor gene has been identified in roughly 85% of tumors located on long arm of chromosome 22 tumor of uncertain histogenesis. Children with epithelioid sarcoma tend to have slightly better outcomes than adults, with 5 year survival rates around 65%.
Although, a progressive clinical course with tumorrelated fatality has been documented in some instances, this lesion does not behave as aggressively as a conventional angiosarcoma 14. Because epithelioid cells lack mobility and adhere closely together, they are less likely to spread compared with sarcomatoid cells. Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues, but a variety of primary sites, including the adrenals, thyroid, skin, and bone, are encountered. Differential diagnosis epithelioid sarcoma surgical. Epithelioid sarcoma, immunohistochemistry, pathology. Pseudomyogenic hemangioendotheliomaepithelioid sarcomalike. The course of epithelioid sarcoma is often unpredictable and it is common for patients to present with extensive disease, lymph node metastases, or distant metastases. Esun has an outstanding medical advisory and editorial board and is a vital source of information for thousands of readers worldwide. Epithelioid sarcoma was first characterized as a distinct clinicopathologic entity by enzinger in 1970,1 after being described as aponeurotic sarcoma in 1961 by laskowski2 and as largecell sarcoma of the tendon shealth in 1968 by bliss and reed. Jul 27, 2016 to explore the clinical characteristics and pathological features of epithelioid inflammatory myofibroblastic sarcoma eims with emphasis on the diagnostic spectrum. The mass was contained within his sartorius muscle and shown in figure a. Evaluation of perineurial differentiation in epithelioid. Selected topics in the pathology of epithelioid soft tissue. Epithelioid sarcoma symptoms, pictures, prognosis, treatment.
277 642 368 661 429 189 835 542 866 156 833 631 1242 1366 1429 1540 938 215 495 1205 1492 1545 56 612 1513 1223 233 724 124 1072 1047 1157 1487 1070 11 1178 74 667 1199 1279 708 675 1471 174