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. Epithelioid sarcoma es is a softtissue tumor, composed of large, polygonal cells resembling carcinomas. Epithelioid hemangioendothelioma ehe, first described by weiss and enzinger in 1982, is a malignant vascular neoplasm with indolent behavior in the majority of cases. Epithelioid sarcomas are rare, mesenchymal tumors of unknown histogenesis and display multidirectional differ entiation, which is predominantly epithelial.
Epithelioid sarcoma es has been a rarely developed malignancy affects soft tissues present in the upper limb. Although the exact pathogenesis of ks is not known, infection with hhv8 ksassociated herpes virus, combined with other genetic and environmental factors, has. Epithelioid sarcoma es is a distinctive soft tissue neoplasm with a predilection for. The histopathological diagnosis of myxoid liposarcoma, epi. The current 20 world health organization who classification of tumors of soft tissue and bone 1 was published 11 years after the prior volume. Evaluation of perineurial differentiation in epithelioid sarcoma. Histopathology showed a large softtissue neoplasm with extensive hemorrhage figure 1. Of the 106 cases, 70 were the conventional subtype and 36 the large cell subtype.
A rare case of pseudomyogenic hemangioendothelioma phe. Epithelioid sarcoma is a malignant tumor with an already poor prognosis, aggravated by the commonly delayed diagnosis and resulting inadequate treatment 2,3. It often occurs in the abdominal cavity, mesentery, omentum majus, and affects mostly adults, and men more frequently than women 5. We report a case of a 65 years old male, with history of abdominal pain and upper gastrointestinal bleeding. Immunohistochemistry of epithelioid soft tissue sarcomas. 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. Histology of soft tissue sarcoma in extremities and trunk wall. In 2011, according to the national cancer institute, only 10,980 people in the united states will be diagnosed with a sarcoma. It is not associated with pax3 rearrangements, a typical finding in alveolar rhabdomyosarcoma and biphenotypic sinonasal sarcoma. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. It was originally recognized as a variant of epitheloid sarcoma. We report a study of 106 cases with clinical, histologic, and immunohistochemical data, including ini1 expression, and followup data. Epithelioid sarcoma is an uncommon slowgrowing soft tissue malignancy.
Proximaltype epithelioid sarcoma of the head and neck hn. 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. Epithelial mesothelioma diagnosis epithelioid mesothelioma accounts for the majority of cases. Epithelioid sarcoma es is rare with a poor prognosis and for which a loss of ini1 expression has been recently reported.
It accounts for less than 1% of all soft tissue sarcomas. Epithelioid sarcoma accounts for less than 1% of soft tissue tumors and usually pre sents as a slow. To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma. Epithelioid sarcoma surgical pathology criteria stanford.
Differential diagnosis of epithelioid histiocytoma pathology. Epithelioid sarcoma sarcoma program at cincinnati childrens. 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. 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. Jul 10, 2008 epithelioid sarcoma es is a softtissue tumor, composed of large, polygonal cells resembling carcinomas. Soft tissue ehe equally affects both sexes, but liver and. Epithelioid sarcoma of the extremities akpinar f, dervis e. A proximal variant of es, proximaltype epithelioid sarcoma pes, described in 1997 by guillou, demonstrates a more aggressive clinical course. 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. The who classification of tumours of soft tissue and bone. Epithelioid sarcoma american journal of clinical pathology. Pathology of epithelioid sarcoma dr sampurna roy md. First described in 1970 by enzinger as epithelioid or spindled cells in a multilobular granulomalike pattern with central hyalinization or necrobiosis cancer 1970.
The mass is often mistaken for a nonsarcomatous lesion, as sarcomas are fortunately extremely rare. This is one of the reasons epithelial mesothelioma has the best prognosis of the different mesothelioma cancers. 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. Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioidlike features. 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.
Characterization of five cases article pdf available in skeletal radiology 427 february 20 with 784 reads how we measure. We use cookies to make interactions with our website easy and meaningful, to better understand the use of our services, and to tailor advertising. Epithelioid sarcoma, immunohistochemistry, pathology. An immunohistochemical analysis evaluating the utility of cytokeratin 56 in distinguishing superficial epithelioid sarcoma from spindled squamous cell carcinoma. An immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis markku miettinf. Pdf background epithelioid sarcoma is a rare, highgrade malignant tumor of the. 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. The treating surgeon operated completely within the sartorius compartment and did not violate any other compartments, however the. Epithelioid sarcoma es is a rare malignancy notorious for its tendency to histologically mimic granuloma annulare and other palisading granulomatous processes. August 2017 soft tissue tumor immunohistochemistry updatewei et al 1073. Epithelioid sarcoma is a rare, slowgrowing type of soft tissue cancer. The 5year survival rate for epithelioid sarcoma patients is 5070%, and the 10year survival rate is 4255%. 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. Pseudomyogenic hemangioendotheliomaepithelioid sarcomalike.
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. Other than the name, classical type epithelioid sarcoma shares only the distinctive immunophenotype of proximal type epithelioid sarcoma. Selected topics in the pathology of epithelioid soft tissue. 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. Nearly all ess with typical histology 94% were positive for keratin 8 k8. Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the royal national orthopaedic hospital. 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. Soft tissue tumor immunohistochemistry update archives of. 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. 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. Kaposis sarcoma ks is a lowgrade, spindlecell neoplasm first described by moritz kaposi, in 1872. Diagnostic criteria handbook in histopathology pdf free download.
Epithelioid inflammatory myofibroblastic sarcoma of. Twentyeight patients were treated for a primary epithelioid sarcoma of the hand. Pdf pseudomyogenic hemangioendothelioma epithelioid. Epithelioid inflammatory myofibroblastic sarcoma is an aggressive variant of inflammatory myofibroblastic tumor. 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. Six cases of epithelioid sarcoma were studied by conventional light microscopy and immunohistochemistry. 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. Cutaneous epitheiloid vascular tumours are heterogeneous groups of vascular proliferations sharing in common the epithelioid morphology of tumour cells. Online mendelian inheritance in man omim 601607 mentzel t march 2010. Endoscopy demonstrated a posterior duodenal wall perforation communicating with a solid retroperitoneal neoformation. Therefore it is absolutely necessary to carry out immunohistochemical studies for the correct diagnosis. Richard l kempson md robert v rouse md department of pathology stanford university school of medicine stanford ca 943055342. Histopathological examination of a skin biopsy from a nodule on the palm. It typically occurs in younger patients, with the peak incidence in the third fourth decade and most cases show at least focal cytokeratin expression.
It may also be found in the legs, trunk, head or neck regions. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. Pseudomyogenic epithelioid sarcoma like hemangioendothelioma. Both epithelioid sarcoma and meningioma showed frequent expression of the perineurial markers glut. Selected topics in the pathology of epithelioid soft. It can affect both children and adults, but is most common in young adulthood. Proximaltype epithelioid sarcoma affects middleaged patients and arises in proximal and deepseated locations, primarily the pelvis, perineum, and genital tract. Melanoma amelanotic melanoma will show similar large epithelioid cells but will generally display more pleomorphism and epidermal involvement. 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. Magnetic resonance imaging, which was available from all cases, was assessed. The average age of the five male and one female patient was 10. Epithelioid sarcoma resembling benign fibrous histiocytoma.
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. Eas is character ized by an epithelioid morphologic appearance. Proximaltype epithelioid sarcoma laboratory medicine. Selected topics in the pathology of epithelioid soft tissue tumors. Pediatric patients also tend to display less lymphatic spread and metastasis. This constellation of morphological and immunohistochemical features strongly supports the diagnosis of epithelioid sarcoma, classic type. Most patients will present at a community hospital setting with a mass lesion. Erg and fli1 protein expression in epithelioid sarcoma. Immunohistochemical study and interphase fluorescence in situ hybridization fish analysis were carried out.
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. University of groningen diagnostic and prognostic aspects in. Curent concepts in pathology of soft tissue sarcoma. We report a case of es on the right hand of a 23yearold man that histopathologically resembled a benign fibrous histiocytoma. Proximal epithelioid sarcomaa misnomer, histopathology. 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. 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 hemangioendothelioma is a malignant vascular tumour. 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. A diagnosis of epithelioid sarcoma was established based on the combination of the histopathological and immunohistochemical findings. 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. Pseudomyogenic hemangioendothelioma is an extremely rare soft tissue tumor, also named as epithelioid sarcoma like hemangioendothelioma, which occurs more frequently in young adult males. Typical for the epithelioid sarcoma is immunohistochemical. Ballard 2 1department of pathology, lawrence general hospital, lawrence, ma 01842 2st georges medical school, grenada, west indies abstract.
Pdf epithelioid sarcoma with multiple lesions on the left arm. 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. 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. Synovial sarcoma has fusion of ss18 with ssx1, ssx2 or ssx4. Always think of epithelioid sarcoma when an extremity lesions looks granulomatous keratin positivity is typically seen in both. Evaluation of perineurial differentiation in epithelioid. Bleeding hand mass in an older man mdedge dermatology. Because epithelioid cells lack mobility and adhere closely together, they are less likely to spread compared with sarcomatoid cells. 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. Jul 27, 2016 to explore the clinical characteristics and pathological features of epithelioid inflammatory myofibroblastic sarcoma eims with emphasis on the diagnostic spectrum.
The final pathology came back as a high grade soft tissue sarcoma. The cells of classicaltype epithelioid sarcoma are typically small, uniform, and relatively bland d. The mass was contained within his sartorius muscle and shown in figure 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. Epithelioid sarcoma is a rare, aggressive keratinpositive sarcoma that coexpresses cd34 in 50% of cases and may mimic an angiosarcoma. Children with epithelioid sarcoma tend to have slightly better outcomes than adults, with 5 year survival rates around 65%. Read proximal epithelioid sarcomaa misnomer, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. Epithelioid sarcoma in children and adolescents springerlink. In vast description of es attaining as a subcutaneous or dermal tissue mass in distal segments of the upper or lower limbs. Epithelioid sarcoma is a type of soft tissue sarcoma that typically appears in the extremities especially in the arms and hands. 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.
Cytopathologic features of epithelioid inflammatory. Epithelioid angiosarcoma, immunohistochemistry, histopathology. Epithelioid sarcoma is a rare soft tissue sarcoma in young adults 2039 year olds involving the upper extremities 60% of the time. Epithelioid sarcoma symptoms, pictures, prognosis, treatment. Whereas focal myxoid change does occur, to our knowledge only two cases of es with diffuse myxoid stroma have been reported. Pathology of epithelioid hemangioendothelioma dr sampurna.
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. Epithelioid sarcoma genetic and rare diseases information. Cytologic features of epithelioid hemangioendothelioma. Esun has an outstanding medical advisory and editorial board and is a vital source of information for thousands of readers worldwide. Histopathology images of epithelioid hemangioendothelioma by. In some cases, histogenesis is uncertain, and the designation reflects the architectural pattern eg, alveolar sarcoma of soft parts, epithelioid sarcoma, clear cell sarcoma. These tumours are usually present in the deep soft tissues but. Differential diagnosis epithelioid sarcoma surgical. 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 sarcoma is rare and commonly misdiagnosed. The epithelioid angiosarcoma ea consisted mostly of irregular slitshaped vessels lined by sheets of atypical endothelial cells figure 2. Fetsch, md epithelioid sarcoma es is a distinctive soft tissue neoplasm with a predilection for the distal extremities of young adults. 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. Examples include liposarcoma, synovial sarcoma, leiomyosarcoma, rhabdomyosarcoma rms, fibrosarcoma, and angiosarcoma.
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. Soft tissue, lung formerly known as intravascular bronchioalveolar tumour, pleura, liver, peritoneum, bone, lymph nodes and skin. Epithelioid sarcoma es is a rare malignant soft tissue tumor, which is. From the department of pathology, the university of alabama at. 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. Case report epithelioid angiosarcoma of the middle ear. Epithelioid sarcoma es, a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. Particular clinical findings included the location of the tumours.
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