When an unexpected diagnosis occurs: a vaginal premenopausal sarcoma / Quando um diagnóstico inesperado ocorre: um sarcoma vaginal na pré-menopausa

Abstract Vaginal cancer is a rare entity. The evidence on its management resides mostly in clinical cases or small case series. Of the histological types, the most frequent is the squamous cell carcinoma, followed by adenocarcinoma. But what to do when identifying an even more infrequent sarcoma in a premenopausal woman? In this study, we describe the case of a 53-year-old woman presenting with metrorrhagia for two months, who was evaluated after an intense episode. A necrotic and ulcerative vaginal swelling was documented and then submitted to biopsy, which revealed a vaginal sarcoma. The patient was referred to radiation therapy with 50 Gy (aiming to control the symptoms and to cause tumor reduction for posterior pelvic exenteration with intraoperative radiotherapy) and developed an extra-pelvic metastization at the end of the treatment, which caused a fast negative outcome. Despite the initial poor prognosis, a chemo-irradiation or primary surgery regimen might have achieved (although with greater side effects) a better survival. This case-report entails a discussion about the strategies to manage vaginal sarcoma in advanced stage and in premenopausal women.

Resumo O cancro vaginal é uma doença rara. A evidência para a sua abordagem reside fundamentalmente em casos clínicos ou pequenas séries de casos. Dentre os tipos de cancro histológicos, o mais frequente é o carcinoma espinocelular, seguido do adenocarcinoma. Mas o que fazer em presença de um sarcoma ainda mais raro numa mulher pré-menopáusica? No presente estudo, descrevemos o caso de uma mulher de 53 anos apresentando metrorragia por dois meses, avaliada após um episódio intenso. Foi então documentada uma tumefacção vaginal necrótica e ulcerativa, submetida a biópsia, que revelou um sarcoma vaginal. A paciente foi encaminhada para radioterapia com50 Gy (comos objetivos de controlo da sintomatologia e de redução tumoral para posterior exenteração pélvica com radioterapia intraoperatória) e desenvolveu, ao final do tratamento, umquadro demestastização extra pélvica, que ocasionou um desfecho negativo rápido. Apesar do mau prognóstico inicial, um esquema de quimiorradiação ou cirurgia primária poderiam ter alcançado (ainda que com maiores efeitos laterais) uma maior sobrevivência. Este estudo de caso aborda uma discussão sobre as estratégias de abordagem do sarcoma vaginal em estádios avançados e na mulher pré-menopáusica.

Tumoración pélvica en una paciente con síndrome de li fraumeni / Pelvic tumor in a patient with li-fraumeni syndrome

El síndrome de Li Fraumeni (SLF) es una rara enfermedad hereditaria asociada con un riesgo incrementado de padecer ciertos tumores malignos. Presentamos el caso de una paciente con diagnóstico de SLF con antecedentes de sarcoma de glúteo con metástasis pulmonares y cáncer de mama bilateral metacrónico. Acudió al Servicio de Urgencias por distensión y dolor abdominal. Se objetivó una masa pélvica y se pensó en un probable origen ovárico de la misma. La paciente fue intervenida en el Servicio de Ginecología, y durante la intervención se descartó dicho origen ya que la tumoración dependía del epiplón. El diagnóstico final fue metástasis de sarcoma.

Li-Fraumeni syndrome (LFS) is a rare, inherited syndrome associated with increased risk of various malignant tumors. We present the case of a patient diagnosed LSF with a history of gluteal sarcoma with lung metastases and metachronous bilateral breast cáncer. She came to the emergency department for abdominal bloating and pain. She had a pelvic mass and we had thought probable ovarian dependence. The patient was operated on at the Department of Gynecology, and during the intervention we realized that the tumor depended on the omentum. The final diagnosis was a metastatic of sarcoma.

Descriptive analysis of and overall survival after surgical treatment of lung metastases / Analise descritiva e sobrevida global do tratamento cirurgico das metastases pulmonares

OBJECTIVE: To describe demographic characteristics, surgical results, postoperative complications, and overall survival rates in surgically treated patients with lung metastases. METHODS: This was a retrospective analysis of 119 patients who underwent a total of 154 lung metastasis resections between 1997 and 2011. RESULTS: Among the 119 patients, 68 (57.1%) were male and 108 (90.8%) were White. The median age was 52 years (range, 15-75 years). In this sample, 63 patients (52.9%) presented with comorbidities, the most common being systemic arterial hypertension (69.8%) and diabetes (19.0%). Primary colorectal tumors (47.9%) and musculoskeletal tumors (21.8%) were the main sites of origin of the metastases. Approximately 24% of the patients underwent more than one resection of the lesions, and 71% had adjuvant treatment prior to metastasectomy. The rate of lung metastasis recurrence was 19.3%, and the median disease-free interval was 23 months. The main surgical access used was thoracotomy (78%), and the most common approach was wedge resection with segmentectomy (51%). The rate of postoperative complications was 22%, and perioperative mortality was 1.9%. The overall survival rates at 12, 36, 60, and 120 months were 96%, 77%, 56%, and 39%, respectively. A Cox analysis confirmed that complications within the first 30 postoperative days were associated with poor prognosis (hazard ratio = 1.81; 95% CI: 1.09-3.06; p = 0.02). CONCLUSIONS: Surgical treatment of lung metastases is safe and effective, with good overall survival, especially in patients with fewer metastases. .

OBJETIVO: Descrever características demográficas, resultados operatórios, complicações pós-operatórias e taxa de sobrevida global em pacientes com metástases pulmonares tratados cirurgicamente. MÉTODOS: Análise retrospectiva de 119 pacientes submetidos a um total de 154 cirurgias de ressecção de metástase pulmonar entre 1997 e 2011. RESULTADOS: Do total de 119 pacientes, 68 (57,1%) eram do sexo masculino, e 108 (90,8%) eram brancos. A mediana de idade foi de 52 anos (variação, 15-75 anos). Nessa amostra, 63 pacientes (52,9%) apresentaram comorbidades, sendo as mais frequentes hipertensão arterial sistêmica (69,8%) e diabetes (19,0%). Tumores primários colorretais (47,9%) e musculoesqueléticos (21,8%) foram os principais sítios de origem das metástases. Aproximadamente 24% dos pacientes foram submetidos a mais de uma ressecção das lesões, e 71% fizeram tratamento adjuvante prévio à metastasectomia. A taxa de recidiva de metástase pulmonar foi de 19,3%. A mediana do intervalo livre de doença foi de 23 meses. A principal via de acesso usada foi toracotomia (78%), e o tipo de ressecção mais frequente foi em cunha e segmentectomia (51%). O índice de complicações pós-operatórias foi de 22% e o de mortalidade perioperatória foi de 1,9%. As taxas de sobrevida global em 12, 36, 60 e 120 meses foram, respectivamente, de 96%, 77%, 56% e 39%. A análise de Cox confirmou que complicações nos primeiros 30 dias pós-operatórios associaram-se a pior prognóstico (hazard ratio = 1,81; IC95%: 1,09-3,06; p = 0,02). CONCLUSÕES: O tratamento cirúrgico das metástases pulmonares oriundas de diferentes sítios tumorais é efetivo e seguro, com boa sobrevida global, especialmente nos casos com um menor número de lesões ...

Proximal-type epithelioid sarcoma - Case report / Sarcoma epitelióide tipo proximal - Relato de caso

Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.

O sarcoma epitelióide, primeiramente descrito por Enzinger, em 1970, é uma neoplasia de partes moles que ocorre principalmente nas extremidades distais de adolescentes e adultos jovens. Em 1997, Guillou e cols. descreveram um tipo diferente de sarcoma epitelióide, que afetava frequentemente a região pélvica, períneo e áreas genitais de pacientes de média idade, com exame histológico caracterizado pela proliferação de células com aspecto epitelióide. Neste trabalho, descreve-se caso de paciente que apresentava há três meses duas lesões na região glútea, cujo exame histológico confirmou diagnóstico de sarcoma epitelioide do tipo proximal, já com presença de metástases pulmonares e cerebrais e que foi a óbito nove meses após o diagnóstico.

Surgical Management of a Locally Advanced Symptomatic Recurrence of Penile Sarcoma Secondary to Prostate Brachytherapy

Background The surgical management of patients with symptomatic metastatic or locally advanced recurrences involving the penis remains poorly characterized. The aim of the present abstract and video is to detail our experience in the surgical management of a specific patient with a locally advanced symptomatic recurrence of penile sarcoma secondary to prostate cancer treated with primary brachytherapy. Materials and Methods A 70 year old male patient initially treated for localized prostate cancer with interstitial brachytherapy at an outside facility developed an unfortunate secondary malignancy consisting of a locally advanced penile sarcoma involving as well the prostate and base of the bladder. Despite our best efforts to control his pain, he developed a very symptomatic local recurrence with a secondary penile abscess and purulent periurethral drainage. At this time, it was felt a surgical resection consisting of a total penectomy, urethrectomy, cystoprostatectomy, and ileal conduit urinary diversion would be the best option for local cancer control in this particular patient. Results The patient underwent the surgical resection without any complications as illustrated in this surgical video, with a jejunal intestinal mass identified at the time of surgery which was resected with a primary bowel anastomosis performed. The patient was discharged from hospital uneventfully with his symptomatic local recurrence being successfully managed and the patient no longer requiring oral narcotics for pain control. The pathological report confirmed a locally advanced sarcoma involving the penile, prostate, and bladder which was resected with negative surgical margins and the jejunal mass was confirmed to represent a small bowel sarcoma metastatic site. Conclusion As highlighted in the present video, the treatment of a symptomatic sarcoma local recurrence contiguously involving the penis can be successfully managed provided the patient ...

Multiple metastatic basal cell carcinoma with concurrent metastatic pleomorphic sarcoma in chronic lymphedema area: case report / Carcinoma basocelular múltiplo e metastático concomitante a sarcoma pleomórfico e metástatico em área de linfedema crônico: relato de caso

Chronic lymphedema presents as interstitial fluid retention due to a failure in the lymphatic system drainage. The affected region becomes more vulnerable immunologically and predisposed to the onset of neoplasms. Basal Cell Carcinoma is the most common sort of neoplasm, nevertheless it rarely metastisizes. Sarcomas are malignant mesenchymal neoplasms, locally aggressive, which can spread. Here is reported an infrequent case of multiple basal cell carcinoma, synchronous to a poorly differentiated pleomorphic sarcoma, both spreading to lymph nodes and arising from tissue compromised by chronic lymphedema.

Linfedema crônico se manifesta pelo acúmulo de líquido intersticial por falha da drenagem linfática. A região afetada torna-se imunologicamente vulnerável e predisposta ao desenvolvimento de neoplasias. Carcinoma basocelular é a neoplasia maligna mais comum, entretanto raramente metastatiza. Sarcomas são neoplasias mesenquimais malignas, localmente agressivas e capazes de metastatizar. Apresentamos um caso raro de múltiplos carcinomas basocelulares concomitantes a sarcoma pleomórfico pouco diferenciado, metastáticos para linfonodos, originando-se em área de linfedema crônico.

Metástase cutânea de sarcoma de bexiga: quando devemos considerar essa hipótese? uma revisão bibliográfica / Cutaneous metastasis of bladder sarcoma: when should we consider this hypothesis? a review of the literature

A incidência de metástases cutâneas é de, aproximadamente, 0,7 a 10 por cento. As de origem urotelial correspondem a menos de 1 por cento. Dos tumores do trato genitourinário, o carcinoma de células transicionais é mais comum. O sarcoma de bexiga é raro, correspondendo a 0,3 por cento. Podem ter múltiplas apresentações clínicas, sendo o nódulo mais comum. O diagnóstico é realizado pela anamnese, quadro clínico e histopatologia. O tratamento é cirúrgico. O prognóstico é muito reservado e depende de um acompanhamento multidisciplinar.

The incidence of cutaneous metastases is approximately 0.7 to 10 percent, while in metastases of urothelial origin the incidence is less than 1 percent. Transitional cell carcinoma is the most common of the genitourinary tract tumors. Sarcoma of the bladder is rare (0.3 percent). It can involve multiple clinical presentations, with a nodule being the most common. Diagnosis is made by a combination of medical history, clinical examination and histopathology. The treatment is surgical. The prognosis is very uncertain and depends on a multidisciplinary approach.

A clinico-morphological study of 95 cases of sarcomas with metastases to the lungs.

Background: Metastasis of soft tissue sarcoma most commonly occurs to the lungs. There are very few studies on histology of pulmonary metastatectomy and hardly any wherein the histology of the primary tumor has been compared with the metastasis. Aims and Objectives: To review histologically all metastatic sarcomas to lung and compare with the primary where available. Materials and Methods: Ninety-five patients with pulmonary metastases from sarcoma were analyzed histologically for type of sarcoma, chemotherapy-related changes, and changes in adjacent lung. Various clinical parameters like laterality, multiplicity, and interval between primary and metastasis were also studied. Results: Osteosarcoma constituted half of the metastatic sarcomas (48 cases, 50.5%) followed by synovial sarcoma (16 cases, 16.8%) and high grade spindle cell sarcoma-NOS (10 cases, 10.5%). The histology of primary and the metastases was similar in 60% of cases of osteosarcoma. Conclusions: Osteosarcoma is the commonest metastatic sarcoma to the lung. There is often a change to fibroblastic histology in patients of conventional osteosarcoma treated with chemotherapy.

Metástasis ocular de un sarcoma pleomórfico de partes blandas / Ocular metastasis of soft tissue pleomorphic sarcoma

Introducción: Desde la primera descripción en 1872 de un tumor metastático en el ojo de un paciente con carcinoma, la literatura ha indicado que los tumores metastáticos son los tumores intraoculares más comunes en adultos. Los sarcomas de partes blandas son un heterogéneo grupo de tumores originados en los tejidos extraesqueléticos no epiteliales del cuerpo humano. Las metástasis a los linfáticos regionales son bastante raras en los sarcomas a diferencia de los carcinomas. Objetivo: Aportar conocimientos acerca del tema. Método: Revisión bibliográfica del tema y estudio completo del paciente. Resultados: Se presenta el caso de un paciente masculino blanco, de 32 años de edad, con antecedentes de ser atendido en consulta de reumatología hace un año por dolores osteomusculares que fueron interpretados como una espondilitis anquilosante, acudió a consulta de retina refiriendo disminución de la agudeza visual del ojo izquierdo. Al examen físico se constataron varios desprendimientos serosos de retina que interesaban el polo posterior en ojo izquierdo y solo dos de estos en el ojo derecho que respetaban la mácula. La agudeza visual del paciente era de movimiento de manos para el ojo izquierdo y la unidad de visión para el derecho. El diagnóstico se realizó por los datos obtenidos del examen físico, ultrasonido ocular, tomografía axial computarizada de órbitas, tórax y abdomen, y biopsia por aspiración con aguja fina. Conclusiones: Se concluyó el caso con el diagnóstico de sarcoma pleomórfico de partes blandas, se le aplicó tratamiento con citostáticos y radiaciones, pero finalmente terminó con la vida del paciente.

Introduction: Since the first description in 1872 of a metastatic tumor in an eye patient with carcinoma, the literature has indicated that metastatic tumors are the most common intraocular tumor in adults. Soft tissue sarcomas are a heterogeneous group of tumors arising from the non-epithelial extraskeletal tissue of human body. Metastases to regional lymph are rare in sarcomas unlike carcinomas. Objective: To provide knowledge about the subject. Method: A literature review and comprehensive review of patient. Results: It is reported a case of a 32 years white male patient, with a history of being treated in rheumatology clinic a year ago with musculoskeletal pain that were interpreted as ankylosing spondylitis, he went to consultation referring loss of visual acuity in her left eye. At physical examination it was found multiple serous retinal detachments that interested the posterior pole in left eye and only two of these in the right eye that respected the macula. The patient's visual acuity was hand movements for the left eye vision and the vision unit to the right eye. The diagnosis was based on data obtained from physical examination, ocular ultrasound, computed tomography of the orbits, thorax and abdomen, and biopsy by fine needle aspiration. Conclusions: The diagnosis was that the patient suffered a pleomorphic soft tissue sarcoma; it was treated with cytostatics and radiation, but finally ended the patient´s life.

Sarcoma de vena cava inferior / Sarcoma of the vena cava inferior

Se presentó un caso infrecuente de una paciente con tumor abdominal y gran circulación colateral superficial tipo cava-cava, el ultrasonido abdominal, el ecocardiograma trans-torácico y transesofágico y la tomografía axial computarizada identificaron un tumor de la vena cava inferior. La citología aspirativa con aguja fina de la lesión confirmó que se trataba de un sarcoma fusopleomórfico de alto grado de malignidad primario de la vena cava inferior

Authors present an infrequent case of a female patient with abdominal tumor a great superficial collateral circulation type cava-cava, the abdominal ultrasound, the transthoracic and transesophageal echocardiogram and the computerized axial tomography (CAT) identified a tumor of the vena cava inferior. The fine-needle aspiration cytology of lesion confirmed the presence of primary fusopleomorphous sarcoma of high degree of malignancy of vena cava inferior

Pulmonary metastasectomy from soft tissue sarcomas

INTRODUCTION: Isolated pulmonary metastases from soft tissue sarcomas occur in 20-50 percent of these(the issue is about metastases, not lung cancer )patients, and 70 percent of these patients will present disease limited only to the lungs. Surgical resection is well accepted as a standard approach to treat metastases from soft tissue sarcomas isolated in the lungs, and many studies investigating this technique have reported an overall 5-year survival ranging from 30-40 percent. The most consistent predictor of survival in these patients is complete resection. The aim of the present study was to determine the demographics and clinical treatment-related variables associated with long-term (90-month) overall survival in patients with lung metastases undergoing pulmonary metastasectomy from soft tissue sarcomas. METHODS: We performed a retrospective review of patients admitted in the Thoracic Surgery Department with lung metastases who underwent thoracotomy for resection following treatment of the primary tumor. Data regarding primary tumor features, demographics, treatment, and outcome were collected. RESULTS: One hundred twenty-two thoracotomies and 273 nodules were resected from 77 patients with previously treated soft tissue sarcomas. The median follow-up time of all patients was 36.7 months (range: 10-138 months). The postoperative complication rate was 9.1 percent, and the 30-day mortality rate was 0 percent. The 90-month overall survival rate for all patients was 34.7 percent. Multivariate analysis identified the following independent prognostic factors for overall survival: the number of metastases resected, the disease-free interval, and the number of complete resections. CONCLUSION: These results confirm that lung metastasectomy is a safe and potentially curative procedure for patients with treated primary tumors. A select group of patients can achieve long-term survival after lung resection.

Estudo piloto do papel do probucol na proteção da lesão pulmonar induzida pela administração de doxorrubicina em ratos: análise comparativa entre microscopia óptica e microscopia eletrônica / Pilot study of probucol protection on pulmonary injury induced by doxorubicin in rats: comparison analysis between optic and eletronic microscopy

Objetivo: Estudar o papel do probucol na lesão pulmonar obtida pela administração de doxorrubicina em ratos. Método: foi realizado um estudo piloto experimental, onde o probucol foi testado como protetor da injúria pulmonar obtida pela administração de doxorrubicina em ratos. Resultados: Na análise comparativa dos grupos, estudados por microscopia óptica, não houve diferença significativa de critérios previamente definidos, exceto pelo edema pleural (p < 0,05). Já na microscopia eletrônica, a agressão da doxorrubicina foi identificada através da desorganização estrutural. No grupo que recebeu probucol e doxorrubicina, não foi observada a mesma desorganização (p < 0,05). Conclusões: os resultados deste estudo piloto sugerem que o probucol exerceu um efeito protetor no tecido pulmonar agredido pela doxorrubicina e que a microscopia eletrônica é mais sensível na identificação de critérios de injúria pulmonar decorrente da exposição à doxorrubicina (AU)

Objective: To study the role of probucol in the pulmonary injury caused by doxorubicin in rats. Methods: An experimental study was carried out to verify where the probucol was protective of the pulmonary injury caused by the administration of doxorubicin in rats. Results: In the comparative analysis of the groups studied by optic microscopy, it did not have significant difference in pre-definite criterions, except for pleural edema (p < 0,05). In eletronic microscopy, the aggression of the doxorubicin was indicated through the structural disorganization. In the group that received probucol and doxorubicin was not observed the same disorganization (p < 0,05). Conclusion: The results suggest that the probucol was effective in the protection of pulmonary injury caused by doxorubicin and that the eletronic microscopy is more sensitive for pre-definite criterions of pulmonary injury (AU)

Pure sarcomatous recurrence of clear cell renal carcinoma following radical nephrectomy and dendritic cell vaccination / Recorrência sarcomatosa pura após nefrectomia radical e vacina de células dendríticas no carcinoma renal de células claras

CONTEXT: Sarcomatous differentiation, which represents transformation to high-grade malignancy, can occur in all histogical types of renal malignancy. CASE REPORT: The authors report on the case of a 66-year-old woman with a right renal mass that was shown to be a clear cell carcinoma. She underwent radical nephrectomy and dendritic cell vaccination and, 3.5 years later, she developed retroperitoneal pure sarcomatous recurrence of the tumor. The authors speculate that the vaccination could have played some role in this differentiation or selection of the sarcomatous component of the primary tumor.

CONTEXTO: Diferenciação sarcomatosa, que representa evolução para malignidade de alto grau, pode ocorrer em todos os tipos histológicos de câncer renal. RELATO DE CASO: Os autores relatam o caso de uma mulher de 66 anos, com uma massa no rim esquerdo que se revelou um carcinoma de células renais. Após 3,5 anos da nefrectomia radical seguida de vacinação com células dendríticas, a paciente desenvolveu uma recorrência sarcomatosa pura no retroperitônio. Os autores especulam que a terapia com vacina de células dendríticas pode ter desempenhado algum papel na diferenciação ou seleção do componente sarcomatoso do tumor primário.

O sistema de endoprótese modular na reconstruçäo após ressecçäo ampla de neoplasias metastáticas e primárias / Modular system of endoprosthesis in reconstruction after metastatic and primary tumor ample resection

Os autores apresentam os resultados iniciais obtidos com o sistema modular de endoprótese em 17 pacientes com diagnóstico de lesoes ósseas metastáticas e primárias. O seguimento variou de três a 15 meses, com média de nove meses. Houve dois casos de luxaçao da endoprótese proximal de fêmur e dois casos de infecçao. Os resultados funcionais foram: excelente, nove; bom, cinco; regular, dois e mau, um. Apesar do curto seguimento e do pequeno número de casos, os autores consideram promissor o sistema de endoprótese, devido à maior rapidez no seu emprego, sua simplicidade e menor custo

Tratamento cirúrgico das metástases pulmonares nodulares / Surgical treatment of the nodular lung metastasis

Cinquenta e dois pacientes com metástases pulmonares nodulares de sarcomas e carcinomas foram tratados cirurgicamente e seus prontuários avaliados retrospectivamente no Serviço de Cirurgia Torácica do Instituto Nacional do Câncer entre agosto de 1979 a agosto de 1989. Entre os sarcomas observamos 23 osteossarcomas, dois fibro-histiocitomas e casos isolados de sarcoma de Ewing, neurofibrossarcoma, leiomiossarcoma, condrossarcoma e sinoviossarcoma. Nos pacientes com carcinomas observamos sete tumores testiculares, quatro tumores de mama, três tumores de cólon e reto, três tumores de pele, doi tumores de parótida e um de cada com tumor de estomago, rim e faringe. O intervalo livre de doença variou de zero a 96 meses e o tempo de duplicaçäo tumoral foi determinado em 22 pacientes variando de 16 a 107 dias. Os 52 pacientes foram submetidos a 73 operaçöes, sendo que 12 necessitaram de mais de uma cirurgia, devido a recidiva. Dentre os 462 nódulos ressecados, apenas seis pacientes com tumores testiculares näo tinha células malignas viáveis. Conclui-se que a cirurgia da metástase pulmonar é o tratamento indicado para pacientes selecionados e a presença de doença bilateral foi o único dado que comprometeu o prognóstico desses pacientes

Epithelioid sarcoma of the upper extremity with cerebral metastases.

A forty year old male patient presented with swollen, deformed right hand with multiple irregular ulcers. The axillary lymph nodes were enlarged, firm and tender. The biopsy of nodules present on the hand revealed areas of haemorrhage and necrosis. The histopathological examination confirmed the clinical diagnosis of epithelioid sarcoma. Following below-elbow amputation, patient failed to regain consciousness and expired. On autopsy, distant metastases were found in the regional lymph nodes, pleura, kidney and cerebrum.