Sarcoma fibromixoide de baixo grau em região paratesticular: um relato de caso / Low-grade fibromyxoid sarcoma in paratesticular area: a case report

Objetivos: relatar um caso raro de sarcoma fibromixoide de baixo grau (SFMBG) em uma localização incomum de modo a reforçar aspectos histopatológicos e imunoistoquímicos relevantes para o reconhecimento desta entidade e o adequado diagnóstico diferencial de massas paratesticulares. Relato de caso: homem de 20 anos, com massa escrotal à direita, cuja análise histopatológica demonstrou a presença de tecido fibroso com áreas mixoides e predominância de células fusiformes. A imunoistoquímica foi positiva para vimentina, com índice de Ki67 de 2%, e negativa para S100, CD-34, beta-catenina, desmina e miogenina. Conclusões: caso raro de SFMBG na região paratesticular que reforça a importância da histopatologia e da imunoistoquímica no diagnóstico desse tumor. Apesar da característica histológica benigna, o SFMBG apresenta altas taxas de recorrência e metástases, sendo essencial o seguimento do paciente.

Objectives: to report a rare case of low-grade fibromyxoid sarcoma (LGFMS) in an unusual location in order to reinforce histopathological and immunohistochemical aspects relevant to the recognition of this entity and the adequate differential diagnosis of paratesticular masses. Case report: 20-year-old man, with a right scrotal mass and histopathological analysis showing the presence of fibrous tissue with myxoid areas and a predominance of spindle cells. Immunohistochemistry was positive for vimentin, with a Ki67 index of 2%, and negative for S100, CD-34, beta-catenin, desmin and myogenin. Conclusions: rare case of LGFMS in the paratesticular region that reinforces the importance of histopathology and immunohistochemistry in the diagnosis of this tumor. Despite the benign histological characteristic, LGFMS has high rates of recurrence and metastasis, and patient follow-up is essential.

Extremities Soft Tissue Sarcomas, more Common and as Dangerous as Bone Sarcomas / Sarcomas de partes moles nos membros, mais comuns e tão graves quanto os sarcomas ósseos

Abstract Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissues arcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bones arcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.

Resumo Os sarcomas musculoesqueléticos são doenças raras que exigem atenção. Frequentemente, apresentam alto grau de malignidade ao diagnóstico e se subestimados podem evoluir de forma agressiva local e sistemicamente. Apresentam-se como sarcoma de partes moles e sarcomas ósseos, sendo os de partes moles quatro a cinco vezes mais comuns. A maioria dos sarcomas de partes moles ocorre nos membros. Os subtipos mais comuns nas crianças e adolescentes são o rabdomiossarcoma e o sarcoma sinovial, nos adultos o sarcoma pleomórfico indiferenciado, lipossarcoma, leiomiossarcoma, mixofibrossarcoma e sarcoma sinovial; todos de alto grau de malignidade histológica. Muitos sarcomas de partes moles são confundidos com tumores benignos de partes moles, 100 vezes mais comuns, por isso são ressecados sem o planejamento necessário, acarretando em amputação de um membro que poderia ter sido preservado. Como em todos os cânceres, o fator prognóstico mais importante é a doença metastática. Na sua vigência, a taxa de sobrevida global cai em torno de 20 a 30%. As taxas de sobrevida no geral são parecidas entre os sarcomas ósseos e de partes moles, portanto o sarcoma de partes moles, além de mais prevalente, mostra-se tão agressivo quanto os sarcomas ósseos, por isso merece muita atenção dos ortopedistas que frequentemente são a primeira linha de atendimento dos portadores destes tumores.

Malignant uterine neoplasms attended at a brazilian regional hospital: 16-years profile and time elapsed for diagnosis and treatment / Neoplasias malignas do útero atendidas em hospital regional brasileiro: Perfil em 16 Anos e tempo dispendido até diagnóstico e tratamento

Abstract Objective The present study aims to evaluate the profile of endometrial carcinomas and uterine sarcomas attended in a Brazilian cancer center in the period from 2001 to 2016 and to analyze the impact of time elapsed fromsymptoms to diagnoses or treatment in cancer stage and survival. Methods This observational study with 1,190 cases evaluated the year of diagnosis, age-group, cancer stage and histological type. A subgroup of 185 women with endometrioid histology attended in the period from 2012 to 2017 was selected to assess information about initial symptoms, diagnosticmethods, overall survival, and to evaluate the influence of the time elapsed from symptoms to diagnosis and treatment on staging and survival. The statistics used were descriptive, trend test, and the Kaplan- Meier method, with p-values < 0.05 for significance. Results A total of 1,068 (89.7%) carcinomas (77.2% endometrioid and 22.8% nonendometrioid) and 122 (10.3%) sarcomas were analyzed, with an increasing trend in the period (p < 0.05). Histologies of non-endometrioid carcinomas, G3 endometrioid, and carcinosarcomas constituted 30% of the cases. Non-endometrioid carcinomas and sarcomas weremore frequently diagnosed in patients over 70 years of age and those on stage IV (p < 0.05). The endometrioid subgroup with 185 women reported 92% of abnormal uterine bleeding and 43% diagnosis after curettage. The average time elapsed between symptoms to diagnosis was 244 days, and between symptoms to treatment was 376 days, all without association with staging (p = 0.976) and survival (p = 0.160). Only 12% of the patients started treatment up to 60 days after diagnosis. Conclusion The number of uterine carcinoma and sarcoma cases increased over the period of 2001 to 2016. Aggressive histology comprised 30% of the patients and, for endometrioid carcinomas, the time elapsed between symptoms and diagnosis or treatment was long, although without association with staging or survival.

Resumo Objetivo O presente estudo avaliou o perfil dos carcinomas endometriais e sarcomas uterinos atendidos em um centro brasileiro de câncer no período de 2001 a 2016, e avaliou o impacto do tempo decorrido entre os sintomas até o diagnóstico ou tratamento no estadiamento e sobrevida pelo câncer. Métodos Estudo observacional com 1.190 casos que analisou o ano do diagnóstico, faixa etária, estágio e tipo histológico do câncer. Um subgrupo de 185 mulheres com histologia endometrioide e atendidas no período de 2012 a 2017 foi selecionado para avaliar informações sobre sintomas iniciais, métodos de diagnóstico, sobrevida global e para analisar a relação entre o tempo decorrido a partir dos sintomas até o diagnóstico e tratamento no estadiamento e sobrevida. Foram realizadas análises estatísticas descritiva, de tendência linear e de sobrevida pelo método de Kaplan-Meier, com valores de p < 0,05 para significância. Resultados Os casos estudados de acordo com a histologia foram 1.068 (89,7%) carcinomas (77,2% endometrioides e 22,8% não endometrioides) e 122 (10,3%) sarcomas, com tendência crescente no período (p < 0,05). Histologias de carcinomas não endometrioides, G3 endometrioides e carcinossarcomas consistiram em 30% dos casos. Carcinomas não endometrioides e sarcomas forammais frequentemente diagnosticados em pacientes acima de 70 anos de idade e em estágio IV (p < 0,05). O subgrupo com185 mulheres com carcinoma endometrioide apresentou 92% de sangramento uterino anormal e 43% de diagnóstico após curetagem. O tempo médio decorrido entre os sintomas e o diagnóstico foi de 244 dias e entre os sintomas e o tratamento, 376 dias, todos sem associação com estadiamento (p = 0,976) e sobrevida (p = 0,160). Apenas 12% das pacientes iniciaram o tratamento em até 60 dias após o diagnóstico. Conclusão O número de casos de carcinomas e sarcomas uterinos aumentaram no período de 2001 a 2016. A histologia agressiva compreendeu 30% dos pacientes e, no caso dos carcinomas endometrioides, o tempo decorrido entre os sintomas e o diagnóstico ou tratamento foi longo, embora sem associação com estadiamento ou sobrevida.

Adult primary liver sarcoma: systematic review / Sarcoma primário de fígado em adultos: revisão sistemática

ABSTRACT Introduction: primary liver sarcoma is a rare type of tumor, more common in children. Among adults, it represents a spectrum of neoplasms with reserved prognosis. There is no consensus on the treatment of choice of these lesions, justifying a systematic review of the literature on treatment options, prognostic factors, and survival. Material/Methods: a systematic review of articles published in Pubmed, Medline, LiLacs e SciElo, from 1966 to March/2019, presenting the keywords: primary-liver-sarcoma and primary-hepatic-sarcoma was undertaken. Studies including patients older than 18 years, and published in English, Portuguese and Spanish were included. Case reports, metastatic tumors and multiple oncologic diagnosis were excluded. The initial search listed 1,318 articles. 1,206 did not meet the inclusion criteria. After reviewing 112 eligible articles, 15 were selected (14 case series and 1 retrospective-cohort). Results: proposed treatment modalities for primary liver sarcoma included surgery and/or chemotherapy and/or radiotherapy or liver transplantation. The most common histological types were angiosarcoma (32%), leiomyosarcoma (29%), epithelioid hemangioendothelioma (15%) and embryonal sarcoma (7%). Histology, degree of differentiation and R0 resection were mentioned positive prognostic factors. Median survival ranged from two to 23 months. Five-year survival rate varied from 0% to 64%, on average 21%. Conclusion: surgical resection (R0 resection) is the main treatment for primary liver sarcomas. Development of effective systemic therapies are required to improve prognosis of patients harboring this type of tumor.

RESUMO Introdução: o sarcoma primário do fígado é uma neoplasia rara, que acomete mais frequentemente crianças. Nos adultos, representa espectro de neoplasias de prognóstico reservado. Entretanto, em nenhuma faixa etária há consenso sobre a terapêutica de escolha dessas lesões, motivando revisão sistemática com objetivo de elencar opções de tratamento, fatores prognósticos e sobrevida. Material/Método: realizamos revisão sistemática dos artigos publicados nas bases de dados Pubmed, Medline, LiLacs e Scielo, de 1966 a março/2019, contendo as palavras-chaves: primary-liver-sarcoma e primary-hepatic-sarcoma. Foram incluídos estudos que incluíram pacientes com idade a partir de 18 anos e publicados em inglês, português e espanhol. Relatos de caso, tumores metastáticos e pacientes com múltiplos diagnósticos oncológicos foram excluídos. Foram encontrados inicialmente 1.318 artigos, desses, 1.206 foram excluídos por estarem fora dos critérios de inclusão. Dos 112 artigos analisados e discutidos, 15 foram incluídos nesse artigo (14 séries de casos e 1 estudo de coorte retrospectivo). Resultado: os tratamentos propostos para o sarcoma primário do fígado em adultos incluíram cirurgia e/ou quimioterapia e/ou radioterapia ou transplante hepático. Os tipos histológicos mais frequentemente relados foram angiossarcoma (32%), leiomiossarcoma (29%), hemangioendotelioma epitelioide (15%) e sarcoma embrionário (7%). Tipo histológico, grau de diferenciação e resseção R0 foram os principais fatores de bom prognóstico. A sobrevida média total variou de dois a 23 meses. A sobrevida em cinco anos variou entre 0 e 64%, em média 21%. Conclusão: ressecção cirúrgica R0 é a base do tratamento dos sarcomas primários do fígado. Esse grupo heterogêneo de tumores requer desenvolvimento de terapias sistêmicas efetivas para melhoria do prognóstico.

Disponibilidad de técnicas moleculares para niños con tumores de difícil caracterización. Un problema en la Oncopatología pediátrica. Aplicación y difusión de técnicas de diagnóstico complementarias en el ámbito público / Availability of molecular techniques for children with difficult-to-characterize tumors. A problem in pediatric oncopathology. Application and diffusion of complementary diagnostic techniques in the public setting

La revolución de la biología molecular y el desarrollo de la investigación biomédica básica para el diagnóstico y posterior manejo del cáncer infantil han llevado a la necesidad de organización de grupos interdisciplinarios de profesionales, los cuales se encargan de afrontar los nuevos desafíos diagnósticos y terapéuticos. Los sarcomas indiferenciados pediátricos constituyen un grupo heterogéneo de neoplasias malignas de aspecto primitivo y polifenotípico. La categorización de gran parte de este tipo de tumores es posible gracias a la aplicación de técnicas moleculares complementarias al estudio histopatológico. El objetivo del presente estudio fue recategorizar sarcomas indiferenciados mediante la implementación de una nueva metodología diagnóstica. Se efectuaron técnicas de inmunohistoquimica (IHQ), FISH de interfase y RT-PCR a partir de tejido fijado en formol e incluido en parafina en 144 casos de sarcomas indiferenciados. Se logró la recategorización del 95.1% de los casos, arribando a 24 diagnósticos diferentes. Sólo un 4.9% permanece aún como sarcoma indiferenciado o inclasificable. Los resultados alcanzados por este estudio demuestran la importancia de contar con nuevas herramientas diagnósticas a nivel molecular y recursos humanos especializados que posibiliten su correcta implementación para el diagnóstico de neoplasias de difícil caracterización (AU)

The revolution of molecular biology and the development of basic medical research for the diagnosis and subsequent management of childhood cancer have led to a need to organize interdisciplinary groups of professionals in charge of facing new diagnostic and treatment challenges. Childhood undifferentiated sarcomas are a heterogeneous group of malignant neoplasms that are primitive in appearance and have polyphenotypic features. Categorization of a large part of this type of tumor has become possible with molecular techniques as a complement to histopathological studies. The aim of this study was to categorize undifferentiated sarcomas using new diagnostic tools. Immunohistochemistry (IHC), interfase FISH, and RT-PCR techniques were used on formalin-fixed and paraffin-embedded tissues of 144 cases of undifferentiated sarcomas. Overall, 95.1% of the cases could be recategorized resulting in 24 different diagnoses. In only 4.9% the diagnosis of undifferentiated or unclassifiable sarcoma was maintained. These results emphasize the importance of the availability of new diagnostic tools at the molecular level and specialized human resources enabling adequate implementation for the diagnosis of difficult-to-characterize neoplasms (AU)

Leiomiosarcoma uterino de alto grado / High grade uterine leiomyosarcoma

Los sarcomas de útero son un grupo diverso de tumores que constituyen 1 por ciento de las neoplasias del aparato genital femenino. La incidencia mundial es de 0,5 a 3,3 casos por cada 100,000 mujeres por año. Se presenta un caso poco frecuente con diagnóstico de leiomiosarcoma uterino de alto grado y se hace una revisión de la literatura sobre este tema(AU)

Uterine sarcomas are a diverse group of tumors that make up 1 percent of the neoplasms of the female genital tract. The worldwide incidence is from 0.5 to 3.3 cases per 100,000 women per year. A rare case with a diagnosis of high-grade uterine leiomyosarcoma is presented and a review of the literature on this subject is made(AU)

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.

Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment / Hemipelvectomia com amputação laparoscópica do reto para tratamento de sarcoma epitelioide

Abstract The epithelioid sarcoma (ES) is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the "proximal-type" in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with "proximal-type" ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The "proximal-type" ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task.

Resumo O sarcoma epitelióide (SE) é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como "tipo proximal". São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com amputação abdominoperineal do reto por videolaparoscopia associada a hemipelvectomia parcial esquerda incluindo o membro inferior esquerdo (MIE). O paciente apresentou boa evolução pós operatória e encontra-se em segmento ambulatorial. O anatomopatológico evidenciou margens cirúrgicas livres.O SE proximal é um tipo raro de sarcoma de partes moles, cujo tratamento curativo é desafiador, em virtude dos altos índices de recidiva local e metástases à distância.

Registro Argentino de sacomas de partes blandas del adulto. Análisis retrospectivos de los datos obtenidos durante dos años en una institución pública y tres instituciones privadas / Argentine Registry of soft tissue sacomas of the adult. Retrospective analysis of the data obtained during two years in a public institution and three private institutions

Se trata de un análisis retrospectivo de los datos obtenidos durante dos años en una institución pública y tres privadas. Los sarcomas de partes blandas constituyen un grupo infrecuente y heterogéneo de tumores. Los registros de esta enfermedad son excepcionales y, en nuestro medio, solamente contamos con datos de instituciones y en general de carácter retrospectivo. El objetivo del estudio fue evaluar en forma multicéntrica las características de los pacientes con diagnóstico histológico de sarcomas de partes blandas en centros de la República Argentina. Fueron evaluadas 458 historias clínicas de cuatro centros especializados en sarcomas en el período 2012-2013. Se analizaron características demográficas de los pacientes así como variables histológicas y clínicas, y se utilizó estadística descriptiva. La distribución de la enfermedad fue mayor en el sexo femenino y la mediana de edad fue de 51 años. Más del 40% se localizó en extremidades y la estadificación evidenció predominio de estadio III. La histología más frecuente fue la de sarcoma pleomórfico y no especificados (NOS), seguido por el leiomiosarcoma. Alrededor del 20% de los casos se presentaron con enfermedad metastásica al diagnóstico. No hubo diferencias estadísticamente significativas entre las características analizadas de los pacientes de hospitales públicos y centros privados. El presente análisis constituye la primera iniciativa de una evaluación cooperativa de los pacientes con sarcomas de partes blandas de Argentina. A pesar del carácter retrospectivo, consideramos estos datos científicamente valiosos y permitirán construir un modelo de evaluación local tanto diagnóstico como de eficacia de los tratamientos realizados (AU)

This is a retrospective analysis of the data obtained during two years in a public institution and three private ones. Soft-tissue sarcomas constitute an uncommon and heterogeneous group of tumors. The records of this disease are exceptional and, in our environment, we only have data from institutions and in general retrospective. The objective of the study was to evaluate in a multicenter way the characteristics of the patients with histological diagnosis of soft tissue sarcomas in centers of the Argentine Republic. A total of 458 clinical histories of four centers specialized in sarcomas were evaluated in the period 2012-2013. Demographic characteristics of the patients as well as histological and clinical variables were analyzed, and descriptive statistics were used. The distribution of the disease was greater in the female sex and the median age was 51 years. More than 40% were localized in extremities and staging showed a predominance of stage III. The most frequent histology was that of pleomorphic sarcoma and NOS, followed by leiomyosarcoma. About 20% of the cases presented with metastatic disease at diagnosis. There were no statistically significant differences between the analyzed characteristics of patients from public hospitals and private centers. The present analysis constitutes the first initiative of a cooperative evaluation of patients with soft tissue sarcomas in Argentina. Despite the retrospective nature, we consider these data to be scientifically valuable and allow us to construct a model of local assessment, both for diagnosis and for the efficacy of the treatments performed (AU)

Liposarcoma: A Rare Case Report with Review of Literature.

Liposarcoma is one of the most common malignant mesenchymal neoplasms, comprising approximately 15% of all soft-tissue sarcomas. First described by “Virchow” in 1857, it has been extensively reported in the literature, although its incidence remains exceedingly rare in the head and neck region with an annual incidence estimated to be 2.5/1 million inhabitants in population-based studies. It is one of the most common malignant mesenchymal neoplasms, comprising approximately 15% of all soft-tissue sarcomas. It is a heterogeneous disease with distinct sub-entities presenting with differential clinical behavior. The purpose of this article is to report an additional case of liposarcoma of the buccal vestibule and to review the literature.

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin / Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case

Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

Histologic Variability and Diverse Oncologic Outcomes of Prostate Sarcomas

PURPOSE: Primary prostate sarcomas are a rare type of prostate cancer that account for less than 0.1% of primary prostate malignancies. We analyzed the experience of a single institution with prostate sarcoma over 20 years. MATERIALS AND METHODS: In this case series, the medical records of 20 patients with prostate sarcoma were reviewed from June 1990 to December 2013 to identify symptoms at presentation, diagnostic procedures, metastasis presence and development, histologic subtype, French Federation Nationale des Centres de Lutte Contre le Cancer grade, primary tumor grade and size, and treatment sequence, including surgery and preoperative and postoperative therapies. The average follow-up period was 23.6 months (range, 1.4-83.3 months). RESULTS: The average patient age was 46.3+/-16.7 years. Most patients presented with lower urinary tract symptoms (55%). The histologic subtype was spindle cell sarcoma in five patients (25%), rhabdomyosarcoma in three patients (15%), synovial sarcoma in three patients (15%), liposarcoma in three patients (15%), stromal sarcoma in three patients (15%), and Ewing sarcoma, nerve sheath tumor, and adenocarcinoma with sarcomatoid component (5% each). For liposarcoma, two patients were alive after complete surgical resection and had a good prognosis. At last follow-up, 15 patients had died of sarcoma. The 2- and 5-year actuarial survival rates for all 20 patients were 53% and 12%, respectively (medial survival, 20 months). CONCLUSIONS: The disease-specific survival rate of prostate sarcoma is poor. However, sarcoma that is detected early shows a better result with proper management including surgical intervention with radio-chemotherapy than with no treatment. Early diagnosis and complete surgical resection offer patients the best curative chance.

A Case of Low-grade Fibromyxoid Sarcoma of the Colon / 대한소화기학회지

Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm that shows benign histologic features but may have clinical course of malignant disease. It has been reported to occur in the thigh, inguinal area, axilla, shoulder, neck, perineum or buttock. However, there have been few cases of abdominal organ involvement. A 21-year-old woman presented with a large palpable abdominal mass. A 7x4 cm sized round soft tissue tumor at right upper quadrant area was identified by abdominopelvic CT scan. Percutaneous ultrasound-guided biopsy revealed features of spindle cell tumor. On exploration, the tumor originated from transvers colon and was attached to gastrocolic ligament, transverse mesocolon and stomach. The tumor could be dissected with transverse colectomy and partial gastrectomy. The excised tumor, measuring 7x5x5 cm, was well demarcated and appeared as an ovoid mass with firm and myxoid cut surface. She was diagnosed with low-grade fibromyxoid sarcoma arising from transverse colon, and is currently being followed-up without recurrence or metastasis.

Two Different Surgical Approaches for Prostatic Stromal Sarcoma: Robot-Assisted Laparoscopic Radical Prostatectomy and Open Radical Cysto-Prostatectomy With Ileal Conduit

Stromal sarcoma of the prostate is very rare and shows rapid growth, which consequently is related to poor prognosis. Recently, we treated two cases of prostatic stromal sarcoma: one with robot-assisted laparoscopic radical prostatectomy and the other with open radical cysto-prostatectomy with an ileal conduit. To the best of our knowledge, this is the first case report of a prostatic stromal sarcoma managed by use of a robotic procedure. Here, we report of our experiences in the treatment of prostatic stromal sarcoma by use of two different methods.

Two Different Surgical Approaches for Prostatic Stromal Sarcoma: Robot-Assisted Laparoscopic Radical Prostatectomy and Open Radical Cysto-Prostatectomy With Ileal Conduit

Stromal sarcoma of the prostate is very rare and shows rapid growth, which consequently is related to poor prognosis. Recently, we treated two cases of prostatic stromal sarcoma: one with robot-assisted laparoscopic radical prostatectomy and the other with open radical cysto-prostatectomy with an ileal conduit. To the best of our knowledge, this is the first case report of a prostatic stromal sarcoma managed by use of a robotic procedure. Here, we report of our experiences in the treatment of prostatic stromal sarcoma by use of two different methods.

Indicações e limites da imunoistoquímica no câncer de cabeça e pescoço / Indications and pitfalls of immunohistochemistry in head and neck cancer

O estudo imunoistoquímico tem sido empregado para a avaliação do diagnóstico diferencial de neoplasia. OBJETIVO: Avaliar o uso do método nos casos de câncer de cabeça e pescoço. MÉTODO: Estudo retrospectivo de casos do Registro Hospitalar de Câncer da instituição. RESULTADOS: De 704 resultados anatomopatológicos, a imunoistoquímica foi realizada em 76 (11%). A maioria correspondeu a carcinomas - 85,80% e, destes, 83,66% eram epidermoides. Todos os exames foram para fins diagnósticos. Houve maior frequência para o uso de 34BE12 (37,18%), AE1/AE3 (35,90%), 35BH11 (28,21%), CD45 (25,64%), CD20 (24,36%), CD30 (24,36%), CK7 (23,08%) e CD3 (23,08%). CONCLUSÃO: A imunoistoquímica foi usada em 10,67% dos casos de câncer de cabeça e pescoço submetidos a exame anatomopatológico, sendo maior para os carcinomas -- 5,26%. Na determinação do carcinoma epidermoide, seu uso foi de 18,42% do total de neoplasias.

Immunohistochemistry (IHC) has been employed in the differential diagnosis of tumors. OBJECTIVE: To assess the use of IHC in cases of head and neck tumor. METHOD: This is a retrospective study of the cases included in the Cancer Registry of the institution. RESULTS: IHC was used in 76 (11%) of 704 pathology tests. Most cases were carcinomas (85.80%), and 83.66% of them were squamous cell carcinomas. All tests were done with diagnostic purposes. The most frequently used antibodies were 34BE12 (37.18%), AE1/AE3 (35.9%), 35BH11 (28.21%), CD45 (25.64%), CD20 (24.36%), CD30 (24.36%), CK7 (23.08%) and CD3 (23.08%). CONCLUSIONS: IHC was used in 10.67% of the head and neck tumor cases submitted to pathology testing, mostly for carcinoma (5.26%). In the determination of squamous cell carcinoma, IHC accounted for 18.42% of all tumors.

Primary extragastrointestinal stromal tumors: A clinicopathological and immunohistochemical study-A tertiary care center experience.

Background: Extra gastrointestinal stromal tumors (EGIST) are uncommon compared to their gastrointestinal counterparts. EGISTs involve omentum, mesentery, retroperitoneum, pancreas, and pelvis. Materials and Methods: Ten EGISTs were analyzed in this study from January 1995 to November 2011. They were analyzed with respect to clinical features, imageological, histopathological, and immunohistochemical findings. The immunohistochemical stains used were Smooth muscle actin (SMA), Desmin, S-100 protein, CD34 and CD-117. Results: There was slight female preponderance with wide age range. Four of the tumors were in retroperitoneum, three in mesentery, and two in omentum and one in pelvis. Histopathologically majority were spindle cell tumors. Immunohistochemically CD117 was consistently positive followed by CD34. Smooth muscle actin was positive in eight cases, S-100 protein and desmin were positive in two cases each. Conclusion: EGISTs are rare and should be considered in the differential diagnosis of the mesenchymal tumors and immunohistochemistry helps to confirm the diagnosis. Further study with better follow-up is desired to characterize these uncommon tumors.

Anaplastic solitary plasmacytoma of mandible, masquerading as sarcoma

Plasma cell neoplasm is characterized by a monoclonal neoplastic proliferation of plasma cells and solitary plasmocytoma of bone [SPB] is a localized form. It usually occurs in vertebrae and secondarily in long bones. Its presence in mandible is extremely rare event. A 48-year-old man consulted to our clinic with a chief complaint of pain in his mandible. Radiography revealed a destructive lesion in body and ramus. The initial pathologic evaluation revealed a high grade pleomorphic neoplasm. The diagnosis was confirmed by immunohistochemical markers. Overall, plasmacytoma with anaplastic features can be confused with high grade sarcoma clinically and histologically