Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium

Epithelioid angiomyolipoma is an uncommon subtype of renal angiomyolipoma associated with potentially malignant behavior and is considered a distinct entity by the World Health Organization classification of renal tumors. We present a case of an epithelioid variant of angiomyolipoma with extension into the renal vein, inferior vena cava reaching up to the right atrium. Pre-operatively, a diagnosis of renal cell carcinoma was considered based on imaging findings. Intra-operatively due to extensive adhesions, surgical resection was not performed and only tissue sampling was performed for histopathology. Microscopic examination revealed short fascicles of spindle cells and perivascular epithelioid cells. A differential diagnosis of renal cell carcinoma with sarcomatoid differentiation was considered. The immunohistochemical profile showed tumor cells that express Melan-A and smooth muscle actin, while they were negative for pan-cytokeratin, PAX8, CK7, CD117 and CD34. Therefore a diagnosis of epithelioid angiomyolipoma was rendered. The presence of intravascular thrombi on radiological investigation and carcinoma-like growth pattern on light microscopy may compound an erroneous diagnosis of renal cell carcinoma. Hence, it is prudent for the urologist to consider differential diagnosis other than renal cell carcinoma when confronted with a renal neoplasm presenting with intravascular thrombi. In these cases, a core biopsy should be planned pre-operatively and diagnosis should be made with aid of appropriate immunohistochemical markers.

Lupus miliaris disseminatus faciei

Abstract: Lupus miliaris disseminatus faciei is a rare inflammatory dermatosis of unknown etiology that primarily affects young adults. Clinically, it is characterized by an asymptomatic papular eruption mainly involving the central face, typically on and around the eyelids. Characteristic histopathological features include dermal epithelioid cell granulomas with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells. Lupus miliaris disseminatus faciei has a spontaneously resolving course, yet can be cosmetically debilitating given the location and potential for scarring. Treatment is difficult and there is a lack of controlled studies. We report a new case of lupus miliaris disseminatus faciei successfully treated with minocycline and systemic steroids, and briefly discuss its nosology and therapeutic options.

Angiomiolipoma renal: ¿es siempre un tumor benigno? / Renal angiomyolipoma: Is it always a benign tumor?

El angiomiolipoma (AML) renal es un tumor sólido compuesto por células de músculo liso, vasos sanguíneos dismórfi cos y tejido adiposo. Esta lesión ha sido considerada siempre como una neoplasia benigna. Reportamos a una paciente de 44 años, asintomática, con una lesión sugerente de AML mayor a 4 cm en el TAC que fue sometida a nefrectomía parcial abierta. La biopsia definitiva informó un angiomiolipoma con componente epiteloídeo focal (AMLE). Controles de imágenes posteriores de esta paciente no han evidenciado recidivia. El angiomiolipoma epiteloídeo (AMLE) es una variante descrita en los últimos años y que sugiere un cambio en el paradigma clásico de “benignidad” asociada al AML Las guías para el manejo de los AML no toman en cuenta la posibilidad de que se trate de un AMLE en sus recomendaciones. Existe muy poca información respecto al manejo de este tipo de lesiones, sólo hay series de casos publicadas. Faltan estudios prospectivos que otorguen herramientas para la toma de decisiones terapéuticas adecuadas en estos pacientes.

Renal angiomyolipoma (AML) is a solid tumor formed by smooth muscle cells, dimorphic blood vessels and adipose tissue. This lesion has been always considered as a benign neoplasm. We report an asymptomatic 44 year-old female patient, with a tumor suggesting an AML in a CT scan greater than 4 cms, who had an open partial nephrectomy. The biopsy report showed an AML with a focal epithelioid component. Follow-up imaging in this case has not showed any recurrence. Epithelioid angiomyolipoma (EAML) is a variant with malignant potential that must be considered when a patient with a renal AML is been evaluated. Guidelines for AML management do not take AMLE as a differential diagnosis. Few studies have been published regarding the management of this kind of lesion, only consisting of case series. There is lack of prospective studies that could give tools for the decision-making process in the treatment of these patients.

Melanoma amelanótico em cães: estudo retrospectivo de 35 casos (2004-2010) e caracterização imuno-histoquímica / Amelanotic melanoma in dogs: a retrospective study of 35 cases (2004-2010) and immunohistochemical characterization

Melanomas são tumores agressivos de melanócitos que ocorrem principalmente na cavidade oral, nas junções mucocutâneas e na pele de cães. Este tipo de neoplasma pode apresentar diversos graus de pigmentação melânica, incluindo total ausência (melanomas amelanóticos [MA]). Os arquivos de biópsia do SPV-UFRGS, que compreendem o período de 2004 a 2010, foram revisados e levantados os casos de neoplasias melanocíticas em cães. Realizou-se estudo retrospectivo de 35 casos de MA e caracterização pela imuno-histoquímica (IHQ). As principais raças acometidas foram o Poodle, Dachshund e Cocker Spaniel, mas o maior número de casos foi observado em cães sem raça definida (SRD). A idade média desses cães foi de 10,7 anos (variação de 5 a 18 anos) e não houve predileção por sexo. As principais localizações incluíram cavidade oral (57,1%) e dígitos (17,1%). Histologicamente, 40% dos MA foram classificados como epitelioides, 34,3% como mistos e 25,7% como fusiformes. Na avaliação IHQ, 86,6% dos casos foram positivos para a vimentina, 70% para a proteína S-100 e 56,6% para o melan-A. Os resultados obtidos neste trabalho possibilitam concluir que os cães com MA caracterizavam-se por serem velhos. A forma celular mais observada foi a epitelioide. Devido a pouca diferenciação desses tumores, ressalta-se a importância da realização do painel imuno-histoquímico, sobretudo da proteína S-100, que apresentou melhor marcação que o melan-A.

Melanomas are aggressive tumors of melanocytes. They are common in dogs and involve mainly the oral cavity, mucocutaneous junction, and skin. Furthermore, these tumors could be highly pigmented or lack pigment. The biopsies archives from SPV-UFRGS, 2004 to 2010, were retrieved and melanocytic neoplasms in dogs were revised. A retrospective study of 35 cases of amelanotic melanomas (AM) was performed, also immunohistochemistry (IHC) characterization was evaluated. The dogs more affected were mixed breed followed by Poodle, Dachshund and Cocker Spaniel. The average age of the dogs was 10.7 years (5-18 years in age) and there was no sex predilection. The locations of the neoplasms were the oral cavity (57.1%) and digits (17.1%). Histologically, 40% were classified as epithelioid, 34.3% mixed and 25.7% spindle. The positive immunostaining for vimentin, S-100 protein and melan-A were 86.6%, 70%, and 56.6% respectively. These results indicated the most affected dogs with AM were elderly. Epiteliod classification was the most observed histologically. It is important to perform IHC, due to lacking of differentiation of AM, mainly, anti S-100 protein that showed to be the best option of positive marker, even better to Melan-A.

Epithelial trophoblastic tumor associated with in situ squamous cell carcinoma of the cervix / Tumor trofoblástico epitelioide associado a carcinoma epidermoide in situ cervical

Epithelioid trophoblastic tumors are rare neoplasias that develop from the neoplastic transformation of chorionic-type intermediate trophoblasts. They show a wide spectrum of differential diagnoses. The authors describe a case of epithelioid trophoblastic tumor with atypical presentation in a 26-year-old patient that had recently undergone conization for in situ squamous cell carcinoma of the uterine cervix. The histopathology, immunohistochemistry and differential diagnoses of the lesion are also discussed. No similar case had been reported in the medical literature and it is an example of the hindrances to the diagnosis of epithelioid trophoblastic tumors.

Os tumores trofoblásticos epitelioides são neoplasias raras que se desenvolvem a partir da transformação neoplásica de trofoblastos intermediários coriônicos, apresentando grande espectro de diagnósticos diferenciais. Os autores descrevem um caso de tumor trofoblástico epitelioide com apresentação atípica em paciente de 26 anos de idade, que apresentava história recente de conização por carcinoma epidermoide in situ do colo uterino. Histopatologia, imuno-histoquímica e diagnósticos diferenciais da lesão também são discutidos. Nenhum caso similar foi descrito na literatura, sendo um exemplo das dificuldades encontradas para diagnosticar os tumores trofoblásticos epitelioides.

Epithelioid hemangioma of the temporal artery: a case report.

Temporal or giant cell arteritis is the most common vasculitis affecting the temporal artery. We encountered an unusual case of involvement of the temporal artery, which showed marked proliferating capillaries admixed with a dense infiltrate of eosinophils affecting all layers of the vessel. It was concluded that these changes were those of an epithelioid hemangioma of the temporal artery.

An unusual presentation of extrapulmonary tuberculosis.

A 22 years old female presented with fever, respiratory distress and a rapidly enlarging, soft left postauricular lump for last two months. She was found anaemic, had a right supraclavicular, non-tender lymph node of about 2.5 cm diameter and mild hepatosplenomegaly. She had a positive Mantoux test, and normal chest x-ray. Ultrasonography of abdomen showed multiple pre-and para-aortic enlarged lymph nodes. Mild pericardial effusion was detected on echocardiography. Fine needle aspiration cytology from the right supraclavicular lymph node showed epithelioid cell granuloma. Excision and biopsy of the dermoid were carried out. The content was pus, which was smear-negative but culture-positive for acid-fast bacilli. The patient responded to antituberculous chemotherapy satisfactorily.

Angioimmunoblastic T-cell lymphoma obscured by concomitant florid epithelioid cell granulomatous reaction--a case report.

Epithelioid cell granuloma occurs in association with many neoplasms including lymphoma. However they have rarely obscured the microscopic features of a lymphoma. We report on a case where a florid epithelioid cell granulomatous reaction caused difficulty in interpretation and delayed the final diagnosis of a case of peripheral T cell lymphoma of the angioimmunoblastic type. We draw attention to this unusual phenomenon because of its practical implications.

Lennert's Lymphoma

Lymphoepithelioid cell lymphoma Lennert's Lymphoma is a rare morphological variant of peripheral T-cell lymphoma characterized by the presence of numerous clusters of epithelioid histiocytes without formation of discrete granulomas and the intervening atypical lymphocytes. Lennert's lymphoma is often misinterpreted as granulomatous lymphadenitis or Hodgkin's disease. This report describes fine needle aspiration cytology and histological findings in a case of Lennert's lymphoma

Tumores epiteliais do ovário. Estádio clínico I: revisåo histopatológica de 100 casos / Epithelial ovarian tumours. Clinical stage I: histopathological revision of 100 cases

Foram revistos histopatologicamente 100 casos de tumores epiteliais de ovário estádio clínico I (ECI), tratados com cirurgia seguida de radioterapia ou quimioterapia. Pudemos reclassificar 88 casos, baseado na classificaçåo recomendada pela OMS e complementado por outros trabalhos, quando aquela era incompleta. Verificamos uma concordância com o diagnóstico histopatológico inicial em apenas 41 por cento. Inicialmente tínhamos 34 por cento dos casos com diagnóstico de adenocarcinoma ou carcinoma, sem outras especificaçöes, que nåo existem mais na classificaçåo supracitada. Verificamos que o diagnóstico de cistoadenocarcinoma seroso foi confirmado em 78 por cento dos casos, o de células claras em todos os dois casos avaliados, o de endometrióide em 50 por cento e o de mucinoso em apenas 25 por cento. Esta grande divergência de diagnóstico entre tumores mucinosos se deveu ao fato de que 50 por cento deles foram reclassificados como tumores "bordeline". O diagnóstico de tumor "bordeline", que havia sido feito em apenas um caso, após a revisåo apareceu em 14 casos(17,5 por cento de tumores epiteliais). Houve também quatro casos de tumores nåo epiteliais e quatro de tumores benignos. A graduaçåo histológica, que praticamente nåo havia sido feita inicialmente, demonstrou 24 por cento de tumores grauI, 47 por cento grau II e 29 por cemto grau III. Se fosse feita uma revisåo prévia, cerca de 50 por cento dos casos nåo necessitariam de tratamento complementar pós-operatório por serem tumores com grau histológico I, "bordeline" ou benigno. Por isso nós consideramos imprescindível a revisåo de lâmina por um patologista experiente. Além disso, é necessária uma cirurgia adequada com estadiamento e informaçöes completas do cirurgiåo, para um planejamento terapêutico adequado

Cytologic diagnosis of tuberculous lymphadenitis in children by fine needle aspiration.

One hundred consecutively diagnosed cases of tuberculous lymphadenitis in children have been analysed retrospectively. All cases were stained by May Grunwald Giemsa for cytomorphology and Ziehl Neelsen stain for acidfast bacilli (AFB). In 52 cases the material was sufficient and AFB cultures were possible. A diagnosis of tuberculosis was made when smears showed epithelioid cell granulomas or AFB on either smears or culture. M. tuberculosis and atypical mucobacteria were cultured in 26 and 3 cases respectively. In 6 cases the diagnosis of tuberculosis would have been missed but for culture studies, the cytologic smears were necrotic and stains for AFB negative.

Dificuldades no emprego da classificaçäo de Ridley e Jopling: uma análise morfológica / Difficulties in the use of Ridley and Jopling classification: a morphological analysis

O autor expöe as dificuldades encontradas na prática diária para o emprego da classificaçäo de Ridley e Jopling. Säo identificados os parâmetros morfológicos, cujas variaçöes permiten distinguir, do ponto de vista histológico, os tipos polares e os sub-grupos dimorfos. Deixando-se de lado as alteraçöes histológicas mais inconstantes, considera-se que esta distinçäo depende basicamente dos parâmetros: célula epitelióide, granuloma de células epitelióides, linfócitos e baciloscopia. É realizada análise crítica de cada um destes parâmetros, concluindo-se que säo escassos, e que há grande dificuldade na identificaçäo e interpretaçäo de suas variaçöes para fins de classificaçäo. Estas dificuldades säo maiores durante as fases reacionais