Multicentric osteoblastoma-like: report of a rare case in the craniofacial region / Osteoblastoma-like multicêntrico: relato de um caso raro na região craniofacial

ABSTRACT The osteoblastoma-like tumor is a rare condition with limited information about its treatment in the current medical literature. The tumor histologically resembles osteoblastoma, although the imaging features are similar to those seen in primary vascular lesions. Due to the uncertainty in the biological behavior of this tumor and because it is an unusual diagnosis, treatment can be aggressive, such as amputation, en bloc resection, and/or chemotherapy. This work reports a rare case of a patient with multicentric osteoblastoma-like in the craniofacial region, treated aggressively with total resection of the lesions.

RESUMEN El tipo osteoblastoma es una afección poco común y la literatura médica actual tiene información limitada sobre su tratamiento. Es histológicamente similar al osteoblastoma, aunque las características de las imágenes son similares a las que se observan en las lesiones vasculares primarias. Por la incertidumbre de su comportamiento biológico y por tratarse de un diagnóstico poco habitual, el tratamiento puede ser agresivo, con amputación, resección en bloque y/o quimioterapia. Este trabajo reporta un caso raro de osteoblastoma multicéntrico en la región craneofacial, tratado de manera agresiva con resección total de las lesiones.

RESUMO O osteoblastoma-like é uma condição rara, e a literatura médica atual tem informações limitadas sobre seu tratamento. Ele se assemelha histologicamente ao osteoblastoma, embora as características imaginológicas sejam semelhantes às observadas nas lesões vasculares primárias. Devido à incerteza do seu comportamento biológico e por se tratar de um diagnóstico incomum, o tratamento pode ser agressivo, com amputação, ressecção em bloco e/ou quimioterapia. Este trabalho relata um caso raro de osteoblastoma-like multicêntrico em região craniofacial, tratado de forma agressiva com ressecção total das lesões.

Sclerosing variant of well-differentiated testicular liposarcoma: a case report / Lipossarcoma testicular bem diferenciado com variante esclerosante: um relato de caso

ABSTRACT Testicular cancers are classified in germ cell and non-germ cell tumors, as well as, liposarcomas. We report the case of a patient with a large testicular liposarcoma, submitted to surgical treatment with excision of scrotal pouch and segment of the spermatic cord, and the left testicle, showing a good evolution. This report presents one of the first cases of a sclerosing variant of well-differentiated testicular liposarcoma, large in size and with no association with another cancer. Due to their location, the diagnosis is difficult and unusual. Complete tumor resection and regular medical follow-up show a good prognosis, less recurrence, and little cellular differentiation.

RESUMEN Los cánceres de testículo se clasifican en tumores de células germinales y células no germinales, así como en liposarcomas. Presentamos el caso de un paciente con un gran liposarcoma testicular, sometido a tratamiento quirúrgico con exéresis de la bolsa escrotal y segmento de cordón espermático y testículo izquierdo, con buena evolución. Este informe presenta uno de los primeros casos de una variante esclerosante de liposarcoma testicular bien diferenciado, de gran tamaño y sin asociación con otro cáncer. Debido a su ubicación, el diagnóstico es difícil e inusual. La resección completa del tumor y el seguimiento médico regular muestran un buen pronóstico, menor recidiva, y poca diferenciación celular.

RESUMO Os tumores testiculares são classificados em células germinativas e não germinativas, assim como os lipossarcomas. Relatamos o caso de um paciente com lipossarcoma testicular de grande dimensão submetido a tratamentos cirúrgicos com ressecção de bolsa escrotal e segmento do cordão espermático e do testículo esquerdo, apresentando boa evolução do quadro. O relato traz um dos primeiros casos de lipossarcoma testicular bem diferenciado com variante esclerosante, de grande dimensão e sem associação a outra neoplasia. Devido à localização, apresenta diagnóstico difícil e pouco habitual. Com a ressecção total do tumor e o acompanhamento médico regular, o paciente apresenta bom prognóstico, menor recidiva e pouca diferenciação celular.

Nodular fasciitis: case report / Fasciíte nodular: relato de caso

ABSTRACT Nodular fasciitis (NF) is a rare fibroblast proliferation of unknown etiology, with benign, rapid clonal growth, from a superficial fascia to the subcutaneous tissue or an adjacent muscular layer. Also known as pseudosarcomatous fasciitis, this clinical syndrome is characterized as a solitary mass of hardened consistency, painless palpation and no gender preference. The definitive diagnosis is made by immunohistochemistry and surgery is the treatment of choice.

RESUMEN La fascitis nodular (FN) es una lesión proliferativa fibroblástica rara, de etiología desconocida, naturaleza benigna, crecimiento rápido y probablemente clonal, que se origina en una fascia superficial hacia el tejido subcutáneo o una camada muscular adyacente. También conocida como fascitis pseudosarcomatosa, este síndrome clínico se caracteriza por un tumor solitario de consistencia endurecida, ligero dolor a la palpación y sin predilección por sexo. Su diagnóstico definitivo se hace por inmunohistoquímica, y el tratamiento de elección es el quirúrgico.

RESUMO A fasciíte nodular (FN) é uma lesão proliferativa fibroblástica rara, de etiologia desconhecida, caráter benigno, crescimento rápido e provavelmente clonal, que se origina a partir de uma fáscia superficial para o tecido subcutâneo ou uma camada muscular adjacente. Também conhecida como fasciíte pseudossarcomatosa, esta síndrome clínica caracteriza-se por uma massa solitária de consistência endurecida, pouco dolorosa à palpação e sem predileção por gênero. O diagnóstico definitivo é feito por imuno-histoquímica, e o tratamento de escolha é o cirúrgico.

Scanning electron microscopy of granuloma annulare

Abstract: Light microscopy of granuloma annulare shows mucin deposition with lympho-histiocytic infiltrate. We describe the ultrastructural three-dimensional aspects of a typical case of granuloma with characteristic histopathological findings. At the ultrastructural level, affected collagen bundles and granular mucin deposition were observed. Round cells corresponding to lymphocytes were identified. Bigger oval cells, corresponding to isolated or palisading histiocytes were also found. The ultrastructural aspects overlap with light microscopy and contribute to its iconographic documentation.

Scanning electron microscopy of granuloma annulare.

Light microscopy of granuloma annulare shows mucin deposition with lympho-histiocytic infiltrate. We describe the ultrastructural three-dimensional aspects of a typical case of granuloma with characteristic histopathological findings. At the ultrastructural level, affected collagen bundles and granular mucin deposition were observed. Round cells corresponding to lymphocytes were identified. Bigger oval cells, corresponding to isolated or palisading histiocytes were also found. The ultrastructural aspects overlap with light microscopy and contribute to its iconographic documentation.

Scanning electron microscopy of lichen sclerosus.

Lichen sclerosus is an acquired inflammatory condition characterized by whitish fibrotic plaques, with a predilection for the genital skin. We performed scanning electron microscopy of the dermis from a lesion of lichen sclerosus. Normal collagen fibers could be easily found in deeper layers of the specimen, as well as the transition to pathologic area, which seems homogenized. With higher magnifications in this transitional area collagen fibers are adherent to each other, and with very high magnifications a pearl chain aspect became evident along the collagen fibers. In the superficial dermis this homogenization is even more evident, collagen fibers are packed together and round structures are also observed. Rupture of collagen fibers and inflammatory cells were not found. These autoimmune changes of the extracellular matrix lead to the aggregation of immune complexes and/or changed matrix proteins along the collagen fibers, the reason why they seem hyalinized when examined by light microscopy.

Tuberculose exclusivamente colônica em paciente com HIV / Colonic tuberculosis in patient with HIV

A coinfecção pelo vírus da imunodeficiência humana (HIV) e pelo Mycobacterium tuberculosis pode elevar em 25 vezes o risco de desenvolver a tuberculose-doença. Mesmo assim, o comprometimento do cólon pela tuberculose é extremamente incomum. Relatamos um caso de paciente HIV positivo com tuberculose colônica.

Co-infection with the human immunodeficiency virus (HIV) and Mycobacterium tuberculosis may increase by 25-fold the risk of developing tuberculosis. Nevertheless, commitment of the colon by tuberculosis is extremely unusual. Here we report the case of a HIV-positive patient with colon tuberculosis.

Actinomicose ovariana em paciente não usuária de dispositivo intrauterino / Ovarian Actinomycosis in a non-IUD user

Neste relato é descrito caso de Actinomicose ovariana, estendendo-se ao epíploon, em mulher de 49 anos não usuária de DIU, com dor abdominal, febre, diarreia, massa abdominal palpável e irritação peritoneal, que foi submetida à laparotomia exploradora, seguida de ooferectomia unilateral, devido a abscesso tubo ovariano esquerdo.

Here we report the case of ovarian Actinomycosis extending to the omentum, in a non-IUD user, a 49-year-old woman with abdominal pain, fever, diarrhea, palpable abdominal mass and peritoneal irritation, who underwent laparotomy followed by unilateral oophorectomy due to left tube ovarian abscess.

Tuberculose cutânea em paciente sem infecção pelo HIV / Cutaneous tuberculosis in patients without HIV infection

Trata-se de um caso de tuberculose cutânea escrofulodérmica, secundária a foco tuberculoso pulmonar. Paciente de 40 anos, masculino, que apresentava duas fístulas externas em região perineal direita. O exame anátomo-patológico de biópsia da lesão demonstrou granulomas tuberculoides cutâneos com necrose caseosa, com pesquisa para bacilos álcool-ácido-resistentes positiva. Baciloscopia direta do escarro e raio X de tórax mostraram o pulmão como sítio primário. Tratamento específico para tuberculose determinou regressão completa das lesões cutâneas.

A case of scrofulodermal tuberculosis secondary to a pulmonary tuberculosis focus. Forty-year-old male patient who had two external fi stulas in the right perineal area. The pathological examination of biopsy showed cutaneous tuberculoid granulomas with caseous necrosis, which tested positive for acid-fast bacilli. Direct sputum smear microscopy and chest X-ray showed the lung as the primary site. Specifi c treatment for tuberculosis determined complete regression of skin lesions.

Scanning electron microscopy of lichen sclerosus / Microscopia eletrônica de varredura do líquen escleroso

Lichen sclerosus is an acquired inflammatory condition characterized by whitish fibrotic plaques, with a predilection for the genital skin. We performed scanning electron microscopy of the dermis from a lesion of lichen sclerosus. Normal collagen fibers could be easily found in deeper layers of the specimen, as well as the transition to pathologic area, which seems homogenized. With higher magnifications in this transitional area collagen fibers are adherent to each other, and with very high magnifications a pearl chain aspect became evident along the collagen fibers. In the superficial dermis this homogenization is even more evident, collagen fibers are packed together and round structures are also observed. Rupture of collagen fibers and inflammatory cells were not found. These autoimmune changes of the extracellular matrix lead to the aggregation of immune complexes and/or changed matrix proteins along the collagen fibers, the reason why they seem hyalinized when examined by light microscopy.

O líquen escleroso é uma afecção inflamatória caracterizada por placas esbranquiçadas fibróticas ocorrendo preferentemente na pele genital. Realizamos exame com microscópio eletrônico de varredura da derme de uma lesão de líquen escleroso. Ao exame as fibras colágenas normais puderam ser facilmente identificadas, bem como a transição para a área alterada, a qual aparece homogeneizada. Nessa área as fibras parecem aderidas umas às outras e com aspecto em colar de pérolas. Na derme superficial essa homogeneização é ainda mais evidente, com as fibras bem aderidas e ainda com as estruturas arredondadas. Ruptura das fibras e células inflamatórias não foram observadas. Os fenômenos autoimunes que ocorrem na matriz extracelular nessa enfermidade devem levar à agregação de imunocomplexos e/ou proteínas alteradas nas fibras colágenas, por essa razão elas aparecem hialinizadas na microscopia óptica.

Intratesticular and intraovarian epidermoid inclusion cysts: report of two cases / Cistos de inclusão epidérmica intratesticular e intraovariano: relato de dois casos

Two rare cases of intragonadal epidermoid inclusion cysts are described. Their etiology remains controversial and a possible hypothesis is monodermal abortive teratomas, with no mesodermal and endodermal components. As the ultrasound test results were inconclusive, it became difficult to rule out the possibility of neoplasm. Therefore, patients were submitted to radical therapy. The definitive diagnosis for both cases was provided by the anatomopathological examination.

Trata-se de dois casos raros de cistos de inclusão epidérmica intragonadais, cuja etiologia permanece controversa, sendo uma hipótese a de teratomas monodérmicos abortivos, sem componentes mesodérmicos e endodérmicos. Devido a resultados inespecíficos dos exames ultrassonográficos, torna-se difícil afastar a possibilidade de neoplasia e o tratamento padrão permanece sendo, portanto, a terapia radical. O diagnóstico definitivo, em ambos os casos, foi firmado pelo exame anatomopatológico.

Light and electron microscopy of classical Ehlers-Danlos syndrome.

A 12-year-old boy with difficulty in wound healing and abnormal scars since early childhood was examined. Light microscopy showed loose and disperse dermal collagen with rare bundles, and fibroblasts show an irregular morphology. The fibrous sheath of hair presented a normal parallel distribution of the collagen fibers with normal spindle-shaped fibroblasts. Transmission electron microscopy also found disorganized collagen fibers, which were seen in a same field in longitudinal and cross sections. With high magnifications, an amorphous substance was seen near to loose collagen fibers, which showed variable diameters in cross sections. Scanning electron microscopy of the dermis showed disorganized collagen fibers and with higher magnification, important collagen disarrangement was observed with isolated and crossed-over fibers.

Classical Ehlers-Danlos syndrome: clinical, Histological and ultrastructural aspects.

A 12-year-old boy with difficulty in wound healing and scars of unusual appearance was examined. Thin, shiny and protruding skin, characterizing pseudotumoral lesions, was observed on his knees and elbows. Loose joints and increased skin elasticity were also observed. Light microscopy showed rare collagen bundles and loosely dispersed collagen fibers. Irregularly distributed elastic tissue was observed, and transmission electron microscopy revealed disorganized collagen fibers. In cross sections under high magnifications, fibers of varying diameters could be seen, as well as an irregular fiber outline. Scanning electron microscopy of the dermis showed disorganized collagen fibers, which were rarely arranged in bundles. Isolated and crossed-over fibers were also found.

Classical Ehlers-Danlos syndrome: clinical, Histological and ultrastructural aspects / Síndrome de Ehlers-Danlos clássica: aspectos clínicos, histológicos e ultraestruturais

A 12-year-old boy with difficulty in wound healing and scars of unusual appearance was examined. Thin, shiny and protruding skin, characterizing pseudotumoral lesions, was observed on his knees and elbows. Loose joints and increased skin elasticity were also observed. Light microscopy showed rare collagen bundles and loosely dispersed collagen fibers. Irregularly distributed elastic tissue was observed, and transmission electron microscopy revealed disorganized collagen fibers. In cross sections under high magnifications, fibers of varying diameters could be seen, as well as an irregular fiber outline. Scanning electron microscopy of the dermis showed disorganized collagen fibers, which were rarely arranged in bundles. Isolated and crossed-over fibers were also found.

Paciente masculino de 12 anos relatou dificuldade em cicatrizar, seguido por cicatrizes de aspecto infrequente . A pele dos joelhos e cotovelos é fina, brilhante e protrusa, caracterizando pseudo- tumores. Além disso apresenta hiperelasticidade cutânea e articular. A microscopia óptica demonstrou raros feixes colágenos, as fibras colágenas encontram-se dispersas. O tecido elástico está presente e secundariamente irregular. A microscopia eletrônica de transmissão também observou fibras colágenas desorganizadas e com cortes transversais em grande aumento evidenciou diâmetros variados e contorno irregular das mesmas. A microscopia eletrônica de varredura da derme demonstrou raros feixes colágenos e fibras colágenas isoladas e entrecruzadas.

Tumor carcinoide na extremidade distal do apêndice no serviço de cirurgia da Irmandade Santa Casa de Misericórdia de Porto Alegre / Carcinoid tumor in the distal end of the appendix in the general surgery unit of ISCMPA

Introdução: Este artigo descreve os resultados de uma coorte retrospectiva dos pacientes da ISCMPA, discutindo as características do tumor carcinoide quando localizado no apêndice. Métodos: Foram revisados os registros do banco de dados do Serviço de Patologia da ISCMPA de todas as apendicectomias realizadas entre 2000 e 2007 com posterior revisão manual dos registros de cada paciente. Resultados: Foram identificadas 3.730 apendicetomias. A prevalência de tumores carcinoides foi 0,4%. A idade mediana do paciente ao diagnóstico de tumor carcinoide foi de 26 anos, com uma distância interquartil de 20 anos. O sexo mais acometido foi o masculino. Em 46,67% dos casos, a extremidade distal foi o local do tumor e em 40% dos casos o tumor ultrapassou a camada serosa. Conclusões: Houve dificuldades em se definir claramente se as alterações no apêndice identificadas no transoperatório correspondiam a tumor carcinoide. A congelação no transoperatório ajudou a definir o tipo de tratamento cirúrgico mais adequado e a identificar metástases de outras patologias sistêmicas

Introduction: This paper describes the results of a retrospective cohort of patients of the ISCMPA, discussing the features of carcinoid tumors occurring in the appendix. Methods: We searched the database of the ISCMPA Department of Pathology for all the records of appendectomies performed between 2000 and 2007, with subsequent manual review of individual patient records. Results: 3,730 appendectomies were identified. The prevalence of carcinoid tumors was 0.4%. The median age at diagnosis of carcinoid tumor was 26 years with an interquartile range of 20 years. Males were more affected than females. In 46.67% of the cases the distal end was the site of the tumor and in 40% the tumor exceeded the serosa layer. Conclusions: There were difficulties in clearly defining if the changes in the appendix identified during surgery corresponded to a carcinoid tumor. Transoperative freezing helped determine the most appropriate type of surgical approach and identify metastases of other systemic diseases