INTRODUCTION: The differential diagnosis of B-cell lymphoproliferative processes remains a challenge for pathologists, dermatologists and oncologists, despite advances in histology, immunohistochemistry and molecular biology. OBJECTIVE: Evaluate aid and limitations of clonality analysis in the diagnosis of primary cutaneous B-cell lymphomas and B-cell pseudolymphomas. METHODS: This study included 29 cases of B-cell lymphoproliferative processes classified as primary cutaneous B-cell lymphomas (13), B-cell pseudolymphomas (6) and inconclusive cases (10) using histology and immunohistochemistry. The clonality analysis was performed by polymerase chain reaction analysis of immunoglobulin light chain and heavy chain rearrangements. RESULTS: DNA quality was shown to be generally poor; eight samples were inadequate for polymerase chain reaction analysis. The results showed monoclonality in eight of the primary cutaneous B-cell lymphomas and polyclonality in four of the B-cell pseudolymphomas. In addition, monoclonality was shown in two of the inconclusive cases by histology and immunohistochemistry, demonstrating the utility of polymerase chain reaction as an ancillary diagnostic tool for primary cutaneous B-cell lymphomas. DISCUSSION: The low quality DNA extracted from these cases demanded the use of an IgH protocol that yielded small fragments and IgK. Both methods used together improved detection. CONCLUSION: Use of the two protocols, immunoglobulin heavy chain FR3-trad and immunoglobulin light chain-Kappa Biomed protocols for clonality analysis improved diagnostic accuracy.

Os autores tecem considerações sobre a estrutura e funções normais das glândulas paratireóides como introdução à patologia e as repercussões clinico - patológicas tanto do excesso como da redução do paratormônio. Maior ênfase é dedicada ao hiperparatireoidismo primário quanto às causas, a fisiopatologia das alterações, os aspectos anatomopatológicos macro e microscópicos das lesões e sua patogenia, na "Osteite fibrocistica" ou "doença de von Recklinghausen dos ossos" com a correlação aos aspectos radiográficos. Apresentam caso de paciente, cuja história clinica demonstra as dificuldades encontradas para o diagnóstico da doença. Referem-se ainda às alterações e patogenia das formas de hiperparatireoidismo secundário e terciário e ao hipoparatireoidismo.

The authors present a summary on the normal anatomy and function of the parathyroid glands as well as a brief review of clinical and pathological repercussions of higher and lower parathyroid hormone production. The emphasis is given on the causes, physiopathology, anatomy, macroscopy and microscopy of the lesions and their role in the genesis of fibrocystic osteitis, also known as Von Recklinhausen disease of the bones. Radiological correlation is also given. The authors show the challenges for the diagnosis in the same cases. We also write about secondary and tertiary hyperparathyroidism, as well as hypoparathyroidism.

OBJETIVOS: Estudar retrospectivamente 18 casos de pacientes com adamantinoma de ossos longos, todos localizados na tíbia; ressaltar a importância da biópsia e a correlação com métodos de imagem para diagnóstico diferencial com osteofibrodisplasia e displasia fibrosa; tecer considerações sobre a natureza do adamantinoma de ossos longos, cujo nome deve-se à analogia histológica com o adamantinoma (ameloblastoma) da mandíbula. MÉTODOS: Foram analisados o quadro clínico, imagens e exames anatomopatológicos complementados com imunohistoquímica e a evolução dos pacientes. Todos foram submetidos a tratamento cirúrgico, 17 com "tibialização" da fíbula e os demais com amputação. RESULTADOS: A evolução pós-cirúrgica mostrou-se imprevisível e não relacionada com os aspectos clínicos ou histopatológicos. Dois pacientes evoluíram com metástases pulmonares e morreram. Seis não tiveram recidivas ou metástases e estão clinicamente curados. Os demais, após alta hospitalar não retornaram à consulta. CONCLUSÕES: Trata-se de rara neoplasia constituída por estruturas epiteliais e mesenquimais que devem ser diagnosticadas com precisão, antes de qualquer procedimento. O tratamento é cirúrgico com ressecção do tumor com boa margem oncológica. O comportamento biológico é variável e imprevisível.

OBJECTIVE: To make a retrospective study of 18 cases of patients with adamantinoma of the long bone, all of them located in the tibia; to point to the relevance of biopsy and the correlation with imaging methods in order to have a differential diagnosis with osteofibrous dysplasia and fibrous dysplasia; to comment on the nature of long bone adamantinoma, whose name is due to the histological analogy with the adamantinoma (ameloblastoma) of the jaw. METHODS: A review was made of the clinical condition, images, and anatomopathological exams supplemented with immunohistochemical essays, and the evolution of the patients. All of them were submitted to surgical treatment, 17 with "tibialization" of the fibula and the others with amputation. RESULTS: The post-surgical evolution showed to be unpredictable and not related to clinical or histopathological aspects. Two patients had an evolution with lung metastasis and died. Six did not present recurrent disease or metastases, and are clinically cured. The others, after being released from hospital, did not return for consultation. CONCLUSIONS: This is a rare neoplasia made of epithelial and mesenchymal structures that must be accurately diagnosed before any procedure is attempted. Treatment is surgical, with tumor resection with a good oncologic margin. The biological behavior is varied and unpredictable.

São relatados os aspectos neurorradiológicos, neuropatológicos e imuno-histoquímicos em um paciente jovem com comprometimento do sistema nervoso central simulando encefalomielite aguda disseminada. Paciente masculino branco, com 17 anos de idade, hígido até há 4 meses, quando desenvolveu hemiparesia direita e, após 2 meses, hemiparesia bilateral com comprometimento progressivo de vários nervos cranianos. A imagem de ressonância magnética mostrou lesões múltiplas, sem efeito de massa, sugerindo perda da mielina. Permaneceu inconsciente durante quase 1 mês, com óbito decorrente de pneumonia. Na autópsia o encéfalo pesou 1505 g, com hérnias e tumefação do hemitronco direito. Havia várias áreas moles e róseas principalmente ao nível do hemitronco direito, cerebelo esquerdo e substância branca do hemisfério cerebral esquerdo. A histopatologia evidenciou proliferação blastomatosa difusa sem substituição ou destruição do tecido original. As células neoplásicas apresentavam fenotipos astrocíticos, oligodendrocíticos e espongioblásticos, algumas delas com reatividade positiva para GFAP. Havia transformação focai anaplásica. O diagnóstico de "gliomatosis cerebri" apenas foi possível com a autópsia.

Neuroradiologic, neuropathologic and immunohistochemical features are reported in a young man with a impairment of the central nervous system mimicking an acute diffuse encephalomyelitis. A white male, 17 years old, healthy till 4 months before, when developed a right hemiparesis and after 2 months a bilateral hemiparesis with a progressive impairment of several cranial nerves. Magnetic resonance imaging showed multiple lesions without a mass effect that suggested myelin loss. He remained unconscious for almost one month before dying of pneumonia. The neuropathologic examination showed a heavy brain (1505 g) with herniations and a large right midbrain. There were several soft and pink areas mainly at the right midbrain, left cerebellum and in the white matter of the left cerebral hemisphere. The histopathologic sections showed diffuse blastomatous proliferation without total replacement or destruction of the original tissue. The tumor cells had astrocytic, oligodendrocytic and spongioblastic phenotypes, some of them with a GFAP-positive reactivity. There were focal anaplastic changes. The diagnosis of "gliomatosis cerebri" was only possible by the autopsy.