Enfermedad de Castleman en glándula salival: informe de un caso pediátrico / Castleman disease in salivary gland: report of a pediatric case

La enfermedad de Castleman (EC) es una rara entidad linfoproliferativa benigna caracterizada por el crecimiento no neoplásico progresivo de los ganglios linfáticos en cualquier parte del cuerpo, aunque en la mayoría de los casos (60%) afecta el mediastino y, con una muy baja frecuencia, compromete la región de la cabeza y el cuello, incluidas las glándulas salivales. Representa un desafío diagnóstico para el médico porque se presenta con síntomas y signos inespecíficos. Existen dos subtipos: uno localizado en forma de adenopatía sin síntomas generales y otro multicéntrico con compromiso de varios grupos ganglionares y con manifestaciones clínicas sistémicas y alteraciones del laboratorio. El diagnóstico requiere un examen físico completo y exámenes complementarios (laboratorio, ecografía, tomografía computarizada o resonancia magnética nuclear o ambas, punción aspiración con aguja fina) y se confirma a través del análisis histopatológico. El tratamiento es quirúrgico, pero la forma multicéntrica requiere además un tratamiento adyuvante. A pesar de su localización inusual en las glándulas salivales, la EC debería ser considerada entre los diagnósticos diferenciales, sobre todo ante la sospecha de procesos linfoproliferativos. Se describe en este trabajo el caso de una paciente pediátrica con diagnóstico de EC localizada en la glándula submaxilar, y se realizó una revisión de la literatura. (AU)

Castleman disease (CD) is a rare lymphoproliferative entity characterized for a non ­ neoplasic progressive growth of the lymph nodes in any part of the body; nevertheless it usually affects the mediastinum (60 %), and with less frequency, it compromises the head and neck region, including the salivary glands. It represents a diagnostic challenge for the doctor, because it has nonspecific symptoms and signs. There are two subtypes: the localized form, that generally is presented through an adenopaty without systemic symptoms, and a multicentric form that compromises several nodal groups and has systemic symptoms and laboratory abnormalities. Diagnosis requires a complete physical examination and complementary exams (blood tests, ultrasonography, computed tomography and/or magnetic resonance, fine needle puncture ­ aspiration), and the final diagnosis is through histopathological analysis. The treatment is surgery, but multicentric form requires adyuvant treatment. Although its unusual presentation in salivary glands, CD should be considered between differential diagnosis, especially when we are thinking of lymphoproliferative entities. In this article, we present a case of a pediatric patient with diagnosis of CD of submaxilar gland and we review the literatura about this disease with special attention in the salivary glands location. (AU)

Castlemans disease in HIV infected patient from eastern India.

Castlemans disease is a rare lymphoproliferative disorder. We report a patient of Castlemans disease with advanced HIV infection who responded well to conventional HAART. This case is interesting because of the rarity of the disease in the eastern part of India and its good out come with antiretroviral therapy.

Doença de Castleman e síndrome de Poems: relato de caso e revisão da literatura / Castleman disease and Poems syndrome: case report and review

A Doença de Castleman é uma desordem linfoproliferativa que apresenta relação etiológica com o herpes vírus tipo 8 e o HIV, manifestando-se de duas formas distintas. A forma unicêntrica é benigna, não cursa com sintomas sistêmicos e geralmente é curada com a ressecção cirúrgica. A forma multicêntrica geralmente apresenta-se com linfadenopatia generalizada, febre, emagrecimento, entre outras manifestações sistêmicas e pode estar associada a malignidades, entre as quais a síndrome de POEMS (polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações de pele). Relatamos o caso de um homem de 50 anos que se apresentava com emagrecimento, febre e linfadenopatia, associado a gamopatia monoclonal, organomegalia, endocrinopatia e neuropatia, cujo diagnóstico foi de Doença de Castleman cursando com síndrome de POEMS.

Castleman’s disease is a lymphoproliferative disorder related with human herpesvirus 8 and HIV, presenting in two different ways. The unicentric form is benign, doesn’t have systemic symptoms and is usually cured with surgery. The multicentric form usually presents with generalized lymphadenopathy, fever, weight loss, and other systemic manifestations and it can be associated with malignancy, especially POEMS syndrome. We report a case of a 50 year old man presenting with weight loss, fever and lymphadenopathy, associated with monoclonal gammopathy, organomegaly, endocrinopathy and neuropathy, which diagnosis was Castleman’s disease with POEMS syndrome.

Lack of evidence of epstein-barr virus infection in patients with castleman's disease molecular genetic analysis

Epstein-Barr virus [EBV] infection is associated with a diverse group of malignancies and many lymphoproliferative disorders. Castleman's disease [CD] is atypical lymphoproliferative disorder. The role of EBV in the pathogenesis of CD is not clear yet. The objective of this study is to investigate the EBV status in CD. We searched medical records for cases of CD at the Toronto General Hospital, Toronto, Canada and King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Twenty cases were found. The presence of EBV was analyzed using polymerase chain reaction. Polymerase chain reaction were performed at the Department of Pathology and Laboratory Medicine, Toronto General Hospital. The study started in 2001 and completed in 2005. The age range was 16-90 years. Seventeen patients manifested the localized form of CD. There were 11 males 9 females. Epstein-Barr virus genome was detected only in 2 cases; both were males and have plasma cell type. One is a localized type and the other is of a multicentric type. One patient revealed clonal rearrangement of the immunoglobulin H. The number of cases is small; however it appears that EBV is less likely to play a significant role in the pathogenesis of CD; however, it seems to be associated with clonal progression

Mediastinal Castleman's disease complicated by follicular dendritic cell tumour.

We describe a rare case of follicular dendritic cell tumour which had arisen over the background of hyaline vascular type of Castleman's disease at the mediastinal location. Constellation of histology and immunohistochemistry using CD21 antibody and non-reactivity to CD15, CD30, cytokeratin and epithelial membrane antigen helped us diagnose this case. The literature is reviewed, specially with reference to the genesis of follicular dendritic cell neoplasm at the backdrop of Castleman's disease and its clinical relevance.