Enfermedad de Castleman de localización torácica / Intrathoracic Castleman's disease

Antecedentes: la enfermedad de Castleman es conocida como hiperplasia linfática angiofolicular y se caracteriza por crecimiento masivo del tejido linfático. Histológicamente hay tres categorías: hialino-vascular, tipo células plasmáticas y mixto. Se describen dos variantes clínicas: monocéntrica (localizada) y multicéntrica. Objetivo: presentar nuestra experiencia en el manejo de esta enfermedad haciendo énfasis en su compromiso mediastinal y pulmonar. Material y métodos: revisión de historias clínicas e informes patológicos. Resultados: se identificaron 5 pacientes, 1 mujer y 4 hombres, con edad promedio de 58,5 años. Tres pacientes presentaron la forma localizada y dos la multicéntrica. Los síntomas fueron: dolor torácico en dos casos, astenia en 1 caso y síndrome POEMS (polineuropata, organomegalia, endocrinopata y pico monoclo-nal) en 2 casos. Se realizó toracotomía y resección en 2 casos, lobectomía superior derecha en bloque con la lesión mediastinal en 1 caso, biopsia de masa apical en 1 caso y biopsia ganglionar por mediastinoscopia en 1 caso. Se realizó reoperación por sangrado en 1 caso. Tres pacientes presentaron la forma hialino-vascular y 2 la variante de células plasmáticas. Un paciente resecado se perdió de seguimiento. Los pacientes con síndrome POEMS fallecieron, uno al 8° día por neumonía intrahospitalaria y otro al 6° mes por sepsis. Los enfermos resecados restantes cursan buena evolución. Conclusiones: se recomienda la resección quirúrgica en pacientes con enfermedad de Castleman variedad localizada, la cual es curativa. Los pacientes con la variedad multicéntrica no se benefician con esta terapéu-tica, pero son pasibles de quimioterapia o radioterapia o combinación de ambas.

Background: Castleman's disease, known as angio follicular lymph node hyperplasia, is characterized by the massive growth of lymphoid tissue. Histollogically there are three categories: hyaline-vascular, plasm cell type and mixed type. Two clinical variantis have been described: monocentric (localized) and multicentric. Objective: to describe our experience in the treatment of this disease, focusing on itis mediastinal and pulmonary afecton. Method: review of clinical records and pathological reportis. Resultis: 5 patentis (1 female and 4 male), within an age -range of 58, 5 years were screened. Three patentis were afected by the localized type and two by the multicentric type. The symptoms were: thoracic pain in two of the cases, asthenia in one and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy and monoclonal changes) in 2 cases. We performed thoracotomy and resecton in two cases, upper right side lobectomy in block with the mediastinal lesion in 1 case, apical mass biopsy in 1 case and lymph node biopsy by mediastinoscopy in one case. We performed reoperaton by bleeding in one case. Three patentis presented the hyaline vascular type and two, the plasma-cell type. We lost track of a resectoned patent. Those patentis with POEMS syndrome died, one of them of intra-hospital pneumonia on the eighth day, and another of sepsis on the sixth month. The remaining resectoned patentis are evolving well. Conclusions: on patentis presenting localized type Castleman's disease, we recommend surgical resecton, which is a curative therapy. Patentis presenting the monocentric type do not beneft from this therapy and are passible of chemotherapy and /or radiotherapy.

Doença de Castleman cervical: relato de três casos / Cervical Castleman's disease: report of three cases

A doença de Castleman (DC) é uma rara e benigna desordem linfoproliferativa descrita por Castleman como "hiperplasia hialinizante linfoide". Sua etiologia e patogênese é desconhecida, porém parece haver hiperestimulação antigênica induzida por vírus de forma crônica e mediada por interleucina 6, com alguns casos ligados ao herpes vírus 8 e vírus da imunodeficiência humana (AU)

Castleman's disease (CD) is a rare and benign lymphoproliferative disorder described by Castleman as "hyelinizing lymphoid hyperplasia". Its etiology and pathogenesis are unknown, but there appears to be antigen hyperstimulation chronically induced by virus and mediated by interleukin 6, with some cases related to herpes virus 8 and human immunodeficiency virus (AU)

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. RESULTS: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. CONCLUSION: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.

Castleman's Disease with Cutaneous Involvement Manifestating as Multiple Violaceous Plaques on Entire Body

Castleman's disease (CD) is an uncommon B-cell lymphoproliferative disorder characterized by lymph node hyperplasia with vascular proliferation. Cutaneous involvement in CD is rare. A 65-year-old man presented with a 7-year history of gradually developing multiple reddish to violaceous indurated plaques on the scalp, trunk, and legs. On physical examination, there were palpable enlarged cervical, axillary, and inguinal lymph nodes. Laboratory examination revealed anemia, thrombocytosis, hyperproteinemia, hypoalbuminemia, and polyclonal hypergammaglobulinemia. An inguinal lymph node biopsy and a skin biopsy were performed and the patient was diagnosed with the plasma cell type of CD. Chemotherapy was started and the lesions have responded to treatment.

An Unusual Stroma-Rich Variant of Castleman's Disease of the Hyaline-Vascular Type: A Case Report

The stroma-rich variant of Castleman's disease of the hyaline-vascular type (CDHV) is a rare entity that shows overgrowth of a variety of stromal cells in the widened interfollicular (IF) area. We report here on a case of a stroma-rich variant of CDHV in an 18-year-old man who presented with an asymptomatic solitary neck mass he'd had for 1 year. Histologically, an enlarged lymph node fulfilled the criteria of CDHV, along with vague nodularity of a widened IF area. The nodular lesion consisted of numerous vessels and a proliferation of spindle cells. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin, they were negative for desmin, CD21, CD34, CD68, ALK-1, and S-100 protein. This stromal lesion is typically hyperplastic and clinically benign, and it must be distinguished from neoplastic stromal proliferation associated with Castleman's disease because of its potential for recurrence and metastasis.