Cytological diagnosis of hyaline-vascular type of Castleman disease.

Castleman disease (CD) is a rare, benign lymphoproliferative disorder, mostly involving the mediastinal lymph nodes, but can occur wherever lymphoid tissue is found. With only a few published case reports, there needs to be more literature on its cytological findings. We report the case of a 63-year-old female presenting with left upper cervical swelling. Fine needle aspiration cytology smears showed variably sized lymphoid follicles with diminished germinal centers, prominence of follicular dendritic cells, and capillaries traversing some of the follicles. The possibility of a hyaline-vascular type of Castleman disease was suggested. Histopathology confirmed the cytological diagnosis. The index case is being presented to discuss the cytological features of the CD along with its histological and immunohistochemical correlation.

Enfermedad de Castleman: un enfoque integral a propósito de un reporte de caso / Castleman's Disease: A Comprehensive Approach Regarding a Case Report

Resumen Se presenta el caso clínico de una paciente del sexo femenino, de 30 años, con adenopatías supraclaviculares y axilares ipsilaterales, quien fue sometida a biopsia excisional con posterior estudio. El análisis histopatológico de la muestra de tejido resecado reveló una serie de características distintivas asociadas con la enfermedad de Castleman variante hialinovascular. La presentación de este caso no solo proporciona información detallada sobre la evolución clínica de la paciente, sino que también sirve como base para ilustrar los aspectos clave del diagnóstico histopatológico y las implicaciones inmunohistoquímicas en la enfermedad de Castleman. Además de hacer una revisión de tema respecto a esta patología poco común, en la cual los informes de casos son fundamentales para aumentar la comprensión de su variabilidad clínica y su abordaje diagnóstico, ilustrando los desafíos en el diagnóstico diferencial y como deben abordarse los mismos.

Abstract The clinical case of a 30-year-old female patient with supraclavicular and ipsilateral axillary lymphadenopathy who underwent excisional biopsy with subsequent study is presented. Histopathological analysis of the resected tissue sample revealed a series of distinctive features associated with hyalinevascular variant Castleman disease. The presentation of this case not only provides detailed information about the clinical evolution of the patient, but also serves as a basis to illustrate Key aspects of histopathological diagnosis and immunohistochemical implications in Castleman disease. In addition to making a review of the topic regarding this rare pathology in which case reports are essential to increase the understanding of its clinical variability and its diagnostic approach, illustrating the challenges in differential diagnosis and how they should be addressed.

Castleman disease. Histopathological and immunohistochemical analysis of 39 cases. / Enfermedad de Castleman. Análisis histopatológico e inmunohistoquímico de treinta y nueve casos.

Introduction: Castleman disease (CD) is a rare lymphoproliferative that comprises two distinct clinical subtypes (unicentric and multicentric) and has two basic histopathology patterns that are hyaline-vascular (HV) and plasma-cell (PC) type. Some cases of multicentric PC disease are associated with HHV-8 infection. Objective: To present the histopathologic and immunohistochemical characteristics of 39 cases of CD. Methods: A review of cases with the diagnosis CD from the files of the Department of Pathology of the ABC Medical Centre in Mexico City was performed. Thirty-nine cases of CD were identified, and a detailed paraffin immunophenotypic study of 9 of them was completed using desmin, cytokeratin OSCAR (CO) and Epidermal growth factor receptor (EGFR), to evaluate the dendritic cell population. Results and Conclusions: Of the 39 cases of CD, 24 were HV and 15 CP. All HV cases were unicentric and only one case of CP was multicentric. The most frequent localization in both subtypes was in lymph nodes; 21/24 cases in HV and 15 cases of CP. All cases were immunostained with CD20 that was expressed in the germinal centers (CGs), CD3 in the paracortical zone, and CD21 in follicular dendritic cells (CDF) within CGs, with expansion towards the area of the hyperplastic mantle zone (only in the HV variant). One case of CD CP was positive for HHV-8. Of the nine cases (6 HV and 3 PC cases) that were detailed with IHC, we found EGFR expression in FDC in all but one of the 9 cases studied and desmin was positive in fibroblastic reticulum cells (FRC) in all, but one of the cases of CD. CO was positive FRC in 3 of 6 cases of HV type and all (3) of the PC type. Clinical, histopathological and HIV and HHV-8 status markers, allow for the classification of CD into groups with markedly different outcomes and disease associations.

Características clinicopatológicas de la enfermedad de Castleman: experiencia en el Instituto Nacional de Cancerología / Clinical and pathological features of Castleman's disease: experience at the Instituto Nacional de Cancerología, Mexico City

Introducción: La enfermedad de Castleman es una entidad patológica poco comprendida, descrita originalmente en pacientes europeos. Informamos nuestra experiencia con esta entidad clinicopatológica en pacientes del Instituto Nacional de Cancerología de la Ciudad de México. Material y métodos: Analizamos retrospectivamente los expedientes de pacientes con enfermedad de Castleman de 1996 a 2003. La enfermedad fue monocéntrica si había solo un ganglio o multicéntrica si se encontraba linfoadenopatía generalizada. Además, se dividió en las variantes histológicas hialinovascular y de células plasmáticas. Resultados: Once pacientes con enfermedad de Castleman fueron diagnosticados en el periodo referido, seis tenían enfermedad monocéntrica y cinco multicéntrica. La mediana de seguimiento fue de 40 meses. Todos los pacientes con enfermedad monocéntrica tenían la variante hialinovascular. De los cinco con multicéntrica, cuatro tenían la variante de células plasmáticas y uno la hialinovascular. Cinco pacientes con enfermedad monocéntrica se trataron con cirugía y uno con quimioterapia; al momento de este informe todos permanecían vivos y sin enfermedad. Tres pacientes con enfermedad multicéntrica recibieron quimioterapia y dos, quimioterapia más radioterapia por enfermedad residual; a dos pacientes se les prescribió quimioterapia de segunda línea, con buena respuesta. Dos pacientes con una condición asociada evolucionaron desfavorablemente. Conclusiones: Las características clínicas, patológicas y los resultados del tratamiento son similares a los señalados en otras poblaciones.

BACKGROUND: Castleman's disease (CD) is a rare, poorly understood pathological entity. We report our experience with this clinicopathological entity. METHODS: We retrospectively analyzed records of all patients with CD from 1996 to 2003. The disease was classified as unicentric if a solitary mass was present or multicentric if generalized lymphadenopathy was present. We further subdivided the disease into hyaline vascular (HV) and plasma cell (PC) histological variants. RESULTS: We found 11 patients with CD. Six patients had unicentric disease and five had multicentric disease. Median follow-up was 40 months. All patients with unicentric disease had the HV variant. Of the five patients with multicentric disease, four had the PC variant and one had the HV. Five patients with unicentric disease were treated surgically with complete resection, and only one patient was treated with chemotherapy. All remain alive without disease. Three patients with multicentric disease were treated with chemotherapy, and two patients received chemotherapy plus radiotherapy for residual disease. Two patients received second-line chemotherapy with a favorable outcome. Two patients with a comorbid condition had a poor outcome. CONCLUSIONS: Clinical characteristics, pathological features and treatment results are similar to that reported in other populations.

Enfermedad de castleman: a propósito de un caso / Castleman disease: a purpose of a case

La enfermedad de Castleman o hiperplasia angiofolicular se caracteriza por hiperplasia linfoide reactiva, crecimiento de tumores benignos del tejido linfático y una mayor predisposición a padecer linfomas. Descrita por Benjamín Castleman en 1956, de etiología desconocida, con probable relación con el herpes virus tipo 8, fallo en la inmunorregulación, expresión aumentada del gen codificador de interleukina-6. En pediatría es excepcional. Clínicamente se distinguen las formas multicéntrica y la localizada (70 por ciento de los casos), de buen pronóstico, localizada en mediastino, cuello, abdomen, menos frecuente en axila, pelvis y páncreas; la resección de la lesión es curativa. Histológicamente se clasifica en dos tipos: hialinovascular (la más frecuente), y variedad de células plasmáticas. Se revisó la literatura y se presentan dos casos clínicos. Caso nº 1: escolar de 6 años, quien desde los 18 meses de vida presentaba masa tumoral en axila izquierda de 0,5 cm. la cual fue resecada a los 4 años. A los 6 años recidivó hasta medir 7 x 4 cms, realizándose exéresis. Presentó además hipergammaglobulinemia, bajo nivel de células NK y del índice CD4/CD8. Caso nº 2: pre-escolar masculino de 4 años, con masa tumoral en axila derecha de 1 cm. de 6 meses de evolución. Se le realizó biopsia excisional. En ambos casos el estudio anatomopatológico e inmunohistoquímico reportó Enfermedad de Castleman de variedad hialinovascular. Los pediatras y cirujanos pediatras debemos maximizar la vigilancia de adenomegalias que puedan ser lesiones centinelas de afección inmunológica o neoplásica curables si son tratadas precozmente.

Castleman’s disease or angiofolicular hiperplasy is characterized by reactive lymphoid hyperplasia, benign tumors of lymphoid tissue and predisposition to develop lymphomas. Described by Benjamin Castleman in 1956, it is of unknown etiology, probably related to herpes virus type 8, immunoregulation failure, increased expression of 6-interleukin gene. Very rare in childhood, the disease has two different clinical types: a multicentric type, and a localized type (70% of the cases). The latter with good prognosis, located inmediastinum, neck, abdomen, and less frequently in axila, pelvis and pancreas. Treatment consists in the resection of the lesion. The histological types are the hyaline-vascular type (most frequent) and the plasma cells type. Literature was reviewed and two clinical cases are reported: Case nº1: 6 year old child, who presents at 18 months of age with a 0.5 cm bulk in his left axila. The lesion was removed surgically at 4 years of age, with reappearance of a 7 x 4 lesion which was removed at 6 years of age. This child had also hipergammaglobulinemia, low levels of NK cells and of the CD4/CD8 index. Case nº2: 4 year old child, who presented with a 1 cm mass in his right axila of 6 months of evolution. An excisional biopsy was performed. In both cases the histological study reported Castleman’s disease of hialinovascular variety. Pediatricians and pediatric surgeons must follow very closely the growth of lymphoid tissue that may represent immunological. or neoplastic lesions, potentially curable if diagnosed and treated early.