A retrospective study of unicentric and multicentric Castleman’s disease: a report of 52 patients

Abstract

Castleman's disease (CD) is a rare disorder characterized by non-cancerous tumor growth that may develop in the lymph node tissue at a single site or throughout the body (Castleman et al. in Cancer 9:822-830, 1956). It involves hyperproliferation of specific B cells that produce the cytokine IL-6. This disorder is often undiagnosed or misdiagnosed. For this reason, only very few patients have been reported, and little information is available in the literature. In hopes of providing a better understanding of this rare disease, this report examines 52 patients with Unicentric Castleman's disease (UCD) and Multicentric Castleman's disease (MCD) treated from 1999-2008 at a single institution. Fifty-two patients with CD, along with their histological diagnoses, were collected. Patients were divided into two groups--the more common UCD and the less common MCD. Relevant clinical, pathological, and laboratory data were examined in order to evaluate treatment responses, with symptom onsets and survival period serving as the endpoints of the assessment. Each of the 48 patients with UCD exhibited benign symptoms and underwent a curative surgical resection with excellent prognosis. All of the four patients with MCD received surgical resection. Three of the four patients relapsed and received radiotherapy and/or chemotherapy. Only one of the three post-treatment patients survived. UCD is manifested in the form of benign, painless, slow lymph node enlargement that is generally asymptomatic. Complete surgical removal is recommended as a course of curative treatment. The multicentric form of CD exhibits a progressive clinical course with potential for malignancy. There is currently no standard therapy for MCD.

A retrospective study of 34�patients with unicentric and multicentric Castleman's disease: Experience from a single institution

“…A total of 5 patients who underwent laparoscopic surgery achieved faster recovery, which was due to the advantage of laparoscopy in gaining access to difficult areas of the abdomen, as well as improved visualization with bloodless dissection. No patients underwent complete excision received further therapies as no evidence of disease recurrence was discovered in these patients, which was consistent with other reports on the critical role of surgical excision in UCD treatment (15,17).…”

“…HVCD has been reported in 90% of patients with UCD, but rarely in patients with MCD, whereas PCCD has been reported in only 10% of patients with UCD and in 80-90% of patients with MCD (9). In the present study, the HV type was identified in 81.5% of patients with UCD, and the PC type was observed in 57.1% of patients with MCD which was slightly lower compared with one recent study from China (17). The histological characteristics of HVCD consisted of distinctive dysplasia follicles with regressed germinal centers and a broad mantle zone of lymphocytes, which formed a concentric ring (known as the ʻonion-skinʼ arrangement) (18).…”

“…These presentations may lead to the initial diagnosis of lymphoma (10,23). Although elevated serum expression levels of IL-6, as well as circulating human herpes virus-8 particles, may be useful for differential diagnosis, these two indicators are not widely nor commonly used in clinical practice (17). The size of the swollen lymph node in UCD, with a mean value of 5.7 cm, may be another useful indicator, as the typical size of lymphoma lymph nodes is smaller (15).…”

The Role of Surgical Resection in Unicentric Castleman’s Disease: A Systematic Review

“…Ye et al [12] conducted a retrospective study of 52 patients with Castleman's disease between 1999 and 2008. 48 patients had UCD and in 22 cases the lesion was located in the mediastinum.…”

Enfermedad de Castleman. Análisis histopatológico e inmunohistoquímico de treinta y nueve casos

“…Aunque no se considera una lesión neoplásica, la variante de CP multicéntrica es tratada como tal y está asociada a riesgo incrementado de desarrollar linfoma difuso de células grandes B y sarcoma de Kaposi 3,5,6 . La EC HV frecuentemente es unicéntrica 7 y, aunque poco frecuente, se puede asociar con la aparición de sarcomas de células dendríticas foliculares de manera sincrónica o metacrónica 8,9 . Hay informes de la expresión del receptor del factor de crecimiento epidérmico (EGFR, por sus siglas en inglés) en las CDF de ambas variedades, y se ha propuesto una posible relación patogénica 2,8,9 .…”