Localized retroperitoneal Castleman’s disease: a case report and review of the literature

Abstract

IntroductionCastleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman’s disease have been reported in the literature.Case presentationA 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected.ConclusionCastleman’s disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.

Castleman's Disease as a Rare Differential Diagnosis of Lymphadenopathy: 2 Case Reports and Review of Literature

“…Patient was finally diagnosed as multicenteric hyaline vascular castleman's disease. Multicenteric Castleman's disease is typically of the plasma cell type but in our case series the multicenteric case is of hyaline vascular type [12]. Multicenteric castleman's disease is characterized by generalized Lymphadenopathy: hepatosplenomegaly and usually associated with constitutional manifestations as fever: night sweating: severe fatigue: and anorexia.…”

“…The second case was also presented in unusual very young age (2.5) years. Castleman's disease is a rare lymph proliferative disorder with an age range from 8years to 66 years [12].…”

Analysis of Clinical Characteristics, Pathological Changes and Changes of Interleukin-6 (IL-6) and C-Reactive Protein (CRP) in Children with Castleman’s Disease

“…Based on the diagnostic criteria for CD, with the exception of the characteristic clinicopathological changes of patients, other related primary changes are often excluded in clinical diagnosis, thus, connective tissue disease, tumor disease, and infectious disease should be excluded in clinical diagnosis of CD, so as to improve the accuracy, sensitivity and specificity of clinical diagnosis [ 20 ]. The limitations in this study were that the potential value of IL-6 and CRP as diagnostic markers of CD children requires further validation within a large sample size, and by using comparison of histological and immunohistochemical analysis, including staining for CD3, CD5, CD10, CD20, CD23, CD79, and Ki-67 as previously reported [ 21 ]. The evaluation also needs to include the incorporation of the comprehensive treatment and follow-up details.…”

POEMS syndrome associated with Castleman disease: a case report and literature review

“…[17] The clinical features of Castleman disease are classified into two categories: localized and multicentric. [18] There The diagnosis of POEMS syndrome is confirmed when both of the mandatory major criteria, 1 of the 3 other major criteria and 1 of the 6 minor criteria are present. *Summary of frequencies of POEMS syndrome features based on largest retrospective series, [2,3,[6][7][8][9]] † Takasuki and Nakanishi series are included, even though only 75% of patients had a documented plasma cell disorder.…”