Castleman Disease- An Uncommon And Intriguing Condition Revisited: A Report Of Two Cases

Shubhangivinayak Agale,Pinkesh Gugaliya,Sushama N Ramraje,Grace F D’Costa,Banasri Devi,Sameer Ansari

doi:10.21276/apalm.1559

Abstract

Castleman disease (CD) also called as angiofollicular hyperplasia or giant lymph node hyperplasia of unknown cause is a benign lymphoproliferative disorder. It is a localised (unicentric) or disseminated (multicentric) disease. This disease is uncommon with an incidence of 25 cases per million person per year. Castleman disease is a heterogeneous group of disorders with four characteristic histological variants. The etiology of this disease is poorly understood. We report two cases of CD, one presenting with a neck mass and the other presenting with para-aortic and mesenteric lymphadenopathy which were classified as hyaline vascular type and mixed type of CD respectively. DOI: 10.21276/APALM.1559

Highlights

Castleman disease (CD), called as angiofollicular hyperplasia or benign giant lymph node hyperplasia, is of unknown cause and is a rare, benign lymphoproliferative disorder.[1,2,3,4,5,6,7,8,9,10]. It was first reported as a localized mediastinal lymph node hyperplasia by Castleman and Towne in 1954. [2,3] Castleman disease commonly involves lymphoid tissues in the thorax (70%), abdomen and pelvis (15%), and the neck (15%).[4]
The multicentric form characterized by involvement of multiple lymph nodes and multiple organs was first described by Gaba et al in 1972.[10,11] Histologically, CD is classified into four variants, hyaline vascular, plasma cell, mixed type and plasmablastic variant.[6]
We report two cases of CD, one presenting with a neck mass and the other presenting with para-aortic and mesenteric lymphadenopathy which were classified as hyaline vascular type and mixed type of CD respectively

Summary

Introduction

Castleman disease (CD), called as angiofollicular hyperplasia or benign giant lymph node hyperplasia, is of unknown cause and is a rare, benign lymphoproliferative disorder.[1,2,3,4,5,6,7,8,9,10] It was first reported as a localized mediastinal lymph node hyperplasia by Castleman and Towne in 1954. [2,3] Castleman disease commonly involves lymphoid tissues in the thorax (70%), abdomen and pelvis (15%), and the neck (15%).[4]. The multicentric form characterized by involvement of multiple lymph nodes and multiple organs was first described by Gaba et al in 1972.[10,11] Histologically, CD is classified into four variants, hyaline vascular, plasma cell, mixed type and plasmablastic variant.[6] We report two cases of CD, one presenting with a neck mass and the other presenting with para-aortic and mesenteric lymphadenopathy which were classified as hyaline vascular type and mixed type of CD respectively. Case 1 - A 16 years old male presented with history of right side neck swelling since 5 years.

Results

Conclusion