N Zhou1, C W Huang, C Huang, W Liao. 1. Department of Dermatology, Changzheng Hospital, and Fudan University, Shanghai, China.
Abstract
OBJECTIVE: Castleman's disease (CD) is a rare disorder of unknown aetiology characterized by lymph node enlargement. This study evaluated the clinical features, diagnosis and outcomes of surgical treatment in patients with CD. METHODS: The medical records of 10 patients with histologically confirmed CD who underwent surgery between 2003 and 2008 were retrospectively reviewed. Study endpoints were survival and recurrence. RESULTS: The age of disease onset ranged from 11 to 63 years (median 38 years). Of the 10 patients included in the study, eight were diagnosed with localized (unicentric) CD and two with multicentric CD. All eight patients with localized (unicentric) CD were asymptomatic. Histological evidence indicated the hyaline vascular form of CD in eight patients and the plasmacytic form in the remaining two patients. The primary treatment was complete surgical resection in nine cases, and these patients were alive with no evidence of recurrence or exacerbation at the time of writing (June 2011). CONCLUSIONS: Localized (unicentric) CD is usually of the hyaline vascular form and complete surgical excision of the tumour allows full recovery in most cases.
OBJECTIVE:Castleman's disease (CD) is a rare disorder of unknown aetiology characterized by lymph node enlargement. This study evaluated the clinical features, diagnosis and outcomes of surgical treatment in patients with CD. METHODS: The medical records of 10 patients with histologically confirmed CD who underwent surgery between 2003 and 2008 were retrospectively reviewed. Study endpoints were survival and recurrence. RESULTS: The age of disease onset ranged from 11 to 63 years (median 38 years). Of the 10 patients included in the study, eight were diagnosed with localized (unicentric) CD and two with multicentric CD. All eight patients with localized (unicentric) CD were asymptomatic. Histological evidence indicated the hyaline vascular form of CD in eight patients and the plasmacytic form in the remaining two patients. The primary treatment was complete surgical resection in nine cases, and these patients were alive with no evidence of recurrence or exacerbation at the time of writing (June 2011). CONCLUSIONS: Localized (unicentric) CD is usually of the hyaline vascular form and complete surgical excision of the tumour allows full recovery in most cases.
Authors: Frits van Rhee; Eric Oksenhendler; Gordan Srkalovic; Peter Voorhees; Megan Lim; Angela Dispenzieri; Makoto Ide; Sophia Parente; Stephen Schey; Matthew Streetly; Raymond Wong; David Wu; Ivan Maillard; Joshua Brandstadter; Nikhil Munshi; Wilbur Bowne; Kojo S Elenitoba-Johnson; Alexander Fössa; Mary Jo Lechowicz; Shanmuganathan Chandrakasan; Sheila K Pierson; Amy Greenway; Sunita Nasta; Kazuyuki Yoshizaki; Razelle Kurzrock; Thomas S Uldrick; Corey Casper; Amy Chadburn; David C Fajgenbaum Journal: Blood Adv Date: 2020-12-08