Najiha Farooqi, MD1, Daoning Liu, MD2, Keaton Joppru1, Greta Berger1, Muhammad Maaz, MD1, Chunyi Hao, MD2, James V Harmon, MD, PhD, FACS1. 1University of Minnesota, 2Peking University Cancer Hospital and Institute
Introduction: Castleman’s disease(CD) is a very rare, lymphoproliferative disorder associated with a Rhadinoviral infection of B lymphocytes and can be either unicentric or multicentric in distribution. There an important role for a surgeon in both unicentric and multicentric types. Approximately 1000 cases have been reported to date, we present 28 unpublished cases.
Methods: A retrospective review of cases from two large teaching hospitals was reviewed. Baseline demographics including age, race, gender, clinical variables such as anatomical site and foci of disease, histopathological type, nature of the surgical approach (resective vs diagnostic), and outcome (disease-free survival vs death due to disease) was collected and analyzed.
Results: A total of 28 patient were reviewed for this study. Mean age at the time of presentation was 45.9 yrs. 64.3% of patients were female. 89.3% of the cases were unicentric and 10.7% were multicentric. 57.1% of the patients presented with an asymptomatic mass; 39.3 % had local symptoms and 3.5% patients had systemic symptoms. Anatomical distribution of disease was: 42.9% intrabdominal, 32.1% retroperitoneal, 10.7% neck, 7.14% pelvis and 3.57% axilla and 3.57% in the epitrochlear region. In terms of histopathological type, hyaline vascular accounted for 57.1% of all cases, 17.9 % of cases were of the plasma cell type. Complete surgical resection was performed 96% of patients with unicentric disease. Diagnostic biopsy and medical therapy were provided to all patients with multicentric disease. Overall survival rate was 92.6%.
Conclusion: Castleman’s disease is a very rare tumor; acute care surgeons are likely to have little experience when encountering this tumor. Significant differences exist in the clinical presentation, surgical approach, and patient outcomes between unicentric and multicentric Castleman's Disease. The anatomic distribution of cases in this series emphasizes its importance to an acute care surgeon. Complete surgical resection for unicentric disease is likely to be curative. In multicentric disease, lymph node biopsy should be performed without complications, so as to avoid delay in initiating medical therapy.
Presented at the SAGES 2017 Annual Meeting in Houston, TX.
Abstract ID: 88481
Program Number: P068
Presentation Session: iPoster Session (Non CME)
Presentation Type: Poster