Castleman's Disease in the Head and Neck Region: Meta-analysis of Reported Cases in Taiwan and Literature Review

Background/Purpose: Castleman's disease (CD) is a rare and complex disease that involves the head and neck region. In our hospital, only three cases of CD in the head and neck region have been encountered in the past 20 years. Methods: To establish a database of Castleman's disease in Taiwan and to compare it to published western studies, we collected case reports of CD in the head and neck region in Taiwan from 1992 to 2007. We used "Castleman's disease" and "Taiwan" as keywords to search medical journal databases. A total of 15 cases were incorporated in our study, including three from our own hospital. These data were integrated for studying the disease characteristics in Taiwan. Results: Submandibular area (level 1) was the most commonly involved site (5 cases, 33.3%). The ratio of unicentric to multicentric CD and the proportion of histological classifications were similar to those in the western studies included in our review. All of the unicentric CD treated by complete resection showed no evidence of recurrence. Multicentric CD was present in two patients, although one died following a heart attack. The other multicentric CD patient was treated by medication and survived for several years. Conclusion: The features of head and neck CD in Taiwan are similar to those reported in western studies. Although CD is not a common disease, it should be considered as a differential diagnosis in patients with a neck mass, especially when the patient is suffering from long-term inflammation. Multicentric CD is also likely to be initially misdiagnosed as unicentric CD. It is important to remember that all patients diagnosed with CD should receive a systemic survey to exclude the possibility of ignored lesions.