A retrospective study of 34 patients with unicentric and multicentric Castleman's disease: Experience from a single
Jin-Peng Jiang1,2, Xiao-Fei Shen2, Jun-Feng Du3
1Department of Rehabilitation Medicine, General Hospital of Beijing Military Command, Beijing 100700, P.R. China.
Insights
This study on Castleman's disease (CD) found complete surgical resection is the standard treatment for Unicentric CD (UCD). For Multicentric CD (MCD), molecular targeted therapy and stem cell transplantation show promise.
Area of Science:
- Oncology
- Hematology
- Surgical Pathology
Background:
- Castleman's disease (CD) is a rare lymphoproliferative disorder.
- CD presents in two main forms: Unicentric CD (UCD) and Multicentric CD (MCD).
- Accurate diagnosis and tailored treatment strategies are crucial for managing CD.
Purpose of the Study:
- To share single-institute experience in diagnosing and treating Castleman's disease.
- To evaluate treatment strategies for both Unicentric CD (UCD) and Multicentric CD (MCD).
- To analyze outcomes based on disease classification and treatment modalities.
Main Methods:
- Retrospective analysis of 34 CD patients hospitalized between 2006 and 2014.
- Classification into UCD (27 patients) and MCD (7 patients) based on anatomical distribution.
- Data collection through medical records review and patient telephone contact; histological confirmation via lymph node biopsy.
Main Results:
- All 27 UCD patients treated with surgical resection survived, barring one unrelated death.
- 5 MCD patients received varied treatments including chemotherapy, targeted therapy, and stem cell transplantation.
- 3 MCD patients survived with a median follow-up of 69 months.
Conclusions:
- Complete surgical resection remains the standard treatment for UCD.
- Multidetector CT and laparoscopic approaches offer advantages in UCD management.
- Molecular targeted therapy and hematopoietic stem cell transplantation show potential for MCD treatment.
Abstract:
The aim of the present study was to share the experience of a single institute in the diagnosis, use of accessory examinations and treatment strategies of Castleman's disease (CD). The present study analyzed 34 patients (13 males and 21 females) with CD who were hospitalized between January 2006 and September 2014. The patients were divided into two groups based on the anatomical distribution of the disease: Unicentric CD (UCD) and multicentric CD (MCD). Histological data was obtained from lymph node biopsies. All clinical data were acquired by reviewing patients' medical records and contacting patients by telephone. A total of 27 patients had UCD and 7 patients had MCD. All 27 patients with UCD with benign symptoms underwent complete diagnostic surgical resection and survived, with the exception of 1 patient who succumbed to pancreatic head carcinoma 13 months after surgery. A total of 7 patients with MCD presented with systemic symptoms and 2 of these patients declined treatment following the definite diagnosis of CD. The remaining 5 patients were treated with various strategies, including surgical resection and further glucocorticoid treatment, intravenous siltuximab, rituximab in combination with cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy or hematopoietic stem cell transplantation. A total of 3 patients with MCD survived, with a median follow-up period of 69 months. The present study indicates that complete surgical resection is currently the standard treatment for UCD. Perioperative use of multidetector computed tomography and the laparoscopic approach have certain advantages in UCD. Molecular target therapy is effective in patients with stable MCD, and hematopoietic stem cell transplantation may be beneficial in certain patients with MCD and disease progression.
