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Published on: September 20, 2018
Castleman disease: A single-center case series
Kyle Pribyl1, Victor Vakayil2, Najiha Farooqi3
1Department of Surgery, University of Minnesota, Minneapolis, MN, USA; University of Minnesota Medical School, Minneapolis, MN, USA.
Insights
Castleman disease (CD) is a rare condition. Surgical excision offers a cure for unicentric CD (UCD), while multicentric CD (MCD) requires multimodal treatment, though surgical benefits for MCD remain unclear.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Castleman disease (CD) is a rare lymphocytic disorder with distinct clinical outcomes based on its type.
- Unicentric CD (UCD) typically has a favorable prognosis post-surgery.
- Multicentric CD (MCD) presents a severe clinical course with poor patient outcomes.
Purpose of the Study:
- To analyze the clinical presentation, treatment, and outcomes of Castleman disease.
- To compare the recurrence and survival rates between unicentric and multicentric CD.
- To evaluate the role of surgery in managing different types of Castleman disease.
Main Methods:
- Retrospective analysis of 28 patients diagnosed with Castleman disease between 1995 and 2017.
- Review of patient demographics, clinical variables, anatomical site, histopathology, and treatment approaches.
- Evaluation of 5-year recurrence and survival rates for UCD and MCD cohorts.
Main Results:
- Castleman disease was diagnosed in 28 patients (57% female, mean age 41.6 years).
- UCD (64%) was often asymptomatic or presented with localized symptoms, while MCD (36%) had a more severe presentation.
- Complete resection achieved 95% 5-year disease-free survival for UCD, contrasted with 33% for MCD; 100% survival was observed in UCD with hyaline vascular variant.
Conclusions:
- Castleman disease is rare, often misdiagnosed due to nonspecific symptoms, necessitating its consideration in differential diagnoses for lymph node hyperplasia.
- Surgical resection is curative for nearly all UCD cases.
- MCD management requires a multimodal approach including surgery, chemotherapy, and immunotherapy, though the specific benefit of cytoreductive surgery for MCD is not yet established.
Background:
Castleman disease (CD) is a rare lymphocytic disorder. Unicentric CD (UCD) has an excellent long-term prognosis after surgical excision; however, multicentric CD (MCD) has a severe clinical course with poor outcomes.
Study Design:
We analyzed the clinical presentation of 28 patients treated at a single institution from 1995 to 2017. Demographics, clinical variables, anatomical site, centricity, histopathology, immunochemistry, and surgical approach were reviewed. We evaluated the 5-year recurrence and survival for patients with UCD and MCD.
Results:
Of the 28 patients, 57 % (n = 16) were female, with a mean age of 41.6 ± 15.6 years. CD was asymptomatic in 57 % (n = 16) of patients, 21 % (n = 6) presented with local symptoms such as pain, and 21 % (n = 6) of patients also had systemic symptoms, including weight loss and fever. CD was unicentric in 64 % (n = 18) and multicentric in 36 % (n = 10). The hyaline vascular variant was noted in 57 % (n = 16) of the tumors, plasmacytoid variant in 36 % (n = 10), and mixed variants in 7% (n = 2) of tumors. Anatomical distributions included: head and neck (20 %), thorax and axilla (24 %), retroperitoneal (13 %), abdominopelvic (30 %) regions, and other (13 %). Complete surgical resection was performed in 95 % of patients with UCD. Surgical biopsy and medical therapy were provided to all patients with MCD. The recurrence rate for UCD and MCD was 6 % (n = 1) and 14 % (n = 1), respectively. The five-year disease-free survival rate for UCD was 95 % (n = 19) and MCD was 33 % (n = 2). We found 100 % survival in patients with UCD and histology demonstrating the HV variant.
Conclusion:
CD is rare and often misdiagnosed due to the absence of specific clinical symptoms. Surgeons should include CD in their differential diagnoses when evaluating patients with lymph node hyperplasia. Surgery can be curative in nearly all patients with UCD. Patients with MCD require a combination of surgical therapy, chemotherapy, and immunotherapy; however, cytoreductive surgery benefits for patients with MCD have not been established.
