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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.

International Journal of Surgery Case Reports
|February 25, 2021
PubMed
Summary

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.

Keywords:
Hyaline vascularMulticentric Castleman diseasePlasmacytoidUnicentric Castleman disease

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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.