UCD with MCD-like inflammatory state: surgical excision is highly effective.

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder.
• Multicentric Castleman disease (MCD) presents with systemic inflammation.
• A subset of UCD cases exhibit MCD-like inflammatory symptoms (UCD-MIS).

Purpose of the Study:

• To compare treatments and outcomes for UCD-MIS versus UCD-non-MIS.
• To evaluate the role of surgery in managing UCD-MIS.
• To understand the biological behavior of UCD-MIS.

Main Methods:

• Retrospective analysis of 116 UCD cases.
• Identification of 19 cases with UCD-MIS.
• Comparison of histopathological subtypes, treatments, and outcomes between UCD-MIS and UCD-non-MIS groups.

Main Results:

• UCD-MIS cases showed a higher prevalence of plasmacytic histopathology (52.6% vs 13.4%).
• Complete surgical excision in 16 UCD-MIS patients led to complete resolution of systemic inflammation.
• No recurrence of lymphadenopathy or inflammatory state was observed after a median follow-up of 64 months.

Conclusions:

• Surgical treatment offers a potential cure for UCD-MIS with resectable disease.
• UCD-MIS shares some biological behaviors with typical UCD.
• Alternative treatments should be considered for unresectable UCD-MIS due to potential disease progression.