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

Abstract

Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder presenting as a single nodal mass with characteristic histopathology. Patients with UCD are typically asymptomatic with normal laboratory markers, whereas patients with multicentric Castleman disease (MCD) demonstrate multicentric lymphadenopathy and cytokine storm–induced systemic inflammatory symptoms. This retrospective analysis of 116 UCD cases identified 19 (16.4%) cases with an MCD-like inflammatory state (UCD-MIS). We compared treatments and outcomes between cases of UCD-MIS and UCD–non-MIS to evaluate the role of surgery and illuminate biological behavior of UCD-MIS. There were differences in the distribution of histopathological subtypes (plasmacytic histopathology was more frequently seen, 52.6% vs 13.4%; P < .001) between the 2 groups. However, both groups demonstrated good responses to surgical treatment, suggesting that UCD-MIS in some patients still shared common biological behavior with UCD in other patients. Sixteen (94.2%) patients with UCD-MIS underwent complete surgical excision alone, and the systemic inflammation resolved completely in all of them. This high response rate suggests surgical treatment as a potential cure for this unique subset of patients. After a median follow-up duration of 64 months (range, 2-239 months), neither lymphadenopathy nor the inflammatory state recurred. However, inflammation may progress in patients with irresectable disease, and treatment options other than surgery should be considered in these patients.

Ongoing symptoms following complete surgical excision in unicentric Castleman disease

“…Though CLE was less effective in this cohort than has been reported in the literature, our data support it as the first-line therapy for UCD. 1,2 We observed a clinical response in 32/53 (74.4%) patients, which is marginally lower than that reported in past literature reviews ($96.0%). 1 There are several limitations to this study.…”

A national, multicenter, retrospective study of Castleman disease in China implementing CDCN criteria

“…For HHV-8-negative MCD, patients who fulfilled both major criteria and at least 2 of 11 minor criteria proposed by the CDCN and did not meet any of the exclusion criteria were diagnosed with iMCD; patients who did not have symptoms or a hyperinflammatory state and thus did not meet the minor diagnostic criteria of iMCD were considered to have asymptomatic MCD (aMCD) according to the China Castleman Disease Network (CCDN) consensus. 3 UCD patients with an MCD-like inflammatory state were defined as UCD-MIS, 13 and iMCD patients were further classified into iMCD-not otherwise specified (iMCD-NOS) and iMCD-TAFRO groups. TAFRO was an acronym for thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis of bone marrow (R) and organomegaly (O).…”

Recent Advances in Castleman Disease

“…Although all lymph node regions of the body may be affected, UCD tends to be a solitary mass in the mediastinum. Of note, constitutional symptoms such as weight loss, fever, or fatigue occur in 10-20% of cases [51].…”

“…Complete surgical excision will usually eliminate any systemic symptomatology and laboratory abnormalities, if present [4,51]. Surgery is also successful in pediatric cases [11].…”