Clinical and Pathological Characteristics of Hyaline-Vascular Type Unicentric Castleman Disease: A 20-Year Retrospective Analysis

Abstract

The first case of hyaline vascular type of unicentric Castleman disease (HV-UCD) was reported more than six decades ago. Since patients with HV-UCD are often asymptomatic and this condition is generally discovered incidentally on imaging tests, most of the previous reports were of mediastinal origin detected by chest radiography. In recent years, improved access to imaging modalities has provided new insights in the diagnosis of this condition. In this study, we reviewed the detailed clinical and pathological findings of 38 HV-UCD cases (20 males and 18 females, mean age: 42.8 years). The most common site involved was the abdominal cavity (34.2%), followed by mediastinum (23.7%) and retroperitoneum (15.8%). In the abdominal cavity, mesenteric origin was the most common. The mean size of masses was 4.8 cm. Pathologically, thick hyalinized collagen fibers surrounding large blood vessels and calcification were observed (81.6% and 23.7%, respectively). Multinucleated giant cells resembling Warthin–Finkeldey cell were also observed in occasional cases (23.7%). This is a unique paper that summarizes detailed clinical and pathological findings of a large series of a rare disease. The clinical information presented in this paper is more plausible than previous views and is useful for accurate diagnosis and understanding of the disease.

Historical and pathological overview of Castleman disease

“…However, we recently reported that intra-abdominal and retroperitoneal origins are more frequent than previously thought. 36 In the cohort of 38 cases, the most common location was the abdominal cavity (34.2%), followed by mediastinal (23.7%) and retroperitoneal (15.8%) regions. 36 In the abdominal cavity, the mesenteric origin is the most common and is often clinically excised due to suspicion of gastrointestinal stromal tumor, leiomyoma, or neurogenic tumor.…”

“… 36 In the cohort of 38 cases, the most common location was the abdominal cavity (34.2%), followed by mediastinal (23.7%) and retroperitoneal (15.8%) regions. 36 In the abdominal cavity, the mesenteric origin is the most common and is often clinically excised due to suspicion of gastrointestinal stromal tumor, leiomyoma, or neurogenic tumor. The mean size of the lesions is approximately 5.0 cm, 35 , 36 which is larger than the lymph nodes observed in MCD.…”

“…In approximately one-fourth of cases, atypical dendritic cells can be found in the germinal center ( Figure 4a ) and interfollicular area ( Figure 4b ), often multinucleated and resembling Warthin-Finkeldey cells. 36 These cells are positive for follicular dendritic cell markers such as CD21 ( Figure 4c ) and CXCL13 ( Figure 4d ). 15 , 36 - 40 …”

“… 36 In the abdominal cavity, the mesenteric origin is the most common and is often clinically excised due to suspicion of gastrointestinal stromal tumor, leiomyoma, or neurogenic tumor. The mean size of the lesions is approximately 5.0 cm, 35 , 36 which is larger than the lymph nodes observed in MCD.…”

Unicentric Castleman Disease and Pheochromocytoma

“…Systemic symptoms are present in a small percentage of cases of UCD, and IL-6 appears to be the responsible cytokine, being better characterized in MCD, where these symptoms predominate [ 3 , 5 ] .…”