Abstract:
Objective: In this study, we aimed to investigate the clinicopathological features and survival of CD, which is quite rare and has many unknowns. Patients and Methods: This study was conducted by retrospectively evaluating patients diagnosed with CD in six different centers in Turkey. Results: The median age of 33 patients included in the study was 49 and 51.5% (n = 17) of these patients were women. 18 (54.5%) patients were in the hyaline vascular subtype and most of the patients were UCD (n = 20, 60.6%). The most common involvement region was head and neck (n = 19, 57.5%). The UCD group was younger than the MCD group (p=0.027). Visceral lymph node involvement was higher in MCD than in UCD (p=0.001). Similarly, it was observed that there was more hepatomegaly (p=0.035) and splenomegaly (p=0.013) in the MCD group. During the median 19.5 months follow-up period, there were no patients who died. Conclusions: It was observed that UCD and MCD are different clinical entities. Promising survival times can be achieved with surgical and systemic treatments in both subtypes of this extremely rare disease. However, this result should be supported by well-designed prospective comprehensive studies. © 2022 Verduci Editore s.r.l. All rights reserved.