Castleman disease: A single-center case series.

Hyaline vascular Multicentric Castleman disease Plasmacytoid Unicentric Castleman disease

Journal

International journal of surgery case reports
ISSN: 2210-2612
Titre abrégé: Int J Surg Case Rep
Pays: Netherlands
ID NLM: 101529872

Informations de publication

Date de publication:
Mar 2021
Historique:
received: 04 01 2021
revised: 09 02 2021
accepted: 11 02 2021
pubmed: 26 2 2021
medline: 26 2 2021
entrez: 25 2 2021
Statut: ppublish

Résumé

Castleman disease (CD) is a rare lymphocytic disorder. Unicentric CD (UCD) has an excellent long-term prognosis after surgical excision; however, multicentric CD (MCD) has a severe clinical course with poor outcomes. We analyzed the clinical presentation of 28 patients treated at a single institution from 1995 to 2017. Demographics, clinical variables, anatomical site, centricity, histopathology, immunochemistry, and surgical approach were reviewed. We evaluated the 5-year recurrence and survival for patients with UCD and MCD. Of the 28 patients, 57 % (n = 16) were female, with a mean age of 41.6 ± 15.6 years. CD was asymptomatic in 57 % (n = 16) of patients, 21 % (n = 6) presented with local symptoms such as pain, and 21 % (n = 6) of patients also had systemic symptoms, including weight loss and fever. CD was unicentric in 64 % (n = 18) and multicentric in 36 % (n = 10). The hyaline vascular variant was noted in 57 % (n = 16) of the tumors, plasmacytoid variant in 36 % (n = 10), and mixed variants in 7% (n = 2) of tumors. Anatomical distributions included: head and neck (20 %), thorax and axilla (24 %), retroperitoneal (13 %), abdominopelvic (30 %) regions, and other (13 %). Complete surgical resection was performed in 95 % of patients with UCD. Surgical biopsy and medical therapy were provided to all patients with MCD. The recurrence rate for UCD and MCD was 6 % (n = 1) and 14 % (n = 1), respectively. The five-year disease-free survival rate for UCD was 95 % (n = 19) and MCD was 33 % (n = 2). We found 100 % survival in patients with UCD and histology demonstrating the HV variant. CD is rare and often misdiagnosed due to the absence of specific clinical symptoms. Surgeons should include CD in their differential diagnoses when evaluating patients with lymph node hyperplasia. Surgery can be curative in nearly all patients with UCD. Patients with MCD require a combination of surgical therapy, chemotherapy, and immunotherapy; however, cytoreductive surgery benefits for patients with MCD have not been established.

Sections du résumé

BACKGROUND BACKGROUND
Castleman disease (CD) is a rare lymphocytic disorder. Unicentric CD (UCD) has an excellent long-term prognosis after surgical excision; however, multicentric CD (MCD) has a severe clinical course with poor outcomes.
STUDY DESIGN METHODS
We analyzed the clinical presentation of 28 patients treated at a single institution from 1995 to 2017. Demographics, clinical variables, anatomical site, centricity, histopathology, immunochemistry, and surgical approach were reviewed. We evaluated the 5-year recurrence and survival for patients with UCD and MCD.
RESULTS RESULTS
Of the 28 patients, 57 % (n = 16) were female, with a mean age of 41.6 ± 15.6 years. CD was asymptomatic in 57 % (n = 16) of patients, 21 % (n = 6) presented with local symptoms such as pain, and 21 % (n = 6) of patients also had systemic symptoms, including weight loss and fever. CD was unicentric in 64 % (n = 18) and multicentric in 36 % (n = 10). The hyaline vascular variant was noted in 57 % (n = 16) of the tumors, plasmacytoid variant in 36 % (n = 10), and mixed variants in 7% (n = 2) of tumors. Anatomical distributions included: head and neck (20 %), thorax and axilla (24 %), retroperitoneal (13 %), abdominopelvic (30 %) regions, and other (13 %). Complete surgical resection was performed in 95 % of patients with UCD. Surgical biopsy and medical therapy were provided to all patients with MCD. The recurrence rate for UCD and MCD was 6 % (n = 1) and 14 % (n = 1), respectively. The five-year disease-free survival rate for UCD was 95 % (n = 19) and MCD was 33 % (n = 2). We found 100 % survival in patients with UCD and histology demonstrating the HV variant.
CONCLUSION CONCLUSIONS
CD is rare and often misdiagnosed due to the absence of specific clinical symptoms. Surgeons should include CD in their differential diagnoses when evaluating patients with lymph node hyperplasia. Surgery can be curative in nearly all patients with UCD. Patients with MCD require a combination of surgical therapy, chemotherapy, and immunotherapy; however, cytoreductive surgery benefits for patients with MCD have not been established.

Identifiants

DOI: 10.1016/j.ijscr.2021.105650 PMID: 33631648 PMC: PMC7907481
pubmed: 33631648
pii: S2210-2612(21)00147-4
doi: 10.1016/j.ijscr.2021.105650
pmc: PMC7907481
pii:
doi:

Types de publication

Journal Article

Langues

eng

Pagination

105650

Informations de copyright

Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.

Auteurs

Kyle Pribyl (K)

Department of Surgery, University of Minnesota, Minneapolis, MN, USA; University of Minnesota Medical School, Minneapolis, MN, USA.

Victor Vakayil (V)

Department of Surgery, University of Minnesota, Minneapolis, MN, USA.

Najiha Farooqi (N)

Department of Surgery, Central Michigan University, Saginaw, MI, USA.

Nivedita Arora (N)

Department of Medicine, Division of Hematology & Oncology, University of Minnesota, Minneapolis, MN, USA.

Benjamin Kreitz (B)

University of Minnesota Medical School, Minneapolis, MN, USA.

Salman Ikramuddin (S)

Department of Neurology, University of Minnesota, Minneapolis, MN, USA.

Michael A Linden (MA)

Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, MN, USA.

James Harmon (J)

Department of Surgery, University of Minnesota, Minneapolis, MN, USA; University of Minnesota Medical School, Minneapolis, MN, USA. Electronic address: harm0031@umn.edu.

Classifications MeSH