Multidisciplinary Clinical Case Study

A Multidisciplinary Approach to Castleman Disease of the Neck

Authors: Alexandra A. Shams, BS, Mostafa M. Ahmed, MD, Nicholas J. Scalzitti, MD, Della L. Howell, MD, Jordan M. Hall, MD, John L. Ritter, MD, Stephen C. Maturo, MD


Castleman disease (CD) is a rare lymphoproliferative disorder that occurs in adults and rarely in the pediatric population. The disease is characterized by slowly enlarging masses that can form anywhere within the lymphatic system. It is an uncommon cause of a neck mass in both children and adults that presents insidiously and nonspecifically.

A 21-year-old woman was referred to the otolaryngology service because of an asymptomatic neck mass found incidentally on computed tomographic imaging 15 months earlier. On repeat imaging, the lesion was characterized as a homogenously enhancing soft tissue mass and appeared stable in size compared with previous studies. Given the nondiagnostic radiologic features, tissue sampling was pursued, first using fine-needle aspiration and ultimately excisional biopsy. The excision revealed histopathology consistent with unicentric, hyaline-vascular CD. Excision is the gold standard for treatment of this variant of CD. The patient was referred to the hematology/oncology service but was subsequently lost to follow-up.

This case illustrates a rare cause of a neck mass in a young adult and exemplifies the extremely broad differential in this setting. In addition, it highlights the importance of a systematic and thorough approach to diagnosing neck masses in children and adults

This content is limited to qualifying members.

Existing members, please login first.

If you have an existing account please login now to access this article or view your purchase options.

Purchase only this article ($15)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.


1. Curtis WJ, Edwards SP. Pediatric neck masses. Atlas Oral Maxillofac Surg Clin North Am 2015;23:15-20.
2. Badaway MK. Pediatric neck masses.Clin Pediatr EmergMed 2010;11:73-80.
3. Amedee RG, Dhurandhar NR. Fine-needle aspiration biopsy. Laryngoscope 2001;111:1551-1557.
4. Anne S, Teot LA, Mandell DL. Fine needle aspiration biopsy: role in diagnosis of pediatric head and neck masses. Int J Pediatr Otorhinolaryngol 2008;72:1547-1553.
5. Rabinowitz MR, Levi J, Conard K, et al. Castleman disease in the pediatric neck: a literature review. Otolaryngol Head Neck Surg 2013;148:1028-1036.
6. Zawawi F, Varshney R, Haegert DG, et al. Castleman’s disease: a rare finding in a pediatric neck.Int J Pediatr Otorhinolaryngol 2014;78:370-372.
7. Ramsay AD. Reactive lymph nodes in pediatric practice. Am J Clin Pathol 2004;122(Suppl):S87-S97.
8. O’Malley DP, George TI, Orazi A, et al. Specific clinical entities. In: King DW, ed. Atlas of Nontumor Pathology: Benign and Reactive Conditions of Lymph Node and Spleen. Washington, DC: American Registry of Pathology/Armed Forces Institute of Pathology, 2009, pp 155-164.
9. Talat N, Belgaumkar AP, Schulte KM. Surgery in Castleman’s disease: a systematic review of 404 published cases. Ann Surg 2012;255:677-684.
10. Matthiesen C, Ramgopol R, Seavey J, et al. Intensity modulated radiation therapy (IMRT) for the treatment of unicentric Castleman’s disease: a case report and review of the use of radiotherapy in the literature. Radiol Oncol 2012;46:265-270.