461 - Charting the Course of Rosai-Dorfman: A Case Study

Friday, February 23, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

KK

Karthik B. Kamath, DO

PM&R Resident
Nassau University Medical Center
Coram, New York, United States

Co-Author(s)

LW

Leslie Wong, DO

PM&R Resident Physician
Nassau University Medical Center
East Meadow, New York, United States
JE

Jenzel Espares, DO

PM&R Resident Physician
Nassau University Medical Center
East Meadow, New York, United States
TP

Thomas Pobre, MD

Attending Physician
Nassau University Medical Center
East Meadow, New York, United States

Case Diagnosis: Rosai-Dorfman Disease

Case Description: A previously healthy 27-year-old man sought medical attention for monitoring of a submandibular mass. He started experiencing vomiting with chronic headaches. A noncontrast CT scan of the neck demonstrated a sizable mass in the left cerebellar pontine region and a smaller satellite mass. Subsequent MRI confirmed the presence of a 3.5x2.5x3.3 cm mass, leading to the patient's admission for a left retrosigmoid craniotomy to remove the tumor. Pathology revealed lymphoplasmacytic rich meningioma consistent with Rosai-Dorfman Disease, positive for S100 and presence of emperipolesis. Patient’s postoperative course was complicated with voice hoarseness, dysphagia and balance problems. Patient followed up with PM&R and began outpatient speech therapy and physical therapy. Patient’s balance improved with physical therapy and the patient was transitioned to a home exercise program. Outpatient Oncology followup showed KRAS positive, Extranodal disease, so the patient is to begin chemotherapy.

Discussions: Rosai-Dorfman Disease (RDD) is a benign disorder characterized by the excessive growth of histiocytes, primarily in lymph nodes. It often presents as painless lymph node enlargement, often in the neck region, along with symptoms such as fever, fatigue, night sweats, and weight loss. RDD is more commonly seen in children and young adults. RDD is idiopathic, and treatment may be necessary, which include corticosteroids, surgery, radiation therapy, or chemotherapy. Prognosis is usually favorable, but recurrence is possible.

Conclusions: A cerebellar pontine mass tumor was found incidentally in our young healthy patient on scan evaluation of a submandibular mass. These tumors can cause mass effects in the area leading to problems with balance, facial weakness, difficulty speaking and swallowing. Prompt removal of the tumor likely prevented further debility. This case highlights the benefits of a multidisciplinary team within the diagnosis, treatment and followup of a patient with an extremely rare disorder.