P2930 - Curious Case of a Pancreatic Mass - Case Report and Review of Literature

Kavya Kelagere Mayigegowda, MD¹, Aayushi Patel, MBBS², Vaibhav Wadhwa, MD¹, Nirav Thosani, MD^3
1University of Texas Health Science Center at Houston, Houston, TX; ²UT Health, Houston, TX; ³McGovern Medical School at UTHealth, Houston, TX

Introduction: Rosai–Dorfman disease (RDD) is primarily manifested as massive cervical lymphadenopathy. Gastrointestinal involvement occurs in less than 1% of cases. RDD in the pancreas is very rare, with only a handful of cases previously described. Herein, we report a case of pancreatic head mass as an extra nodal presentation of this disease.

Case Description/Methods: A 70-year-old female with a past medical history of Sjogren syndrome and left suprasellar mass was referred after an abnormal PET scan. Patient had a history of suprasellar mass and was previously treated with chemotherapy. A surveillance PET CT showed a new uptake at the level of the pancreatic head. Subsequently, a pancreatic protocol MRI showed 2.2cm x 1.9cm pancreatic head mass. Patient underwent an endoscopic ultrasound (EUS) with fine needle biopsy which showed a 15 mm x 21 mm homogenous well-circumscribed mass in the head of the pancreas. The surrounding pancreatic parenchyma was normal without evidence of atrophy. Using doppler guidance to confirm avascular access, 4 passes of the mass were performed using a 22-gauge biopsy needle. Pathology showed significant stromal fibrosis, mixed inflammatory cells including many plasma cells, and some reactive ducts. CD-68 staining showed multiple histiocytes.

Discussion: RDD is a rare idiopathic disorder of proliferative histiocytes which is now classified under R group non-Langerhans histiocytosis. First described in 1965 by Destombes and later in 1969 by Rosai and Dorfman, it is primarily manifested as a massive cervical lymphadenopathy. Extra-nodal involvement is typically seen in about 43% of cases. Gastrointestinal involvement is very rare but occurs in less than 1%. RDD in the pancreas is very rare, with only 12 cases previously described. Diagnosis is confirmed by sampling of nodal or extra-nodal disease. Typical findings seen on histology are sinusoidal expansion, infiltrates of plasma cells, histiocytes and lymphocytes. Few key clinicopathological characteristics are emperipolesis and positive immunostaining for S-100 protein and CD68. Sporadic RDD is usually self-limited and has a good outcome, with spontaneous remission reported in up to 50% of cases. Systemic therapies include corticosteroids, sirolimus, radiotherapy, chemotherapy and immunomodulatory therapy. Extra nodal RDD should be considered in differential diagnosis while evaluating nonmalignant solid pancreatic tumors.


Disclosures:


Kavya Kelagere Mayigegowda, MD¹, Aayushi Patel, MBBS², Vaibhav Wadhwa, MD¹, Nirav Thosani, MD³. P2930 - Curious Case of a Pancreatic Mass - Case Report and Review of Literature, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.