1979 GI Bleed Secondary to Gangliocytic Paraganglioma Treated With Radiation Monotherapy

Abstract

INTRODUCTION: Gangliocytic Paraganglioma (GP) is a rare, usually benign tumor typically found in the 2nd part of the duodenum. The rarity of the tumor hasn’t allowed development of treatment guidelines. Mainstay of treatment involves endoscopic or surgical resection. Adjuvant chemotherapy or radiation has been utilized in a few cases. The case described is of a patient with GP presenting with hematochezia. The patient’s gastrointestinal (GI) bleed was treated with radiotherapy alone, which is the first case to our knowledge in the literature. CASE DESCRIPTION/METHODS: A 78 year old male with past medical history of multiple comorbid cardiovascular conditions and Multiple Myeloma presented with a six month history of hematochezia. Complete Blood Count showed a hemoglobin (hgb) of 8.6 g/dL with a baseline hgb of 11 g/dL four months prior. Esophagogastroduodenoscopy (EGD) showed a 3 cm ulcerated, friable periampullary mass. Biopsies demonstrated epithelioid, spindle and “large” cells associated with GP. Computed Tomography (CT) scan of the abdomen showed a 5.1 cm soft tissue density appearing to originate from the pancreatic head and abutting the duodenum with no regional lymphadenopathy or distant metastasis (mets). Due to the patient’s comorbidities and high perioperative mortality risk, radiation monotherapy was pursued for treatment. After fourteen radiotherapy sessions, the patient’s GI bleed resolved. Repeat EGD showed decreased vascular markings in the duodenal mass. DISCUSSION: GP is a rare tumor composed of epithelioid, spindle and ganglion cells. Tumor behavior is usually benign with rare invasion of nearby structures and lymph node mets. CT scan and endoscopic ultrasound (EUS) are generally recommended for staging. Our patient underwent CT Scan, but EUS was deferred due to little utility with an increased perioperative mortality. Local surgical resection is the most common treatment, but endoscopic resection has shown lower morbidity and mortality in selected cases. The size of the tumor and invasion depth dictates treatment modality. Our patient’s tumor size (>5 cm) and pancreatic head origin would have necessitated pancreaticoduodenectomy in a healthy patient. Due to his comorbid conditions and positive literature data on adjuvant radiotherapy for benign paragangliomas of the pancreatic head and neck, our patient underwent successful monotherapy with radiation for control of bleeding. This case shows benefit of radiotherapy alone for control of bleeding due to GP in high risk surgical patients.