Amit R. Hudgi, MBBS1, Mahima Zandu, MBBS2, Lakshmi K. Vemavarapu, MD3, Subbaramiah Sridhar, MBBS, MPH, FRCP, FRCPC, FACG4, Eula P. Tetangco, MD, MPH5, Jayanth Keshavamurthy, MD3; 1Augusta University Medical Center, Augusta, GA; 2Mahatma Gandhi Medical College, Jhamshedpur, Jharkhand, India; 3Charlie Norwood VA Medical Center, Augusta, GA; 4AUMC, Augusta, GA; 5Medical College of Georgia at Augusta University, Augusta, GA
Introduction: Rhabdomyosarcoma is rare in adults, with even fewer patients presenting with pancreatic metastasis. While most cases are asymptomatic, they may present as pancreatitis or bowel obstruction.
Methods: A 63-year old African American male with seizure disorder, hypertension, alveolar rhabdomyosarcoma of the right sinus 6 months prior, presented with constipation. He had undergone maxillectomy and selective bilateral neck dissection with ongoing adjuvant chemotherapy. He denied difficulty swallowing, abdominal pain and distention, nausea, vomiting, jaundice, bloody or acholic stools, and loss of appetite. He was a former smoker with 30 pack-years of smoking cigarettes and former alcoholic abstinent for the past 30 years. Examination revealed a thin, cachectic appearing man with no localized abdominal tenderness or organomegaly. Bloodwork revealed anemia with hemoglobin of 9.4 g/dL and a Mean Corpuscle Volume (MCV) of 83.5. Serum electrolytes, hepatic and pancreatic enzymes were within normal limits. Magnetic resonance imaging (MRI) of the abdomen showed a hypo-enhancing mass in the pancreatic tail measuring 2.7 x 2.2 cm. Esophageal endoscopic ultrasound (EUS) confirmed a heterogeneous lesion in the tail of the pancreas. Fine needle aspiration (FNA) confirmed cytomorphologic features consistent metastatic rhabdomyosarcoma. Discussion: Rhabdomyosarcoma (RMS) is an aggressive and highly malignant cancer that develops from skeletal (striated) muscle cells, commonly seen in children. Rarely seen in adults, accounting for 3% of all adult soft tissue sarcomas. The rarity of detection of pancreatic metastasis is linked to the fact that most cases are asymptomatic as reported in a case series. One of the studies showed an incidence of 67% in children having RMS on autopsy with no clinical or radiological diagnosis of the metastasis. 18F-fluoro-2-deoxy-D-glucose Positron Emission Tomography- Computed Tomography (FDG PET-CT) scan is most accurate in diagnosing and staging RMS. Endoscopic Ultrasound (EUS)-guided Fine Needle Aspiration (FNA) gives the definitive diagnosis, provides vital information for treatment planning, and rules out other diagnoses for pancreatic lesions. Studies have shown that adults with RMS treated in accordance with multimodality approaches -adopted from pediatric protocol have shown significant improvement in survival. Accurate staging is important as metastatic disease have a poor prognosis and with more aggressive treatment approach.
CT abdomen: Mass on T1 slightly more intense than liver
PET/CT - Metabolically active mass in the tail of the pancreas
Disclosures: Amit Hudgi indicated no relevant financial relationships. Mahima Zandu indicated no relevant financial relationships. Lakshmi Vemavarapu indicated no relevant financial relationships. Subbaramiah Sridhar indicated no relevant financial relationships. Eula Tetangco indicated no relevant financial relationships. Jayanth Keshavamurthy indicated no relevant financial relationships.