Dr St. Joseph's Regional Medical Center Paterson , NJ
Elias Estifan, MD1, Mina Fransawy Alkomos, MD2, Gabriel Melki, MD2, Youssef Botros, MD2, Yana Cavanagh, MD2, Matthew Grossman, MD1; 1St. Joseph Regional Medical Center, Paterson, NJ; 2St. Joseph's Regional Medical Center, Paterson, NJ
Introduction: Neuroendocrine gallbladder neoplasms (NENs) are rare. They account for only 0.5% of all NENs and 2.1% of gallbladder cancers. The current WHO classification of NENs of the gastrointestinal and hepatopancreatobiliary tract includes: Neuroendocrine tumors (grade 1, grade 2, and grade 3), Neuroendocrine carcinoma (large cell and small cell ), and Mixed adeno-neuroendocrine carcinoma. Only 16 cases of pure Large cell neuroendocrine neoplasms (LCNEC) without other histologic components have been reported. Therefore, the clinical manifestation, biological behavior, management, and overall patient prognosis remains unclear.
Methods: A 73-year-old female with a past medical history of hypertension, moderate persistent asthma, diabetes type 2, and morbid obesity, presented with complaints of intermittent right upper quadrant (RUQ) pain of two days associated with nausea and one episode of nonbilious non-bloody emesis. The physical exam was significant for RUQ tenderness. No guarding or rigidity was present. Bloodwork was unremarkable. The abdominal US revealed gallbladder stones with nonspecific gallbladder wall thickening and no evidence of infection or obstruction. CT scan of the abdomen confirmed gallstones, with enlarged retroperitoneal lymph nodes measuring up to 2.2 x 1.4 cm. The patient underwent routine laparoscopic cholecystectomy under the diagnosis of cholecystitis and was discharged on a postoperative day 3 with no complications. Interestingly, a mass measuring 2 x 1.7 x 1 cm was identified in the fundus of the gallbladder specimen. Pathology confirmed the presence of large cell neuroendocrine carcinoma [Figure 1,2], with immunohistochemical stain positive for pan-cytokeratin, synaptophysin [Figure 3]. Subsequently, the patient underwent a PET/CT gallium 68 scans that revealed regional lymphadenopathy. Following a multi-disciplinary conference discussion, the decision to start chemotherapy was pursued. The patient received four cycles of Etoposide and Carboplatin with no local recurrence at six months follow-up. Discussion: Limited data is available to support any management approach for LCNEC. However, aggressive surgical management with a multimodal approach appears to increase overall survival. We hope to add to the growing body of evidence in defining this pathology as the scientific community better understands the biological behavior, management, and prognosis of pure large cell neuroendocrine carcinoma of the gallbladder.
Figure 1 Simple columnar epithelium of the gall bladder (white arrow), necrotic tumor tissue with chronic inflammatory cells ( green arrow ).
Figure 2 (Large cell neuroendocrine carcinoma under a high power microscope. Tumor cells have abundant eosinophilic cytoplasm, coarsely granular chromatin, and prominent nucleoli).
Figure 3 ( Immunohistochemical stain positive for Synaptophysin ).
Disclosures: Elias Estifan indicated no relevant financial relationships. Mina Fransawy Alkomos indicated no relevant financial relationships. Gabriel Melki indicated no relevant financial relationships. Youssef Botros indicated no relevant financial relationships. Yana Cavanagh indicated no relevant financial relationships. Matthew Grossman indicated no relevant financial relationships.