Staten Island University Hospital Staten island, NY
Samyak Dhruv, MD1, Dhineshreddy Gurala, MD2, Abhishek Polavarapu, MD3, Anupma Agarwal, MD1, Jocelyn D. Villanueva, MD1, Stephen Mulrooney, MD3; 1Staten Island University Hospital, New York, NY; 2Staten Island University Hospital, Northwell Health, Staten Island, NY; 3Staten Island University Hospital, Staten Island, NY
Introduction: Neuroendocrine neoplasm (NEN) is a rare heterogenous neoplasm that originates from the neuroendocrine cells with the incidence of 5.25 per 100,000 people. These tumors are frequently found in the gastrointestinal (GI) tract followed by bronchopulmonary system. In the GI tract appendix, jejunum, ileum and rectum are most frequently involved. Gallbladder NEN is very rare contributing to 0.5% of all NEN and 2.1% of gallbladder cancers, with highest incidence in sixth and seventh decade. NEN are classified into well differentiated neuroendocrine tumor (NET) and poorly differentiated neuroendocrine cancer (NEC). NEC is divided based on histopathology into small cell and large cell NEC (#Figure 1). Large cell NEC is exceedingly rare aggressive tumor with worse prognosis. Herein we report a rare case of Large cell gall bladder NEC with extensive hepatic involvement in a patient presented with constipation.
Methods: A 72-year-old male presented to emergency department with constipation for last three months. Vital signs were stable. On physical examination abdomen was distended and non-tender. Computed tomography (CT) of the abdomen demonstrated ill-defined 7.2X4.9 cm hypodense lesion in the gall bladder fossa with extension into the hepatic parenchyma. Needle biopsy of the gall bladder was performed. Histopathology of the biopsy is shown (# Figure 3). Immunohistochemical analysis showed tumor cells were positive for CD56, synaptophysin, chromogranin, CK 20, Ki67 and negative for TTF1, CK7 favoring gastrointestinal origin of NEC. Positron Emission Tomography (PET) Scan showed extensive involvement (#Figure 2). He was started on palliative chemotherapy and eventually made comfort care per his request. Discussion: Definitive diagnosis of NEN requires histopathology and immunohistochemical analysis. Radiological imaging studies are helpful in staging. Surgery along with chemotherapy is the gold standard for the early detected cancers. Palliative chemotherapy has been used in tumors with advanced stage and widespread metastasis. Increasing tumor size, depth of invasion and metastasis are associated with the worse prognosis. Gastroenterologist should be aware of this rare and aggressive carcinoma and consider NEN gallbladder in the differential diagnosis of gallbladder mass. Differentiating large cell NEC from other types of cancers is essential given the different treatment options and higher mortality rate of NEC approaching 83% in 5 years and median survival of 11 months after diagnosis.
Histopathological classification of the Neuroendocrine Neoplasms. (NEN)
PET scan images showing, FDG (Fluorodeoxyglucose) Avid Liver mass Contiguous with Gallbladder with extensive Retroperitoneal, Portacaval and Left Supraclavicular Lymphadenopathy in our patient with known diagnoses of the Large Cell Neuroendocrine Carcinoma of the Gallbladder.
Right Lobe of the Liver/Gallbladder mass core biopsy showing poorly differentiated carcinoma consistent with Large Cell Neuroendocrine Carcinoma. Foci of individual cell necrosis and Mitosis are present. (H AND E 400X. INSET 40X)
Disclosures: Samyak Dhruv indicated no relevant financial relationships. Dhineshreddy Gurala indicated no relevant financial relationships. Abhishek Polavarapu indicated no relevant financial relationships. Anupma Agarwal indicated no relevant financial relationships. Jocelyn Villanueva indicated no relevant financial relationships. Stephen Mulrooney indicated no relevant financial relationships.