Introduction: Small cell carcinoma most commonly originates in the lung. Colorectal small cell carcinoma is very rare, comprising 0.2% of all colorectal cancers. The incidence of small cell lung cancer (SCLC) was found to be 22-times that of extrapulmonary small cell cancer. Colorectal SCC is highly aggressive and carries a poor prognosis. Case Description/Methods: Patient is a 42-year-old female with a history of GERD and tobacco abuse who presented for rectal pain, 2 episodes of rectal bleeding, and a 2-month history of worsening constipation. She also endorsed night sweats, fatigue, nausea, and poor appetite. CT abdomen/pelvis showed a perirectal mass measuring 3.3 3 2.2 cm with adjacent mildly enlarged lymph nodes (Figure 1a). Colonoscopy then showed an eroded, nodular, and ulcerated mucosa in the distal rectum (Figure 1b). The mass was biopsied and pathology revealed small cell carcinoma (Figure 1c). Sigmoidoscopy with EUS and rectal biopsy confirmed poorly differentiated small cell carcinoma. Immunohistochemistry revealed that the tumor cells were positive for CD56 (Figure 1d), chromogranin, AE1/AE3 and TTF1. MRI showed T4N2 disease with possible involvement of the left levator muscle in addition to positive suspicious left inguinal lymph nodes. Patient was started on cisplatin and etoposide therapy. Discussion: Small cell carcinoma accounts for 0.1% to 1% of all GI malignancies, with the mean age at diagnosis of 60 years old. Symptoms of rectal small neuroendocrine cancers are similar to those of rectal adenocarcinomas including defecation difficulties, anal discomfort and blood per rectum. Most patients present with distant metastasis on presentation and have generalized symptoms of malignancy including fatigue, weight loss, and anorexia. The prognosis of colorectal SCC is generally poor. The rate of lymph node and liver metastases in colorectal SCC patients are 60%-89% and 20%-71%, respectively. Median survival in previous studies was 11 months with palliative chemotherapy and 1 month with best supportive care (BSC) only. Based on their established role in metastatic SCLC, cisplatin and etoposide have been one of the most widely used regimens in gastroenteropancreatic NEC, improving median survival to around one year. Curative surgery is usually attempted in localized disease, although retrospective series indicate that it is rarely curative as a sole therapeutic modality. Given the high relapse rate observed following radical surgery, platinum-based adjuvant therapy is recommended.[2085] Figure 1. (a) CT scan demonstrating perirectal mass measuring 3.3 3 2.2 cm concerning for metastases with adjacent mildly enlarged lymph nodes. (b) Colonoscopy image showing an eroded, nodular and ulcerated mucosa in the distal rectum. (c) H&E staining at 103 magnification demonstrating the rectal tumor (black arrow) adjacent to normal rectal tissue d. demonstrates Special staining demonstrating positive CD56 tumor cells.
Introduction: Primary duodenal neuroendocrine carcinoma (NEC) is a rare and highly aggressive malignancy with very poor prognosis. There is no established treatment due to its rarity. Treatment regimens used for small cell lung cancer (SCLC) are used to treat neuroendocrine carcinoma, due to its clinical and histopathological similarities. Therapeutic strategies are poorly understood and not well defined. There is no standardization of therapy even for limited disease and usually multimodal treatment approach is used. Etoposide based treatment regimens have been used mostly in advanced stages. We herein report a case of an aggressive primary duodenal neuroendocrine tumor with excellent response to oxaliplatin-based chemotherapy regimen. Case Description/Methods: We describe a case of a 72-year-old caucasian male who presented to emergency room with abdominal bloating and constipation. He was found to have peritoneal carcinomatosis and marked hydronephrosis in computed tomography (CT) of the chest/abdomen/pelvis. He was further evaluated by urology, oncology, and gastroenterology team. He had paracentesis with removal of 4 L of ascitic fluid. He underwent esophagogastroduodenoscopy, endoscopic ultrasound, and a colonoscopy for further assessment. He was found to have a large mass in the duodenum which upon biopsy was consistent with grade 4 poorly differentiated neuroendocrine carcinoma. Positron emission tomography dotadate scan, peritoneal biopsy and peritoneal fluid cytology further confirmed metastatic neuroendocrine carcinoma. His tumor markers showed elevated Ca 19-9 and chromogranin levels at presentation. Due to his poor performance status and concerns for intolerability to etoposide, he was started on FOLFOX chemotherapy. He completed 12 cycles of chemotherapy with near complete resolution of his disease as evidence by his positron emission tomography dotadate scan and improvements in his tumor markers. Due to excellent response and disease remission, he is currently on xeloda with omission of oxaliplatin. Discussion: Primary duodenal neuroendocrine carcinoma is a relatively rare malignancy with reported incidence of 0.4-2% among all duodenal malignancies. The prognosis is dismal due to presentation as advanced stage at diagnosis. Oxaliplatin based regiments have been shown to have promising anti-tumor activity in gastrointestinal neuroendocrine cancers, however the available data in duodenal gastrointestinal neuroendocrine cancers are very limited.
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