A 57-year-old male with periampullary duodenal mass was diagnosed as grade 3 duodenal neuroendocrine carcinoma with multiple liver metastasis. After nine cycles of cisplatin and etoposide, abdominal computed tomography (CT) findings showed complete regression of primary duodenal mass with marked size reduction of liver metastasis. Positron emission tomography findings showed metabolic complete response in both duodenal and liver mass. Pylorus-preserving pancreaticoduodenectomy was done and pathologic finding showed 5 mm sized remnant neuroendocrine tumor. The patient has remained alive with no evidence of disease for 43 months after initial diagnosis. This case suggests the possibility of heterogeneous nature of grade 3 neuroendocrine carcinoma and selected population may have extreme sensitivity to cisplatin and etoposide chemotherapy leading to complete response.
Carcinoma, Neuroendocrine ; Cisplatin ; Diagnosis ; Drug Therapy ; Etoposide ; Humans ; Liver ; Male ; Middle Aged ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pancreaticoduodenectomy ; Positron-Emission Tomography

PURPOSE: Chromogranin A (CgA) has been considered to be valuable not only in the diagnosis but also in monitoring the disease response to treatment. However, only a few studies have been published on this issue. We purposed to evaluate whether biochemical response using plasma CgA level is reliable in concordance with the clinical response of grade 1-3 nonfunctiong gastroenteropancreatic neuroendocrine tumors (GEP-NETs). MATERIALS AND METHODS: Between March 2011 and September 2013, a total of 27 cases in 18 patients were analysed, clinically and radiologically while serial CgA tests were also conducted during treatment. Tumor responses were defined by both Response Evaluation Criteria in Solid Tumors (RECIST) criteria ver. 1.1 and biochemical criteria based on the CgA level. RESULTS: Among the 27 cases analysed, no difference in the basal CgA level was observed with regard to gender, primary tumor site, tumor grade (World Health Organization classification), liver metastasis, number of metastatic site, and line of chemotherapy. The overall response rate (RR) by RECIST criteria ver. 1.1 was six out of the 27 cases (22.2%) and eight out of the 27 cases (29.6%) for biochemical RR. The overall concordance rates of the response based on RECIST and biochemical criteria were 74%. In grades 1 and 2 GEP-NETs (n=17), the concordance rate of the disease control was 94.1%. There was a significant difference for progression-free survival (PFS) between responders and non-responder in accordance to biochemical criteria (35.73 months vs. 5.93 months, p=0.05). CONCLUSION: This study revealed that changes of the plasma CgA levels were associated with tumour response. Additionally, biochemical response based on serial CgA may be a predictive marker for PFS in GEP-NETs.
Chromogranin A* ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Liver ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Plasma*

Neuroendocrine tumor is a very heterogenous group arising from the neuroendocrine cells. Especially, large cell neuroendocrine tumor of the ovary is a extremely rare aggressive neoplasm, characteristically arising in association with a surface epithelial tumor. This report describes a mixed type of large cell neuroendocrine carcinoma and adenocarcinoma of the ovary. A 63-year old woman presented with abdominal distension and discomfort underwent staging laparotomy under the impression of ovarian cancer. The operation revealed an small ovarian mass with invasion of multiple region in peritoneal cavity by the tumor. Immunohistochemical and ultrastructural analysis confirmed the neuroendocrine nature of the tumor. The adenocarcinoma in this case is mixture of mucinous and endometrioid type. A diagnosis of stage IIIc mixed large cell neuroendocrine tumor and adenocarcinoma of the ovary was rendered. She is subsequently being treated with Paclitaxel and Carboplatin combination chemotherapy.
Adenocarcinoma* ; Carboplatin ; Carcinoma, Neuroendocrine ; Diagnosis ; Drug Therapy, Combination ; Female ; Humans ; Laparotomy ; Middle Aged ; Mucins ; Neuroendocrine Cells ; Neuroendocrine Tumors* ; Ovarian Neoplasms ; Ovary* ; Paclitaxel ; Peritoneal Cavity

Primary duodenal adenocarcinoma is a rare malignant neoplasm accounting for 0.3% of all gastrointestinal tract carcinomas. We herein present one case of duodenal adenocarcinoma after duodenal neuroendocrine carcinoma. Poorly differentiated duodenal neuroendocrine carcinoma with liver metastasis (TxNxM1) was confirmed, and eight cycles of palliative chemotherapy (5-fluorouracil/etoposide/cisplatin) were administered. The patient was then in a clinically complete response status. About 1 year later, newly developed adenocarcinoma was detected at the same site. It was completely surgically resected, and the patient was cured.
Adenocarcinoma/*diagnosis/drug therapy ; Antineoplastic Agents/therapeutic use ; Duodenal Neoplasms/*diagnosis/drug therapy ; Humans ; Male ; Middle Aged ; Neoplasms, Second Primary/*diagnosis/drug therapy ; Neuroendocrine Tumors/*diagnosis/drug therapy

Vasoactive intestinal polypeptide-secreting tumors (VIPomas) cause VIPoma syndrome, which is characterized by watery diarrhea, hypokalemia, and achlorhydria. The treatment options for metastatic VIPomas include somatostatin analogs, cytoreductive surgery, and chemotherapy. We report the case of a 54-year-old male who presented with a peripancreatic mass with multiple hepatic metastases on computed tomography. After resection, the peripancreatic mass was demonstrated pathologically to be a neuroendocrine tumor. Although the patient received systemic chemotherapy and somatostatin analogs for the hepatic metastatic masses, the tumor increased in size. The patient then experienced severe diarrhea, despite treatment with the somatostatin analogs. Elevated serum VIP levels (3,260 pg/mL) and typical symptoms confirmed the diagnosis of VIPoma. We performed hepatic artery embolization (HAE) to reduce the tumor volume and control his symptoms, which led to a very rapid symptomatic response. The patient has remained symptom-free for 18 months with repeated HAE.
Achlorhydria ; Diagnosis ; Diarrhea ; Drug Therapy ; Hepatic Artery* ; Humans ; Hypokalemia ; Liver* ; Male ; Middle Aged ; Neoplasm Metastasis* ; Neuroendocrine Tumors ; Somatostatin ; Tumor Burden ; Vipoma*

PURPOSE: Very few cases of neuroendocrine tumor of the stomach have been reported in Korea. The prognosis of gastric neuroendocrine tumors is known to be poorer than that of ordinary gastric carcinomas. The purpose of this retrospective study was to review the clinicopathologic features of seven cases of this unusual gastric tumor. METHODS: Excluding the tumor containing only sparse neuroendocrine differentiation, we resected 7 typical neuroendocrine tumors from January 1995 to December 1997 at Asan Medical Center; 2,346 gastric cancer patients were treated surgically during the same period. Average follow up period was 22.6 months. RESULTS: A typical well demarcated tumor margin was observed in four of the seven cases. The entire tumor was located on the distal stomach. Even when the tumor had deep ulcerations, the tumor cells had not infiltrated the serosal layer. Three cases showed recurrence of disease. All the recurrences were found in the liver within 12 months, and two of them, who were not indicated for chemotherapy, died within 10 months after diagnosis of recurrence. One patient with a recurrence was treated with systemic chemotherapy just after the diagnosis and was still alive after 29 months. CONCLUSION: Gastric neuroendocrine tumor frequently recurs the liver even in an early stage and especially when preoperative CEA is increased. We experienced a case of successful control of hepatic metastasis by using systemic chemotherapy.
Chungcheongnam-do ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Korea ; Liver ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Prognosis ; Recurrence ; Retrospective Studies ; Stomach Neoplasms ; Stomach* ; Ulcer

PURPOSE: To report the first case of pigmented choroidal metastases without enucleation diagnosed with fine-needle aspiration biopsy. CASE SUMMARY: A 47-year-old male was referred to our clinic with a suspected diagnosis of choroidal melanoma in his left eye. Positron emission tomography-computer tomography used to evaluate systemic metastases revealed lung cancer. Pathology of the detected lung mass was primary lung neuroendocrine tumor. To differentiate choroidal metastasis and primary choroidal melanoma, we performed a fine-needle aspiration biopsy for choroidal tumors. The cytology showed results favoring metastatic atypical carcinoid and the patient was started on systemic chemotherapy. CONCLUSIONS: All pigmented choroidal tumors are not choroidal melanomas and choroidal metastases may be pigmented. The present case suggests that proper systemic evaluation and biopsy for suspected choroidal tumor could be helpful in diagnosis.
Biopsy ; Biopsy, Fine-Needle ; Carcinoid Tumor ; Choroid* ; Diagnosis ; Drug Therapy ; Electrons ; Humans ; Lung ; Lung Neoplasms ; Male ; Melanoma* ; Middle Aged ; Neoplasm Metastasis* ; Neuroendocrine Tumors ; Pathology

A 31-yr-old man with abdominal pain was diagnosed with a pancreatic endocrine tumor and multiple hepatic metastases. Despite optimal treatment with interferon alpha, a somatostatin analog, local therapy with high-intensity focused ultrasound ablation for multiple hepatic metastases, and multiple lines of chemotherapy with etoposide/cisplatin combination chemotherapy and gemcitabine monotherapy, the tumor progressed. As few chemotherapeutic options were available for him, sorafenib (800 mg/day, daily) was administered as a salvage regimen. Sorafenib was continued despite two episodes of grade 3 skin toxicity; it delayed tumor progression compared to the previous immunotherapy and chemotherapy. Serial computed tomography scans showed that the primary and metastatic tumors were stable. Thirteen months after beginning targeted therapy, and up to the time of this report, the patient is well without disease progression. We suggest that sorafenib is effective against pancreatic endocrine tumors.
Adult ; Antineoplastic Agents/adverse effects/*therapeutic use ; Benzenesulfonates/adverse effects/*therapeutic use ; Humans ; Liver Neoplasms/drug therapy/pathology/secondary ; Male ; Neuroendocrine Tumors/*diagnosis/drug therapy/pathology ; Pancreatic Neoplasms/*diagnosis/drug therapy/pathology ; Pyridines/adverse effects/*therapeutic use ; Salvage Therapy ; Skin Diseases/chemically induced ; Tomography, X-Ray Computed

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2–1.2 mg/dL), AST 200 IU (normal 0–40 IU), ALT 390 IU (normal 0–40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0–60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0–37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.
Bile Duct Neoplasms ; Bile Ducts, Extrahepatic* ; Bilirubin ; Carcinoma, Neuroendocrine* ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Common Bile Duct ; Diagnosis ; Dilatation ; Drainage ; Drug Therapy ; Follow-Up Studies ; Hepatic Duct, Common ; Humans ; Jaundice ; Liver ; Middle Aged ; Neuroendocrine Tumors ; Radiotherapy ; Recurrence ; Synaptophysin ; Tomography, X-Ray Computed ; Transferases