RESUMO
We report the case of a 55-year-old-male with a large cell metastatic pancreatic neuroendocrine carcinoma treated for 14 months with lanreotide autogel having a stable disease (SD) and not responding to chemotherapy. The somatostatin analogues (SSA) were introduced after an episode of diarrhea and controlled the disease. Progression-free survival (PFS) as determined by Computerized Tomography (CT) scans was obtained for 14 months. After more than a year, the patient's health state deteriorated along with progressive disease. The capecitabine-temozolomide regimen was challenged, but after three cycles, a rapid clinical decline was noted. CONCLUSION: This unexpected event (diarrhea) in the course of the disease could represent the beginning of carcinoid syndrome. While the lanreotide autogel helped the episode of diarrhea pass, it also helped gain control over the disease itself.
RESUMO
A 55-year-old female patient was admitted for flushing and abdominal pain in the right upper quadrant. Her past medical history revealed high blood pressure and a recent echocardiography showed thickened appearance of tricuspid valve with coaptation defect and grade II tricuspid regurgitation. Contrast enhanced abdominal CT scan and MRI were subsequently performed and revealed a large macronodular liver mass, as well as other micronodular lesions disseminated in the liver parenchyma. CT guided biopsy from the main liver mass revealed neuroendocrine tumor of unknown origin (probably GI) with Ki-67 of 8%. Surgical exploration was decided. During laparotomy, the primary tumor was found in the proximal ileum and the patient underwent segmental enterectomy. Non-anatomical hepatectomy was also performed to remove the bulk of the tumor burden (more than 90%). Postoperative course was uneventful and the carcinoid syndrome relieved. At present, 15 months postoperatively, the patient is under treatment with somatostatin analogue for its antiproliferative effect, with good clinical, biochemical and tumoral control and stable heart disease. In patients with neuroendocrine liver metastases from unknown primary, surgical exploration could allow detection (and resection) of the primary tumor and surgical debulking of liver metastases to control carcinoid syndrome and carcinoid heart disease.
RESUMO
Resection represents the single hope for long-term survival in a patient diagnosed with a hilar cholangiocarcinoma (Klatskin tumor). However, the largest part of these patients develops a recurrent disease. Second metachronous periampullary cancers after a curative-intent surgery for a Klatskin tumor represent an exceptional pathology, and the management of these patients was poorly documented. Hereby, it is presented a 32-year-old patient with bile duct resection, left hemi-hepatectomy and loco-regional lymph nodes dissection, for a type IIIB Bismuth-Corlette Klatskin tumor, which, furthermore, 6 years later, underwent a pancreaticoduodenectomy for a metachronous carcinoma of the ampulla of Vater. The management and outcomes were discussed in the reported case, along with a literature review of the previously published patients. In conclusion, a metachronous periampullary carcinoma after resection of a Klatskin tumor should be distinguished from a loco-regional recurrent disease. While most of the patients with recurrences are suitable to only chemotherapy and or radiotherapy, a second curative-intent surgery (i.e., pancreaticoduodenectomy) is feasible in the largest part of the patients with a metachronous cancer, with good long-term outcomes.
