Results of liver transplantation in the treatment of metastatic neuroendocrine tumors. A 31-case French multicentric report.

Department of Surgery, Hopital de La Conception, Marseilles, France.
Annals of Surgery (Impact Factor: 8.33). 05/1997; 225(4):355-64.
Source: PubMed


The purpose of this study was to assess the value and timing of orthotopic liver transplantation (OLT) in the treatment of metastatic neuroendocrine tumors (NET).
Liver metastasis from NET seems less invasive than other secondary tumors. This observation suggests that OLT may be indicated when other therapies become ineffective. However, the potential benefit of this highly aggressive procedure is difficult to assess due to the scarcity and heterogeneity of NET.
A retrospective multicentric study was carried out, including all cases of OLT for NET performed in France between 1989 and 1994. There were 15 cases of metastatic carcinoid tumor and 16 cases of islet cell carcinomas. Hormone-related symptoms were present in 16 cases (55%). Only 5 patients (16%) had no previous surgical or medical therapy before OLT. Median delay from diagnosis of liver metastasis and OLT was 19 months (range, 2 to 120).
The primary tumor was removed at the time of OLT in 11 cases, by upper abdominal exenteration in 7 cases and the Whipple resection in 3. Actuarial survival rate after OLT was 59% at 1 year, 47% at 3 years, and 36% at 5 years. Survival rates were significantly higher for metastatic carcinoid tumors (69% at 5 years) than for noncarcinoid apudomas (8% at 4 years), because of higher tumor- and non-tumor-related mortality rates for the latter.
OLT can achieve control of hormonal symptoms and prolong survival in selected patients with liver metastasis of carcinoid tumors. It does not seem indicated for other NET.

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    • "Initial results were disappointing owing to the lack of patient selection. Noncarcinoid tumors, nongastrointestinal carcinoids, high-grade tumors, or tumors not drained by the portal vein are considered to be associated with worse outcomes [15, 16]. A retrospective analysis of the United Network for Organ Sharing database on LT performed in the US between 1988 and 2008 included 150 patients with metastatic NET who had an overall 1-, 3- and 5-year survival rate of 81%, 65%, and 49%, respectively [17]. "
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    ABSTRACT: Primary hepatic carcinoids are rare tumors that are often diagnosed at a locally advanced stage. Their primary nature can only be ascertained after thorough investigations and long-term follow-up to exclude another primary origin. As with secondary neuroendocrine liver tumors, surgical resection remains the mainstay of therapy. Despite their large size and often central location liver resection is often feasible, offering long-term survival and cure to most patients. In selected patients liver transplantation appears to be a good indication for tumors not amenable to liver resection. An aggressive surgical attitude is therefore warranted. We report a large and unusually fast-growing liver carcinoid that appeared only marginally resectable in a patient who remains free of disease four years after surgery.
    Full-text · Article · Feb 2014
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    • "Majority of patients undergoing orthotopic liver transplantation (OLT) ultimately develop recurrent disease and reported 5-year recurrence-free survival ranges from 24 to 45% with an overall survival range of 36–57% [100–105]. "
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    ABSTRACT: Neuroendocrine neoplasms (NENs) are a distinctive entity, and nearly 10% of patients already have liver metastases at presentation. The management of neuroendocrine liver metastases (NEN-LM) is complex with differing patterns of metastatic presentation. An aggressive approach should be used to resect the primary tumor, to remove regional lymph nodes, and to resect or treat appropriate distant metastases (including liver tumors). Despite having an indolent course, NENs have a significantly reduced survival when liver metastases are untreated. Though a wide range of therapies are now available with a multimodal approach to the treatment, surgical treatment offers the only chance for a significant survival prolongation and/or improvement of symptoms and quality of life. A review of the existing surgical modalities for NEN-LM is discussed in this paper.
    Full-text · Article · Jan 2012
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    • "Immunohistochemically, chromogranin-A, NSE, and synaptophysin can be used to confirm the diagnosis of a neuroendocrine tumor such as a PHCT. We diagnosed the tumor as a PHCT because of histologic and immunohistochemical findings [7, 8]. In addition, preoperative examination and intraoperative observation revealed no other primary lesion. "
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    ABSTRACT: We report a primary hepatic carcinoid tumor occurring in a 47-year-old man. The patient consulted our hospital complaining of epigastralgia. Abdominal ultrasonography, computed tomography scanning, and magnetic resonance imaging showed a large mass in the right lobe of the liver. FDG-PET revealed 18F-FDG uptake by the right hepatic lobe. The tumor was a solid mass with cystic components, approximately 15 cm in diameter. We conducted an extended right lobectomy of the liver. The resected specimen was a solid tumor with cystic components and hemorrhagic lesion. Microscopic findings showed that the tumor cells had round nuclei and formed trabecular patterns. Immunohistologically, tumor cells were stained positive for chromogranin A, neuron specific enolase, CD56, and S-100. Careful examinations before and after the operation revealed no other possible origin of the tumor. Based on these findings, the tumor was diagnosed as a primary hepatic carcinoid. This is a report of a rare case of a primary hepatic carcinoid tumor with a discussion of several other relevant reports.
    Full-text · Article · Apr 2009 · Case Reports in Gastroenterology
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