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Long-term management of a patient with well-differentiated pulmonary neuroendocrine carcinoma: a case report.

ABSTRACT: Pulmonary neuroendocrine tumors (NET) are rare, and very few published reports have described the long-term treatment of patients with this disease. Current treatment options for patients with metastatic well-differentiated pulmonary NET are limited. This case report details the long-term treatment of a 62-year-old female patient with well-differentiated pulmonary NET and multiple liver metastases. The heavily pretreated patient achieved radiographic stability in measurable disease, improvement in nonmeasurable disease, and symptomatic improvement over 3 years while receiving the combination of everolimus and octreotide long-acting repeatable (LAR). Treatment was well tolerated without mucositis, rash, or pneumonitis. This case report suggests that the combination of everolimus and octreotide LAR may be a novel treatment option for heavily pretreated patients with metastatic well-differentiated pulmonary NET, but these findings require further analysis in clinical trials.

SUBMITTER: Beck T

PROVIDER: S-EPMC3636965 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature

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Long-term management of a patient with well-differentiated pulmonary neuroendocrine carcinoma: a case report.

Beck Thaddeus T Mantooth Ryan R

Case reports in oncology 20130101 1

Pulmonary neuroendocrine tumors (NET) are rare, and very few published reports have described the long-term treatment of patients with this disease. Current treatment options for patients with metastatic well-differentiated pulmonary NET are limited. This case report details the long-term treatment of a 62-year-old female patient with well-differentiated pulmonary NET and multiple liver metastases. The heavily pretreated patient achieved radiographic stability in measurable disease, improvement ...[more]

PMID: 23626563

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Project description:IntroductionRecent classification of neuroendocrine neoplasms has defined well-differentiated high-grade neuroendocrine tumors (NET G3) as a distinct entity from poorly differentiated neuroendocrine carcinoma. The optimal treatment for NET G3 has not been well-described. This study aimed to evaluate metastatic NET G3 response to different treatment regimens.Materials and methodsThis was a retrospective study of patients with NET G3 within the Mayo Clinic database. Patients' demographics along with treatment characteristics, responses, and survival were assessed. Primary endpoints were progression-free survival (PFS) and overall survival. Secondary endpoints were objective response rate (ORR) and disease control rate (DCR).ResultsTreatment data was available in 30 patients with median age of 59.5 years at diagnosis. The primary tumor was mostly pancreatic (73.3%). Ki-67 index was ≥55% in 26.7% of cases. Treatments included capecitabine + temozolomide (CAPTEM) (n = 20), lutetium 177 DOTATATE (PRRT; n = 10), Platinum-etoposide (EP; n = 8), FOLFOX (n = 7), and everolimus (n = 2). CAPTEM exhibited ORR 35%, DCR 65%, and median PFS 9.4 months (95% confidence interval, 2.96-16.07). Both EP and FOLFOX showed similar radiographic response rates with ORR 25.0% and 28.6%; however, median PFS durations were quite distinct at 2.94 and 13.04 months, respectively. PRRT had ORR of 20%, DCR of 70%, and median PFS of 9.13 months.ConclusionAmong patients with NET G3, CAPTEM was the most commonly used treatment with clinically meaningful efficacy and disease control. FOLFOX or PRRT are other potentially active treatment options. EP has some activity in NET G3, but responses appear to be short-lived. Prospective studies evaluating different treatments effects in patients with NET G3 are needed to determine an optimal treatment strategy.Implications for practiceHigh-grade well-differentiated neuroendocrine tumors (NET G3) are considered a different entity from low-grade NET and neuroendocrine carcinoma in terms of prognosis and management. The oral combination of capecitabine and temozolomide is considered a good option in the management of metastatic NET G3 and may be preferred. FOLFOX is another systemic option with reasonable efficacy. Similar to other well-differentiated neuroendocrine tumors, peptide receptor radionuclide therapy seems to have some efficacy in these tumors.

Dataset Information

Long-term management of a patient with well-differentiated pulmonary neuroendocrine carcinoma: a case report.

Publications

Long-term management of a patient with well-differentiated pulmonary neuroendocrine carcinoma: a case report.

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