Carcinoid Syndrome and Costs of Care During the First Year After Diagnosis of Neuroendocrine Tumors Among Elderly Patients

Purpose: The purpose of this study is to study the main epidemiological, clinical, para clinical, pathological, therapeutic, and evolutionary features of patients with testicular neuroendocrine tumors (TNET). Materials and Methods: Nine case series and sixteen case reports were identified by searching PubMed database and qualified for inclusion in this study. We added the data of one case treated in the department of urology in Habib Bourguiba Hospital in Sfax, to the published cases. Results: A total of 132 cases were collected. Median age at diagnosis was 39 years old (range 10- 83 years). The most common presenting symptom was either a testicular mass or a swelling in 38.46% of cases. Carcinoid syndrome was documented in 10.60% of patients. The clinical examination revealed a palpable mass in 44.70% of patients. This mass was painless and firm in most cases. Serum tumor markers (β-gonadotrophine chorionique humaine, α-feto protein, and lactate dehydrogenase) were within normal limits in all patients except in one case. Most testicular neuroendocrine tumors (76.52%) were primary and pure. The tumors were positive for chromogranin (100%), synaptophysin (100%) and cytokeratin (93.10%). Metastases were detected at time of diagnosis in eight cases (6.06%). The main treatment was radical orchiectomy performed in 127 patients (96.21%). The 5-year overall survival rate was 78.70% and the 5-year specific survival rate was 84.30%. Conclusion: The diagnosis of testicular carcinoids is based on the immunohistochemistry study. The treatment of choice for these tumors is radical orchiectomy. Somatostatin analogues were reported to be effective in patients with carcinoid syndrome.

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Comment on the Paper by Lemelin et al. Entitled “Elderly Patients with Metastatic Neuroendocrine Tumors Are Undertreated and Have Shorter Survival: The LyREMeNET Study”

Neuroendocrinology ◽

10.1159/000504550 ◽

2019 ◽

Vol 110 (7-8) ◽

pp. 721-722

Author(s):

Alberto Bongiovanni ◽

Chiara Liverani ◽

Federica Recine ◽

Laura Mercatali ◽

Stefano Severi ◽

...

Keyword(s):

Elderly Patients ◽

Neuroendocrine Tumors

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Chromogranin A: From Laboratory to Clinical Aspects of Patients with Neuroendocrine Tumors

International Journal of Endocrinology ◽

10.1155/2018/8126087 ◽

2018 ◽

Vol 2018 ◽

pp. 1-12 ◽

Cited By ~ 15

Author(s):

Paola Di Giacinto ◽

Francesca Rota ◽

Laura Rizza ◽

Davide Campana ◽

Andrea Isidori ◽

...

Keyword(s):

Neuroendocrine Tumors ◽

Phase Ii ◽

Chromogranin A ◽

Carcinoid Syndrome ◽

Phase Ii Trial ◽

Randomized Study ◽

Clinical Aspects ◽

Clinical Activity ◽

Octreotide Lar ◽

Well Differentiated

Background. Neuroendocrine tumors (NETs) are characterized by having behavior and prognosis that depend upon tumor histology, primary site, staging, and proliferative index. The symptoms associated with carcinoid syndrome and vasoactive intestinal peptide tumors are treated with octreotide acetate. The PROMID trial assesses the effect of octreotide LAR on the tumor growth in patients with well-differentiated metastatic midgut NETs. The CLARINET trial evaluates the effects of lanreotide in patients with nonfunctional, well-, or moderately differentiated metastatic enteropancreatic NETs. Everolimus has been approved for the treatment of advanced pancreatic NETs (pNETs) based on positive PFS effects, obtained in the treated group. Sunitinib is approved for the treatment of patients with progressive gastrointestinal stromal tumor or intolerance to imatinib, because a randomized study demonstrated that it improves PFS and overall survival in patients with advanced well-differentiated pNETs. In a phase II trial, pasireotide shows efficacy and tolerability in the treatment of patients with advanced NETs, whose symptoms of carcinoid syndrome were resistant to octreotide LAR. An open-label, phase II trial assesses the clinical activity of long-acting repeatable pasireotide in treatment-naive patients with metastatic grade 1 or 2 NETs. Even if the growth of the neoplasm was significantly inhibited, it is still unclear whether its antiproliferative action is greater than that of octreotide and lanreotide. Because new therapeutic options are needed to counter the natural behavior of neuroendocrine tumors, it would also be useful to have a biochemical marker that can be addressed better in the management of these patients. Chromogranin A is currently the most useful biomarker to establish diagnosis and has some utility in predicting disease recurrence, outcome, and efficacy of therapy.

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Gastrointestinal Carcinoid Tumors (Gastrointestinal Neuroendocrine Tumors) and the Carcinoid Syndrome

Sleisenger and Fordtran's Gastrointestinal and Liver Disease ◽

10.1016/b978-1-4160-6189-2.00031-7 ◽

2010 ◽

pp. 475-490.e4 ◽

Cited By ~ 1

Author(s):

Kjell Öberg

Keyword(s):

Neuroendocrine Tumors ◽

Carcinoid Syndrome ◽

Carcinoid Tumors ◽

Gastrointestinal Carcinoid

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Clinical, pathological, and demographic factors associated with development of recurrences after surgical resection in elderly patients with neuroendocrine tumors

Annals of Oncology ◽

10.1093/annonc/mdx164 ◽

2017 ◽

Vol 28 (7) ◽

pp. 1582-1589 ◽

Cited By ~ 4

Author(s):

C. Shen ◽

A. Dasari ◽

Y. Chu ◽

D.M. Halperin ◽

S. Zhou ◽

...

Keyword(s):

Elderly Patients ◽

Surgical Resection ◽

Neuroendocrine Tumors ◽

Demographic Factors ◽

Factors Associated

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Factors associated with octreotide LAR use among elderly patients with carcinoid syndrome: A population-based analysis.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.e14549 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. e14549-e14549

Author(s):

Ya-Chen T. Shih ◽

Ying Xu ◽

Colleen Leary ◽

James C. Yao

Keyword(s):

Carcinoid Syndrome ◽

Multivariate Logistic Regression Analysis ◽

Population Based ◽

First Year ◽

Delay Tumor Growth ◽

Octreotide Lar ◽

Neighborhood Socioeconomic Status ◽

Factors Associated ◽

Urban Rural ◽

Medicare Database

e14549 Background: Octreotide LAR is indicated for the treatment of carcinoid syndrome, diarrhea related to VIPoma, and may delay tumor growth in neuroendocrine tumors (NETs). The pattern of octreotide LAR use in clinical practice has not been well documented. Methods: Using the SEER-Medicare database, we identified patients diagnosed with NETs from July 1999 to Dec 2007. We then excluded those patients with poorly differentiated histology, under age 65 at diagnosis, enrolled in HMOs, and who did not have continuous Part A & B enrollment within 12 months of diagnosis. We further identified patients with potential indication for octreotide LAR use as those having two or more claims indicating carcinoid syndrome, flushing, diarrhea, or malignant islet cell neoplasm within the first year of NETs diagnosis, with the first claim occurring within 6 months of diagnosis. HCPCS codes, J2352 and J2353, were used to identify octreotide LAR use. Multivariate logistic regression analysis was performed to ascertain factors associated with octreotide use; covariates included age, race, region, comorbidity score, urban/rural status, tumor characteristics, neighborhood socioeconomic status and year of diagnosis. Results: Of the 2000 NETs patients with potential indication for octreotide LAR use, 478 (23.9%) were started on octreotide LAR within 6 months of diagnosis. Multivariate analysis showed that older age (≥ 80) (OR=0.61, 95 CI=0.44 – 0.86), southern region (OR =0.67; 95% CI =0.44 – 1.00) and tumor of undetermined grade (OR=0.71, 95% CI=0.52 – 0.98) were associated with lower odds of octreotide use while the odds was positively associated with advanced stage and more recent diagnosis years. By primary site, no clear pattern was identified. Conclusions: Our analyses suggest only a small (< 25%) fraction of patients with eligible diagnoses are receiving indicated therapy with octreotide LAR. Further studies and improved education are needed to improve the management of carcinoid syndrome.

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Octreotide LAR use among elderly patients with carcinoid syndrome and survival outcomes: A population-based analysis.

Journal of Clinical Oncology ◽

10.1200/jco.2013.31.4_suppl.322 ◽

2013 ◽

Vol 31 (4_suppl) ◽

pp. 322-322

Author(s):

Chan Shen ◽

Ying Xu ◽

James C. Yao ◽

Ya-Chen T. Shih

Keyword(s):

Logistic Regression ◽

Elderly Patients ◽

Survival Benefit ◽

Islet Cell ◽

Carcinoid Syndrome ◽

Survival Outcomes ◽

P Value ◽

Proportional Hazard ◽

Multivariate Logistic Regression ◽

Octreotide Lar

322 Background: Octreotide LAR is indicated for the treatment of carcinoid syndrome, diarrhea related to VIPoma, and may delay tumor growth in neuroendocrine tumors (NETs). The pattern of octreotide LAR use in clinical practice and its impact on survival outcomes has not been well-documented. Methods: Using the SEER-Medicare database, we identified NET patients diagnosed between 7/1999 and 12/2007. We excluded those under age 65, enrolled in HMOs, and without continuous Part A and B enrollment within 12 months of diagnosis. Potential indication for octreotide use was defined as ≥ 2 claims for carcinoid syndrome, flushing, diarrhea, or malignant islet cell neoplasm within first year of diagnosis, with the first claim occurring within 6 months of diagnosis. HCPCS codes, J2352 and J2353, were used to identify octreotide LAR treatment. Multivariate logistic regression and Cox proportional hazard survival analyses were performed to ascertain factors associated with octreotide use and evaluate impact of octreotide use on survival; covariates included age, race, region, comorbidity score, urban/rural status, tumor characteristics, neighborhood socioeconomic status and year of diagnosis. Results: Among those with potential indication for octreotide use (carcinoid syndrome and islet cell), 264 (49%) out of 539 distant stage patients, and 83 (10%) out of 850 patients with local regional disease were started on octreotide LAR within 6 months of diagnosis. Multivariate logistic regression showed that patients living in the southern region were less likely to use octreotide. Multivariate proportional hazard model showed that octreotide LAR provided a significant 5-year survival benefit for distant stage patients (HR=.80, p-value=.047); while this survival benefit was not shown for the local/regional stage patients (HR=.97, p-value=.89). Conclusions: This retrospective study suggests a possible survival benefit for use of octreotide LAR in distant stage elderly patients with carcinoid syndrome. Data also suggest that many elderly patients with claims for carcinoid syndrome did not receive octreotide LAR therapy within 6 months of diagnosis. Further studies and improved education are warranted.

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