Background: Data to guide the management of advanced pulmonary carcinoid (APC) come from retrospective reports and subgroup analyses of trials that included mainly extrapulmonary carcinoid tumors. We report the largest series to date of 49 patients with locally advanced or metastatic pulmonary carcinoid.
Methods: The Johns Hopkins Pathology Database was reviewed for APC patients treated between January 1992 and December 2012. Data on time to recurrence, progression-free survival, and overall survival were estimated by using the Kaplan-Meier method.
Results: Forty-nine patients were treated for APC in the specified time period. Median time to recurrence after surgical resection was 2.5 years (atypical carcinoid [AC] versus typical carcinoid [TC], 2.5 versus 6.3 years; p = 0.063). Median survival with advanced disease was 7.1 years and significantly longer for TC compared with AC (10.2 versus 4 years; p = 0.009). Among the diverse systemic therapies used, responses occurred in four of 17 patients (23.5%) who received platinum/etoposide with a median progression-free survival of 7 months.
Conclusion: Although systemic chemotherapy has moderate activity for APC, novel approaches are required. TC and AC, although both classified as pulmonary carcinoid, are clearly different clinical and molecular entities and require separate treatment paradigms in the advanced/metastatic setting.
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http://dx.doi.org/10.1097/JTO.0000000000000065 | DOI Listing |
AACE Clin Case Rep
August 2024
Division of Endocrinology, Department of Medicine, Duke University, Durham, North Carolina.
Background/objective: Pulmonary carcinoid tumors are a rare cause of Cushing's syndrome and usually present with an indolent course. Here, we present a case of rapid onset and severe Cushing's syndrome due to a typical pulmonary carcinoid tumor.
Case Report: A 32-year-old woman developed diabetes, hypertension, and weight gain of 50 pounds over 3 months.
J Med Case Rep
December 2024
RineCynth Advisory Limited, Kampala, Uganda.
Background: Pulmonary carcinoids are rare neuroendocrine tumors accounting for less than 1% of all lung cancers. They are classified into two subcategories; typical and atypical carcinoids with the latter tending to grow faster. Historically, open thoracotomy was the standard approach for pulmonary resection.
View Article and Find Full Text PDFCancers (Basel)
November 2024
Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, ENDO-ERN (European Reference Network on Rare Endocrine Conditions), 00280 Helsinki, Finland.
Small intestinal neuroendocrine tumors (SI-NETs) are characterized by carcinoid syndrome and carcinoid heart disease (CHD). The aim of the present study was to identify early risk markers for carcinoid heart disease and survival in a prospective median-term follow-up setting. We measured 5-HIAA and cumulative 5-HIAA exposure (Cum-5-HIAA) based on repeated measurements, proBNP, vascular function, hepatic tumor load, and transthoracic echocardiography (TTE) at baseline and during the median 5-year follow-up.
View Article and Find Full Text PDFClin Respir J
December 2024
Department of Internal Medicine, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China.
Neuroendocrine tumor (NET) is a deadly malignancy disease that can be found anywhere in the body. The lack of tumor-specific treatment led to the worse prognosis of NET. Anaplastic lymphoma kinase-tyrosine kinase inhibitors (ALK-TKIs), such as alectinib and crizotinib, have been used in the treatment of NET patients with ALK rearrangement.
View Article and Find Full Text PDFCureus
November 2024
Endocrinology and Metabolism, University of Rochester School of Medicine and Dentistry, Rochester, USA.
Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing is a rare syndrome. We present a case that illustrates the diagnostic and therapeutic challenges of ectopic Cushing. A 35-year-old woman presented to the outpatient clinic for evaluation of progressive weight gain, muscle weakness, easy bruising, uncontrolled hypertension, and hyperglycemia.
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