Lung Cancer |

Primary Pulmonary Carcinoid: A Rare Presentation of a Rare Disease FREE TO VIEW

Rassull Suarez, MD
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Yale New Haven Hospital, New Haven, CT

Chest. 2014;146(4_MeetingAbstracts):670A. doi:10.1378/chest.1995258
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SESSION TITLE: Cancer Student/Resident Case Report Posters III

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Carcinoid syndrome is the term used to describe the variety of symptoms caused by humoral factors elaborated by carcinoid tumors. This syndrome varies in its presentation depending on the primary origin of the tumor. There are three different sites of origin depending on its embryonic derivative which include: Foregut, Midgut and Hindgut. We report a case of Primary pulmonary carcinoid, deriving from the foregut.

CASE PRESENTATION: A 56-year-old Hispanic woman with a known history of persistent asthma, papillary thyroid carcinoma and GERD presented to our practice for evaluation of a chronic cough that started 6 years before evaluation. Despite being on optimal therapy for her asthma, GERD and upper airway cough syndrome her symptoms kept progressively worse to the point when she developed a ventral hernia as consequence of severe coughing. The pulmonary function tests did not reveal any obstructive airway disease, chest radiograph showed new development of diffuse interstitial markings bilaterally (Figure.1). Chest CT confirmed the presence of multiple scattered nodules through both lungs, highly concerning for metastatic tumor. Bronchoscopy with endobronchial washings were negative for endobronchial pathology. Given high concerns for malignancy, the decision was made to perform wedge biopsy/resection of the lung. Pathology revealed carcinoid tumor. There were no other lesions detected on abdominal CT scan, PET or colonoscopy. Urinary 5-HIAA and serum Chromogranin A and octreotide scan all were negative. The patient was started on subcutaneous injections of Somatostatin analogs and had significant improvement of her symptoms.

DISCUSSION: The disease spectrum ranges from being indolent and asymptomatic to malignant and devastating. The known evidence suggests that this tumor arises from an endodermal derivative; the foregut. Carcinoid tumor cells are typically known to metabolite Tryptophan into Serotonin. Unfortunately the tumor cells that rise from the foregut derivative lack the aromatic amino acid decarboxylase this results in the accumulation of the intermediate hydroxyl-Tryptophan and no production of Serotonin; the clinical significance of this unexpected metabolism of Tryptophan results in no production of the common urinary and serologic markers typically used for diagnosing and staging this disease creating uncertainty in detection, staging and management.

CONCLUSIONS: Carcinoid tumors that originate primarily in the lung are very rare. Due to its epidemiology and different forms of presentation, primary pulmonary carcinoid is a field that needs further investigation.

Reference #1: 1. Cakir M, Grossman A. The molecular pathogenesis and management of bronchial carcinoids. Expert Opin Ther Targets. 2011 Apr;15(4):457-91.

Reference #2: 2. Modlin IM, Lye KD, Kidd M. 5-decade analysis of 13,715 carcinoid tumors. Cancer 2003;97-934.

Reference #3: 3. Froudarakis M, Fournel P, Burgard G, et al. Bronchial carcinois. A review of 22 cases. Oncology 1996; 53:153.

DISCLOSURE: The following authors have nothing to disclose: Rassull Suarez

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