INTRODUCTION:Chronic cough is often explained by diagnoses we see daily, but there are instances where our diagnostic modalities can lead us to diagnoses we have never considered.
CASE PRESENTATION:A 65 year old African American female presents with a chief complaint of chronic cough. Cough had been occurring chronically for approximately 15 years, but had recently worsened and was associated with clear mucous and occasional wheezing. The patient became short of breath upon walking approximately one city block or one flight of stairs and had two pillow orthopnea but no appreciable PND. Her symptoms were somewhat improved with albuterol. Her past medical history was positive for asthma and multiple aeroallergens. She had positive skin testing for mold, wheat, grass, trees, weeds, and dust mites. She received allergy injections through 1983-1984 with some relief. Her history was also positive for hypertension, GERD, and diabetes and a recent diagnosis of rheumatoid arthritis. A tuberculosis skin test, recently administered, was positive at 20 mm. She did not use tobacco or alcohol. Her medication list included salmeterol/fluticasone, esomeprazole, montelukast, and albuterol prn. Recently started were weekly methotrexate and prednisone 5 mg daily. Her physical exam was unremarkable.Inhaled corticosteroids and oral anti-histamines were initiated without a change in her symptomatology. Workup included pulmonary function tests which showed an FVC of 50% of predicted, FEV1/FVC ratio of 80%, and a TLC of 71%. She also had a mild decreased diffusing capacity. A CT of the sinuses was negative. An EGD showed mild erosive gastritis and her proton pump inhibitor was increased to BID without symptomatic change. An HRCT of the chest revealed diffuse bilateral pulmonary nodules ranging from two mm to one centimeter with upper lobe ground glass opacities. No hilar or mediastinal lymphadenopathy was observed. Bronchoscopy was negative. She was scheduled for lung biopsy with video assisted thoroscopic surgery.The differential diagnosis, prior to biopsy, included parenchymal lung diseases associated with rheumatoid arthritis, other forms of interstitial lung disease, reactivation of tuberculosis, atypical presentation of sarcoidosis, or disseminated cancer of unknown etiology. Following thorocoscopic lung biopsy, the initial pathology results of the nodules were consistent with a neuroendocrine tumor. When immunohistochemical stains were applied, the final diagnosis was multifocal carcinoid tumorlets, which was the likely etiology of the patient’s primary symptom of intractable coughing.
DISCUSSIONS:Carcinoid tumorlets are rare phenomenon and not well described in the literature. They are neuroendocrine tumors that occur predominantly in the gastrointestinal tract but can also be found in the bronchopulmonary system. They are more common in the bronchial tree, but as in our patient, can be seen in the lung parenchyma and interstitium as well. Average age of presentation is usually 45 to 65. The main symptoms are coughing and wheezing. These tumors secrete serotonin, but advancement to carcinoid syndrome is rare. Symptoms of this disease may respond well to octreotide therapy. Carcinoid tumorlets are typical carcinoid tumor cell type, rarely metastasize, and have an overall good prognosis with an average five year survival of 82%. Carcinoid tumorlets are rarely considered in the diagnosis of chronic cough and documentation of this phenomenon in the literature is extremely sparse. This diagnosis would be very difficult to make prior to lung biopsy and clouded by her multiple comorbidities, allergies, GERD, and asthma, which may all produce chronic cough syndrome.
CONCLUSION:The presentation of carcinoid tumorlets is an unusual diagnosis that can be seen in the presentation of a very common symptom.
DISCLOSURE:Stephen Kovacs, No Financial Disclosure Information; No Product/Research Disclosure Information