Laryngeal neuroendocrine neoplasms are rare neoplasms. Only 13 cases of well-differentiated carcinoid tumors of larynx are reported so far. We present a case of elderly female who presented with involvement of larynx, trachea and bronchi with carcinoid tumors treated with local resection.
A 69 year old black female with past medical history of hypertension, coronary heart disease, congestive heart failure, diabetes mellitus, 30 pack year smoking presented with 2 months history of worsening heart failure symptoms with ocassional blood streaked sputum, wheezing, hoarseness and an acute attack of supraventricular tachycardia.ct angiogram to rule out pulmonary embolism excluded embolism but showed nodular filling defects within the trachea and bilateral mainstem bronchi. Bronchoscopy revealed right vocal cord polyp and multiple polypoid lesions in trachea and bilateral bronchi. Biopsy showed low grade, well differentiated neuroendocrine tumour with tumor markers positive for moc 31, synaptophysin, chromogranin and s100. Metastatic workup was negative and patient underwent sucessful local resection of the tumors.DISCUSSION: Carcinoids are neuroendocrine neoplasms. Most of the carcinoids occur in appendix, rectum and ileum and they arise from enterochromaffin cells. Pulmonary carcinoids arise from kulchitsky’s cells and they constitute very small percentage among the carcinoid tumors. Bronchial carcinoids usually involve the central airways and show well defined margins. Histologically they vary from low grade typical to more aggressive atypical ones. Similary very few typical laryngeal carcinoids have been reported, most of them being atypical variants. Patients in 3rd to 7th decade are usually affected. Symptoms are cough, hemoptysis and other local obstructive symptoms. Carcinoid syndrome is unusual in laryngeal and bronchial carcinoids. Bronchoscopic biopsy is effective usually given the central location of these tumors. Various tumor markers like moc 31,protein s100 for sustentacular cells, chromogranin and synaptophysin corroborate the diagnosis.Treatment is based on the histology of the tumor and extent of involvement. Most of them are amenable to local resection or endoscopic ablation. Hilar and mediastinal lymphnode involvement could influence resection procedure and long term outcome. Chemo and radiation therapy are not effective in improving long term outcome in advanced disease. Somatostatin analogues may be of benefit in early stages of metastatic carcinoid tumors with carcinoid syndrome. However the overrall 5-year survival rate in the typical variety following local resection is around 90%.
To our knowledge this kind of a presentation of carcinoid tumor is extremely rare. Because of the low incidence of laryngeal and tracheal carcinoids it is important to explore the identity of these tumors further to refine diagnostic and therauptic strategies suited for dealing with this kind of tumors.
S.C. Chada, None.