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Laryngeal paraganglioma: a case report
Apresentação do caso
Case presentation: Female, 78-year-old, with no tobacco and alcoholic history, complaint of progressive hoarseness, and difficulty in swallowing solid food started clinical investigation. She had diabetes, hypothyroidism, and heart disease as comorbidities. The head and neck surgeon evaluation confirmed a nodule vocal folds lesion by the video laryngoscopy without lymph nodes enlargement by the clinical palpation. The computed tomography (CT) showed a lesion with central mass in the pyriform sinus, with aryepiglottic fold undefined and extension to the glottic larynx. The tumor with the 38 x 30 x 30 mm dimension led to anatomy distortion and airway narrowing. The CT thorax was normal. The magnetic resonance imaging (MRI) evidenced a glottic and infraglottic tissue thickening measuring 47 x 34 x 30 mm with the right vallecula, pyriform sinuses, and vocal folds bilaterally involvement. Right deep inferior cervical lymph nodes were found adjacent to the tumor measuring 5 and 6 mm. Because of the imminent airway risk obstruction, the patient was urgently tracheostomized. The first biopsy analysis was not conclusive. The immunohistochemistry (IHC) expressed synaptophysin and chromogranin, and it was negative for the cytokeratin and epithelial membrane antigen. Therefore, from the pathological test, a laryngeal paraganglioma was diagnosed. Due to the poor performance status patient and locally advanced disease, the primary tumor resection was not indicated. The patient treatment was radiation therapy with 50 Gy in 25 fractions with no recurrence description until the moment.
Discussion: Laryngeal paragangliomas are rare submucosal tumors that arise from paraganglion cells located in the false vocal fold and subglottic larynx. Diagnosis is based on pathology and IHC with neuroendocrine markers expression and chief cells negative for the epithelial markers. The symptoms are hoarseness and deglutition disorders, and subglottic tumors can cause airway obstruction. MRI can detect these lesions and permit characterization of the vascularity. Surgery is preferable to radiation for treatment, but conventional irradiation can be considered for extensive disease, skull base involvement, multiple or bilateral tumors, and not-candidate resection patients.
Final comments: The standard treatment for paraganglioma is surgery however more studies are necessary for laryngeal presentation, especially for the unresectable disease when definitive radiation therapy is indicated.
Keywords: Paraganglioma; Laryngeal Neoplasms; Radiation Therapy
FARLEY SOARES CANTIDIO, GABRIEL OLIVEIRA BERNARDES GIL, IZABELLA NOBRE QUEIROZ, MARCOS REGALIN, INÊS VILLELA COSTA PINTO, GUSTAVO VILLELA COSTA PINTO, CONCEIÇÃO APARECIDA MEDEIROS ALMEIDA, PAOLA PALMER REIS CALDEIRA, ANDRÉ HENRIQUE FAIS DA SILVA