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2018

ANUÁRIO DO HOSPITAL DONA ESTEFÂNIA
REPOSITÓRIO MÉDICO CIENTÍFICO

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GRANULAR CELL TUMOR OF TRACHEA: A RARE CAUSE OF DYSPNEA IN PEDIATRIC AGE

Ana Palhinha1, Ana Casimiro2, Fátima Abreu2, José Cavaco2, José Oliveira Santos2

1- Serviço de Imunoalergologia, Hospital de Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E
2- Unidade de Pneumologia, Hospital de Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E

- XVI Congresso Internacional de Pneumologia Pediátrica (CIPP-XVI), Lisboa, 22 a 25 de Junho de 2017
- Apresentação sob a forma de poster; publicação de resumo
- Reunião Internacional

Resumo:
Introduction: Granular Cell Tumor (GCT) is an infrequent tumor, extremely rare in pediatric population, but 98% of the cases are benign. They can have any location, but more than 50% are in head and neck region, and the trachea is the less common place. Most tracheal GCT are asymptomatic, but they can present with hemoptysis, wheezing, cough, or post obstructive pneumonitis. GCT have a neurogenic origin, derived from Schwann cells, which is supported by positive immunostaining for S-100 protein. The treatment is still controversial, but bronchoscopic excision is adequate for tumors less than 1 cm in diameter, since a proper follow-up is assured, to detect possible recurrences.
Case report: 
A 15-years old girl was evacuated from Cape Verde with dyspnea of effort with two years of evolution, persistent cough and stridor of progressive worsening. In the last six months, she refers dyspnea for small efforts and a loss of 10 Kg (17% of body weight). She denied hemoptysis, and fever. She was observed in her country, where she was diagnosed with asthma and medicated with inhaled beta-agonist and corticoid without improvement. She did a chest radiography that showed a reduction in the air column of the superior trachea, and a thoracic computed tomography scan that identified a solid image in the 1/3 proximal trachea, with 80% lumen obstruction. For investigation, she was evacuated to Portugal.In our hospital, stood out stridor, decreased vesicular murmur, limitation of daily activities, and an episode of syncope of effort, with necessity of oxygen therapy. She did bronchofibroscopy (BF), in which was observed a mass emergent from the left post lateral wall of the trachea, with irregular surface, pink color and vascularized, in the 1st/2nd tracheal rings, with 80% of lumen obstruction. A rigid bronchoscopy (RB) was made and the mass was removed partially, with complete resolution of the symptoms. The biopsy revealed a granular cell tumor, with pavement epithelium metaplasia, positive for S-100 protein. Given the diagnosis and the high risk of recurrence, a second BF was made, which demonstrated the presence of residual mass with a lumen obstruction of 25 to 30%. The RB was repeated for removal of the residual mass with tweezers and argon plasma ablation, which was confirmed in a FB 3 days after the treatment, with biopsy of the lesion margins, that were free of neoplastic tissue.
Conclusion: The GCT are extremely rare, and a high level of suspicion is needed for the diagnosis. They have a slow grow, and when localized in the trachea, can be asymptomatic until the lumen obstruction is about 50 to 80%, like in our case it evolved for two years. Although most are benign, they have a high rate of recurrence, whereby the removal must be total, and the follow up should be individualized and kept at least for 5 years. Our patient follow up will include a new BF in 4 months, to detect a possible recurrence of the tumor, and in that case, will be considered a chirurgical approach.

Palavras Chave: granular cell tumor, tracheal tumor