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[Laryngeal myoblastoma in childhood]
1Dipartimento di Fisiopatologia Clinica, II Clinica ORL, Università di Torino.
Insights
This study details a rare granular cell tumor of the larynx in an 11-year-old boy, highlighting its neurogenic origin and the importance of conservative surgical approaches for this benign neoplasm.
Area of Science:
- Oncology
- Pediatric Pathology
- Laryngeal Neoplasms
Background:
- Laryngeal tumors are rare, especially those of neurogenic origin.
- Granular cell tumors typically affect adult males, with few cases reported in children.
Observation:
- A case of a granular cell tumor in the larynx of an 11-year-old boy is presented.
- The tumor caused progressive dyspnea due to its size and location in the right laryngeal ventricle.
Findings:
- Histological analysis revealed neoplastic cells with preserved structure, closely associated with nerve fibers and exhibiting nerve cell-like metabolic activity.
- Immunohistochemistry confirmed neurogenic origin, with positive S-100 staining indicating neural crest derivation.
Implications:
- The findings underscore the polymorphic histology of granular cell tumors and the necessity of tailored surgical interventions.
- Conservative surgery is recommended to prevent recurrence while avoiding extensive tissue removal for this benign laryngeal tumor.
Abstract:
Laryngeal tumors originating in the nerves are extremely rare, particularly in infants. Indeed, this type of neoplasm normally arises in adult males. Only 10 cases have been described in the 5 to 17 year age range. The present work reports a clinical case of granulous tumor of the larynx in an 11-year-old boy. Histologically this tumor showed cells which preserved their original structure and were in close relation with the nerve fibers, but not with the myocytes. Their metabolic activity was similar to that of the nerve cells. The neurogenic origin of the neoplastic granulous cells shows the presence of neuroectodermic cytoplasmic S-100 specific for cells derived from the neural crest. The boy manifested ingravescent dyspnea due to the presence of a myoblastoma in the right laryngeal ventricle. A thyrotomic approach was adopted because of the significant size of the neoplasm although it was possible to perform conservative surgery given that the lesion was benign. The authors underline the polymorphic histology of this neoplasm and the importance of the type of surgery which should both avoid widespread demolition since the neoplasm is benign and, at the same time, prevent recurrences.