Comunicación en congreso Tumor de Abrikossof: Tumor infrecuente de etiología controvertida

Rare Tumor of Controversial Etiology

Abstract

Introduction and objective: Granular cell tumor (GCT) is a rare tumor described by Abrikosoof in 1926. It is a neoplasm whose etiology is still controversial today. Most are benign (1) and clinically appear as a single nodule, usually in the oral cavity, although it can develop in many other organs.(2) In recent years, some cases associated with Noonan syndrome and neurofibromatosis have been described, included within rasopathies, diseases of genetic origin that affect genes that encode proteins of the Ras family.(1) To analyze the clinical, histological and immunohistochemical characteristics of this tumor and to assess its neural origin. Method: Study of two cases of this type of neoplasm treated in the ENT Service of the Segovia Healthcare complex, having removed them under local anesthesia. In collaboration with the Pathological Anatomy Service, histological analyzes and immunohistochemical tests were carried out. Results: In our two patients, both men, the location of the tumor was in the tongue, appearing as a small nodular tumor, hard, non-painful and slow-growing. In both cases, the presumptive diagnosis was fibroma. Histologically, GCT is a nonencapsulated tumor made up of large polyhedral cells with a small hyperchromatic central nucleus and cytoplasm filled with eosinophilic granules. The overlying epithelium presented pseudoepitheliomatous hyperplasia. No mitoses were observed. Immunohistochemistry was positive for S100 protein, CD68 antigen, and neuron-specific enolase. Discussion: The origin of Abrikossoff's tumor is still controversial, having suggested a myogenic histogenesis, in mesenchymal cells or in histiocytes, although today there is evidence of its origin from Schwann cells (3). In a recent review, more than 60% are women (4), unlike the cases presented by us, which were both men. The most common location is the skin and oral mucosa (1). Clinically, it is usually a non-painful hard touch nodular lesion, coinciding with our two cases. The evil TCG is extremely rare (4). As in other studies, the presumptive clinical diagnosis was fibroma (1) The association with Noonan Syndrome and Neurofibromatosis type I may indicate a common pathogenic link between Rasopathies due to alterations in Ras proteins, which play an important role in cell proliferation and differentiation (1). Histologically, it is made up of large cells with PAS-positive eosinophilic granules in the cytoplasm. Pseudoepitheliomatous hyperplasia is common in the epithelium covering the lesion, and can be confused with squamous cell carcinoma. Immunohistochemistry shows positivity for the S100 protein and CD68 antigen, and specific neuronal enolase. These findings demonstrate its neural origin and are comparable to those of other authors (1,3, 4) Simple excision is the treatment of choice (3). Conclusions: - The most frequent location is the tongue. - Most are benign. - The basis of diagnosis is histology as it presents a non-specific clinical appearance. - Immunohistochemistry supports its neurogenic origin. - Treatment is surgical excision of the lesion.