Multiple oral and cerebral relapses of a Granular cell tumor (Abrikossoff Tumor) in a young girl affected by congenital dyserythropoietic anemia type II: doi: 10.7417/CT.2022.2388

Abstract

Case Report: A 14-year-old girl came to our observation for 1 month whitened lesion on the ventral surface of the tongue, about 1 cm in diameter. Past history showed congenital dyserythropoietic anemia type II. The lesion was excised and microscopic and immunohistochemical analysis was compatible with benign Abrikossoff tumor. Total body MRI was negative. After six months the patient presented a second tongue lesion and four months later an another large diffused painful swelling lesion in the soft palate, with the same istological diagnosis. After that, we had other multiple lesions: two apperead at pharyngeal levels (not biopsied) that remain stable on time, and one at level of pituitary gland.

Conclusion: Granular cell tumors, with or without multiple lesions, are rare in children. About 50% of cases involve the head and neck region, with the tongue being the most affected site. Therapy is based on the surgical excision of the lesions; however some tumor forms, like our case, although the histological aspect of benignity, often have important infiltrative power similar to the malignant picture, making the therapeutic approach difficult.