Tumeur d’Abrikossof (tumeur à cellules granuleuses) du nerf ulnaire au bras. À propos d’un cas et revue de la littérature des tumeurs à cellules granuleuses du nerf ulnaire

Abstract

Granular cell tumours are extremely rare on peripheral nerves, with an incidence of 0.029% of pathologic samples. In a literature review, we found only 5 cases involving the ulnar nerve, although considered the most frequently involvement nerve. A 32 year-old female from the French West Indies presented a severe arm pain with deficit of interosseous hand muscles. Imaging studies were in favour of a Schwanoma, but during surgery, we found an unremovable intra-neural tumour. Nerve biopsy revealed a granular cell tumour. Initial decision was observation only. However, within two years, tumour increased in size, along with pain aggravation and functional deficit. We performed a nerve resection (with adequate margins) with reconstruction using sural nerve graft associated with a neurotisation of the motor branch with the anterior interosseus nerve. At two years follow-up, no recurrence was observed. The scar is hypersensitive with moderate neuropathic pain. There is a sensory reinnervation of the fourth finger, with no motor recovery of the hand. We observed a slight recovery of flexor profundus tendons, which, in turn increased the claw hand. The five cases described in the literature were managed differently (biopsy only, excision, excision with reconstruction), with modest results. There is no recommended treatment. Our case is the first at arm level. We were able to perform complete resection, but functional result is poor. Granular cell tumours require treatment if symptomatic (pain, function loss), but, at the moment, there is no recommended treatment.