Cas Clinique / Case Report Neural

Neural fibrolipoma, also known as fibrolipomatous hamartoma of the nerve, is a benign and rare nerve sheath tumor that involves the upper and lower extremities; mainly the digits of the hand and foot. It does not become symptomatic until it has been present for many years and its symptomatology stems from compression of the nerve rather than intraneural involvement by the tumor [1]. A fibrolipomatous hamartoma is usually unilateral and has no known genetic component. Although it can present in childhood and early adulthood, it is believed to be of congenital origin [2]. While most commonly found in the median nerve, studies have reported the lesion at other sites such as the radial, ulnar, sciatic, and plantar nerves [3-5] and in the lungs as well [6]. When found in the median nerve, the lesion is usually at the level of the wrist or hand. It is pathologically composed of a fatty and fibrous tissue with intermingling nerve fibers. Magnetic resonance imaging (MRI) demonstrates fusiform nerve enlargement that is caused by fatty proliferation and thickening of nerve bundles. Nerve bundles appear as serpentine tubular structures, hypointense on both T1 and T2 weighted images [7]. Although the World Health Organization (WHO) grouped intraneural lipoma, fibrolipomatous hamartoma of the nerve, fatty infiltration, and neural fibrolipoma under lipomatosis of the nerve in 2002 [8], the clinical findings and treatments of these conditions differ. In early 2013, the new classification of tumors of soft tissue and bones was released and published under the auspices of the WHO [9]. The revised classification places neural fibrolipomas under benign nerve sheath tumors [9]. Careful preoperative planning based on clinicopathological and radiological findings is necessary for the optimal treatment of neural fibrolipomas of the median nerve [10]. Besides, surgical intervention is only necessary when the patient is symptomatic. This communication presents a case of this uncommon disease with a review of the literature.