• Journal of Korean Neurosurgical Society
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• v.63 no.5
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• pp.664-670
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• 2020

Lipofibromatous hamartoma (LFH) is a rare tumor of the peripheral nerves, which usually involves the median nerve. The authors reported on two rare cases of carpal tunnel syndrome due to LFH of the median nerve. A 49-year-old female patient complained of the mass and symptoms consistent with LFH. Magnetic resonance imaging (MRI) showed typical LFH findings. The symptoms were successfully ameliorated with carpal tunnel release and external neurolysis. A 37-year-old female patient complained of weakening thumb abduction and the mass where the MRI showed atypical findings. Opponensplasty and debulking operations were performed after which thumb abduction was improved; however, neurological sequelae remained. LFH of the median nerve is managed on a case-by-case basis as treatment guidelines are not very clearly defined yet. However, the less invasive treatment such as carpal tunnel release and external neurolysis than more aggressive surgical treatment should be recommended as a treatment option.

• Archives of Plastic Surgery
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• v.36 no.3
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• pp.356-360
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• 2009

Purpose: Lipofibromatous Hamartoma(LFH) of nerve is a tumor - like lipomatous process principally involving the young persons. This is rare disease characterized by a soft slowly growing mass surrounding and infiltrating major nerves and their branches of the palm and digits. LFH of nerve usually affects the median nerve, with the most common sites of presentation being the distal forearm and hand in the wrist or palm. It may cause symptoms of compression neuropathy and is associated with macrodactyly. Recently, MRI plays a major role in confirming the diagnosis of LFH. Therefore, we present two cases of LFH in the hand with MRI features and surgical management. Methods: One is 6 - years - old female who presented with macrodactyly involving both the soft tissue and bony parts of the second, third and forth digits of her right hand. The other one is 16 - years - old man who presented involving the soft tissue of the second and third digits of his right hand, with pain and numbness, along with motor and sensory deficits in the median nerve distribution. To evaluation about LFH, we enforced preoperative MRI and physical examination. After confirming the diagnosis of LFH, we proposed decompression of all compromised peripheral nerve to help alleviate pain and paresthesia to reduce the likelihood of permanent motor and sensory sequelae. Results: A characteristic feature on MRI is the appearance of serpentiform nerve fascicle surrounded by fibro - fatty tissue within the expended nerve sheet. Distribution of fat between fascicles is asymmetric. Two cases were treated by limited debulking of the redundant tumor tissue and excision of epineurial fatty tissue. These cases were performed with relief of symptom. Conclusion: MRI not only confirms the diagnosis, it also provides a detailed assessment of nerve involvement preoperatively. Especially, on coronal images, the nerve has a spagetti - like appearance that is pathognomonic of LFH. Recommendations for early treatment include decompression of the carpal tunnel, debulking of the fibro - fatty sheath, microsurgical dissection of the neural elements and excision of involve nerve with or without grafting.

• Archives of Plastic Surgery
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• v.49 no.5
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• pp.656-662
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• 2022

The median nerve can be compressed due to a tumor along the course of the median nerve, causing typical compression symptoms or even persistence or recurrence after an operation. The aim of this review is to provide a comprehensive overview of rare tumors described in recent publications that cause median nerve compression and to evaluate treatment options. The PubMed, Embase, and Web of Science databases were searched for studies describing median nerve compression due to a tumor in adults, published from the year 2000 and written in English. From 94 studies, information of approximately 100 patients have been obtained. Results The rare tumors causing compression were in 32 patients located at the carpal tunnel, in 21 cases in the palm of the hand, and 28 proximal from the carpal tunnel. In the other cases the compression site extended over a longer trajectory. There were 37 different histological types of lesions. Complete resection of the tumor was possible in 58 cases. A total of 8 patients presented for the second time after receiving initial therapy. During follow-up, three cases of recurrence were reported with a mean follow-up period of 11 months. The most common published cause of median nerve compression is the lipofibromatous hamartoma. Besides the typical sensory and motor symptoms of median nerve compression, a thorough physical examination of the complete upper extremity is necessary to find any swelling or triggering that might raise suspicion of the presence of a tumor.