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Lipomatous, vascular, and chondromatous benign tumors of the peripheral nerves

Representative cases and review of the literature

Claude-Edouard Châtillon, Marie-Christine Guiot and Line Jacques

Benign peripheral nerve lesions of lipomatous, vascular, and chondromatous origin are very rare. Only one previous case of brachial plexus involvement by such a tumor has been reported. The authors report on their experience with peripheral nerve tumors in three patients and review the available literature on these topics. The three cases discussed include a 44-year-old woman with an intraneural lipoma of the right middle trunk, a 40-year-old woman with an intraneural hemangioma infiltrating the right posterior cord, and a newborn male with a predominantly cartilaginous hamartoma originating from the right C-5 nerve root.

The literature review yielded six previous cases of intraneural lipoma, approximately 50 cases of lipofibromatous hamartoma, 13 cases of intraneural hemangioma, and no previous case of cartilaginous hamartoma originating from a nerve. Intraneural lipomas are well encapsulated, and gross-total resection can be achieved. Lipofibromatous hamartomas are diffusely infiltrative; decompressive debulking and neurolysis is often the most appropriate initial approach for patients with symptomatic lesions. Resection of intraneural hemangiomas can be achieved but may require nerve resection and repair in some cases. Debulking has been reported to provide prolonged symptomatic relief in these lesions, and preoperative embolization and postoperative radiotherapy were beneficial in the case presented here. To the authors' knowledge, this is the first reported case of a cartilaginous hamartoma infiltrating a peripheral nerve. Gross-total resection of symptomatic intraneural lipomas is feasible and apparently curative. The optimal treatment for lipofibromatous hamartomas and vascular and chondromatous lesions of the peripheral nerves is uncertain and should be guided by the severity of symptoms.

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Robert J. Spinner, Bernd W. Scheithauer, Kimberly K. Amrami, Doris E. Wenger and Marie-Noëlle Hébert-Blouin

) depicting the existing classification scheme, which includes lipomatosis of nerve with or without overgrowth (red) , intraneural lipoma (yellow), and extraneural lipoma (blue) , all in primary colors. These groups have been considered mutually exclusive. The 2 larger overlapping circles (right) highlight our current understanding, emphasizing the classic forms (lipomatosis and lipoma) and their combinations. A secondary color (green) reflects the joined lipoma categories. Subsets of combined intraneural and extraneural localizations are represented by horizontal

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Paolo Missori, Sergio Pandolfi, Manila Antonelli and Maurizio Domenicucci

N eural fibrolipoma is a rare tumor of the peripheral nerves. In 1953, Mason 17 reported on a patient with a “neuroma of the median nerve” and another with “a fusiform mass involving the median nerve.” In 1964, the lesion was histologically identified as an “intraneural lipoma” because the fibers of the median nerve were spread out over the surface of a fatty tumor containing very scant fibrous supporting stroma. 7 , 20 , 21 , 30 Since that time, various alternative names have been reported, including neural fibrolipoma, fibrolipomatosis of nerve

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Thomas J. Wilson, Jacob R. Joseph, Jonathan R. Dillman, Amer Heider and Lynda J. S. Yang

. In our patient, the imaging characteristics and clinical scenario were thought preoperatively to be most consistent with a fibrolipomatous hamartoma of the median nerve. Fibrolipomatous hamartomas comprise hypertrophied nerves secondary to fibrofatty ingrowth interspersed between thickened nerve bundles. 4 , 10 , 13 These should be distinguished from intraneural lipomas, in which a well-encapsulated fatty mass displaces nerve fibers that then course along the periphery of the mass. 13 These lesions occur most commonly in association with the median nerve. 10

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Andrés A. Maldonado, Benjamin M. Howe and Robert J. Spinner

AL , Hudson AR , Hunter DA : Chronic nerve compression—an experimental model in the rat . Ann Plast Surg 13 : 112 – 120 , 1984 10.1097/00000637-198408000-00004 14 Matsuo T , Sugita T , Shimose S , Kubo T , Yasunaga Y , Ochi M : Intraneural lipoma of the posterior interosseous nerve . J Hand Surg Am 32 : 1530 – 1532 , 2007 18070639 10.1016/j.jhsa.2007.08.007 15 Rempel DM , Diao E : Entrapment neuropathies: pathophysiology and pathogenesis . J Electromyogr Kinesiol 14 : 71 – 75 , 2004 10.1016/j.jelekin.2003.09.009 14759752

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Tomas Marek, Robert J. Spinner, Akshay Syal and Mark A. Mahan

this disorder. While fibrolipomatous hamartoma remains one of the most commonly used terms, 81 this entity is also reported as lipofibromatous hamartoma, fibrolipoma, lipofibroma, interfibrillar lipoma of the nerve, and intraneural lipoma, 10 , 22 , 27 , 37 , 73 among others. LN has also been referred to as macrodystrophia lipomatosa (MDL) when it presents with massive overgrowth of soft tissue, 32 including soft-tissue lesions (e.g., lipomas) and bony changes (e.g., macrodactyly, exostoses). In 2002, the WHO adopted the term “lipomatosis of nerve” to replace