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Ana Canga, Faustino Abascal, Luis Cerezal, Manuel Bustamante, Luis Pérez-Carro and Alfonso Vázquez-Barquero

distal forearm through the carpal tunnel into the palm with posterior displacement of the flexor tendons and bulging of the retinaculum flexor. The composition of the mass is demonstrated in Fig. 1 left and center . At surgery the median nerve was enlarged by a fusiform lipomatous mass extending from the distal third of the forearm to the palm ( Fig. 1 right ). Biopsy sampling of the mass and surgical division of the retinaculum flexor was performed. Histopathological examination revealed fibrolipomatous hamartoma. One year after the surgery the patient

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.89.4.0682 Fibrolipomatous hamartoma of the median nerve Ana Canga Faustino Abascal Luis Cerezal Manuel Bustamante Luis Pérez-Carro Alfonso Vázquez-Barquero October 1998 89 4 683 683 10.3171/jns.1998.89.4.0683 J Neurosurg Journal of Neurosurgery 0022-3085 Journal of Neurosurgery Publishing Group 1 10 1998 October 1998 89 4 10.3171/jns.1998.89.issue-4

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Daniel H. Kim, Judith A. Murovic, Robert L. Tiel, Gregory Moes and David G. Kline

, Hill DR , Bovill EG Jr , de Lorimier AA , Beckstead JH , et al : Desmoid tumors: local control and patterns of relapse following radiation therapy. Int J Radiat Oncol Biol Phys 9 : 1167 – 1171 , 1983 Leibel SA, Wara WM, Hill DR, Bovill EG Jr, de Lorimier AA, Beckstead JH, et al: Desmoid tumors: local control and patterns of relapse following radiation therapy. Int J Radiat Oncol Biol Phys 9: 1167–1171, 1983 48. Meyer BU , Roricht S : Fibrolipomatous hamartoma of the proximal ulnar nerve associated

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

spinal nerves includes schwannomas, neurofibromas, perineuriomas, and malignant peripheral nerve sheath tumors, 50 but not lipomatous lesions. The latter are, however, described in the WHO classification of soft tissue tumors. 30 With reference to nerve, it includes only the all-encompassing designation “lipomatosis of nerve,” an umbrella term adopted in 2002 to denote what was previously called “fibrolipomatous hamartoma” or “lipofibromatous hamartoma.” 74 Less well-known designations, including “perineural lipoma,” “fatty infiltration of nerve,” “intraneural

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Marie-Noëlle Hébert-Blouin, Bernd W. Scheithauer, Kimberly K. Amrami, Susan R. Durham and Robert J. Spinner

and soft tissue elements. Collectively the features suggested NMC, rather than lipomatosis of nerve (fibrolipomatous hamartoma), in that 1) undergrowth of the affected limb, rather than the overgrowth often seen in patients with lipomatosis of nerve, as well as 2) relative paucity of intralesional adipose tissue on MR imaging, were noted. Intraoperative stimulation of the tibial and peroneal divisions of the sciatic nerve and of a hamstring branch resulted in contraction of the nerve(s) ( Video 1 ), thus confirming the diagnosis of NMC in which myocytes are present

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Paolo Missori, Sergio Pandolfi, Manila Antonelli and Maurizio Domenicucci

, fibrolipomatous hamartoma of nerve, lipofibroma of nerve, and neurolipomatosis. The WHO classification 24 published in 2002 designated this benign tumor as “lipomatosis of nerve.” A striking predilection exists for the upper extremities. Approximately 80% of upper-extremity lesions originate in the median nerve. 26 A neural fibrolipoma originating from a thoracic nerve and extending from the epidural space into the thoracic cavity is exceedingly rare. A patient with an epidural thoracic neural fibrolipoma is reported. Case Report Examination This 49-year

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Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami and Robert J. Spinner

– 408 , 2012 2 Lam S , Grandhi R , Wong R , Hamilton R , Greene S : Neuromuscular hamartoma of the sciatic nerve: case report and review of the literature . Surg Neurol Int 4 : 8 , 2013 3 Mahan M , Amrami KK , Spinner RJ : Fibroproliferative neuromas may occur after iatrogenic injury for lipomatosis of nerve . Neurosurgery [in press], 2013 4 Marom EM , Helms CA : Fibrolipomatous hamartoma: pathognomonic on MR imaging . Skeletal Radiol 28 : 260 – 264 , 1999 5 Niederhauser BD , Spinner RJ , Jentoft ME , Everist

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Mark A. Mahan, Caterina Giannini and Robert J. Spinner

nerve is commonly associated with bony and soft tissue overgrowth (macrodactyly) in the distribution of affected nerve(s) 1 and occasionally with overgrowth syndromes such as Proteus and Klippel-Trenaunay syndromes. While the names may be similar, the entities are different. Disclosure The authors report no conflict of interest. References 1 Amadio PC , Reiman HM , Dobyns JH : Lipofibromatous hamartoma of nerve . J Hand Surg Am 13 : 67 – 75 , 1988 2 Boren WL , Henry RE Jr , Wintch K : MR diagnosis of fibrolipomatous hamartoma

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Barrow Neurological Institute;  Mayo Clinic 03 2014 36 3 A15 A15 Copyright held by the American Association of Neurological Surgeons. You may not sell, republish, or systematically distribute any published materials without written permission from JNSPG. 2014 Introduction Lipomatosis of nerve (LN), also known as fibrolipomatous hamartoma, is a condition of massive nerve enlargement due to fibrofatty proliferation. It is also variably associated with nerve-territory overgrowth. Although numerous case studies exist, there is little data on

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Mark A. Mahan, Kimberly K. Amrami, B. Matthew Howe and Robert J. Spinner

L ipomatosis of nerve (LN) is part of a spectrum of adipose lesions of nerves, 25 wherein the nerve is enlarged by fibrofatty proliferation within the epineurium. 10 , 24 It has been variously called “lipofibromatous hamartoma,” “fibrolipomatous hamartoma,” “intraneural fibrolipoma,” and “fibrofatty tumor,” among other names, but we prefer the WHO terminology of “lipomatosis of nerve.” 7 It is most commonly found in the median nerve as well as the digital nerves of the hands or feet. The neural appearance of LN is pathognomonic on MRI, obviating the