Lipofibromatous Hamartoma of the Upper Extremity: A Review of the Radiologic Findings for 15 Patients

Toms, Andoni P.; Anastakis, Dimitri J.; Bleakney, Robert; Marshall, T.

doi:10.2214/ajr.04.1717

Cited by 86 publications

(87 citation statements)

References 29 publications

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“…pathology On gross examination, these fusiform tumours appear as an irregular palpable yellow/tan-coloured mass within the peripheral nerves due to proliferation of mature adipocytes and associated connective tissue overgrowth (2,6,15,42). Involved nerves may be elongated and enlarged (up to 17 cm and 30 mm, respectively).…”

Section: Differential Diagnosismentioning

confidence: 99%

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

Senger¹,

Classen²,

Bruce³

et al. 2014

CAN J PLAST SURG

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A lthough carpal tunnel syndrome (CTS) is the most common entrapment neuropathy encountered in an adult surgical practice, it is relatively rare in children. In the majority of adults, CTS is believed to be idiopathic in origin. However, in children, CTS is usually secondary to an underlying pathology, such as lysosomal storage diseases, particularly mucopolysaccharidosis and mucolipidosis. Other causes of CTS in children include genetic bony dysplasia, malformations of the anatomical structures of the carpal tunnel, or enlargement of the median nerve by intraneural lipoma, ganglionic cysts, traumatic neuromas, vascular malformations or neurofibromas. An unusual cause of childhood CTS is fibrolipomatous hamartoma (FLH), a rare benign lesion characterized histologically by the presence of mature fat cells admixed with fibroblastic proliferation within the distorted, expanded nerve bundles.We report the first case in the published literature of acute bilateral CTS secondary to bilateral FLH of the median nerves without macrodactyly in a three-year-old boy who presented with incessant crying due to acute bilateral compression neuropathy. Case presentationA three-year-old boy was brought to the emergency room by his mother due to 'incessant crying' that began earlier that morning. His distress had been progressive since he awoke and, on arrival to the emergency room, he was inconsolable. The mother was unable to identify a precipitating cause. On further questioning, the mother recalled his complaining of discomfort in the right wrist for the past three months. The boy was otherwise healthy, took no medications and his immunizations were up to date.On physical examination, a 4 cm to 5 cm swelling of the flexor aspect of the right wrist and thenar atrophy of this hand were recognized with associated weakness in the hand ( Figures 1A and 1B). Tinel's test was positive. The boy's distress escalated with active and passive movement of the wrist, which impeded the assessment of sensation and range of motion. His physical examination was otherwise unremarkable except for an incomplete syndactyly of the second and third toes.Suspecting a neurofibroma secondary to neurofibromatosis, dermatology and ophthalmology were consulted. A complete workup for the same showed no stigmata of neurofibromatosis. Plastic surgery was subsequently consulted to perform an examination under anesthetic for exploration of the right wrist. Intraoperatively, a fusiform enlargement of the median nerve causing carpal tunnel compression was identified (Figures 2A to 2C). The surrounding tissue was otherwise normal. A biopsy of the median nerve was taken for diagnostic purposes, and a carpal tunnel release (CTR) was performed to alleviate compression symptoms. A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swe...

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Section: Differential Diagnosismentioning

confidence: 99%

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

Senger¹,

Classen²,

Bruce³

et al. 2014

CAN J PLAST SURG

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“…In addition, MRI characteristics of various peripheral nerve tumor masses have been reported [14][15][16]. Although the MRI findings of these entities are well described, the role of MRI in the evaluation of peripheral nerve lesions remains unclear, especially with regard to the impact of MRI on patient management [14,17].…”

Section: Introductionmentioning

confidence: 99%

Upper extremity peripheral neuropathies: role and impact of MR imaging on patient management

et al. 2008

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To investigate the role of MR imaging (MRI) in the evaluation of peripheral nerve lesions of the upper extremities and to assess its impact on the patient management. Fifty-one patients with clinical evidence of radial, median, and/or ulnar nerve lesions and unclear or ambiguous clinical findings had MRI of the upper extremity at 1.5 T. MR images and clinical data were reviewed by two blinded radiologists and a group of three clinical experts, respectively, with regard to radial, median, and/or ulnar nerve, as well as muscle abnormalities. MRI and clinical findings

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“…4 On gross examination, neurofibrolipomas present as a soft, gray-yellow, fusiform, mass that has diffusely infiltrated parts of a large nerve and its branches, varying from small simple lesion to extensive complex lipomatosis involving all the nerves of the upper extremity. 3 Microscopy reveals mature adipose and fibrous tissue infiltrating epineural and perineural compartments. The involved nerves may show pseudoonion bulb and metaplastic new bone formation.…”

Section: Discussionmentioning

confidence: 99%

“…It also affects other nerves such as the ulnar, radial and brachial plexus. [1][2][3] Clinically, it presents as a soft, slow-growing, fusiform swelling usually accompanied by pain, redness and decreased sensation. Here, we report a case of a child presenting with a neurofibrolipoma of the dorsum of left hand which was surgically excised without post-operative deficits.…”

Section: Introductionmentioning

confidence: 99%

Neurofibrolipoma of the dorsum of hand: a case report

et al. 2017

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Lipofibromatous Hamartoma of the Upper Extremity: A Review of the Radiologic Findings for 15 Patients

Abstract: Although there are pathognomonic features that characterize lipofibromatous hamartoma on MRI, the range of appearances is broad. Sonography appears to show equally characteristic features and may be a useful tool for assessing this condition.

Cited by 86 publications

References 29 publications

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

Upper extremity peripheral neuropathies: role and impact of MR imaging on patient management

Neurofibrolipoma of the dorsum of hand: a case report

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