Lipofibromatous hamartoma of the median nerve in association with or without macrodactyly

Kini, Jyoti R.; Kini, Hema; Rau, Aarathi R.; Kamath, Jagannath B; Kini, Anand

doi:10.5146/tjpath.2014.01282

Cited by 15 publications

(17 citation statements)

References 14 publications

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“…Most scholars advocate early carpal tunnel decompression and brous lipid sheath decompression, anatomical and microsurgical resection is usually reserved for those patients who have undergone carpal tunnel decompression but still have progressive and disabling median nerve damage. However, this method may lead to permanent loss of motor and sensory functions, and the effect is not obvious 3 .…”

Section: Discussionmentioning

confidence: 99%

Fibrolipomatous Hamartoma of the Median Nerve of the Hand: A Case Report and Literature Review

Yang

et al. 2021

Preprint

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Introduction: Fibrolipomatous Hamartoma (FLH) is a rare tumor-like disease, mainly occurs in benign tumors of the peripheral nerves of the upper limbs, which characterized by the overgrowth of neuronal fat and fibrous tissue.Case presentation: A patient had numbness of fingers in the left hand for more than 5 years. Three years ago, the superficial sensation at the metacarpophalangeal joint of the hypothenar of the left hand was weakened. Two years later, the distal ulnar side of the index finger and the distal radial side of the middle finger were secondary to weakened superficial sensation and megalodactyly symptoms of the middle finger and index finger of the left hand were developed. Physical examination showed a mass on the left palm side. MRI showed an abnormal subcutaneous fusiform signal at the palmar side of the left wrist: T1W1 isointensity, T2W1 inhomogeneous slightly hyperintense signal. The clinical features, radiologic presentations, treatment choice, and pathologic characteristic were illustrated. During the operation, the appearance of the nerve was hypertrophic and variant, without obvious masses, the proliferated fibrous tissues were showed a fusiform change, and the nerve fibers were degenerated and atrophied. Immunohistochemical results showed S-100 (+), NSE (focus +), EMA (portion +), GFAP (-), ERG (blood vessel +), SMA (blood vessel +), Ki-67 (1%+), NF (small region +). The patient's finger numbness symptom improved obviously and the condition was stable when paying a return visit 3 months after the operation. Conclusions：The clinical symptoms of FLH of median nerve mainly include gradually enlarged painless mass, macrodactyly, pain, numbness, paresthesia and carpal tunnel syndrome. The diagnosis mainly depends on ultrasonic examination and MRI. The pathological sections of FLH sometimes show perineurioma-like changes, which need to be identified by clinical and MRI features. The treatment methods include prophylactic carpal tunnel release (CTR) and nerve transplantation after the whole nerve resection. CTR, has been proven to be helpful for many patients suffering from carpal tunnel symptoms and can relieve most of the symptoms, is the main method. However, the pathogeny needs to be further explored.

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Section: Discussionmentioning

confidence: 99%

Fibrolipomatous Hamartoma of the Median Nerve of the Hand: A Case Report and Literature Review

Yang

et al. 2021

Preprint

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“…[8][9][10] Young patients are more likely to also exhibit macrodactyly. 11 Symptoms are often related to the location of its presentation; when occurring in the median nerve, it may lead to dysfunction of median nerve innervated muscle, sensory impairment of the skin, and most commonly this can mimic carpal tunnel syndrome due to its mass effect. Benign masses such as a ganglion cyst, lipoma, or neurolemmoma may be high on the differential.…”

Section: Discussionmentioning

confidence: 99%

Lipofibromatous Hamartoma of the Palmar Cutaneous Branch of the Median Nerve

Hoellwarth

Goitz

2018

J Hand Microsurg

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Lipofibromatous hamartoma (LFH) is a benign tumor of peripheral nerves, most commonly reported in the median nerve, and often associated with carpal tunnel symptoms. There have been nearly 200 reported cases of LFH in the median nerve, but to the authors' knowledge, this 10-year-old girl represents the first case report involving the palmar cutaneous branch. An excisional biopsy of the mass was performed via a standard midline palmar incision. The tumor was found encapsulating the palmar cutaneous branch, and thus it was sacrificed. The incision healed without issue, but the patient reported numbness in the palm of her hand as was expected. LFH is a benign tumor, although it can cause symptoms due to mass effect. If the diagnosis is uncertain or a patient is symptomatic, excisional biopsy can be performed even if involving a cutaneous nerve.

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“…Previous studies have indicated that resection was the main method for treating the enlarged nerve in type Ⅱ macrodactyly[ 8 ]; However, the sensorimotor function of the nerves in the hands is important to the patients, especially in children, and it is difficult to determine whether the enlarged nerve should be retained or excised. In addition, the enlarged nerve often influences the efficiency of defatting surgery[ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Morphological and neurophysiological impairment of the nerve in type II macrodactyly

et al. 2018

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BackgroundMacrodactyly is a congenital malformation characterized by aggressive overgrowth of multiple tissues, including subcutaneous fat, nerves, and bones in digits or limbs. In type II macrodactyly, the peripheral nerve is enlarged; however, the morphological and functional characteristics of the affected peripheral nerves have rarely been evaluated.MethodsIn this research, six macrodactyly patients and three polydactyly patients (control) were studied. Pre-operative sensory nerve action potential and intra-operative nerve action potential tests were performed. The microstructure and ultrastructure of the enlarged nerves were observed and neurofilament (NF) expression was evaluated using immunofluorescent staining.ResultsAxon impairment of the digital nerves originating from the median nerve (MN) was observed. A compensatory reinnervation from the ulnar nerve (UN) was found in two of the six patients, and significant morphological changes were observed in the enlarged nerve. The myelinated nerve fibers decreased, the lamellar structure of the myelin sheath changed, and the density of the NFs of the unmyelinated fibers decreased. There was aberrant distribution of NFs in the macrodactylous nerve tissues. In patients with compensatory UN reinnervation, the number of myelinated and unmyelinated fibers increased to normal levels; however, the diameter of the myelinated fibers apparently decreased.ConclusionsThe morphology and function of the macrodactylous enlarged nerve was impaired in type II macrodactyly patients; however, the unaffected UN partially compensated for the lost function of the affected MN under specific situations. Electrophysiological tests should be performed to determine the function of the affected nerve and surgical treatment for type II macrodactyly could be refined.

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Lipofibromatous hamartoma of the median nerve in association with or without macrodactyly

Cited by 15 publications

References 14 publications

Fibrolipomatous Hamartoma of the Median Nerve of the Hand: A Case Report and Literature Review

Fibrolipomatous Hamartoma of the Median Nerve of the Hand: A Case Report and Literature Review

Lipofibromatous Hamartoma of the Palmar Cutaneous Branch of the Median Nerve

Morphological and neurophysiological impairment of the nerve in type II macrodactyly

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