Lipofibromatous hamartoma of the median nerve

Abstract

Lipofibromatous hamartoma is a rare tumour of peripheral nerves which is characterised by an excessive infiltration of the epineurium and perineurium by fibroadipose tissue. To the best of our knowledge, only approximately 88 cases are reported in the literature. We report a rare case of lipofibromatous hamartoma of the median nerve causing secondary carpal tunnel syndrome in a 25 year old patient. This patient was treated conservatively with decompression and biopsy and experienced a complete resolution of symptoms post-operatively. Magnetic resonance imaging may be used to diagnose this lesion as it has very distinctive characteristics. Multiple conditions have been associated with this lesion and a greater understanding of these associations may clarify the pathogenesis. The architecture of the tumour makes excision very challenging and the surgical management remains controversial. A review of the literature regarding the etiology, pathogenesis and surgical management of lipofibromatous hamartoma is included.

Lipofibromatous Hamartoma in a Hispanic Patient with macrodactyly: a rare peripheral nerve tumor causing recurrent carpal tunnel syndrome

“…To date, the influence of ethnicity in LFH is unknown despite its description in multiple cases [ 5 , 6 , 8 ]. Silverman and Enzinger described the ethnic distribution of LFH in 14 white patients and 1 black patient [ 5 ].…”

“…Silverman and Enzinger described the ethnic distribution of LFH in 14 white patients and 1 black patient [ 5 ]. Additionally, two other case reports presented LFH in Caucasians [ 6 , 8 ]. We believe that ethnicity should be evaluated as a possible risk factor of LFH in future studies.…”

“…Due to its rarity, there are no guidelines regarding LFH treatment in symptomatic patients with recurrent CTS. A variety of surgical options ranging from the release of carpal tunnel to the complete median nerve excision have been described [ 8 ]. Nowadays, complete nerve excision is rarely recommended due to possible fibrofatty tissue infiltration within nerve bundles, and risk of permanent neurologic deficit [ 10 ].…”

“…Current treatment options are now focused on a conservative approach toward the relief of patient symptoms. A common approach is the open CTR by excision of transverse carpal ligament, which has shown adequate results with pain relief and improvement in the strength of the opponens pollicis muscle [ 7 , 8 ].…”

Role of Ultrasonography in Evaluation of Peripheral Nerves

“…Also there was increased thickness of soft tissues in thenar eminence, mid palm and in thumb with suggestion of hypertrophied subcutaneous fat and fatty infiltration of muscles (Figures 5(c)-(f)) and regional osseous gigantism thumb with premature osteoarthrosis of interphalyngeal joints.Usually patients present with longstanding painless mass. Compression of the affected nerve with paresthesia, motor deficit and pain are known late symptoms[11] [13]. But compressive symptoms may occur only in median nerve involve-M. Aruna Devi, A. Meena DOI: 10.4236/ojim.2018.84022 240 Open Journal of Internal Medicine ment due to encroachment by the flexor retinaculum [11].…”