Lipofibromatous hamartoma of nerve

Seventeen cases of lipofibromatous hamartoma of nerve (14 with histologic confirmation) were treated between 1935 and 1985. One patient had bilateral involvement. Fourteen lesions were located in the upper extremity and four in the lower extremity. Twelve of the 18 lesions were associated with combined soft tissue and skeletal enlargement, or true macrodactyly. Three other lesions had soft tissue enlargement other than that noted intraneurally. Three patients were treated with carpal tunnel release alone and 14 with combined procedures to decrease the size of the affected part. Sensibility in the affected part often appeared unrelated to removal or preservation of hamartomatous nerve tissue. Complications related to nerve surgery included recurrence of carpal tunnel syndrome in one patient, recurrent soft tissue mass in one patient, and painful calcification of the involved tissues 20 years postoperatively in one patient.     

Lipofibromatous hamartoma of the digital nerve without macrodactyly: follow-up of an unusual case

Lipofibromatous hamartoma (LFH) of nerve is a rare benign, tumour-like fibro-fatty malformation of the epi- and perineurium. Major peripheral nerves of the upper extremity are mostly involved, most commonly the median nerve and its branches. Although there is a relatively large number of publications on LFH, involvement of digital nerves is rarely reported or described. A case of LFH involving the digital ulnar nerve of the index finger without macrodactyly is presented; the aetiology, pathogenesis, differential diagnosis and surgical management are described.     

Lipofibromatous hamartoma of the median nerve: A case report and review of the literature

A case of lipofibromatous hamartoma of the median nerve in an adult is described in this article. A 33-year-old male presented with tingling, numbness and swelling in the palm of the left non dominant hand that had been present for a few months. Examination revealed that there was fullness in the volar aspect of the wrist and in the inter-thenar area. Another mass was present at the base of the index finger, which appeared to be involving subcutaneous tissues. The clinical diagnosis was carpal tunnel syndrome due to a space occupying tumor mass in the carpal tunnel. On exploration of the carpal tunnel, a large median nerve was seen 4 cm proximal to the wrist crease line and extending distally until it divided into its branches. Another mass was present at the base of the index finger, which was adherent to the skin. The radial digital nerve of the index finger was normal in size. For the enlarged nerve, an epineurotomy was performed and a biopsy was taken. Another biopsy was taken from the distal mass. His postoperative period was uneventful. In July 2004, at the end of 5 years, the patient had no symptoms and the size of the tumor had not increased.     

Lipofibromatous hamartoma of the median nerve

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 of the median nerve

Lipofibromatous hamartoma of the nerve is a very uncommon, congenital, benign, peripheral nerve tumor. It is mostly encountered in the extremities of young adults, involving the median nerve in the majority of cases. The nerve tissue is infiltrated by diffuse fibroadipose tissue which dissociates the fasciculi without invasion. Patients with lipofibromatous hamartoma of the median nerve usually present with symptoms of carpal tunnel syndrome, usually accompanied by marked macrodactyly. Lipofibromatous hamartoma of the median nerve was encountered in an 18-year-old female patient, involving the wrist, causing macrodactyly of the index finger, and resulting in symptoms of carpal tunnel syndrome. Median nerve release and partial excision of the adipose tissue along the mass were performed. Fourteen months postoperatively, the patient had no complaints and the mass decreased in size without any motor or sensory functional losses.     

Lipofibromatous hamartoma of the median nerve and its associated conditions

This study reports on ten cases of lipofibromatous hamartoma of the median nerve, with specific references to its associated conditions. Macrodactyly was the most common associated condition and was seen in 6 cases. Two cases had associated fatty tumours and another two had bony tumours. The literature was reviewed and a classification of the sites of involvement of the median nerve and its associated conditions is suggested.     

Lipofibromatous hamartoma of the median nerve with macrodactyly of middle finger

Lipofibromatous Hamartoma (LFH) is a rare tumour affecting the peripheral nerves, mostly the median nerve. It is benign and slowly growing. Less than 100 cases have been reported in the literature, about one quarter of them are associated with macrodactyly in the field of the affected nerve.We reported a case of a 13 years old female with LFH of the median nerve at the level of the wrist associated Macrodactyly of the ipsilateral middle finger treated surgically by decompression only.     

Endobronchial hamartoma. A case report.

A patient with a very rare benign bronchial tumour who presented in a most unusual manner is described. An endobrachial hamartoma was expectorated 24 hours after bronchoscopic manipulation for a suspected malignant tumour.     

Malignant schwannoma of the digital nerve in a child. A case report

An extremely rare case of malignant schwannoma (malignant peripheral nerve sheath tumor) arising in the digital nerve in the right ring finger of a child is described. The patient was an 8-year-old boy who did not have neurofibromatosis (von Recklinghausen's disease). He noticed a small tender mass on the tip of his right ring finger in 1991. The lesion was excised twice. However, the tumor recurred soon after each surgery. Histologic examination revealed malignant schwannoma. He eventually underwent disarticulation of the ring finger at the metacarpophalangeal joint in 1995. There was no recurrence or distant metastasis at followup in July 1999. Solitary malignant schwannomas can occur in the small peripheral nerves and in the major nerves.     

Anomalous digital nerve loop: A case report

In this case, a digital nerve loop was observed during the dissection of the digital nerves to the third web space; the common digital artery was passing through this loop. This rare case is presented and its importance is discussed. © 1998 Wiley-Liss, Inc. MICROSURGERY 18:170–171 1998     

Haemangioma of the digital nerve: a case report

An intra-neural haemangioma of the digital nerve of the left index finger is described, the first report of such a lesion in a digit. Treatment consisted of excision of the tumour and the affected portion of the nerve, followed by direct coaptation of the nerve ends.     

Lipofibromatous hamartoma: review of early diagnosis and treatment.

BACKGROUND: Lipofibromatous harmartoma (LFH) is a rare tumour that requires early diagnosis and treatment. To alert physicians to the possibility of this tumour, we review the epidemiologic and pathological characteristics, the presentation, diagnosis and treatment of LFH in the upper extremity. METHODS: We obtained data from a MEDLINE search of the English literature from 1966 to June 2003, using the search terms "lipofibromatous," "hamartomas of the nerve," "macrodactyly" and "intraneural lipoma." STUDY SELECTION: Articles that demonstrated institutional experience (epidemiologic, diagnostic and therapeutic, and complications) and those that provided general reviews were selected for inclusion. Of 501 identified articles, 42 fulfilled the review criteria. DATA EXTRACTION: Relevant clinical, diagnostic and therapeutic evaluations were extracted from the literature and synthesized. DATA SYNTHESIS: LFH is distinguished pathologically from other intraneural tumours by fibrofatty infiltration around the nerve fascicles. Typically, patients present either early with macrodactyly or later with a forearm mass lesion or symptoms consistent with a compressive neuropathy of the affected nerve. Although MRI is an important tool in the diagnosis of LFH, the diagnosis should be supported with a tissue biopsy. Treatment for patients with no neurologic deficit involves prophylactic decompression of affected nerves at all points of possible compression. Patients with macrodactyly may benefit from debulking, joint reconstruction or amputation. CONCLUSIONS: LFH differs from other benign nerve tumours with respect to clinical presentation, pathological characteristics, MRI features and management. Because LFH is extensive and inseparable from the nerve fascicles, excision is not recommended.