Lipofibromatous hamartoma (LFH) of the median nerve is a rare condition in the hand and often remains asymptomatic for a significant period. MRI imaging can reveal unique tumour characteristics; however, the definitive diagnosis is confirmed through a tissue biopsy. In this report, a 38-year-old male presented with a gradually growing mass on his right hand. Physical examination revealed a large soft tissue mass extending from the thenar area to the wrist, causing compression of the median nerve. MRI confirmed the presence of a distinct soft tissue mass on the volar side of the hand. The mass was excised along with a fascicle and confirmed by histological examination. One year after surgery, sensation has improved, but weakness remains and opponensplasty was offered to the patient. Although the treatment strategy of LFH of the median nerve remains controversial, delayed treatment can result in severe compressive neuropathy and irreversible nerve damage. Level of Evidence: Level V (Therapeutic).

Herein, we report a 48-year-old male with lipofibromatous hamartoma-induced secondary carpal tunnel syndrome, treated successfully with decompression. The patient presented to the emergency with complaints of paresthesia and oedema of the wrists. When non-invasive methods failed to ease symptoms and nerve damage became a big concern, surgical intervention was recommended. Surgery to remove the nerve sheath and expose the carpal tunnel alleviated pressure on the median nerve. After a 3-year follow-up, the patient reported no discomfort, paresthesia, or limitation in wrist or finger movements, indicating a remarkable recovery. The tumour did not recur, and the sensorium was unharmed.

Lipofibromatous hamartoma, first reported in 1953, is a rare, slowly progressive soft tissue tumor, the characteristics of which include the enlargement of the affected nerve via the epineurial and perineurial proliferation of adipose and fibrous tissues. Out of 200 previously reported cases of lipofibromatous hamartoma of the median nerve, there have been approximately 25 pediatric cases under the age of 18. Herein, we report a case of lipofibromatous hamatoma of the median nerve in a 3-year-old female patient who was surgically decompressed via carpal tunnel release and epineurolysis. The patient was followed-up on an outpatient clinic basis annually with sonographic evaluations, and the postoperative 10th-year follow-up did not show recurrence or any deficits in motor and sensory functions.

Lipofibromatous hamartoma (LFH) is a rare benign peripheral nerve tumor. The median nerve (MN) is most commonly affected in the upper extremity. We report a case of a 39-year-old male with LFH of the median nerve presented with swelling and symptoms of carpal tunnel syndrome treated successfully with decompression. LFH is reported with various descriptions because of the proliferative nature of its adipocytes and the fibrofatty infiltration within the peripheral nerves. Swelling around the volar aspect of the wrist remains the most frequent presentation of LFH. Surgical decompression without tumor resection can result in symptom improvement. In addition, post-decompression nerve coverage can be a solution to improve the residual hyperesthesia symptoms.

BACKGROUND: Macrodystrophia lipomatosa (MDL) is characterized by progressive overgrowth affecting soft tissues and bony structures and is part of lipomatous overgrowth syndromes. MDL has been associated with lipomatosis of the nerve (LN), an adipose lesion of nerve that has a pathognomonic magnetic resonance imaging (MRI) appearance as well as a mutation in the PIK3CA gene. The authors present a case of occult LN in the setting of MDL.
METHODS: A 2-year-old boy with progressive soft tissue overgrowth of his proximal right lower extremity was initially diagnosed with neurofibromatosis type 1 (NF1). At our institution, NF1 as well as other overgrowth syndromes including PTEN hamartoma tumor syndrome were excluded. He was diagnosed as having so-called MDL. Upon reinterpretation of the patient\'s MRI studies, short-segment LN involving the proximal sciatic nerve and part of lumbosacral plexus was identified. He underwent 2 debulking/liposuction procedures for soft tissue overgrowth. Genetic testing of tissue revealed a mutation in PIK3CA.
CONCLUSIONS: Thorough clinical examination (for signs of overgrowth) as well as an MRI study of the entire neural pathway is a critical part of the diagnostic workup to evaluate for LN. The authors believe that an increasing association of LN, even when occult, will emerge that will explain many cases with marked nerve-territory overgrowth.

Lipofibromatous Hamartoma (LFH) is a rare and slow growing benign tumor affecting the peripheral nerves, which usually involves the median nerve. Median nerve involvement commonly causes pain, numbness, paresthesia and carpal tunnel syndrome (CTS). This article describes a case of lipofibromatous hamartoma in a 6-years-old girl, complained of the mass and numbness in her left distal forearm. Microsurgical interfascicular dissection operation was performed to remove the epineural proliferation tissue, numbness disappeared after the operation. At the 12-months follow-up appointment she remained asymptomatic and there was no change in mass size.

Lipofibromatous hamartoma (LFH) is a rare benign slow growing fibrofatty tumour. It comprises a neurogenic mass of non-inflamed peripheral nerve bundles surrounded and separated by mature fat. LFH has a tendency for the median nerve; however, involvement of the brachial plexus, ulnar, radial, peroneal and plantar nerves has also been described. Patients typically experience pain, with or without sensory and motor deficits. The diagnosis of LFH is highly supported by imaging findings. Ultrasound is increasingly being recognised as a useful tool in aiding diagnosis.

Lipofibromatous hamartoma (LFH) is a benign tumor that causes nerve enlargement due to fatty adipose tissue infiltration around bundles of peripheral nerves. It most commonly occurs at the median nerve with associated macrodactyly. We present an uncommon case of LFH that affected a digital branch of the median nerve without macrodactyly.

Peripheral Nerve Sheath Tumors (PNSTs) are extremely uncommon and it is almost certain that no individual upper limb surgeon will gain great experience in a lifetime with these lesions. Benign and malignant PNSTs are separately analyzed in this descriptive review and discussed focusing the attention towards the most important features. A comprehensive and summarized overview of this topic is offered to the reader in order to improve the complex management of these tumors from diagnosis to treatment. A systematic search in PubMed was carried out using the keywords (and synonyms) written below in order to find relevant and most cited papers. Reckoning the rarity of the pathology, few selected case reports were taken into account. A compendium of each PNST was created to sum up the personal experience of the Authors who wrote the articles, critically inspected and analyzed. Every section of the paper is meant to provide useful tips to the reader.

Adipose lesions of nerve primarily include intra- and extraneural lipomas and lipomatosis of nerve (LN). This paper will summarize the advances that have been made in the past decade, particularly related to LN and nerve territory overgrowth that have improved our understanding of the natural history, genetic background, diagnosis, imaging features, and clinical management.
Articles about adipose lesions of nerve were reviewed from 2011, when the last comprehensive review on this topic was published. Papers reporting advances on natural history, genetic background, diagnosis, imaging features, and clinical management were screened using PubMed and Google Scholar databases and then analyzed. Case reports and small case series were included only if they reported model examples of discussed pathologies, as these types of articles were summarized in recent systematic reviews on intraneural lipomas and LN. All eligible papers were assessed by the authors, who are subject matter experts.
The first screen revealed 404 articles. After careful evaluation, a total of 53 articles were analyzed which includes advances in diagnosis (especially imaging), classification of the lesions, the role of somatic mutations in PIK3CA in LN, and treatment approaches for all adipose lesions of the peripheral nerve.
Many advances have been made in the understanding of adipose lesions of nerve in the past decade. These pathologic entities are more readily recognized as a spectrum of lesions that share common phenotypic features.