Adipose Tumors in Peripheral Nerve Systems

Adipose tumours that arise within the peripheral nerve system encompass a spectrum of benign, slow-growing lesions characterised by the proliferation of mature adipocytes and fibrous tissue within neural sheaths. These lesions, which include fibrolipomatous hamartoma and intraneural lipoma, typically affect upper limbs and present with symptoms of compressive neuropathy, macrodactyly or palpable masses. Clinical diagnosis relies on a combination of neurological examination and imaging modalities such as magnetic resonance imaging, which often reveals characteristic coaxial-cable or spaghetti-like appearances. Histologically, these tumours demonstrate fibroadipose infiltration of the epineurium and perineurium with relative preservation of nerve fascicles, guiding management towards conservative decompression or selective debulking rather than radical excision to preserve function. Although rare, their study offers insights into adipocyte–nerve interactions and guides surgical techniques that balance symptom relief with nerve preservation. Recent advances have illuminated potential genetic and molecular pathways underlying aberrant adipogenesis and neural sheath expansion, with implications for targeted therapies and improved prognostic stratification. Globally, understanding these tumours is critical for optimising patient outcomes and sparing neurological function.

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Adipose Tumors in Peripheral Nerve Systems publication trend

The graph below shows the total number of articles in adipose tumors in peripheral nerve systems across all publications each year (not limited to Nature Index journals).

Technical terms

Fibrolipomatous hamartoma (FLH): A benign lesion characterised by fibroadipose proliferation within the nerve sheath, leading to nerve enlargement.

Macrodystrophia lipomatosa (MDL): A congenital condition in which disproportionate adipose and fibrous tissue growth causes localised limb overgrowth, often associated with FLH.

Epineurium: The outermost connective tissue layer surrounding a peripheral nerve, within which adipose tumours may infiltrate.

Perineurium: The protective sheath encasing individual nerve fascicles, commonly infiltrated by fibroadipose tissue in these tumours.

Intraneural lipoma: A true encapsulated lipoma arising within the nerve trunk, distinct from diffuse fibrolipomatous proliferation.

References

1. Fibrolipomatous hamartoma in the median nerve in the arm - an unusual location but with MR imaging characteristics: a case report*. Journal of Brachial Plexus and Peripheral Nerve Injury (2014).
2. Fibrolipomatous hamartoma of the median nerve: An unusual cause of carpal tunnel syndrome. Clinical Case Reports (2023).
3. 10-Year Clinical Follow-Up after Decompression of Lipofibromatous Hamartoma of the Median Nerve in a 3-Year-Old Patient: Case Report and Review of the Literature. Children (2023).
4. Fibrolipomatous hamartroma with macrodactyly in a 4 years old female patient: A case report. International Journal of Surgery Case Reports (2024).