Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 547
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

LETTER TO EDITOR Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 1  |  Page : 177-178
Lipofibromatous hamartoma of median nerve

Department of Pathology, JSS Medical College, Mysore, Karnataka, India

Click here for correspondence address and email

Date of Web Publication19-Jan-2010

How to cite this article:
Sheela Devi C S, Suchitha S, Sunila R, Manjunath G V. Lipofibromatous hamartoma of median nerve. Indian J Pathol Microbiol 2010;53:177-8

How to cite this URL:
Sheela Devi C S, Suchitha S, Sunila R, Manjunath G V. Lipofibromatous hamartoma of median nerve. Indian J Pathol Microbiol [serial online] 2010 [cited 2023 Sep 29];53:177-8. Available from:


The term lipofibromatous hamartoma (LFH) of the nerve is most commonly used to describe the proliferation of mature adipocytes within the peripheral nerves, resulting in a palpable yellow neurogenic mass. LFH is a rare disorder that most commonly manifests in the nerves of the upper extremity. [1] The median nerve is the most commonly involved peripheral nerve. Patients typically present in their third or fourth decade of life, with a long history of painless swelling in the distal forearm that often is first noted in childhood. [1]

A 20-year-old male patient presented with a soft, mobile swelling of two years duration in the palmar aspect of right hand in the region of the thenar eminence and extending to the middle finger. No neurological deficits were elicited. An X-ray of his right hand was done and the radiologist's provisional diagnosis was hemangioma. Per-operatively, a gray-yellow fusiform mass was seen attached to the median nerve, measuring 6x4x3cm. He underwent an excision biopsy of the mass.

Grossly, the specimen consisted of multiple gray-yellow fragments, totally measuring 5x3cm. Histologically, the epineurium of the nerve was expanded by infiltrating mature fibro-fatty tissue [Figure 1] with perineural fibrosis and septation [Figure 2] and [Figure 3]. The nerve fascicles were atrophic. A diagnosis of LFH of the nerve was made. The patient had deficits in hand function following the biopsy. He was advised a nerve graft for the same. However, he was lost to follow-up.

LFH, first described in English literature by Mason in 1953, is a rare condition characterized by diffuse enlargement of the nerve caused by overgrowth and proliferation of fibrofatty tissue. The lesion is described as a hamartoma because fat and fibrous tissues are normal constituents of the connective tissue stroma of a nerve. [2] Although most commonly reported in the median nerve, the lesion also occurs in the radial, sciatic, ulnar, cranial and plantar nerves. [2],[3] More than one-third of the cases are associated with macrodactyly when it is referred to as macrodystrophia lipomatosa. The most frequent presentation of LFH is a swelling or mass on the volar aspect of wrist or distal forearm. Neurologic deterioration occurs after some years. [3]

Magnetic Resonance Imaging (MRI) has a diagnostic role in the investigation of a suspected LFH. [1],[3] A characteristic feature is the appearance of serpentiform nerve fascicles surrounded by fibro fatty tissue within the expanded nerve sheath. On coronal images, the nerve has a spaghetti-like appearance and a coaxial cable-like appearance on axial images is pathognomonic of LFH. [3],[4] MRI should be always supported with a tissue biopsy, particularly in cases presenting as an asymptomatic mass lesion. [3] The biopsy, however, has to be of a small cutaneous nerve. [5]

At surgery, LFH of the nerve appears as a soft gray-yellow, fusiform mass that has diffusely infiltrated and replaced portions of a large nerve and its branches not adherent to surrounding tissues. [5],[6] Microscopic examination reveals the epineurium expanded by adipose tissue. [6] This infiltration causes atrophy of the neural elements often accompanied by extensive perineural fibrosis. [3]

The histologic differential diagnoses include neuroma, neurofibroma, intraneural lipoma and diffuse lipomatosis. However, in LFH, there is atrophy of neural elements, is diffusely infiltrative and it primarily affects the nerves. [5]

Treatment of LFH is difficult because the lesion cannot be completely excised without sacrificing the involved nerve. [2] Inadvertent excision, as in this case, may inevitably lead to a significant neurological deficit. [6] LFH of the nerve is a rare cause of nerve associated swelling. A combination of MRI and biopsy is diagnostic. However, the importance of recognizing its possibility lies in the fact that a poorly planned surgical treatment is a greater hazard than the natural clinical outcome. This underlines the need for a greater awareness of this condition by the trinity of clinician, radiologist and pathologist.

   References Top

1.Toms AP, Anastakis D, Bleakney RR, Marshall TJ. Lipofibromatous Hamartoma of the Upper Extremity: A review of the radiologic findings for 15 patients. AJR 2006;186:805-11.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Cui Q, Chhabra AB, Leo BM, Pannunzio ME. Lipofibromatous Hamartoma of a Digital Nerve. Am J Orthop 2008;37:146-8.   Back to cited text no. 2      
3.Razzaghi A, Anastakis DJ. Lipofibromatous hamartoma: review of early diagnosis and treatment. Can J Surg 2005;48:394-9.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Jain TP, Srivastava DN, Mittal R, Gamanagatti S. Fibrolipomatous hamartoma of median nerve. Australasian Radiology 2007;51:98-100.  Back to cited text no. 4      
5.Weiss SW, Goldblum JR. Soft tissue tumors. In: Enzinger and Weiss, editors. Benign lipomatous tumors. 4th ed. St. Louis, Missouri: Mosby, Inc; 2001.p. 617-9.   Back to cited text no. 5      
6.Hsu CC, Chen DJ, Chen HC. Lipofibromatous hamartoma of the median nerve with long term follow up. Chang Gung Med J 2005;28:111-5.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  

Correspondence Address:
C S Sheela Devi
367, Adarsha, 6th cross, T K layout, Kuvempunagar, Mysore-570 009, Karnataka
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.59225

Rights and Permissions


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Extensive intraneural fascicular dissection of a lipofibromatous hamartoma of the ulnar digital nerve of the thumb
Mohamed Elsaid Abdelshaheed
European Journal of Plastic Surgery. 2018; 41(5): 605
[Pubmed] | [DOI]
2 Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review
Jenna-Lynn Senger,Dale Classen,Garth Bruce,Rani Kanthan
Plastic Surgery. 2014; 22(3): 201
[Pubmed] | [DOI]
3 Lipomatosis of nerve: A clinicopathologic analysis of 15 cases
Mao, R.-J., Yang, K.-F., Wang, J.
Chinese Journal of Pathology. 2011; 40(3): 165-168


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded105    
    Comments [Add]    
    Cited by others 3    

Recommend this journal