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CORRESPONDENCE
Year : 2016  |  Volume : 61  |  Issue : 4  |  Page : 457-459
Superficial acral fibromyxoma


Department of Dermatology, Leprology and Venereology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Web Publication7-Jul-2016

Correspondence Address:
Ramya C
Department of Dermatology, Leprology and Venereology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.185734

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How to cite this article:
Ramya, Nayak C, Tambe S. Superficial acral fibromyxoma. Indian J Dermatol 2016;61:457-9

How to cite this URL:
Ramya, Nayak C, Tambe S. Superficial acral fibromyxoma. Indian J Dermatol [serial online] 2016 [cited 2022 Dec 5];61:457-9. Available from: https://www.e-ijd.org/text.asp?2016/61/4/457/185734


Sir,

Superficial acral fibromyxoma (SAFM) is a rare slow growing myxoid tumor in the subungual area that was first described in 2001. [1] Histopathologically, SAFM is composed of stellate cells in a myxocollagenous matrix with a poorly circumscribed margin. This tumor is thought to be benign, but its natural course is not fully understood.

A 20-year-old male patient presented with a slow growing fleshy growth under right thumbnail of 1 year duration without any preceding trauma. There was no history of pain on exposure to cold water or cold environment. Cutaneous examination revealed single firm fleshy nodule of size 1 cm × 1 cm over the lateral side of the subungual area of right thumb, displacing the nail with dystrophy without bleeding, eschar, and erosion [Figure 1].
Figure 1: Fleshy subungual growth in the right thumb displacing the nail

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Based on history and cutaneous examination differential diagnosis of subungual exostosis, subungual fibroma and glomus tumor was considered.

Skin biopsy revealed abundant myxoid stroma in the dermis with spindle-shaped cells without any signs of malignancy or cytological atypia [Figure 2] and [Figure 3]. Alcian blue stain confirmed abundant myxoid stroma [Figure 4], suggesting the final diagnosis of SAFM.
Figure 2: Myxoid matrix with dilated blood vessels in the dermis (H and E, ×100)

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Under ring block anesthesia, partial nail avulsion was done and tumor was excised. There has been no recurrence of the tumor 2 years after excision [Figure 5].
Figure 3: Multiple spindle cells in myxoid matrix in (H and E, ×400)

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Figure 4: Abundant myxoid matrix in alcian blue stain, ×400

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Figure 5: No recurrence after 2 years of excision

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SAFM commonly affects subungual region with a predilection for the great toe, although the ventral aspect of fingers, heel, palm, and ankle can also be affected. [2],[3] Preceding trauma has been reported in few cases. [1] Young adults are commonly affected (mean age - 46 years), with male predominance (male:female ratio - 2:1). [1]

It usually presents as a slow growing and painless mass, firm in consistency, ranging in size from 0.6-5 cm. [1] Pain may be noted in up to 10% of the cases. [4] Nail involvement is seen in 50% of cases, with hyperkeratosis or onycholysis.

The tumor is a dome-shaped, polypoid, or verrucous, well-circumscribed, nonencapsulated tumor, which extends throughout the entire dermis. [5] Radiological studies might show underlying bony erosion and scalloping due to mass effect of the tumor. Fine-needle aspiration cytology shows loose cluster of spindle cells in the myxoid material. [6]

On cross-section, it shows yellowish-white jelly like substance. Histopathologically, the tumor is composed of stellate and spindle-shaped fibroblast-like tumor cells with storiform and fascicular or random growth pattern in a variable degree of admixed myxoid or collagenous matrix with lobular pushing or infiltrative margin in the dermis. The collagenous matrix will become predominant over a period. They may show epidermal hyperplasia and hyperkeratosis. There is also blood vessel proliferation, and mast cells are present throughout the dermis with mild nuclear atypia. Tumor cells show positivity for CD34, CD99, vimentin, but epithelial membrane antigen results are inconsistent. Malignant transformation though rare is possible [1] and local recurrence is seen in 22% of the cases. [7]

The differential diagnosis of a mucinous tumor is vast. A close mimic is a solitary superficial acral angiomyxoma which may be the manifestation of the Carney's syndrome. It is locally recurrent, nonmetastatic but occurs more commonly in the fourth decade, and histologically, there is prominent vascularization with myxoid stroma and mucin pools with inflammatory cell component. [8] Digital myxoid cyst arises on or around the distal interphalangeal joint and shows a well-circumscribed, deposit of mucin but few fibroblasts without more vascularity. Cutaneous focal mucinosis lacks a lobular architecture and is acellular with few blood vessels. Low-grade myxofibrosarcomas have small stellate cells with pleomorphic nuclei and pseudolipoblasts. Acquired digital fibrokeratomas are mainly composed of paucicellular and vertically oriented collagen bundles with a collarette.

In conclusion, SAFMs are recently described rare soft tissue tumors. The possibility of an SAFM should be considered when a patient presents with a periungual or subungual slow growing mass. Excision is the treatment, but there are also chances of recurrence, therefore, follow-up is necessary.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Fetsch JF, Laskin WB, Miettinen M. Superficial acral fibromyxoma: A clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes. Hum Pathol 2001;32:704-14.  Back to cited text no. 1
    
2.
Park SW, Kim JH, Shin HT, Park JH, Lee JH, Lee DY, et al. Superficial acral fibromyxoma on the palm. Ann Dermatol 2014;26:123-4.  Back to cited text no. 2
    
3.
Wei C, Fleegler EJ. Superficial acral fibromyxoma of the thumb. Eplasty 2013;13:ic13.  Back to cited text no. 3
    
4.
Wang QF, Pu Y, Wu YY, Wang J. Superficial acral fibromyxoma of finger: Report of a case with review of literature. Zhonghua Bing Li Xue Za Zhi 2009;38:682-5.  Back to cited text no. 4
    
5.
Ashby-Richardson H, Rogers GS, Stadecker MJ. Superficial acral fibromyxoma: An overview. Arch Pathol Lab Med 2011;135:1064-6.  Back to cited text no. 5
    
6.
Raghupathi DS, Krishnamurthy J, Kakoti LM. Cytological diagnosis of superficial acral fibromyxoma: A case report. J Cytol 2015;32:39-41.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Al-Daraji WI, Miettinen M. Superficial acral fibromyxoma: A clinicopathological analysis of 32 tumors including 4 in the heel. J Cutan Pathol 2008;35:1020-6.  Back to cited text no. 7
    
8.
Kura MM, Jindal SR. Solitary superficial acral angiomyxoma: An infrequently reported soft tissue tumor. Indian J Dermatol 2014;59:529.  Back to cited text no. 8
[PUBMED]  Medknow Journal  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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