Indian Journal of Dermatology
CORRESPONDENCE
Year
: 2016  |  Volume : 61  |  Issue : 4  |  Page : 457--459

Superficial acral fibromyxoma


Ramya C, Chitra Nayak, Swagata Tambe 
 Department of Dermatology, Leprology and Venereology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Ramya C
Department of Dermatology, Leprology and Venereology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra
India




How to cite this article:
Ramya, Nayak C, Tambe S. Superficial acral fibromyxoma.Indian J Dermatol 2016;61:457-459


How to cite this URL:
Ramya, Nayak C, Tambe S. Superficial acral fibromyxoma. Indian J Dermatol [serial online] 2016 [cited 2022 Sep 27 ];61:457-459
Available from: https://www.e-ijd.org/text.asp?2016/61/4/457/185734


Full Text

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}

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}

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}{Figure 4}{Figure 5}

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.

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Conflicts of interest

There are no conflicts of interest.

References

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