Journal of Plastic, Reconstructive & Aesthetic Surgery (2014) 67, e282ee283

CORRESPONDENCE AND COMMUNICATION Truncal myxoid Dermatofibrosarcoma Protuberans in a 11-month old child Dear Sir, Dermatofibrosarcoma Protuberans (DFSP) is a rare lowgrade sarcoma of the dermis and usually seen adults that may present in childhood with an incidence of 1 per million children. Fewer than 200 cases have been described.1 A number of subtypes of DFSP exist and in children, only 4 cases of myxoid DFSP have been previously described and none on the trunk.2,3 A 11-month old child was referred by the GP with a short history of an erythematous lesion over her left lumbar region (Image 1). In view of the new onset and growth, a punch biopsy was performed and diagnosis of myxoid DFSP made. An MRI scan was also performed due to the proximity of the plaque to the midline due to concerns regarding involvement with the spinal canal and showed no subfascial involvement or deeper connections (Image 2). Due to the known infiltrative nature of DFSP at its the margins and the risk of local recurrence, excision of the lesion with a margin of 2 cm was performed. This included skin, fascia and muscle, due to the infiltration of the fat by the tumour centrally. The defect was closed with a myocutaneous latissimus dorsi flap. Microscopically, the tumour was similar to that in the biopsy and consisted of bland spindle cells in a pale myxoid stroma with scattered thick collagen fibres. Immunohistochemistry showed diffuse CD34 positive staining in the spindle cells and negative staining for CD31, NSE, desmin, SMA, S100 and EMA. No mitoses, necrosis, giant cells, calcification or fascicles were seen. There were no nodular foci or areas of pleomorphism. It extended through the dermis, focally penetrating into the subcutaneous fat. There was no involvement of the deep fascia or muscle. The tumour margins were clear microscopically. The overlying epidermis was abnormal with mild acanthosis and mild papillomatosis. The karyotype was normal female 46 XX.

The patient was reviewed at regular intervals over a 2year period post-excision, with no evidence of recurrence. The scars have healed well. The myxoid variant of DFSP is rare occurring in 4.3% of all cases. It most commonly affects the limbs, with decreasing frequency in the head and neck followed by the trunk. This is the opposite of the classical defined DFSP that predominantly involves the trunk.3 There have only been 4 previously described cases in childhood (3 male: 1 female).2,3

Figure 1 Photograph illustrating the site of the myxoid DFSP lesion, lying in close proximity to the midline.

http://dx.doi.org/10.1016/j.bjps.2014.07.037 1748-6815/Crown Copyright ª 2014 Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. All rights reserved.

Correspondence and communication

e283 rates of 50e75%, highlighting the importance of achieving adequate excision margins.4 In this case, resection with a 2 cm margin in this young child (18 months at time of surgery) was done due to risks difficulty of reconstruction, but proved sufficient, as no recurrence has been observed to date. In summary, DFSP, especially the myxoid variant, is rare in young children. Early clinical and histological identification would be beneficial to increase the chance of complete excision while avoiding complex reconstruction in small children. Due to some similarities with benign lesions, when presented with a plaque-like lesion DFSP should be borne in mind.

Funding None.

Ethical approval Not required.

Conflict of interest None.

References

Figure 2 MRI image of myxoid DFSP (Identified by the blue arrow) not infiltrating the underlying muscles in a T1-weighted image.

Clinically, DFSP presents as a firm nodular skin lesion that can manifest as a thickened plaque. It tends to be slow growing and resemble those of vascular origin; with a normal, reddish or bluish tinge to the overlying skin,4 as seen here. It may be painless, or painful, with ulceration.5 In this case the lesion was painless, and therefore differential diagnoses of haemangiomatous lesion or birthmark were made. In general, the differential diagnoses for DFSP include haemangiomas, dermatofibromas, neurofibromas, spindle-cell sarcomas and giant cell fibroblastomas.4,5 DFSP originates from the reticular dermis and display pseudopods that infiltrate into the surrounding tissue.4 The myxoid variant is characterised by mucous-like (myxoid) change in >50% of the stroma. CD34 immunohistochemistry is the most specific marker of DFSP (positive in upto 100%) as in with our case. The mainstay of management is surgical excision with adequate excision margins. Previous studies have shown that 3 cm wide local excision of DFSP leads to a recurrence rate of 0e21% but when extended to margins upto 5 cm, recurrence drops to 7%.4 Achieving these margins can be difficult when functional or cosmetic considerations are significant, such as the head and neck region. Inadequate margins in these anatomical areas have led to recurrence

1. Gooskens SL, Oranje AP, van Adrichem LN, et al. Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP). Pediatr Blood cancer 2010;55:369e73. 2. Reimann JD, Fletcher CD. Myxoid dermatofibrosarcoma protuberans: a rare variant analyzed in a series of 23 cases. Am J Surg Pathol 2007;31:1371e7. 3. Campos M, Zarco C, Acquadro F, Riveiro-Falkenbach E, Rodriguez-Peralto JL. Myxoid dermatofibrosarcoma protuberans in childhood. Actas Sifiliogr 2012;103:422e6. 4. Reddy C, Hayward P, Thompson P, Kan A. Dermatofibrosarcoma protuberans in children. J Plast Reconstr Aesthet Surg JPRAS 2009;62:819e23. 5. McKee PH, Fletcher CD. Dermatofibrosarcoma protuberans presenting in infancy and childhood. J Cutan Pathol 1991;18: 241e6.

Mallappa K. Kolar Department of Plastic and Reconstructive Surgery, Clarendon Wing, Leeds General Infirmary, Great George Street, Leeds, LS1 3EX, UK E-mail address: [email protected] Catherine Cullinane Department of Cellular Pathology, St. James’s University Hospital, Beckett Street, Leeds LS9 7TF, UK Grainne Bourke Department of Plastic and Reconstructive Surgery, Clarendon Wing, Leeds General Infirmary, Great George Street, Leeds, LS1 3EX, UK 6 January 2014

Truncal myxoid Dermatofibrosarcoma Protuberans in a 11-month old child.

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