|Year : 2015 | Volume
| Issue : 1 | Page : 45-47
Dermatofibrosarcoma protuberans in a child
Jyoti Prakash Swain
Department of Skin V.D., Chhattisgarh Institute of Medical Sciences, Bilaspur, Chhattisgarh, India
|Date of Web Publication||16-Jan-2015|
Jyoti Prakash Swain
Department of Skin V.D, Chhattisgarh Institute of Medical Sciences, Bilaspur - 495 001, Chhattisgarh
Source of Support: None, Conflict of Interest: None
Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of low-grade malignant potential. Although rare, DFSP is especially challenging to recognize and diagnose in children. In children, the clinical appearance may be heterogeneous, and a high index of suspicion is necessary to avoid delays in diagnosis that can lead to further morbidity. Prompt and definitive treatment is essential based on the tumor's high rate of recurrence. Histologic examination, often with the use of appropriate immunostains, is necessary for diagnosis. Surgery with wide local excision or Mohs micrographic surgery is the mainstay of treatment.
Keywords: Dermatofibrosarcoma protuberans, histopathology, Mohs micrographic surgery
|How to cite this article:|
Swain JP. Dermatofibrosarcoma protuberans in a child. Indian J Paediatr Dermatol 2015;16:45-7
| Introduction|| |
Dermatofibrosarcoma protuberans (DFSP) is a relatively rare slow growing neoplasm affecting the skin. It is an infiltrative tumor of intermediate grade neoplasm originating from the dermal layer of the skin  with a limited potential for metastasis but a high rate of recurrence. It is most often found on the trunk and proximal limbs. It commonly favors young to middle-aged adults. However, it can also occur in infancy and childhood. The incidence in children is even less frequent, although the proportion of those identified in adulthood may reflect a delay in diagnosis of childhood DFSP. Because of the rarity of the tumor and its variegated appearance, diagnosis of DFSP in children is quite difficult. Here we are presenting a case of DFSP in pediatric age group.
| Case report|| |
A 10-year-old girl presented with a 2 years history of a rapid growing, violaceous, multinodular mass located on the left upper abdomen. No other of his family member had similar illness. We observed a firm light darkish nodular swelling of 15 cm diameter with a slightly atrophic epidermal cover in the left upper abdomen [Figure 1]. The lesion was moveable to the underlying tissue but not the epidermis. There was no tenderness upon palpation of the lesion. There were no other skin/mucosal lesions, nail changes or any recent weight loss, night sweats and fevers. The lymph nodes in the head and neck region and the axillae were nonpalpable. Fine needle aspiration cytology was performed with 23 G needle, and provisional diagnosis of the mesenchymal neoplasm was made. Subsequently an excision biopsy was performed, and final diagnosis of DFSP was made based on the histopathological findings. The biopsy showed a spindle-cell neoplasm located primarily in the dermis with infiltration of the subcutaneous fat. The tumor was composed of uniform large spindle cells organized in a storiform or cartwheel pattern. Except for a subepidermal grenz zone, tumor cells were infiltrating the whole dermis and the upper subcutaneous fat. The neoplastic cells showed uniform slender and elongated nuclei with scanty pale cytoplasm [Figure 2]. The biopsy findings were consistent with the diagnosis of DFSP. The patient was then referred to oncosurgery department of Raipur Medical College. Complete surgical excision (Mohs micrographic surgery) was performed. A regular follow-up was recommended for the next 5 years.
|Figure 1: Violaceous, multinodular mass located on the left upper abdomen of size 15 cm × 10 cm|
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|Figure 2: Section showing uniform spindle cells arranged in short fascicles and storiform pattern (H and E, ×10). The tumor was seen infiltrating into the underlying subcutis|
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| Discussion|| |
Dermatofibrosarcoma protuberans is a low-grade, relatively uncommon, soft tissue sarcoma with a marked recurrence rate, but it rarely metastasizes. In 1924, Darier and Ferrand first described the entity of DFSP.  In 1925, Hoffman reported three new cases and coined the term DFSP.  This tumor usually presents between 20 and 50 years of age, and is rare in children with a slight male predominance.  It is most often found on the trunk and proximal limbs and is rare on the head and neck.  The tumor most commonly presents as a slow-growing, asymptomatic, skin-colored, indurated, firm plaque that eventually develops violaceous to red-brown nodules of varying size from one to several centimeters in diameter.  DFSP may present as an atrophic plaque,  which may resemble morphea and can be misdiagnosed.
Cytological smears of DFSP usually show scanty to moderate cellularity comprising scattered single cells and clusters with naked nuclei. The biopsy typically shows the lesion to be located primarily in the dermis with irregular infiltration of the subcutaneous fat in a lace-like pattern. The epidermis is usually spared. The lesion usually comprises highly cellular neoplasm consisting of fairly uniform spindle cells with elongated nuclei and scanty pale cytoplasm arranged primarily in storiform pattern. Pleomorphism is minimal or absent. Immunohistochemically the spindle-shaped cells are strongly positive for CD34 immunostaining.  The general immunostaining pattern of DFSP is CD34 positive and factor XIIIa negative. Dermatofibromas are CD34 negative and factor XIIIa positive. A genetic link has been found in some patients with DFSP, which frequently (more than 90%) exhibits translocation of chromosomes 17 and 22, t (17;22). 
The DFSP in pediatric age group should be differentiated with other fibrohistiocytic tumors like dermatofibroma, leiomyoma, neurofibroma, dermatomyofibroma, infantile myofibromatosis, and fibrous hamartoma of infancy. DFSP in children is often misdiagnosed, leading to delay in treatment. Wide local excision is necessary, since DFSP often exhibits extensive infiltration beyond gross margins. Surgical excision with clear margins is critical for cure. Micrographic control of excision margins improves outcome and reduces recurrence rates while limiting the need of closure by flaps or grafts.  Adjuvant radiotherapy reduces the local recurrence rate when wide local excision is impossible because of anatomical constraints or functional and cosmetic concerns.
| Conclusion|| |
Dermatofibrosarcoma protuberans is rare in patients younger than 16 years of age. But early histological confirmation of diagnosis will improve the outcome and avoid larger excisions.
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[Figure 1], [Figure 2]