Dermatologic SurgeryChildhood dermatofibrosarcoma protuberans: Role of preoperative imaging
Section snippets
Case 2
An otherwise healthy 15-year-old girl had an asymptomatic birthmark on her chest. Initially, the lesion was a pink patch, which gradually enlarged and thickened during adolescence. On physical examination she had a 2.0- × 2.5-cm violaceous indurated plaque with two distinct 1.2-cm erythematous nodules with visible superficial telangiectases. The initial biopsy specimens revealed an atypical spindle cell tumor infiltrating into the subcutis consistent with DFSP. Immunohistochemistry with CD34
Discussion
DFSP, a rare spindle cell tumor of the skin, is an intermediate-grade neoplasm characterized by frequent local recurrence and low metastatic potential. The tumor usually appears as a slow-growing violaceous plaque or nodule in adults between the second and fifth decades. The most common locations are the trunk and proximal extremities. Rarely, tumors may develop on the head and neck. Men and women are affected equally. Generally, the tumor grows slowly, often starting as a plaque and later
References (43)
Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature
J Am Acad Dermatol
(2000)Dermatofibrosarcoma protuberans
J Am Acad Dermatol
(1996)- et al.
Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population
J Pediatr Surg
(1998) - et al.
Stromelysin 3 expression: a useful marker for the differential diagnosis dermatofibroma versus dermatofibrosarcoma protuberans
J Am Acad Dermatol
(2002) Recognition and management of unusual cutaneous tumors
Dermatol Clin
(2000)- et al.
Cutaneous and subcutaneous sarcomas
Clin Dermatol
(2001) - et al.
Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans
J Am Acad Dermatol
(1997) - et al.
The role of radiation therapy in the management of dermatofibrosarcoma protuberans
Int J Radiat Oncol Biol Phys
(1998) - et al.
Dermatofibrosarcoma protuberans: treatment of 35 cases
Radiother Oncol
(2000) - et al.
Dermatofibromas progressifs et recidivants ou fibrosarcomas de la peau
Ann Dermatol Syph
(1924)
Uber das Krollentreibende Fibrosarkom der Haut (Dermatofibrosarkoma protuberans)
Dermat Ztshcr
Dermatofibrosarcoma protuberans
Int J Dermatol
Role of surgery in the management of dermatofibrosarcoma protuberans
Ann Surg
Congenital dermatofibrosarcoma protuberans: variability in presentation
Arch Dermatol
Dermatofibrosarcoma protuberans: MR imaging features
AJR Am J Roentgenol
Genetics of nonmelanoma skin cancer
Arch Dermatol
Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans
J Clin Oncol
Dermatofibrosarcoma protuberans presenting in infancy and childhood
J Cutan Pathol
Dermatofibrosarcoma protuberans in childhood
Pediatr Dermatol
Tumors of the fibrous tissue involving the skin
Storiform neurofibroma, pigmented and nonpigmented
Cancer
Cited by (86)
Dermatofibrosarcoma protuberans case report: Aggressive tumor in a preadolescent child
2023, Journal of Pediatric Surgery Case ReportsCutaneous Mesenchymal Sarcomas
2023, Dermatologic ClinicsCitation Excerpt :Sentinel lymph node biopsy (SLNB) or regional node dissection is currently not recommended because there is no known benefit and node-positive disease is rare. MRI may be useful in larger or recurrent tumors to evaluate for infiltrative disease.14 Treatment should follow the NCCN guidelines as summarized here.12,13
Mohs micrographic surgery for dermatofibrosarcoma protuberans in 7 patients aged 10 years and younger
2022, Journal of the American Academy of DermatologySystematic review of Mohs micrographic surgery in children: Identifying challenges and practical considerations for successful application
2021, Journal of the American Academy of DermatologyDermatofibrosarcoma Protuberans: Pathological and Imaging Review
2021, Current Problems in Diagnostic RadiologyThe role of imaging in the management of patients with nonmelanoma skin cancer: When is imaging necessary?
2017, Journal of the American Academy of DermatologyCitation Excerpt :On areas with minimal adipose tissue, such as the hands and the feet, tumor can more easily extend to muscle and even tendon.24 Imaging can play an important role in planning for reconstruction, particularly when muscle or other critical structures are involved.24-26 The superior soft tissue contrast of MRI makes it the ideal imaging modality to assess the extent of a soft tissue neoplasm that may involve muscle.9,27,28
Funding sources: None.
Conflicts of interest: None identified.