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Abstract
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow-growing, locally aggressive soft tissue sarcoma of the skin. The most common age of presentation for this entity of dermis is the third to fifth decade of life, whereas the most common site of involvement is the trunk and lower extremities, accounting for a total of 70 to 90 percent of the cases. Head and neck regions account for 10 to 15 percent of cases with the involvement of the scalp being the most common presentation in this region of the body.[1]
Although DFSP frequently shows local recurrence with the incidence of 20-50 percent of the cases, distant metastasis has been reported rarely.[2] So the cure rate in early diagnosed cases is high with a 10-year survival rate of 99.1 %, so prompt diagnosis of the entity is important along with awareness about the disease.[3] Here in this article, we are going to present a case of DFSP along with a review of the literature regarding the imaging characteristics of the disease.
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References
- Millare GG, Guha-Thakurta N, Sturgis EM, et al. Imaging findings of head and neck dermatofibrosarcoma protuberans. AJNR. American Journal of Neuroradiology 2014;35(2):373-8.
- Rockley PF, Robinson JK, Magid M, et al. Dermatofibromasarcoma protuberans of the scalp: a series of cases. J Am Acad Dermatol 1989;21(2 Pt 1):278-82.
- Brooks J, Ramsey ML. Cancer, dermatofibrosarcoma protuberans. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2020.
- Skoulakis CE, Papadakis CE, Datseris GE, et al. Subcutaneous benign fibrous histiocytoma of the cheek: case report and review of the literature. Acta Otorhinolaryngo lItal 2007;27(2):90-3.
- Lin J, Martel W. Cross-sectional imaging of peripheral nerve sheath tumors: characteristic signs on CT, MR imaging, and sonography. AJR Am J Roentgenol 2001;176(1):75-82.
- Kim HK, Kim SM, Lee SH, et al. Subcutaneous epidermal inclusion cysts: ultrasound (US) and MR imaging findings. Skelet Radiol 2011;40(11):1415–19.
- Wu JS, Hochman MG. Soft-tissue tumors and tumor like lesions: a systematic imaging approach. Radiology 2009;253(2):297-316.
- Walker EA, Fenton ME, Salesky JS, et al. Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am 2011;49(6):1197–217.
- Patnana M, Bronstein Y, Szklaruk J, et al. Multi method imaging, staging, and spectrum of manifestations of metastatic melanoma. Clin Radiol 2011;66(3):224-36.
- Lee HJ, Im JG, Goo JM, et-al. Peripheral T-cell lymphoma: spectrum of imaging findings with clinical and pathologic features. Radiographics 2003;23(1):7-26.
- Darier J, Ferrand M. Dermatofibrosarcoma progressives et recidivants on fibrosarcomas de la plan. Ann Dermatol Venereol 1924;5:545-62.
- Hoffman E. Uber das knollentriebende Fibrokom der Haut. Dermat Zeitschr 1925;43:1-8.
- Mbonde MP, Amir H, Kitinya JN. Dermatofibrosarcoma protuberans: a clinicopathological study in an African population (review). East Afr Med J 1996;73(6):410-3.
- McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans - an analysis of 86 cases - Five with metastasis. Ann Surg 1967;166(5):803-16.
- Burkhardt BR, Soule EH, Winkelmann RK, et al. Dermatofibrosarcoma protuberans. Study of fifty six cases. Am J Surg 1966;111(5):638-44.
- Maire G, Pédeutour F, Coindre JM. COL1A1 PDGFB gene fusion demonstrates a common histogenetic origin for dermatofibrosarcoma protuberans and its granular cell variant. Am J Surg Pathol 2002;26(7):932 7.
- Bhambri S, Desai A, Del Rosso JQ, et al. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol 2008;1(1):34 6.
- Weinstein JM, Drolet BA, Esterly NB, et al. Congenital dermatofibrosarcoma protuberans: variability in presentation. Arch Dermatol 2003;139(2):207 11.
- Kvransdorf MJ, Meis-Kindblom JM. Dermatofibro sarcoma protuberans-radiologic appearance. AJR Am J Roentgenol 1994;163(2):391-4.
- Williams LS, Mancuso AA, Mendenhall WM. Perineural spread of cutaneous squamous and basal cell carcinoma: CT and MR detection and its impact on patient management and prognosis. Int J Radiat Oncol Biol Phys 2001;49(4):1061-69.
- Chang PC, Fischbein NJ, McCalmont TH. Perineural spread of malignant melanoma of the head and neck: clinical and imaging features. AJNR Am J Neuroradiol 2004;25(1):5-11.
- Kim JH, Kim JY, Chun KA, et al. MR imaging manifestations of skin tumors. Eur Radiol 2008;18(11):2652-61.
- Schwarze HP, Loche F, Gorguet MC. Invasive cutaneous squamous cell carcinoma associated with actinic keratosis: a case with orbital invasion and meningeal infiltration. Dermatol Surg 1999;25(7):587-89.
- Brenner W, Schaefler K, Chhabra H, et al. Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review. Cancer 1975;36(5):1897-902.
- Simstein NL, Tuthill RJ, Sperber EE, et al. Dermatofibrosarcoma protuberans: case reports and review of literature. South Med J 1977;70(4):487-89.
- Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer 2004;101(11):2503-8.
- Nakra T, Cook T, Douglas RS, et al. Dermatofibrosarcoma protuberansmetastatic to the orbit. Arch Ophthalmol 2004;122:1240–01
- Mark RJ, Bailet JW, Tran LM, et al. Dermatofibrosarcoma protuberans of the head and neck: a report of 16 cases. Arch Otolaryngol Head Neck Surg 1993;119(8):891-96.
- Voth H, Landsberg J, Hinz T, et al. Management of dermatofibrosarcomatous transformation: an evidence-based review of the literature. J Eur Acad Dermatol Venereol 2011;25(12):1385-91.
- Angouridakis N, Kafas P, Jerjes W, et al. Dermatofibrosarcoma protuberns with fibrosarcomatous transformation of the head and neck. Head Neck Oncol 2011;3:5.
References
Millare GG, Guha-Thakurta N, Sturgis EM, et al. Imaging findings of head and neck dermatofibrosarcoma protuberans. AJNR. American Journal of Neuroradiology 2014;35(2):373-8.
Rockley PF, Robinson JK, Magid M, et al. Dermatofibromasarcoma protuberans of the scalp: a series of cases. J Am Acad Dermatol 1989;21(2 Pt 1):278-82.
Brooks J, Ramsey ML. Cancer, dermatofibrosarcoma protuberans. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2020.
Skoulakis CE, Papadakis CE, Datseris GE, et al. Subcutaneous benign fibrous histiocytoma of the cheek: case report and review of the literature. Acta Otorhinolaryngo lItal 2007;27(2):90-3.
Lin J, Martel W. Cross-sectional imaging of peripheral nerve sheath tumors: characteristic signs on CT, MR imaging, and sonography. AJR Am J Roentgenol 2001;176(1):75-82.
Kim HK, Kim SM, Lee SH, et al. Subcutaneous epidermal inclusion cysts: ultrasound (US) and MR imaging findings. Skelet Radiol 2011;40(11):1415–19.
Wu JS, Hochman MG. Soft-tissue tumors and tumor like lesions: a systematic imaging approach. Radiology 2009;253(2):297-316.
Walker EA, Fenton ME, Salesky JS, et al. Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am 2011;49(6):1197–217.
Patnana M, Bronstein Y, Szklaruk J, et al. Multi method imaging, staging, and spectrum of manifestations of metastatic melanoma. Clin Radiol 2011;66(3):224-36.
Lee HJ, Im JG, Goo JM, et-al. Peripheral T-cell lymphoma: spectrum of imaging findings with clinical and pathologic features. Radiographics 2003;23(1):7-26.
Darier J, Ferrand M. Dermatofibrosarcoma progressives et recidivants on fibrosarcomas de la plan. Ann Dermatol Venereol 1924;5:545-62.
Hoffman E. Uber das knollentriebende Fibrokom der Haut. Dermat Zeitschr 1925;43:1-8.
Mbonde MP, Amir H, Kitinya JN. Dermatofibrosarcoma protuberans: a clinicopathological study in an African population (review). East Afr Med J 1996;73(6):410-3.
McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans - an analysis of 86 cases - Five with metastasis. Ann Surg 1967;166(5):803-16.
Burkhardt BR, Soule EH, Winkelmann RK, et al. Dermatofibrosarcoma protuberans. Study of fifty six cases. Am J Surg 1966;111(5):638-44.
Maire G, Pédeutour F, Coindre JM. COL1A1 PDGFB gene fusion demonstrates a common histogenetic origin for dermatofibrosarcoma protuberans and its granular cell variant. Am J Surg Pathol 2002;26(7):932 7.
Bhambri S, Desai A, Del Rosso JQ, et al. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol 2008;1(1):34 6.
Weinstein JM, Drolet BA, Esterly NB, et al. Congenital dermatofibrosarcoma protuberans: variability in presentation. Arch Dermatol 2003;139(2):207 11.
Kvransdorf MJ, Meis-Kindblom JM. Dermatofibro sarcoma protuberans-radiologic appearance. AJR Am J Roentgenol 1994;163(2):391-4.
Williams LS, Mancuso AA, Mendenhall WM. Perineural spread of cutaneous squamous and basal cell carcinoma: CT and MR detection and its impact on patient management and prognosis. Int J Radiat Oncol Biol Phys 2001;49(4):1061-69.
Chang PC, Fischbein NJ, McCalmont TH. Perineural spread of malignant melanoma of the head and neck: clinical and imaging features. AJNR Am J Neuroradiol 2004;25(1):5-11.
Kim JH, Kim JY, Chun KA, et al. MR imaging manifestations of skin tumors. Eur Radiol 2008;18(11):2652-61.
Schwarze HP, Loche F, Gorguet MC. Invasive cutaneous squamous cell carcinoma associated with actinic keratosis: a case with orbital invasion and meningeal infiltration. Dermatol Surg 1999;25(7):587-89.
Brenner W, Schaefler K, Chhabra H, et al. Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review. Cancer 1975;36(5):1897-902.
Simstein NL, Tuthill RJ, Sperber EE, et al. Dermatofibrosarcoma protuberans: case reports and review of literature. South Med J 1977;70(4):487-89.
Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer 2004;101(11):2503-8.
Nakra T, Cook T, Douglas RS, et al. Dermatofibrosarcoma protuberansmetastatic to the orbit. Arch Ophthalmol 2004;122:1240–01
Mark RJ, Bailet JW, Tran LM, et al. Dermatofibrosarcoma protuberans of the head and neck: a report of 16 cases. Arch Otolaryngol Head Neck Surg 1993;119(8):891-96.
Voth H, Landsberg J, Hinz T, et al. Management of dermatofibrosarcomatous transformation: an evidence-based review of the literature. J Eur Acad Dermatol Venereol 2011;25(12):1385-91.
Angouridakis N, Kafas P, Jerjes W, et al. Dermatofibrosarcoma protuberns with fibrosarcomatous transformation of the head and neck. Head Neck Oncol 2011;3:5.