Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck. 2011

Nikolaos Angouridakis, and Panagiotis Kafas, and Waseem Jerjes, and Stefanos Triaridis, and Tahwinder Upile, and Georgios Karkavelas, and Angelos Nikolaou
Otorhinolaryngology, Head and Neck Surgery Department, AHEPA University Hospital, Thessaloniki, Greece. nikos.angouridakis@gmail.com

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up.Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.

UI MeSH Term Description Entries
D002471 Cell Transformation, Neoplastic Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill. Neoplastic Transformation, Cell,Neoplastic Cell Transformation,Transformation, Neoplastic Cell,Tumorigenic Transformation,Cell Neoplastic Transformation,Cell Neoplastic Transformations,Cell Transformations, Neoplastic,Neoplastic Cell Transformations,Neoplastic Transformations, Cell,Transformation, Cell Neoplastic,Transformation, Tumorigenic,Transformations, Cell Neoplastic,Transformations, Neoplastic Cell,Transformations, Tumorigenic,Tumorigenic Transformations
D005260 Female Females
D006258 Head and Neck Neoplasms Soft tissue tumors or cancer arising from the mucosal surfaces of the LIP; oral cavity; PHARYNX; LARYNX; and cervical esophagus. Other sites included are the NOSE and PARANASAL SINUSES; SALIVARY GLANDS; THYROID GLAND and PARATHYROID GLANDS; and MELANOMA and non-melanoma skin cancers of the head and neck. (from Holland et al., Cancer Medicine, 4th ed, p1651) Cancer of Head and Neck,Head Cancer,Head Neoplasm,Head and Neck Cancer,Head and Neck Neoplasm,Neck Cancer,Neck Neoplasm,Neck Neoplasms,Neoplasms, Upper Aerodigestive Tract,UADT Neoplasm,Upper Aerodigestive Tract Neoplasm,Upper Aerodigestive Tract Neoplasms,Cancer of Head,Cancer of Neck,Cancer of the Head,Cancer of the Head and Neck,Cancer of the Neck,Head Neoplasms,Head, Neck Neoplasms,Neoplasms, Head,Neoplasms, Head and Neck,Neoplasms, Neck,UADT Neoplasms,Cancer, Head,Cancer, Neck,Cancers, Head,Cancers, Neck,Head Cancers,Neck Cancers,Neoplasm, Head,Neoplasm, Neck,Neoplasm, UADT,Neoplasms, UADT
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D018223 Dermatofibrosarcoma A sarcoma of the deep layers of the skin. The tumors are locally aggressive tends to recur but rarely metastatic. It can be classified into variants depending on the cell type tumors are derived from or by its characteristics: Pigmented variant from MELANIN-containing DERMAL DENDRITIC CELLS; Myxoid variant, myxoid STROMAL CELLS; Giant cell variant characterized by GIANT CELLS in the tumors; and Fibrosarcomatous variant chracterized by tumor areas histologically indistinguishable from FIBROSARCOMA. Bednar Tumor,Bednar's Tumor,DFSP, Fibrosarcomatous,DFSP, Juvenile,DFSP, Myxoid,DFSP, Pigmented,Darier-Ferrand Tumor,Darier-Hoffmann Tumor,Dermatofibrosarcoma Protuberan,Dermatofibrosarcoma Protuberans, Familial,Dermatofibrosarcoma Protuberans, Fibrosarcomatous,Dermatofibrosarcoma Protuberans, Giant Cell,Dermatofibrosarcoma Protuberans, Myxoid,Dermatofibrosarcoma Protuberans, Pigmented,FS-DFSP,Familial Dermatofibrosarcoma Protuberans,Fibrosarcomatous Dermatofibrosarcoma Protuberans,Giant Cell Fibroblastoma,Giant Dermatofibrosarcoma Protuberans,Metastatic Dermatofibrosarcoma Protuberans,Bednars Tumor,Cell Fibroblastoma, Giant,Cell Fibroblastomas, Giant,DFSPs, Fibrosarcomatous,DFSPs, Juvenile,DFSPs, Myxoid,DFSPs, Pigmented,Darier Ferrand Tumor,Darier Hoffmann Tumor,Dermatofibrosarcoma Protuberan, Familial,Dermatofibrosarcoma Protuberan, Fibrosarcomatous,Dermatofibrosarcoma Protuberan, Giant,Dermatofibrosarcoma Protuberan, Metastatic,Dermatofibrosarcoma Protuberan, Myxoid,Dermatofibrosarcoma Protuberan, Pigmented,Dermatofibrosarcoma Protuberans,Dermatofibrosarcoma Protuberans, Giant,Dermatofibrosarcoma Protuberans, Metastatic,Dermatofibrosarcomas,Familial Dermatofibrosarcoma Protuberan,Fibroblastoma, Giant Cell,Fibroblastomas, Giant Cell,Fibrosarcomatous DFSP,Fibrosarcomatous DFSPs,Fibrosarcomatous Dermatofibrosarcoma Protuberan,Giant Cell Fibroblastomas,Giant Dermatofibrosarcoma Protuberan,Juvenile DFSP,Juvenile DFSPs,Metastatic Dermatofibrosarcoma Protuberan,Myxoid DFSP,Myxoid DFSPs,Myxoid Dermatofibrosarcoma Protuberan,Myxoid Dermatofibrosarcoma Protuberans,Pigmented DFSP,Pigmented DFSPs,Pigmented Dermatofibrosarcoma Protuberan,Pigmented Dermatofibrosarcoma Protuberans,Protuberan, Dermatofibrosarcoma,Protuberan, Familial Dermatofibrosarcoma,Protuberan, Fibrosarcomatous Dermatofibrosarcoma,Protuberan, Giant Dermatofibrosarcoma,Protuberan, Metastatic Dermatofibrosarcoma,Protuberan, Myxoid Dermatofibrosarcoma,Protuberan, Pigmented Dermatofibrosarcoma,Protuberans, Dermatofibrosarcoma,Protuberans, Familial Dermatofibrosarcoma,Protuberans, Fibrosarcomatous Dermatofibrosarcoma,Protuberans, Giant Dermatofibrosarcoma,Protuberans, Metastatic Dermatofibrosarcoma,Protuberans, Myxoid Dermatofibrosarcoma,Protuberans, Pigmented Dermatofibrosarcoma,Tumor, Bednar,Tumor, Bednar's,Tumor, Darier-Ferrand,Tumor, Darier-Hoffmann
D018450 Disease Progression The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis. Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease

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