title: Dermatofibrosarcoma protuberans on the right neck with superior vena cava syndrome: case report and literature review.
abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal soft tissue tumour of intermediate malignancy. A 44-year-old man presented to the hospital with a large lesion on the right upper chest and neck. Despite eight previous surgical excisions, the tumour had continued to recur. Contrast-enhanced computed tomography showed recurrence of the tumour, associated with superior vena cava (SVC) syndrome. He declined radical surgical resection of the recurrent tumour, which may have required right upper limb amputation. Targeted therapy with sunitinib malate was therefore introduced. This case demonstrates the recurrent nature of DFSP and the association of this lesion on the upper chest/neck with SVC syndrome. Primary wide radical resection is essential for better local control and to avoid the development of SVC syndrome.
authors.affiliation: Department of Oral and Maxillofacial Surgery, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, PR China.
authors.forename: H S
authors.initials: HS
authors.lastname: Ong
authors.forename: T
authors.initials: T
authors.lastname: Ji
authors.forename: L Z
authors.initials: LZ
authors.lastname: Wang
authors.forename: Z W
authors.initials: ZW
authors.lastname: Yu
authors.forename: C P
authors.initials: CP
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D018223:Dermatofibrosarcoma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006258:Head and Neck Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D006801:Humans; D007211:Indoles / Q000627:therapeutic use; D008297:Male; D009333:Neck / Q000473:pathology* / Q000601:surgery; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D011758:Pyrroles / Q000627:therapeutic use; D000077210:Sunitinib; D013479:Superior Vena Cava Syndrome / Q000209:etiology* / Q000473:pathology; D013909:Thorax / Q000473:pathology; D014683:Vena Cava, Superior
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; D000077210:Sunitinib