Darier-Ferrand Dermofibrosarcoma: A Case Report of a Cervical Localization  

作　　者：Aminata Mbaye Ndiassé Ndiaye Ndeye Fatou Thiam Ousmane Sano Rachid Vitamine Mouhamed Salem Ould Abderrahmane Maïmouna Diagne Malick Ndiaye Aminata Mbaye;Ndiassé Ndiaye;Ndeye Fatou Thiam;Ousmane Sano;Rachid Vitamine;Mouhamed Salem Ould Abderrahmane;Maïmouna Diagne;Malick Ndiaye(ENT Department, Thiès Regional Hospital, Thies, Senegal;ENT Department, Ouakam Military Hospital, Dakar, Senegal;Radiology Department, Thies Regional Hospital, Thies, Senegal;ENT Department, Diamniadio Children’s Hospital, Dakar, Senegal)

机构地区：[1]ENT Department, Thiès Regional Hospital, Thies, Senegal [2]ENT Department, Ouakam Military Hospital, Dakar, Senegal [3]Radiology Department, Thies Regional Hospital, Thies, Senegal [4]ENT Department, Diamniadio Children’s Hospital, Dakar, Senegal

出　　处：《International Journal of Otolaryngology and Head & Neck Surgery》2023年第5期317-325,共9页耳鼻喉（英文）

摘　　要：Darier-Ferrand dermatofibrosarcoma (DFS) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. DFS is a tumor whose prognosis and evolutionary risk are mainly linked to the delay in diagnosis and the quality of the first excision. Late diagnosis makes excision and reconstruction surgery difficult. The chances of recovery in the case of well-performed primary surgery are significantly greater than in the case of salvage surgery. To improve prognosis, early, codified, multidisciplinary management is essential. In our African context, and especially in the case of patients living in rural areas, errant diagnoses are often found hence the importance of raising awareness and providing information to healthcare personnel. We report a case of an enlarged left supraclavicular Darier-Ferrand dermatofibrosarcoma. The patient had come for a late consultation at a stage when the tumour was large. The CT scan was a great help in the pre-operative phase. The patient underwent complete surgical excision, the postoperative course was straightforward and the histology of the surgical specimen confirmed the diagnosis. The resection margins were healthy. The patient is alive at one year with no recurrence or metastasis.Darier-Ferrand dermatofibrosarcoma (DFS) is a cutaneous mesenchymal tumor of intermediate malignancy. It is a rare but not exceptional tumor, accounting for 0.1% of malignant skin tumors. Histological examination is essential for diagnosis. Wide surgical excision is the standard treatment. DFS is a tumor whose prognosis and evolutionary risk are mainly linked to the delay in diagnosis and the quality of the first excision. Late diagnosis makes excision and reconstruction surgery difficult. The chances of recovery in the case of well-performed primary surgery are significantly greater than in the case of salvage surgery. To improve prognosis, early, codified, multidisciplinary management is essential. In our African context, and especially in the case of patients living in rural areas, errant diagnoses are often found hence the importance of raising awareness and providing information to healthcare personnel. We report a case of an enlarged left supraclavicular Darier-Ferrand dermatofibrosarcoma. The patient had come for a late consultation at a stage when the tumour was large. The CT scan was a great help in the pre-operative phase. The patient underwent complete surgical excision, the postoperative course was straightforward and the histology of the surgical specimen confirmed the diagnosis. The resection margins were healthy. The patient is alive at one year with no recurrence or metastasis.

关 键 词：Cervical Localization DERMATOFIBROSARCOMA Skin Tumor Wide Surgery 

分 类 号：R73[医药卫生—肿瘤]