Ouédraogo Nde N-A, Ouédraogo N A, Bambara H A, Tiemtoré-Kambou B M A, Traoré F, Zongo N, Ouédraogo M S, Tapsoba G P, Thouraya Chtioui Achach, Lamien/Sanou A
Hôpital de District de Bogodogo, Service de Radiologie et d'Imagerie Médicale, Ouagadougou, Burkina Faso.
Unité de Recherche en Sciences de la Santé, Université Ouaga I Professeur Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
J Med Case Rep. 2017 Apr 8;11(1):96. doi: 10.1186/s13256-017-1257-8.
Dermatofibrosarcoma protuberans is a rare tumor, representing about 0.1% of skin malignant tumors. It is characterized by local aggressiveness with significant potential for recurrence. Although metastasis is rare, it does occur. We report a case of a Burkinabe woman with dermatofibrosarcoma protuberans.
A 27-year-old Burkinabe woman consulted our institution for a recurrent scalp nodule that had been evolving for 13 years. At clinical examination, she was in good condition with a dry cough. An atrophic scarring alopecic plaque of 15-cm diameter in the right parietal region of the scalp, topped by an erythematous firm nodule measuring 3 × 2 × 2 cm, was noted, as well as a mobile nodule located in the axillary tail of the right breast. Cerebral computed tomodensitometry had not objectified the reach of the vault or the brain. A thoracic scan revealed four intrathoracic tissue masses straight to pleural touch. There were no evolutionary lesions in the abdominopelvic region. Histopathologic examination of the biopsy of the scalp nodule showed a proliferation of fibrous background, with fusiform cells carrying a storiform appearance. These cells had dark, elongated nuclei and showed some mitosis without atypia. The cells expressed CD34 intensely and diffusely. The test results were negative for PS100 and smooth muscle actin. The breast nodule showed the same profile as the scalp nodule.
We concluded on the diagnosis of scalp dermatofibrosarcoma protuberans with breast metastasis and probable pleuropulmonary metastasis.
隆突性皮肤纤维肉瘤是一种罕见肿瘤,约占皮肤恶性肿瘤的0.1%。其特点是具有局部侵袭性,复发潜力大。虽然转移罕见,但确实会发生。我们报告一例患有隆突性皮肤纤维肉瘤的布基纳法索妇女病例。
一名27岁的布基纳法索妇女因一个反复出现且已发展13年的头皮结节前来我院就诊。临床检查时,她状况良好,有干咳症状。在头皮右顶叶区域发现一个直径15厘米的萎缩性瘢痕性脱发斑块,顶部有一个3×2×2厘米的红斑性坚实结节,以及一个位于右乳腋尾部的可移动结节。脑部计算机断层扫描未发现颅骨或脑部有病变。胸部扫描显示有四个胸腔内组织肿块,直接与胸膜接触。腹盆腔区域没有病变进展。对头皮结节活检的组织病理学检查显示纤维背景增生,梭形细胞呈席纹状外观。这些细胞有深色、细长的细胞核,有一些无异型性的有丝分裂。细胞强烈且弥漫性表达CD34。PS100和平滑肌肌动蛋白检测结果为阴性。乳房结节与头皮结节表现相同。
我们诊断为头皮隆突性皮肤纤维肉瘤伴乳房转移及可能的胸膜肺转移。