Yang Yusheng, Yang Fang, Zhu Yin, Yu Jingjing, Zheng Jiangjiang, Chen Dong, Tang Xuezhi, Hu Tingting
Department of Pathology, Yinzhou Second Hospital Ningbo, China.
Department of Pathology, Clinical Pathological Diagnosis Center Ningbo, China.
Int J Clin Exp Pathol. 2019 Oct 1;12(10):3862-3869. eCollection 2019.
Desmoplastic melanoma (DM) is considered a variant of melanoma, characterized by a paucicellular proliferation of malignant spindled melanocytes with an abundant collagenous or "desmoplastic" stroma and an intense inflammatory response. As DMs lack pigmentation, their appearances vary and can mimic many benign and malignant conditions, thus presenting a diagnostic challenge. Here, we are presenting one case involving the head and two cases involving fingers. One of our cases distinctively invade nerves and bones. We reviewed the literature for many similar cases. Most cases presented positive staining of S-100 and negative or focal positive staining of Melan-A and Melanoma. So far, the mechanisms of the rare entity have not been clearly recognized. Early accurate diagnosis and complete excision of this tumor is necessary. Some researchers considered BRAF-targeted therapy may be limited to a small number of patients with DM. Advanced DM may respond well to anti-PD-1 monotherapy.
促纤维增生性黑色素瘤（DM）被认为是黑色素瘤的一种变体，其特征是恶性梭形黑素细胞的少细胞增殖，伴有丰富的胶原性或“促纤维增生性”间质以及强烈的炎症反应。由于促纤维增生性黑色素瘤缺乏色素沉着，其外观各异，可模仿许多良性和恶性病症，因此带来了诊断挑战。在此，我们呈现1例累及头部的病例和2例累及手指的病例。我们的其中1例病例显著侵犯神经和骨骼。我们查阅文献以寻找许多类似病例。大多数病例S-100染色呈阳性，Melan-A和黑色素瘤染色呈阴性或局灶性阳性。迄今为止，这种罕见实体的机制尚未被明确认识。早期准确诊断并完整切除该肿瘤很有必要。一些研究人员认为BRAF靶向治疗可能仅限于少数促纤维增生性黑色素瘤患者。晚期促纤维增生性黑色素瘤可能对抗PD-1单药治疗反应良好。
