Acta Dermatovenerol Croat. 2020 Jul;28(1):49-50.
Dear Editor, Desmoplastic melanoma (DM) is a rare histological subtype of melanoma, usually presenting as a slowly-growing, amelanotic, discoid, and/or firm lesion composed of spindle cells with abundant collagen (1). It is more common on sun-exposed areas, especially on head and neck in elderly patients (2). Regional lymph node involvement is reported to be less frequent than in other cutaneous melanomas (3). Desmoplastic melanoma can clinically mimic a wide spectrum of benign and malignant lesions, including Bowen's disease, desmoplastic nevus, basal cell carcinoma, squamous cell carcinoma, lentigo maligna, dermatofibrosarcoma protuberans, peripheral nerve sheath tumors, cysts, or hypertrophic/keloid scars (4). Regarding its appearance, at the time of diagnosis DM frequently presents as advanced lesions with deep infiltration. A 60-year-old man presented with an one-year history of an asymptomatic, erythematous, well-defined plaque in the right lumbar region (Figure 1). Dermatological examination revealed a 5×5 cm, pink/red infiltrated plaque accompanied by a 6 mm dark-brown melanocytic lesion. Dermoscopically, atypical vascular structures in the form of linear, irregular, and dotted vessels, milky-red areas, and atypical pigment network, and streaks were observed near the melanocytic lesion (Figure 2). A 4 mm punch biopsy was performed on the erythematous plaque next to the melanocytic lesion, and a dermal-based, paucicellular proliferation of atypical spindle cells without melanin in a sclerotic stroma was found histologically (Figure 3, a). Immunohistochemically, dermal spindle cells were stained with S-100 and HMB45 antibodies (Figure 3, b). The patient was histologically diagnosed with melanoma, of the desmoplastic subtype. The lesion was totally excised with 2 cm clear margins. A diagnosis of nonulcerated nodular melanoma with a Breslow thickness of 4 mm and a mitotic index 1/mm2 was established. Sentinel lymph node biopsy revealed no metastases. No systemic metastases were detected in PET-CT scanning and cranial magnetic resonance imaging. The patient remained under follow-up and has been free of any local recurrence or primary or systemic metastasis for 3 years. Dermoscopic characteristics of DM are not well known, probably due to it not being considered a melanocytic lesion. Debarbieux et al. first reported the dermoscopic features of desmoplastic melanoma in six cases (5). They found that only half of the cases presented one classical feature of a melanocytic lesion, whereas the other cases were diagnosed based on the presence of figures of regression such as white scar-like and "peppering", multiple (>4) color, and melanoma-related vascular patterns (five out of six) such as linear-irregular vessels and milky-red areas (5). In the largest DM case series, Jaime et al. reported that all DM featured at least 1 melanoma-specific structure, with atypical vascular structures being the most common (6). Similarly, in our patient dermoscopy showed an atypical pigment network and streaks, atypical vascular structures, and milky-red areas, which is predictive for melanoma. We reported this case to serve as a reminder to consider desmoplastic melanoma in the differential diagnosis of pink tumoral lesions despite its rarity and atypical localization.
致编辑,促结缔组织增生性黑色素瘤(DM)是一种罕见的黑色素瘤组织学亚型,通常表现为生长缓慢、无色素、盘状和/或坚实的病变,由富含胶原的梭形细胞组成(1)。它在暴露于阳光的部位更为常见,尤其是在老年患者的头部和颈部(2)。据报道,区域淋巴结受累的频率低于其他皮肤黑色素瘤(3)。促结缔组织增生性黑色素瘤在临床上可模拟广泛的良性和恶性病变,包括 Bowen 病、促结缔组织增生性神经纤维瘤、基底细胞癌、鳞状细胞癌、恶性雀斑样痣、隆凸性皮肤纤维肉瘤、周围神经鞘瘤、囊肿或肥厚/瘢痕疙瘩(4)。就其外观而言,在诊断时 DM 通常表现为晚期病变,具有深部浸润。一名 60 岁男性,右侧腰部出现了一年的无症状、红斑、界限清楚的斑块(图 1)。皮肤科检查显示一个 5×5cm 的粉红色/红色浸润斑块,伴有一个 6mm 的深褐色黑色素细胞病变。皮肤镜下观察到黑色素细胞病变附近有不规则、点状和线性血管结构,出现乳糜红色区域和非典型色素网以及条纹(图 2)。在靠近黑色素细胞病变的红斑处进行了 4mm 的皮肤打孔活检,组织学检查发现真皮基底、少细胞增生的非黑色素细胞梭形细胞在硬化基质中生长(图 3a)。免疫组化染色显示真皮梭形细胞 S-100 和 HMB45 抗体染色阳性(图 3b)。患者被组织学诊断为促结缔组织增生性黑色素瘤。病变完全切除,切缘 2cm 无肿瘤。病理诊断为非溃疡性结节性黑色素瘤,Breslow 厚度为 4mm,有丝分裂指数为 1/mm2。前哨淋巴结活检未发现转移。PET-CT 扫描和颅磁共振成像未发现全身转移。患者仍在随访中,3 年来无局部复发、原发性或全身转移。DM 的皮肤镜特征尚不清楚,可能是因为它不被认为是一种黑色素瘤病变。Debarbieux 等人首次报道了 6 例促结缔组织增生性黑色素瘤的皮肤镜特征(5)。他们发现只有一半的病例表现出一个黑色素瘤病变的典型特征,而其他病例则根据白色瘢痕样和“胡椒状”、多个(>4 个)颜色以及与黑色素瘤相关的血管模式(6 例中的 5 例)等退行性病变的存在来诊断,如不规则线性血管和乳糜红色区域(5)。在最大的 DM 病例系列中,Jaime 等人报告说,所有的 DM 都至少有 1 个黑色素瘤特异性结构,以非典型血管结构最为常见(6)。同样,在我们的患者中,皮肤镜显示非典型色素网和条纹、非典型血管结构和乳糜红色区域,这提示黑色素瘤的可能。我们报告了这个病例,以提醒大家在鉴别诊断粉红色肿瘤性病变时考虑促结缔组织增生性黑色素瘤,尽管它很罕见,且位置不典型。