Rhabdomyosarcoma Arising in a Giant Congenital Melanocytic Nevus

A number of lesions have been documented to arise within congenital melanocytic nevi (CMNs). Although the most frequent malignancy arising within a CMN is melanoma, the association between rhabdomyosarcoma and CMN has rarely been documented. We present a case arising in a 4‐month‐old girl with a giant CMN. She presented for evaluation of a pedunculated lesion at the superior gluteal crease that had been present since birth and exhibited rapid growth. Biopsy of the lesion revealed two distinct components: an expansile proliferation of pleomorphic cells with varying degrees of cellularity and a proliferation of banal‐appearing melanocytic nevic cells. The cells of the expansile proliferation displayed a wide range of morphologic features, including nests of round cells, spindle‐shaped cells, and more differentiated rhabdomyoblasts within a myxoid, highly vascularized stroma. Cross‐striations, a marker of skeletal muscle differentiation, were present. These tumor cells were strongly immunoreactive with desmin, myo‐D1, and myogenin. Fluorescence in situ hybridization analysis with PAX3/7‐FKHR probes was negative. A diagnosis of embryonal rhabdomyosarcoma in association with CMN was made. Initial excision revealed tumor at the margins, and the patient underwent reexcision with subsequent chemotherapy with vincristine, actinomycin D, and cyclophosphamide. She was disease‐free at the 6‐year follow‐up. It has been postulated that the combination of melanocytic and rhabdomyoblastic cells within the same lesion may imply derivation from a common pluripotent stem cell or neural crest cell. Clinicians following patients with giant CMN should consider rhabdomyosarcoma in the differential diagnosis of lesions arising within the nevus.     

色素性毛表皮痣

患者,男,12岁。主诉：左面颊浅褐色色素沉着斑2年。现病史：2年前无明显诱因于左面颊部出现黄豆大小淡褐色斑,无疼痛瘙痒,当时未做任何处理。数月后皮损变大且数目增多,渐有扩大融合的趋势。日晒后皮损处有灼热感,随后颜色加深,夏季加重,冬季减轻。1年前皮损处有黑色粗毛生长,且骸下处也开始出现类似性质的淡褐色斑片。曾在当地医院就诊,拟“色素沉着”予“维生素C”,“六味地黄丸”,及“氢醌霜”外用,效果欠佳,遂于2009年7月15日到我科就诊。发病以来睡眠精神、食欲佳、大小便无异常。     

Woolly Hair Nevus with an Ipsilateral Associated Epidermal Nevus and Additional Findings of a White Sponge Nevus

Abstract:  We report a case of a 16-year-old male with a woolly hair nevus, an associated ipsilateral epidermal nevus who also had a white plaque on his tongue, clinically diagnosed as a white sponge nevus. The concurrent findings of a white sponge nevus, a woolly hair nevus, and an ipsilateral epidermal nevus, to our knowledge, have not been previously reported.     

Clear Cell Acanthoma Developing in Epidermal Nevus

A 33-year-old Japanese woman presented with a black papule on a pigmented lesion which had been on her right thigh since her early childhood. A hematoxylin-eosin-stained section revealed a sharply demarcated, acanthotic epidermis composed of enlarged clear cells, which stained positively for epithelial membrane antigen and negatively for carcinoembryonic antigen. With antikeratin antibodies, the tumor cells stained for AE1 and AE3, but did not stain for CAM5.2. They contained abundant glycogen. Histologically, we diagnosed the case as a clear cell acanthoma which developed in the pre-existing epidermal nevus. This is the second such case in the literature.     

The Spectrum of Epidermal Nevi: A Case of Verrucous Epidermal Nevus Contiguous with Nevus Sebaceus

During the normal development of skin, pluripotential cells give rise to keratinocytes, sebaceous glands, hair follicles, apocrine glands, and eccrine glands. In epidermal nevi, these components emerge in an abnormal mixture within a circumscribed site. Many authors have categorized epidermal nevi based on their predominant component; however, there is often notable overlap that occurs within a single area or within contiguous areas. We report a verrucous epidermal nevus contiguous to a nevus sebaceus of Jadassohn. The categories of epidermal nevi are somewhat artificial. Our case supports the view that epidermal nevi have a spectrum of manifestations, including verrucous epidermal nevi and nevus sebaceus of Jadassohn.     

泛发性炎症性线形疣状表皮痣

患者女。26岁。主诉：躯干、四肢条索状斑丘疹26年。现病史：患者出生2月后双下肢出现呈线状排列的淡红色丘疹,伴轻度瘙痒,未予重视,随年龄增长皮疹渐增多,泛发躯干四肢,部分皮疹融合成片,颜色逐渐转为淡褐色,患者自行涂擦药膏（具体不详）,皮疹无明显改善,近一年自觉瘙痒难忍,遂来我院门诊就诊。既往史及家族史：既往体健,无化工原料及重金属接触史;无急慢性传染疾病史,家族中无类似病史,无银屑病病史及家族史。     

Giant Inflammatory Linear Verrucous Epidermal Nevus: Successfully Treated with Full Thickness Excision and Skin Grafting

Inflammatory linear verrucous epidermal nevus (ILVEN) is a pruritic, erythematous scaly epidermal nevus which follows a Blaschko''s lines. Lichen striatus, linear Darier disease, linear porokeratosis, linear lichen planus, linear psoriasis, and the verrucous stage of incontinentia pigmenti may all have similar clinical presentations as the linear verrucous epidermal nevus. ILVEN can be distinguished from true nevoid psoriasis by pruritus and lack of response to antipsoriatic treatments. Various therapeutic modalities have been described, but no one therapy has been successful consistently. Though giant ILVEN is a relative contraindication to surgical excision, here we report a case showing effectiveness of full thickness excision and skin grafting for this condition.     

Basal Cell Carcinoma Arising in Nevus Sebaceous during Childhood

Abstract: Nevus sebaceous usually occurs as a solitary lesion located mostly on the scalp and the face. The potential for nevus sebaceous to develop malignancy is well recognized, though it is generally thought that this transformation occurs only postpubertally or later. However, few cases of basal cell carcinoma arising in nevus sebaceous before puberty were reported. Herein, we report a case of basal cell carcinoma arising in a nevus sebaceous on the scalp of a 10‐year‐old boy, and a review of the literature on prepubertal cases and the need for prophylactic surgery.     

Spitz Nevus Arising upon a Congenital Glomuvenous Malformation

Abstract: There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque‐like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.     

Keratoacanthoma Arising in an Organoid Nevus During Childhood: Case Report and Literature Review

We report an 11-year-old girl with an organoid nevus of the cheek. She represents the first report of a keratoacanthoma arising within an organoid nevus in childhood.     

巨型脑迥状痣一例报告

巨型脑迥状痣六先天性巨型色素痣之罕见类型,现将我们诊治的一例报告如下.