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儿童、青少年及青年的黑色素瘤和恶性潜能不确定的黑素细胞肿瘤——斯坦福大学1995 - 2008年的经验

Melanoma and melanocytic tumors of uncertain malignant potential in children, adolescents and young adults--the Stanford experience 1995-2008.

作者信息

Berk David R, LaBuz Elizabeth, Dadras Soheil S, Johnson Denise L, Swetter Susan M

机构信息

Department of Dermatology, Pigmented Lesion and Melanoma Program, Stanford University Medical Center, Stanford, California 94063, USA.

出版信息

Pediatr Dermatol. 2010 May-Jun;27(3):244-54. doi: 10.1111/j.1525-1470.2009.01078.x. Epub 2010 Apr 9.

Abstract

Pediatric melanoma is difficult to study because of its rarity, possible biological differences in preadolescents compared with adolescents, and challenges of differentiating true melanoma from atypical spitzoid neoplasms. Indeterminant lesions are sometimes designated as melanocytic tumors of uncertain malignant potential (MelTUMPs). We performed a retrospective, single-institution review of melanomas, MelTUMPs and Spitz nevi with atypical features (SNAFs) in patients at 21 years of age and younger from 1995 to 2008. We identified 13 patients with melanoma, seven with MelTUMPs, and five with SNAFs. The median age for melanoma patients was 17 years, 10 for MelTUMPs, and six for SNAFs. Of the 13 melanoma patients, only four were younger than 15 years, while six were adolescents, and three were young adults. Nine melanoma patients (69%) were female. The most common histologic subtype was superficial spreading. The median depth for melanomas was 1.2 mm, and 3.4 mm for MelTUMPs. Microscopic regional nodal involvement detected on elective or sentinel lymph node (SLN) dissection was present in 2/10 (20%) of primary melanomas and 2/6 (33%) of Mel-TUMPs. Complete lymphadenectomy was performed on four melanoma patients, with three positive cases. Patient outcome through March 31, 2009 revealed no in-transit or visceral metastasis in patients with MelTUMPs or SNAFs. One SLN-positive patient (8%) with melanoma developed recurrent lymph node and liver metastasis and died 15 months after primary diagnosis. Our data highlight the rarity, female predominance, and significant rate of SLN positivity of pediatric melanoma. The high rate of MelTUMPs with regional nodal disease reinforces the need for close follow-up.

摘要

由于小儿黑色素瘤较为罕见,青春期前儿童与青少年可能存在生物学差异,且难以将真正的黑色素瘤与非典型梭形细胞肿瘤区分开来,因此对其进行研究颇具难度。不确定病变有时被指定为恶性潜能不确定的黑素细胞肿瘤(MelTUMPs)。我们对1995年至2008年间21岁及以下患者的黑色素瘤、MelTUMPs和具有非典型特征的Spitz痣(SNAFs)进行了一项单机构回顾性研究。我们确定了13例黑色素瘤患者、7例MelTUMPs患者和5例SNAFs患者。黑色素瘤患者的中位年龄为17岁,MelTUMPs患者为10岁,SNAFs患者为6岁。在13例黑色素瘤患者中,只有4例年龄小于15岁,6例为青少年,3例为青年。9例黑色素瘤患者(69%)为女性。最常见的组织学亚型是浅表扩散型。黑色素瘤的中位深度为1.2毫米,MelTUMPs为3.4毫米。在选择性或前哨淋巴结(SLN)清扫术中检测到的显微镜下区域淋巴结受累情况,原发性黑色素瘤患者中有2/​​10(20%)出现,Mel-TUMPs患者中有2/​​6(33%)出现。对4例黑色素瘤患者进行了根治性淋巴结清扫术,其中3例呈阳性。截至2009年3月31日的患者预后显示,MelTUMPs或SNAFs患者未出现局部或内脏转移。1例黑色素瘤SLN阳性患者(8%)出现复发性淋巴结转移和肝转移,并在初次诊断后15个月死亡。我们的数据突出了小儿黑色素瘤的罕见性、女性优势以及SLN阳性的显著比例。MelTUMPs伴区域淋巴结疾病的高比例强化了密切随访的必要性。

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