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宫颈原发性恶性黑色素瘤:一例病例报告及文献综述

Primary Malignant Melanoma of the Cervix: A Case Report and a Review of the Literature.

作者信息

Diakosavvas Michail, Fasoulakis Zacharias N, Kouroupi Maria, Theodora Marianna, Inagamova Lola, Tsatsaris Georgios, Nikolaou Panagiotis, Frangia-Tsivou Konstantina, Giatromanolaki Alexandra, Kontomanolis Emmanuel N

机构信息

Department of Obstetrics and Gynecology, National and Kapodistrian University of Athens, Greece.

Department of Pathology, Democritus University of Thrace, Greece.

出版信息

Case Rep Oncol Med. 2020 Sep 3;2020:7206786. doi: 10.1155/2020/7206786. eCollection 2020.

Abstract

BACKGROUND

Gynecologic melanomas are extremely rare malignancies, and primary malignant melanoma of the cervix (PMMC) is the rarest among them all, with less than 100 cases reported so far. Although some conditions have been correlated with the pathogenesis of this entity, no specific risk factor has been yet identified, with vaginal bleeding being the most common symptoms. The diagnosis is based on physical examination with speculum assessment and cytologic and histopathologic findings accompanied with immunohistochemical staining of lesion's biopsies. . We report a case of PMMC in a 34-year-old para-2 patient, among the youngest cases of PMMC reported, that presented to our clinic for routine examination. Gynecologic examination demonstrated a dark, heavily fully pigmented cervical growth completely covering the entire external cervical os. Biopsy obtained and showed malignant melanoma. She underwent radical hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. The pathological diagnosis was FIGO stage IB1 PMMC. Despite 2 courses of anti-PD-1 antibody (Nivolumab) treatment, the patient passed away 13 months after diagnosis (12 months after surgery).

CONCLUSIONS

Early diagnosis and subsequently early treatment are of high importance regarding patients' prognosis and survival. No standardized protocols or treatment guidelines specific for this rare cancer have been issued; thus, clinicians are called to assess each case individually. Current treatment options are based on surgical excision mostly with radical hysterectomy, but in advanced or recurrent state of the disease, other treatment modalities, such as chemotherapy, radiotherapy, and immunotherapy, can be employed. Prognosis for these patients is very poor, and survival rate remains extremely low, with the median OS reported being less than 2 years. Reporting and publishing of such cases are both of paramount importance for the better understanding of this uncommon cervical malignancy, and further biological and clinical investigations are required for more suitable and effective therapies to be determined. A new staging system, specific to PMMC, could be of great use for the better correlation of the disease's stage and prognosis of these patients.

摘要

背景

妇科黑色素瘤是极为罕见的恶性肿瘤,其中宫颈原发性恶性黑色素瘤(PMMC)最为罕见,迄今为止报道的病例不足100例。尽管某些情况与该疾病的发病机制相关,但尚未确定具体的危险因素,阴道出血是最常见的症状。诊断基于窥器检查的体格检查以及细胞学和组织病理学检查结果,并伴有病变活检的免疫组织化学染色。我们报告一例34岁、孕2产2的PMMC患者,这是报道的最年轻的PMMC病例之一,因常规检查前来我院就诊。妇科检查发现宫颈有一个深色、色素沉着严重的肿物,完全覆盖了整个宫颈外口。活检结果显示为恶性黑色素瘤。她接受了根治性子宫切除术、双侧输卵管卵巢切除术和盆腔淋巴结清扫术。病理诊断为国际妇产科联盟(FIGO)分期IB1期PMMC。尽管接受了2个疗程的抗程序性死亡受体1(PD-1)抗体(纳武单抗)治疗,患者在诊断后13个月(手术后12个月)去世。

结论

早期诊断及随后的早期治疗对患者的预后和生存至关重要。尚未发布针对这种罕见癌症的标准化方案或治疗指南;因此,临床医生需要对每个病例进行个体化评估。目前的治疗选择主要基于手术切除,大多采用根治性子宫切除术,但在疾病的晚期或复发状态下,可采用其他治疗方式,如化疗、放疗和免疫治疗。这些患者的预后非常差,生存率仍然极低,报道的中位总生存期不到2年。报告和发表此类病例对于更好地了解这种罕见的宫颈恶性肿瘤至关重要,需要进一步进行生物学和临床研究以确定更合适、有效的治疗方法。一个专门针对PMMC的新分期系统可能对更好地关联疾病分期和这些患者的预后非常有用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/523f/7898279/8dd7b90b67bc/CRIONM2020-7206786.001.jpg

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