Lazhar Hanaa, Slaoui Aziz, Rostoum Soufiane, Caidi Nawfel, Chat Latifa, Baydada Aziz
Gynaecology-Obstetrics and Endoscopy Department, Maternity Souissi, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco.
Gynaecology-Obstetrics and Endoscopy Department, Maternity Souissi, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco; Gynaecology-Obstetrics and Endocrinology Department, Maternity Souissi, University Hospital Center IBN SINA, University Mohammed V, Rabat, Morocco.
Int J Surg Case Rep. 2023 Apr;105:107950. doi: 10.1016/j.ijscr.2023.107950. Epub 2023 Feb 24.
Cervix mucinous adenocarcinomas have been defined by WHO classification into different subtypes: gastric, intestinal and ring signet cell. Ring signet cell subtype represent a diagnostic challenge due to the small number of cases described in the literature. We report hereby the 31st case worldwide, which is all the more exceptional as its atypical clinical presentation with mainly urological clinical signs represented a real diagnostic challenge.
We present the uncommon case of a multiparous, menopausal 68 years-old-woman, who presented in our department for pelvic pain evolving for 4 months associated with dysuria. Gynecological examination showed a bulging mass on the anterior vaginal wall with mucoid urinary discharge. Perineal ultrasound and pelvic MRI showed an anterior vaginal mass arising from the anterior vaginal wall, invading the bladder, urethra and respecting vagina's upper third and the rectum associated with multiple metastatic left iliac lymph nodes. Anatomopathological analysis revealed a moderately differentiated mucinous adenocarcinoma with a signet-ring cell appearance. IHC stain for P16, marker for high-risk HPV, was strongly positive. Due to the advanced stage, the patient was not a candidate for upfront surgery and received definitive chemoradiation with palliative intent. The patient succumbed to her disease after only one month of chemotherapy.
Primary signet ring cell carcinoma of the cervix is rare and associated with a poor outcome. Prognosis is related to the clinical stage. Differentiate primary from metastatic signet cell carcinoma is compulsory. IHC is very helpful but not decisive and the diagnosis is often made by exclusion.
宫颈黏液腺癌已被世界卫生组织分类为不同亚型：胃型、肠型和环状印戒细胞型。环状印戒细胞亚型由于文献中描述的病例数量较少，构成了诊断挑战。我们在此报告全球第31例该病例，因其非典型临床表现（主要为泌尿外科临床症状）而更显特殊，这构成了真正的诊断挑战。
我们呈现了一例罕见病例，患者为68岁多产绝经女性，因盆腔疼痛4个月并伴有排尿困难前来我科就诊。妇科检查发现阴道前壁有一膨出肿物，伴有黏液性尿液排出。会阴超声和盆腔磁共振成像显示阴道前壁有一肿物，侵犯膀胱、尿道，未累及阴道上三分之一及直肠，伴有左侧髂骨多发转移性淋巴结。解剖病理学分析显示为中分化黏液腺癌，呈印戒细胞外观。P16（高危人乳头瘤病毒标志物）免疫组化染色呈强阳性。由于疾病分期较晚，患者不适合进行前期手术，遂接受了姑息性根治性放化疗。患者在化疗仅一个月后死于疾病。
宫颈原发性印戒细胞癌罕见，预后较差。预后与临床分期相关。必须区分原发性和转移性印戒细胞癌。免疫组化非常有帮助，但并非决定性因素，诊断往往需通过排除法做出。
