Neyaz Azfar, Husain Nuzhat, Deodhar Manish, Khurana Rohini, Shukla Saumya, Arora Aditi
Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, LUCKNOW, INDIA.
Turk Patoloji Derg. 2019;35(3):247-253. doi: 10.5146/tjpath.2017.01406.
We describe an unusual case of a Peutz-Jeghers syndrome associated with a composite synchronous cervical neoplasia comprising precursor "lobular endocervical glandular hyperplasia (LEGH)", "minimal deviation adenocarcinoma (MDA)" and "gastric-type adenocarcinoma (GTA)" along with a serous tubal intraepithelial lesion (STIL) in the right fallopian tube. A 24-year-old woman presented with a white mucoid discharge and bleeding per vaginum for one year. Histopathological evaluation showed MDA & GTA in FIGO grade III with pelvic lymph node metastasis despite a deceptively bland tumour morphology and low Ki-67 index, indicating an aggressive tumour course and poor prognosis. Diagnostic marker profile in the cervix showed gastric type mucin and positive expression of CK-7, CK-20 (patchy), CEA, and negative CDX-2, p16, ER and PR. Further an attempt at eliciting the oncogenesis pathway in view of the p16 and HPV negative nature of the gastric type cervical adenocarcinoma showed negativity for p53 but activation of cyclin D1. Growth factors including Her2 and EGFR were negative while VEGFR was over-expressed. She was treated by radical hysterectomy and pelvic radiation. She was free from recurrence at the 12-month follow-up. This is a first-time report of a STIL in the fallopian tube which was validated by a unilateral mutant type p53 expression and increased Ki67 index, associated with synchronous gastric type adenocarcinoma of the cervix in all stages of evolution.
我们描述了一例罕见的黑斑息肉综合征病例,该病例伴有复合性同步宫颈肿瘤,包括前驱病变“小叶状宫颈管腺增生(LEGH)”、“微小偏离腺癌(MDA)”和“胃型腺癌(GTA)”,同时右侧输卵管存在浆液性输卵管上皮内病变(STIL)。一名24岁女性出现白色黏液性分泌物和阴道出血一年。组织病理学评估显示,尽管肿瘤形态看似温和且Ki-67指数较低,但该患者的MDA和GTA处于国际妇产科联盟(FIGO)III级且伴有盆腔淋巴结转移,提示肿瘤病程侵袭性强且预后不良。宫颈的诊断标志物谱显示为胃型黏液,CK-7、CK-20(局灶性)、CEA呈阳性表达,而CDX-2、p16、雌激素受体(ER)和孕激素受体(PR)呈阴性。鉴于胃型宫颈腺癌的p16和人乳头瘤病毒(HPV)阴性特性,进一步尝试探究其肿瘤发生途径,结果显示p53为阴性,但细胞周期蛋白D1激活。包括人表皮生长因子受体2(Her2)和表皮生长因子受体(EGFR)在内的生长因子为阴性,而血管内皮生长因子受体(VEGFR)过度表达。她接受了根治性子宫切除术和盆腔放疗。在12个月的随访中未出现复发。这是首次报道输卵管存在STIL,经单侧突变型p53表达和Ki67指数升高得到验证,且在其发展的各个阶段均与宫颈同步胃型腺癌相关。