Minimal deviation adenocarcinoma of the cervix and tumorlets of sex-cord stromal tumor with annular tubules of the ovary in Peutz-Jeghers syndrome
A 36-year-old single, virgin woman presented with lower abdominal pain and hypermenorrhea for 2 months. Her past medical history was unremarkable. There were several melanin pigments on the tips of the patient's fingers and lips. The gynecological examination was not performed. Pelvic magnetic resonance imaging (MRI) revealed a 5.6×4.8×4.0 cm sized lobulating solid mass at the posterolateral wall of the cervix, and both ovarian multiseptated cystic tumors were suspected to be mucinous borderline neoplasm (). The serum levels of CA-125 (109.8 U/mL) and CA 19-9 (9,296 U/mL) were elevated and hemoglobin was markedly reduced (6.4 g/dL). The first biopsy of cervix was reported to be atypical endocervical glands. However, the re-biopsy of cervix was confirmed to be minimal deviation adenocarcinoma. The patient underwent radical hysterectomy with bilateral salpingooophorectomy, bilateral pelvic node dissection, paraaortic node sampling, omentectomy, and bladder biopsy.\nGossly, the cervix was markedly dilated and an infiltrating tumor mass was noted, measuring 5.5×4.0×4.5 cm (). Microscopic findings of the cervix were compatible with minimal deviation adenocarcinoma (). Histologically, both cystically dilated ovarian masses were reported to be metastatic MDA in mucinous cystadenoma with microscopic foci of SCTATs (). The colonic polyps were confirmed to be hamartomatous polyp (). The biopsy from the bladder flap was diagnosed as a direct extension of tumor cells. Immunohistochemically, in case 1, a reaction of rabbit polyclonal antibody to LKB1 (Novus Biologicals, Cambridge, UK) expression was not found in the atypical infiltrative glands (). FIGO stage of case 1 was IIIA due to the extension into the lower third of vagina with pelvic lymph node metastasis.\nIn case 1, DNA sequencing analysis was revealed a frame shift mutation of codon 132 (TGC to CGC) in exon 3 of the STK11/LKB1 gene ().\nDuring the follow-up period, the patient underwent a colonoscopic polypectomy and was diagnosed with multiple hamartomatous polyps throughout the small intestine and entire colon. Her father had also been diagnosed with PJS and died due to colon cancer 10 years earlier.\nThe patient received combined chemo-radiation therapy with 3 cycles of irinotecan and cisplatin. Nevertheless, she complained of ever-intensifying lower abdominal pain due to the increasing masses in the pelvic cavity and the re-growing vaginal stump mass; she finally expired 1 year after the operation.