Pentalogy of Cantrell with Unilateral Kidney Evisceration: A Case Report and Review of Literature.

Pentalogy of Cantrell (PC) is a congenital malformation syndrome characterized by midline thoracoabdominal wall defect resulting from defective development in the septum transversum. Major hallmarks of this rare anomaly are omphalocele and ectopic heart. In most cases, the diagnosis is made by two-dimensional ultrasound in second trimester. The prognosis of PC relies on the presence and severity of cardiac anomalies but in most cases outcome is fatal. To the best of our knowledge, the presence of kidneys into the omphalocele in Cantrells' pentalogy has not been reported yet. In this article, we report a case of PC associated with unilateral kidney evisceration.

Small-cell neuroendocrine carcinoma of the cervix associated with adenocarcinoma in situ: A case report with analysis of molecular abnormalities.

OBJECTIVE: We report a case of small-cell neuroendocrine carcinoma (SNEC) of uterine cervix associated with adenocarcinoma in situ (AIS), and we discuss prognosis, treatment benefit and goals of care. CASE REPORT: A 36-year-old pluriparous woman presented with vaginal bleeding. Bimanual pelvic examination revealed a exophytic mass arising from the posterior lip of the cervix. A transvaginal ultrasound revealed endometrium thickness of 7mm and exophytic 39x19mm mass arising from the posterior lip of the cervix. Histopathological analysis of the tumorous lesion revealed a small-cell neuroendocrine carcinoma admixed with adenocarcinoma in situ. Differential immunohistochemistry of the small-cell neuroendocrine carcinoma component was CKAE1/AE3, CD 56, TTF -1 positive with diffuse expression of chromogranin and synaptophysin. HPV type 18 has been detected through PCR sequencing analysis. The next generation sequencing based on a 324-gene panel showed that tumor was microsatellite stable (MSS) with low mutational burden (TMB). Only missense mutations of FGF10, HSD3B1, NBN, PBRM1, RICTOR, SDHA were detected. Radical surgery was performed and the patient received adjuvant chemotherapy consisting of cisplatin/etoposide for six cycles. During 12 months of follow up the patient is free of disease. CONCLUSION: Neuroendocrine tumour of uterine cervix is an extremely rare and aggressive cancer. Because of its low incidence there is still no standardized treatment approach based on controlled trials. Radical surgery and adjuvant or neoadjuvant chemotherapy is the mainstay of treatment.

In-vitro fertilization resulting in heterotopic pregnancy, ovarian hyperstimulation and paralytic ileus: A case report.

Heterotopic pregnancy followed by ovarian hyperstimulation syndrome and paralytic ileus are rare and potentially fatal complications associated with assisted reproduction. A 37-year-old nulliparous woman, after in-vitro fertilization and embryo transfer, presented to the gynaecology department with severe abdominal distension, diffuse abdominal pain and vaginal bleeding. Transvaginal ultrasound examination revealed an intrauterine pregnancy, with both ovaries enlarged, measuring 10cmx10cm, with free fluid in the pouch of Douglas. Another gestational sac was visualized in the left adnexal region with a viable pregnancy, crown-rump length (CRL) 6.6 mm at 6 weeks of gestation. Left salpingectomy via laparotomy and uterine evacuation were performed. The patient's postoperative course was complicated by the development of ovarian hyperstimulation syndrome and paralytic ileus. The patient recovered well after receiving supportive therapy. Clinicians should always be aware of the complications associated with assisted reproductive techniques.