Minimally invasive management for multifocal pelvic retroperitoneal malignant paraganglioma: a neuropelveological approach.

BACKGROUND: Pheochromocytoma and Paraganglioma (PGL) are rare neuroendocrine tumors, with an estimated incidence of about 0.6 cases per 100.000 person/year. Overall, 3-8% of them are malignant. These tumors are characterized by a classic triad of symptoms (headaches, palpitations, profuse sweating) due to hypersecretion of catecholamines. Despite several advantages of minimally invasive surgery (MIS) for PGL debulking, the surgical approach is not standardized yet. In this scenario, we aimed to report a case of a multiple recurrent PGL with metastatic retroperitoneal localization involving the pelvic sidewall, excised with MIS. CASE PRESENTATION: We performed complete laparoscopic-assisted neuronavigation (LANN technique) with isolation of the sacral routes and the sciatic nerve to obtain complete exposure of the main anatomic landmarks. Robotic surgery was used to perform neurolysis of sacral plexus, and partial resection of left splanchnic nerves was needed. After the resection of the first mass, extensive neurolysis of all sacral routes, obturator nerve, pudendal nerve till the entrance of the pudendal (Alcock) canal, and sciatic nerve was performed. Finally, the mass was identified after trans gluteal incision and dissection of the maximum gluteal muscle, and a partial resection of the superior gluteal nerve and slicing of the sciatic nerve were needed to obtain a radical excision of the mass. Then neurorrhaphy of the sectioned nerve fibers of the superior gluteal nerve was performed, and nerve protection was obtained using a collagen nerve wrap. After 18 months of follow-up, the patient is free of disease at the MRI imaging and 123I-metaiodobenzylguanidine scintigraphy. CONCLUSIONS: Minimally invasive gynecological surgery with neuropelveological approach could be considered as a feasible option in case of multifocal pelvic retroperitoneal malignant paraganglioma of the pelvic side wall.

Second trimester uterine arteries pulsatility index is a function of placental pathology and provides insights on stillbirth aetiology: A multicenter matched case-control study.

INTRODUCTION: The aim of this study was to investigate the relationships between maternal vascular malperfusions (MVM) and second trimester uterine arteries pulsatility index (UtA-PI) in cases of stillbirth (SB), compared to live-birth (LB) matched controls. METHODS: This was a multicentre, observational, matched case-control study performed at five referral maternity centres over a 4-year period including SB and LB control pregnancies at high-risk for preeclampsia (PE) and/or fetal growth restriction (FGR), matched and stratified for UtA-PI MoM quartiles values of the SB cases. Logistic regression was used to assess the rates of each MVM finding, within each increasing MoM quartile subcategory in SB and matched LB controls. RESULTS: 82 SB and 82 LB matched high-risk pregnancies were included. Placental hypoplasia, placental infarction, retroplacental hematoma, distal villous hypoplasia and accelerated villous maturation showed a significant correlation with UtA-PI. At univariable analysis, placental infarction and distal villous hypoplasia were more highly associated with the increasing quartile uterine Doppler measurements (odds ratio 2.24 and 2.23, respectively). Logistic regressions showed a significant positive and independent association between rates of retroplacental hematoma or distal villous hypoplasia and stillbirth within corresponding UtA-PI MoM quartiles (odds ratio 5.21 and 2.28, respectively). DISCUSSION: We are providing evidence for characterization of two major etiological stillbirth categories, characterized by a positive or absent association with UtA-PI impairment and specific histopathological placental MVM lesions. Our results support a strict third trimester follow-up of cases with increased second trimester UtA-PI, in order to improve the reproductive chances of these pregnant patients.

Fitz-Hugh-Curtis Syndrome Secondary to Postpartum Endometritis: Case Report and Literature Review.

We report a case of Fitz-Hugh-Curtis syndrome (FHCS) 1 week after an urgent cesarean section. Although its onset in the postpartum period is poorly reported in the literature, we suggest that FHCS can complicate puerperal endometritis; therefore, it should be consider in differential diagnosis in puerpera with right upper quadrant pain. Again, it can be useful to perform a contrast computed tomography that allows early diagnosis and conservative treatment.

Incidence, predictors and clinical outcome of pancreatic fistula in patients receiving splenectomy for advanced or recurrent ovarian cancer: a large multicentric experience.

PURPOSE: To evaluate the incidence, predictors and clinical outcome of pancreatic fistulas in patients receiving splenectomy during cytoreductive surgery for advanced or recurrent ovarian cancer. METHODS: Data of women who underwent splenectomy during cytoreduction for advanced or recurrent ovarian cancer from December 2012 to May 2018 were retrospectively retrieved from the oncological databases of five institutions. Surgical, post-operative and follow-up data were analysed. RESULTS: Overall, 260 patients were included in the study. Pancreatic resection was performed in 45 (17.6%) women, 23 of whom received capsule resection alone, while 22 required tail resection. Hyperthermic intraperitoneal chemotherapy (HIPEC) was administered in 28 (10.8%) patients. In the overall population, a pancreatic fistula was detected in 32 (12.3%) patients, and pancreatic resection (p-value = 0.033) and HIPEC administration (p-value = 0.039) were associated with fistula development. In multivariate analysis, HIPEC (OR = 2.573; p-value = 0.058) was confirmed as a risk factor for fistula development in women receiving splenectomy alone, while concomitant cholecystectomy (OR = 2.680; p-value = 0.012) was identified as the only independent predictor of the occurrence of pancreatic fistulas in those receiving additional distal pancreatectomy. Although the median length of hospital stay was higher in women with pancreatic leakage (p-value = 0.008), the median time from surgery to adjuvant treatment was not significantly increased. CONCLUSION: HIPEC was identified as a risk factor for pancreatic fistulas in patients who underwent splenectomy alone, while concomitant cholecystectomy was the only independent predictor of fistula in those receiving additional pancreatectomy. The development of pancreatic leakage was not associated with increased post-operative mortality or delay in the initiation of chemotherapy.

What is the impact of a novel MED12 variant on syndromic conotruncal heart defects? Analysis of case report on two male sibs.

BACKGROUND: Syndromic congenital heart disease accounts for 30% of cases and can be determined by genetic, environmental or multifactorial causes. In many cases the etiology remains uncertain. Many known genes are responsible for specific morphopathogenetic mechanisms during the development of the heart whose alteration can determine specific phenotypes of cardiac malformations. CASE PRESENTATION: We report on two cases of association of conotruncal heart defect with facial dysmorphisms in sibs. In both cases the malformations' identification occurred by ultrasound in the prenatal period. It was followed by prenatal invasive diagnosis. The genetic analysis revealed no rearrangements in Array-CGH test, while gene panel sequencing identified a new hemizygous variant of uncertain significance (c.887G > A; p.Arg296Gln) in the MED12 gene, located on the X chromosome and inherited from the healthy mother. CONCLUSION: No other reports about the involvement of MED12 gene in syndromic conotruncal heart defects are actually available from the literature and the international genomic databases. This novel variant is a likely pathogenic variant of uncertain significance and it could broaden the spectrum of genes involved in the development of congenital heart diseases and the phenotypic range of MED12-related disorders.