Comparison Between Diffusion-Weighted MRI and 123 I-mIBG Uptake in Primary High-Risk Neuroblastoma.

BACKGROUND: High-risk neuroblastoma (HR-NB) has a variable response to preoperative chemotherapy. It is not possible to differentiate viable vs. nonviable residual tumor before surgery. PURPOSE: To explore the association between apparent diffusion coefficient (ADC) values from diffusion-weighted magnetic resonance imaging (DW-MRI), 123 I-meta-iodobenzyl-guanidine (123 I-mIBG) uptake, and histology before and after chemotherapy. STUDY TYPE: Retrospective. SUBJECTS: Forty patients with HR-NB. FIELD STRENGTH/SEQUENCE: 1.5T axial DW-MRI (b = 0,1000 s/mm2 ) and T2 -weighted sequences. 123 I-mIBG scintigraphy planar imaging (all patients), with additional 123 I-mIBG single-photon emission computed tomography / computerized tomography (SPECT/CT) imaging (15 patients). ASSESSMENT: ADC maps and 123 I-mIBG SPECT/CT images were coregistered to the T2 -weighted images. 123 I-mIBG uptake was normalized with a tumor-to-liver count ratio (TLCR). Regions of interest (ROIs) for primary tumor volume and different intratumor subregions were drawn. The lower quartile ADC value (ADC25prc ) was used over the entire tumor volume and the overall level of 123 I-mIBG uptake was graded into avidity groups. STATISTICAL TESTS: Analysis of variance (ANOVA) and linear regression were used to compare ADC and MIBG values before and after treatment. Threshold values to classify tumors as viable/necrotic were obtained using ROC analysis of ADC and TLCR values. RESULTS: No significant difference in whole-tumor ADC25prc values were found between different 123 I-mIBG avidity groups pre- (P = 0.31) or postchemotherapy (P = 0.35). In the "intratumor" analysis, 5/15 patients (prechemotherapy) and 0/14 patients (postchemotherapy) showed a significant correlation between ADC and TLCR values (P < 0.05). Increased tumor shrinkage was associated with lower pretreatment tumor ADC25prc values (P < 0.001); no association was found with pretreatment 123 I-mIBG avidity (P = 0.17). Completely nonviable tumors had significantly lower postchemotherapy ADC25prc values than tumors with >10% viable tumor (P < 0.05). Both pre- and posttreatment TLCR values were significantly higher in patients with >50% viable tumor than those with 10-50% viable tumor (P < 0.05). DATA CONCLUSION: 123 I-mIBG avidity and ADC values are complementary noninvasive biomarkers of therapeutic response in HR-NB. LEVEL OF EVIDENCE: 4. TECHNICAL EFFICACY STAGE: 3.

Robot-assisted laparoscopic pyeloplasty (RALP) in children with complex pelvi-ureteric junction obstruction (PUJO): results of a multicenter European report.

PURPOSE: This study aimed to report a multi-institutional retrospective case series of outcomes after robot-assisted laparoscopic pyeloplasty (RALP) in pediatric patients with complex pelvi-ureteric junction obstruction (PUJO). METHODS: All patients undergoing complex RALP over the last 5 years were included. RALP was defined complex in the following cases of PUJO: anatomic variations including ectopic, malrotated, horseshoe, or duplex kidney and recurrent PUJO after failed open pyeloplasty. RESULTS: Forty-eight patients underwent complex RALP in four European centers in the study period and included 18 girls and 30 boys with a median age of 8 years (range 5-12). The PUJO was associated with anatomic variations in 35/48 (72.9%), whereas a recurrent PUJO was present in 13/48 (27.1%). A dismembered Anderson-Hynes pyeloplasty was performed in all patients. The median operative time including docking was 178.5 min (range 117-255) and the median anastomotic time was 64.8 min (range 50-76). All patients were discharged on 2nd postoperative day (POD). The median follow-up was 18.2 months (range 14-43). The overall success rate was 95.8% (46/48). Early postoperative complications (< 30th POD) included urinary tract infections (UTIs) and stent-related irritative symptoms in 4/48 (8.3%) [II Clavien], whereas late complications (> 30th POD) included recurrence of PUJO in 2/48 (4.2%), who needed re-operation [IIIb Clavien]. CONCLUSIONS: RALP was safe, feasible, and with good mid-term outcome in complex PUJO. An accurate pre-operative planning, a standardized technique, and an experienced surgical robotic team represented key points to manage successfully such complex cases.

Massive pelvic recurrence of uterine leiomyomatosis with intracaval-intracardiac extension: video case report and literature review.

BACKGROUND: Uterine leiomyomas represent the gynecological neoplasm with the highest prevalence worldwide. This apparently benign pathological entity may permeate into the venous system causing the so-called intravenous leiomyomatosis of the uterus (IVL). IVL may seldom extend to large caliber veins and reach the right cardiac chambers or pulmonary arteries and cause signs of right sided congestive heart failure and sudden death. Due to its low incidence, however, IVL with intracardiac extension is often misdiagnosed resulting in deferred treatment. No consensus has been obtained regarding the standard surgical approach to be used for this rare condition. We describe the case of a massive pelvic recurrence of uterine leiomyomatosis with intracardiac extension and provide a review of the literature, analyzing management and surgical outcomes. CASE PRESENTATION: We present the case of a 46-year-old premenopausal woman presenting with lower-extremity edema, recurrent syncopes and a history of subtotal hysterectomy for multiple uterine fibroids. She was diagnosed with pelvic recurrence of uterine leiomyomatosis and IVL with cardiac involvement. A two-stage surgical excision of the intracardiac-intracaval mass and pelvic leiomyomatosis was performed. The patient had an uneventful recovery and no evidence of recurrence was observed on follow-up. CONCLUSIONS: By virtue of the rarity of the present pathology, awareness is widely scarce and diagnosis is often delayed. Early recognition is difficult due to initial aspecific and subtle clinical manifestations. Nevertheless, suspicion should be held high in premenopausal women with known history of uterine leiomyomata, presenting with cardiovascular symptoms and evidence of a free-floating mass within the right cardiac chambers. In-depth imaging is crucial for defining its anatomical origin and relations. Prompt surgical treatment with radical excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence, whereas delayed diagnosis and treatment exposes to increased risk of congestive heart failure and sudden death.

Dynamic Changes in Microvascular Density Can Predict Viable and Non-Viable Areas in High-Risk Neuroblastoma.

Despite aggressive treatments, the prognosis of high-risk NB remains poor. Surgical oncology needs innovative intraoperative devices to help surgeons discriminate malignant tissue from necrotic and surrounding healthy tissues. Changes within the tumor vasculature could be used intraoperatively as a diagnostic tool to guide surgical resection. Here, we retrospectively analyzed the mean vascular density (MVD) of different NB subtypes at diagnosis and after induction chemotherapy using scanned histological samples. One patient was prospectively enrolled, and an ex vivo photoacoustic imaging (PAI) scan was performed on two representative sections to assess its capacity to discriminate different tumor regions. We found that post-chemotherapy, viable areas of differentiating NBs and ganglioneuroblastomas are associated with higher MVD compared to poorly differentiated NBs. Early necrotic regions showed higher MVD than late necrotic and viable regions. Finally, calcified areas showed significantly lower MVD than any other histological component. The acquired PAI images showed a good high-resolution ex vivo 3D delineation of NB margins. Overall, these results suggest that a high-definition preclinical imaging device such as PAI could potentially be exploited to guide surgical resection by identifying different vasculature signatures.

Long-term active problems in patients with cloacal exstrophy: A systematic review.

BACKGROUND: Cloacal exstrophy (CE) is the most severe end of the Exstrophy-Epispadias Complex malformations spectrum. Improvements in postnatal management and well-established operative techniques have resulted in survival rates approaching 100%. This systematic review aims to define the prevalence of long-term active medical problems affecting CE patients after the first decade of life. METHODS: PubMed/Medline, Embase, Scopus, and ISI Web of Knowledge databases were used for the literature search. Original articles related to medical, surgical, and psychosocial long-term problems in CE patients >10 years of age were included in the study. Quality assessment of the articles was performed through the Newcastle-Ottawa Scale. Prevalence estimates and 95% CI were assessed for each outcome. RESULTS: Twelve studies were included. The most common long-term active problems identified were: urinary incontinence with a prevalence ranging from 9.1% to 85%; sexual function issues related to vaginal anomalies with a prevalence ranging from 8.3% to 71.3%, and uterine anomalies, with a prevalence from 14.3% to 71%; gender identity issues in 46, XY patients raised female had a prevalence from 11.1% to 66.7%. There is no documented history of paternity. Impairment of ambulatory capacity was recorded in 13.8% of patients. Only one paper studied psychological well-being, reporting significantly higher levels of depression among gender reassigned patients. CONCLUSIONS: Teenagers and adults born with CE have well defined long-term problems compared to the general population. Recognition and expert management are crucial to improve care and quality of life during and after the transition into adulthood.

Near-InfraRed PhotoImmunoTherapy (NIR-PIT) for the local control of solid cancers: Challenges and potentials for human applications.

Near-InfraRed PhotoImmunoTherapy (NIR-PIT) is a novel cancer-targeted treatment effected by a chemical conjugation between a photosensitiser (e.g. the NIR phthalocyanine dye IRDye700DX) and a cancer-targeting moiety (e.g. a monoclonal antibody, moAb). Delivery of a conjugate in vivo leads to accumulation at the tumour cell surface by binding to cell surface receptors or antigens. Upon deployment of focal NIR-light, irradiation of the conjugate results in a rapid, targeted cell death. However, the mechanisms of action to produce the cytotoxic effects have yet to be fully understood. Herein, we bring together the current knowledge of NIR-PIT from preclinical and clinical studies in a variety of cancers highlighting the key unanswered research questions. Furthermore, we discuss how to enhance the local control of solid cancers using this novel treatment regimen.

Robotic lobectomy in children with severe bronchiectasis: A worthwhile new technology.

BACKGROUND/PURPOSE: Lobectomy is required in children affected by non-responsive, symptomatic, localized bronchiectasis, but inflammation makes thoracoscopy challenging. We present the first published series of robotic-assisted pulmonary lobectomy in children with bronchiectasis. METHODS: Retrospective analysis of all consecutive patients who underwent pulmonary lobectomy for severe localized bronchiectasis (2014-2019) via thoracoscopic versus robotic lobectomy. Four 5 mm ports were used for thoracoscopy; a four-arm approach was used for robotic surgery (Da Vinci Surgical Xi System, Intuitive Surgical, California). RESULTS: Eighteen children were operated (robotic resection, n = 7; thoracoscopy, n = 11) with infected congenital pulmonary malformation, primary ciliary dyskinesia, and post-viral infection. There were no conversions to open surgery with robotic surgery, but five with thoracoscopy. Total operative time was significantly longer with robotic versus thoracoscopic surgery (mean 247 ± 50 versus 152 ± 57 min, p = 0.008). There were no significant differences in perioperative complications, length of thoracic drainage, or total length of stay (mean 7 ± 2 versus 8 ± 3 days, respectively). No blood transfusions were required. Two thoracoscopic patients had a type-3 postoperative complication. CONCLUSIONS: Pediatric robotic lung lobectomy is feasible and safe, with excellent visualization and bi-manual hand-wrist dissection - useful properties in difficult cases of infectious pathologies. However, instrumentation dimensions limit use in smaller thoraxes.

Learning curve for robotic surgery in children: a systematic review of outcomes and fellowship programs.

The learning curve (LC) of a new technique is fundamental for its application and assessment, and for the training. Literature was analysed to define the LCs of different surgical procedures and the impact of fellowship programs. MEDLINE, EMBASE and paediatric surgical journals' databases from January 1995 to December 2018 were systematically analysed. Two independent residents sought for articles providing description of robotic-assisted procedures' LCs in paediatric age/population. Seventeen articles were selected, describing LC of robotic-assisted pyeloplasty (n = 9), fundoplication (n = 4), cholecystectomy (n = 2), choledochal cyst resection (n = 1) and lingual tonsillectomy (n = 1), with 721 procedures. Ten studies refer to one single surgeon; six to more than one; one does not specify the number of operators. Eleven studies are unicentric retrospective, two multicentric retrospective, three prospective and one is a comparative analysis between a retrospective case series and a prospective cohort. The most recruited parameter is operative time alone in 3 articles, associated with complications in 12, length of hospital stay in 6, blood loss in 3, resolution in 4 and narcotic use in 2. The LC is described as impacting procedural planning (n = 17), training (n = 9) and economic costs (n = 2). To date, operative time is the most reported outcome to measure LC and proficiency. Efforts are needed to consider measures of surgical expertise and patient status. Robotic training should be standardized on targeted programs planned upon well-defined LCs.

Umbilical cord sparing technique for repair of congenital hernia into the cord and small omphalocele.

BACKGROUND/PURPOSE: Current repair of small omphaloceles and hernias into the umbilical cord is a straightforward procedure, whose repair may result in a suboptimal cosmetic outcome. We describe a novel repair technique retaining the umbilical cord elements in an attempt to improve the cosmetic appearance of the umbilicus. METHODS: Eight neonates were consecutively treated more than a ten-year period. Size of the fascial defects ranged 1 to 3cm (median, 2). Present technique entails incision of the amniotic sac without its detachment from the skin, reduction of the extruded contents under direct vision, and closure of the abdominal wall defect by circumferential suturing of peritoneum and fascia around the base of the amniotic sac. The amniotic sac is then re-approximated and folded to create an umbilical stump, which is trimmed and left to shed naturally. RESULTS: All patients achieved a scarless abdomen with a normal appearing umbilicus in 6. The remaining 2 patients are awaiting surgery for persisting umbilical hernia repair and umbilicoplasty, respectively. Poor esthetic outcome was significantly associated with initial fascial defect ≥2.5cm in size (p=0.03). CONCLUSIONS: Present technique is a simple and cosmetically appealing repair for umbilical cord hernias and small omphaloceles, especially effective when the size of the fascial defect is less than 2.5cm. LEVEL OF EVIDENCE: IV (Treatment Study).