An abdominal approach alone is often not sufficient for complete resection of neuroblastoma, as the limits of the surgical field become narrower while proceeding deeper into the dissection. There is a lack of literature addressing the thoraco-abdominal approach for resection of neuroblastoma in children. The aim of this study was to evaluate the efficacy of this approach based on our experience in a reference pediatric oncosurgical center.
Between 2003 and 2019 eighteen patients (12 female, 6 male) with neuroblastic tumors (12 neuroblastomas, 5 ganglioneurobastomas, 1 ganglineuroma) underwent tumor resection via a two-cavity-approach. A retrospective review of patient’s records was carried out.
Median age at operation was 48 months (5-132). The two-cavity-approach consisted of thoraco-abdominal access (n=6), upper transverse laparotomy with incision of the diaphragm (n=9), transverse laparotomy with lateral thoracotomy (n=2), or transverse laparotomy with thoracoscopy (n=1). Gross total resection (GTR) was achieved in 13 patients, a near-GTR in 4 cases and an incomplete resection in 1 case. Median duration of surgery was 307.5 minutes (99-990), median intraoperative blood loss was 62 m/kg (12.5-180). Postoperative complications occurred in 8 patients, 2 patients required surgical re-intervention. The 5-year overall survival (OS) was 83%, the 5-year event free survival (EFS) was 41.5%. A tumor relapse occurred in 5 patients (2 combined relapses, 2 metastatic relapses, 1 local relapse), 3 patients had progressive disease (PD). None of the patients with tumor relapse and one of the patients with PD died.
The two-cavity-approach for resection of abdominal neuroblastoma in children is a feasible technique, which is generally well tolerated. This approach allows GTR even in highly complex cases. Associated morbidities, complications, and postoperative courses are not relevantly different than in cases with a pure abdominal access.
Supraclavicular lymph node (SCLN) metastatectomy was performed as part of our efforts to render neuroblastoma patients into complete remission. Due to the lack of published data, we aimed to establish the clinicopathological correlation of patients with SCLN metastases and thus understand the pattern of lymphatic metastatic dissemination.
We retrospectively reviewed our centre’s database from Apr 2008 to Mar 2020. Patients who underwent surgical excision of SCLN at our centre were included. Those who presented at tumor relapse were excluded. Demographic, clinical and surgico-pathological data were analysed.
69 patients underwent excision of SCLN, of which 4 were upfront and 65 were delayed, after mean 4.5 (range 2-10) cycles of neoadjuvant chemotherapy. All 4 upfront excisions yielded mean 2.8 (range 1-5) metastatic lymph nodes. 59 (90.8%) patients with delayed excisions yielded mean 4.5 (range 1-20) metastatic lymph nodes, while 6(9.2%) patients yielded no tumor infiltration in mean 8 (range 3-27) lymph nodes. Initial diagnostic tests that detected SCLN metastases included PET-CT(n=50), MIBG(n=5), CT(n=17) and biopsy(n=9). 8 of 65 Stage 4 patients had SCLN-only metastases. Left-sided SCLN was found in 59 patients, 9 were right-sided and 1 was bilateral. Primary tumors were distributed in thorax-only(n=14), abdomen-only(n=31), and thoraco-abdomen(n=24). All 10 patients with right SCLN were associated with synchronous right thoracic tumors [thorax-only (n=7), thoraco-abdomen (n=3)]. Among the 60 patients with left SCLN, 7 were associated with left thorax-only tumors while the rest had abdomen-only(n=31) or thoraco-abdomen(n=22) tumors. All 55 abdominal tumors, including 36 adrenal-primaries, with SCLN metastases had involved the suprahilar peri-aortic region, hence representing the “abdominal sentinel” to the SCLN.
This study reaffirmed the pattern of lymph node metastases in neuroblastoma. In abdominal primary tumors, the suprahilar peri-aortic tumor location emerged as the prerequisite for SCLN metastases. In thoracic primary tumors, the metastases followed the ipsilateral lymphatic chain of dissemination.
Patients with Neuroblastoma (NB) are now stratified at diagnosis according to the presence of image-defined risk factors (IDRF). Although each IDRF is numerically worth one, from a surgical complexity point of view they are not all equivalent. The aim of the study is to investigate the impact of each IDRF on surgical complexity.
A retrospective single-center cohort of patients with NB from 2016 to 2019 was included. For each patient, data regarding adjuvant chemotherapy, type of surgical approach, intraoperative blood loss, duration of the intervention, complications, and completeness of resection were scored to calculate the “surgical complexity index” (SCI). The presence and type of each IDRF was then correlated to the SCI to determine the impact on surgical outcome.
Fifty-five patients were included. Thirty-nine of them (71%) presented with one or more IDRFs. Median SCI was 8 in these patients (range 4-15) and 0.5 (range 0-6) in the 16 without IDRFs (p < 0.0001). Patients presenting with involvement of two adjacent body compartments, tracheal and/or bronchial compression, encasement of abdominal aorta, and invasion of renal vessels had a higher SCI compared to those without these IDRFs. Superior mesenteric artery and/or coeliac axis encasement, tumor extending at the porta hepatis, and thoracic aorta encasement were associated with a higher SCI compared to other IDRFs and therefore received a maximum score (i.e. 3 points). A score of 2 or 1 was then attributed to the remaining IDRFs, according to the median SCI value associated. IDRF score was significantly correlated with SCI (Spearman’s coefficient = 0.499, p = 0.001).
Scoring IDRFs helps in standardizing the surgical risk and helps surgeons to safely plan the operation and to address an appropriate informed consent. A proposal of multicentric prospective cooperative study to validate these findings will be presented.
Minimally invasive adrenalectomy has been well described in adults, with less pain, smaller scars and shorter hospital admissions. However, there are limited reports in children. Here we describe our experience of laparoscopic adrenalectomy in children.
A retrospective notes review was performed from Oct-2006 to Jan-2020. Data collected included demographics, tumour type/size, operative time, length of stay (LOS), complications and follow up. Transperitoneal approach involved a 12mm umbilical and three 5mm working ports; on the left side the spleen was mobilised and reflected to the right. The resected mass was retrieved in a specimen bag.
Twenty-two adrenalectomies were performed in 19 children [M:F 16:3; median age 6.2 years (Interquartile range (IQR) 2.6-12)]; diagnosis neuroblastoma(11), phaechromocytoma(6), adenoma(1), carcinoma(1), ganglioneuroma(1) and bilateral adrenal hyperplasia(1). Five patients had metastatic disease at presentation. All patients had preoperative cross-sectional imaging; median size was 33mm (IQR 16–42); side (8 left, 8 right and 3 bilateral).
A transperitoneal approach allowed early visualisation and control of the main vessels to the adrenal gland. Median operative time was 137 minutes (IQR 124–227). Median LOS for cases completed with a minimally invasive approach was 2 days (IQR 2–3). Two cases were converted to open [bleeding (1), bowel distension restricting view (1)]. Cases requiring conversion stayed 2.5 days longer (median LOS – 4.5 days) in hospital. One case required a transfusion. There were no cases of capsule breach or injury to adjacent organs.
Children were followed up for a median of 377 days (IQR 80–1329). There was no incidence of local recurrence but one contra-lateral lesion was identified.
Our data suggests that laparoscopic adrenalectomy can be safely performed in children and neither age nor diagnosis was a contra-indication. The transperitoneal approach is our preferred option because it allows early safe control of the major blood supply.