Fetal neck tumors - antenatal and intrapartum management.

In this review article we describe the ante- and perinatal management of fetal neck tumors. Although there are rare congenital anomalies, the clinical consequences for the fetus may be fatal and include airways obstruction, heart insufficiency, pulmonary hypoplasia and cosmetic effect. The right management allows to decrease the fetal and neonatal mortality and morbidity associated with the disease. It includes intrauterine therapy in some cases, mostly in a goitrus hypothyroidism of the fetus, but firstly, an assessment of the fetal airways patency with a subsequent, eventual Ex-Utero Intrapartum Treatment (EXIT).

Surgical treatment of pediatric retroperitoneal tumors with invasion of major blood vessels: a single-center experience.

BACKGROUND: This study assessed operative experiences with common pediatric retroperitoneal tumors invading major blood vessels. METHODS: Forty-seven pediatric patients with retroperitoneal tumors were enrolled. They included 22 neuroblastomas, 6 gangliocytomas and 19 Wilms' tumors, and underwent primary surgical resection after vascular skeletonization and manipulations of involved vessels. RESULTS: In the above tumors, the one-stage gross total resection rates of, respectively, 95.45%, 100% and 100% were obtained. There was only one complication, namely post-operational early acute renal failure that recovered with dialysis. There were no deaths. CONCLUSIONS: Vascular skeletonization and other vessel manipulations prior to surgery improve the gross total resection rates of pediatric retroperitoneal tumors.

Forearm skeletal muscle neuroblastoma in a child: a rare primary location.

Neuroblastoma is the most common neoplasm in the first year of life and almost always arises from the adrenal glands or the sympathetic nervous system chain. We present an original case of an isolated forearm neuroblastoma in a 17-month-old child. The case was not treated with adjuvant chemotherapy after surgical resection because we interpreted it as a peripheral, INSS-stage 1, favorable histology neuroblastoma. Up to date the child remains in complete remission 4 years after diagnosis. Management of peripheral skeletal muscle neuroblastoma is discussed together with a review of the literature.

Neuroblastoma of the Lumbar Spine.

Neuroblastoma is a primitive small-round-blue-cell tumor predominately found in pediatric patients. Few cases of neuroblastoma involving the adult spine have been reported. Herein we present the case of a healthy 34-year-old man treated for a large neuroblastoma involving the L3-5 vertebral bodies and prevertebral great vessels. Neoadjuvant chemotherapy led to significant cytoreduction, enabling en bloc resection of the initially unresectable tumor. Adjuvant differentiating therapy was employed, and curative radiation therapy was used to address the first instance of locoregional recurrence. The patient remains alive with stable disease more than 2 years after his initial diagnosis.

A rare case of pediatric primary central nervous system differentiating neuroblastoma: an unusual and rare intracranial primitive neuroectodermal tumor (a case report).

Neuroblastoma represents the most common solid extracranial tumor in children under 5, accounting for 8% to 10% of all childhood cancers. Primary central nervous system (CNS) neuroblastomas are a very rare location and only few cases are available in the literature. It was first described in 1973 by Hart and Earl as supratentorial primitive neuroectodermal tumors. Clinical presentation is highly variable and depends on the initial location of the tumor. Regarding imaging, primary brain neuroblastoma shows no pathognomonic appearance on brain computed tomography (CT) whether or not enhanced or magnetic resonance imaging (MRI). There were no standard guidelines available for the adjuvant treatment in case of primary CNS neuroblastoma. Surgery remains the main and the first tool toward these lesions. Radiotherapy associated or not to chemotherapy is offered based on patient´s age. Here, the authors report a new pediatric case of primitive central nervous system neuroblastoma revealed by an intracranial hypertension syndrome and confirmed by both histopathological and immunohistochemistry study after a gross total surgical excision. The postoperative course was uneventful and the child had good recovery.

Clinical features and surgical outcome of a suprarenal mass detected before birth.

PURPOSE: With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass has become more common. Most of these masses prove to be congenital neuroblastomas (CNB), but the diagnosis is often confused with other benign lesions and the postnatal management remains to be controversial. METHODS: The medical records of 18 patients that underwent primary surgical excision for an antenatally detected suprarenal mass, between January 1995 and April 2009, were reviewed. The clinical, radiological, surgical, and pathological data were collected. Staging evaluation was performed after histological confirmation of the CNB. RESULTS: There were 13 cases of CNB, 1 adrenal cyst, 2 adrenal hemorrhages, and 2 pulmonary sequestrations. The differential diagnosis was impossible before surgery. Most of the CNBs were stage I (N = 11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had more than one copy of N-myc gene. The stage I patients were cured by surgery alone, and stage IV patients underwent nine cycles of adjuvant chemotherapy and currently have no evidence of disease. The five benign lesions were cured with excision alone. There were no postoperative complications. CONCLUSION: For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an antenatally detected suprarenal mass. The surgery can be safely performed during the neonatal period and provides a cure in most cases.

[Retroperitoneal mass in children : clinical cases of Wilms tumor and neuroblastoma]. / Le cas clinique du mois. Les masses rétropéritonéales chez l'enfant : a propos d'une tumeur de Wilms et d'un neuroblastome.

Detection of a retroperitoneal mass in children needs a fast and accurate exploration. We present the case-reports of 2 children under the age of 5 years admitted to the University Hospital of Liège, one with a Wilms tumor and one with a neuroblastoma.

Definitive Tumor Resection after Myeloablative High Dose Chemotherapy Is a Feasible and Effective Option in the Multimodal Treatment of High-Risk Neuroblastoma: A Single Institution Experience.

BACKGROUND/PURPOSE: The delayed local treatment approach (DL) in high-risk neuroblastoma (HR-NB) refers to the process in which tumor resection is performed after the completion of all the courses of chemotherapy, including myeloablative high-dose chemotherapy (HDC). Alternatively, in the conventional local treatment approach (CL), tumor resection is performed during induction chemotherapy. In this study, we compared the surgical outcomes in HR-NB patients treated by CL and DL. METHOD: Forty-seven patients with abdominal HR-NB underwent primary tumor resection from 2002 to 2018. The timing of surgery was generally determined by following the trials and guidelines available at the time. The outcomes and surgical complications between the two strategies were compared. RESULT: Operation time, blood loss, and postoperative WBC counts were lower in the DL group (n = 25) when compared to the CL group (n = 22), statistical significance notwithstanding. Major vascular structures were less frequently encased in the DL group tumors, while immediate surgical complications were significantly more frequent in the CL group (P < 0.05). Furthermore, the 3-year EFSs were 50.0% and 53.9% in the DL and CL groups, respectively. CONCLUSION: DL appears to be a feasible and effective treatment option for HR-NB. Nonetheless, further verifications using larger cohorts are warranted. LEVEL OF EVIDENCE: Treatment study, Level III.

Primary thoracic neuroblastoma in an adult: A rare case report.

RATIONALE: Neuroblastoma is one of the most common malignant tumors in childhood, which mainly occurs in adrenal glands and peripheral sympathetic nerve system. Neuroblastoma occurring in adulthood is rare, and adults with neuroblastoma arising from thorax are exceedingly rare. A case of neuroblastoma that originated from thorax was reported, and was treated by resection operation. PATIENT CONCERNS: A 46-year-old woman was admitted to our hospital with left side chest pain for 5 days. Laboratory examinations were all normal. Chest computerized tomogram (CT) showed a lesion with clear boundary that was located at the left dorsal pleura. The nature of the mass was heterogeneous, showing slight heterogeneous enhancement after contrast and there was no obvious necrosis. DIAGNOSES: Based on the morphologic and immunohistochemical features, the tumor diagnosis was favorable for neuroblastoma. INTERVENTIONS: A resection operation was carried out. OUTCOMES: Three years postoperative, no sign of recurrence or metastasis has been observed. LESSONS: Primary neuroblastoma in adulthood is rare and has poor prognosis. Resection can be an important treatment option, and combining with other methods like chemotherapy, stem cell transplantation, the survival rate may be improved.

Neoadjuvant bleomycin, etoposide, and cisplatin in adult neuroblastoma arising from the broad ligament of the uterus.

BACKGROUND: A case of primary neuroblastoma arising from the broad ligament with excellent response to neoadjuvant bleomycin, etoposide, and cisplatin (BEP) is reported. CASE: A 48-year-old woman, G0, who presented with acute renal failure, an enlarged pelvic mass, and abdominal pain was diagnosed with adult neuroblastoma arising from the broad ligament of the uterus. She received three cycles of neoadjuvant therapy consisting of bleomycin, etoposide, and cisplatin (BEP) given every 3 weeks and had an excellent initial response. She then underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and appendectomy, with pathologic analysis revealing small residual disease on the broad ligament of the uterus and omentum. The patient died of recurrent disease 20 months after her initial diagnosis. CONCLUSIONS: The clinical management of cancer in the broad ligament of the uterus must be tailored to the pathologic diagnosis. Although our patient had an excellent initial response to BEP, further study is needed to identify a treatment that can reduce recurrences and improve clinical outcomes.

[Anesthetic management in two infants undergoing hemilaminectomy for giant mediastinal neuroblastoma].

We report the anesthetic management in two infants who were a three-month-old boy (case 1) and a two- month-old girl (case 2), undergoing hemilaminectomy for giant mediastinal neuroblastoma. Neuroblastoma invades into the intrathecal space in a rare occasion, resulting in paraplegia, which requires emergent operation to save the spinal cord. Mediastinal neuroblastoma also possesses significant respiratory and cardiovascular risks due to the close anatomic relationship with vital organs. In the present report, we show that preoperative computed tomography (CT) scan and/or echocardiography revealed tracheal deviation in case 1 and mass compression of the left atrium in case 2. Therefore, the patients with mediastinal masses should be carefully evaluated before subjecting them to anesthesia. Especially, there is a possibility that the masses which do not appear to compress the airway and cardiovascular systems develop the airway obstruction and cardiovascular collapse after induction of general anesthesia. In fact, before induction of general anesthesia in the case 2, trial of the prone positioning which was scheduled for the hemilaminectomy, resulted in bradycardia and hypotension, presumably due to the compression of the left atrium. In summary, the authors here discussed the preoperative evaluation and anesthetic management for giant mediastinal neuroblastoma in infants.

The Application of an Evidence-Based Clinical Nursing Path for Improving the Preoperative and Postoperative Quality of Care of Pediatric Retroperitoneal Neuroblastoma Patients: A Randomized Controlled Trial at a Tertiary Medical Institution.

BACKGROUND: A clinical nursing path (CNP) that encourages patients and their families to become actively involved in healthcare decision-making processes may improve outcomes of pediatric retroperitoneal neuroblastoma (NB) patients. OBJECTIVE: The aim of this study was to evaluate the utility and value of an evidence-based CNP provided to pediatric retroperitoneal NB patients undergoing resection surgery. METHODS: One hundred twenty NB cases were assigned to a control group or a CNP group. The control group was provided with standard nursing care. The CNP group was provided with nursing care in accordance with an evidence-based CNP. The utility and value of the CNP were compared with standard nursing care. Outcome measures included rates of postoperative complications, lengths of hospital stay, and cost of hospitalization, as well as preoperative and postoperative quality of care and patient satisfaction with care. RESULTS: The rates of postoperative complications, length of preoperative hospitalization, total length of hospital stay, and costs of hospitalization were significantly lower for patients receiving the CNP compared with the control group. Preoperative and postoperative quality of care and patient satisfaction with care were significantly higher in patients receiving the CNP compared with the control group. CONCLUSION: Adoption of a CNP for preoperative and postoperative care of pediatric retroperitoneal NB patients undergoing resection surgery improves clinical outcomes and patient satisfaction with care. IMPLICATIONS FOR PRACTICE: A CNP can increase families' participation in a patient's recovery process, enhance nurses' understanding of the services they are providing, and improve the quality of healthcare received by patients.

Unremitting watery diarrhoea in early childhood period.

Chronic watery diarrhoea can be a presentation of gastrointestinal disease itself or a less-evident systemic disease. A 17-month-old boy presented with intractable diarrhoea, failure to gain weight, refractory tachycardia and severe hypertension. The ability to recognise and make a quick diagnosis of secretory type of diarrhoea dictated the outcome of patients with this ailment. Catecholamine hypersecretion was considered with the additional clues of refractory tachycardia and hypertension, a well-recognised phenomenon of neuroblastic tumours. A neuroblastic tumour can lead to vasoactive intestinal peptide (VIP) overexpression, which may result in secretory diarrhoea. In this situation, measurements of plasma VIP enabled crucial diagnosis. Imaging studies were used to identify and localise a neuroblastic tumour. Subsequent removal of the tumour was curative and led to the resolution of the symptoms.

The efficacy of delayed surgery in children with high-risk neuroblastoma.

CONTEXT: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies. AIMS: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma. MATERIALS AND METHODS: Thirty-one children with high-risk neuroblastoma were enrolled into the study. STATISTICAL ANALYSIS USED: Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) for windows 10.0. RESULTS: There 'were 15 male and 16 female patients with a median age of 3.0 ± 3.2 years. Primary tumor site was adrenal in 27, non-adrenal in two, pelvic in one, and mediastinal in one patient. MYCN gene was amplified in four and non-amplified in 11 children on totally 15 children with available data. Lactate dehydrogenase was elevated in 30 children. The tumor volumes at diagnosis and before surgery in the whole group were 154.3 and 12.5 mL, respectively. The decline in tumor volume was statistically significant (P < 0.0001). Initial surgery was performed in three and delayed in 20 children, and eight children were inoperable. Surgical complication rate was 66.6% (two out of three patients) in initial surgery group; however, the rate was 15% (3 out of 20 patients) in delayed surgery group. The 5-year event-free survival and overall survival rates in the whole group were 44.8% and 50.8%, respectively. Primary tumor area control rate was 95% CONCLUSIONS: In conclusion, the delayed surgery with intensive chemotherapy and radiotherapy has been successful for primary control in high-risk neuroblastoma patients.

First line targeted radiotherapy, a new concept in the treatment of advanced stage neuroblastoma.

33 previously untreated advanced stage neuroblastoma patients were treated with [131I]meta-iodobenzylguanidine (MIBG). The number of treatments varied between 2 and 7 per patient (mean 3). Toxicity was seldom severe. Only thrombocytopenia WHO-grade 4 was noticed. Response was documented before surgery for the primary tumour was performed. There was one complete response (CR), 18 partial responses (PR), 11 had stable disease (SD) and 3 had progressive disease (PD). After MIBG therapy and surgery, 12 of 33 patients achieved a CR. This approach is feasible, comparable to multidrug chemotherapy in efficacy and less toxic. Long term results are not known yet.

Superficial temporal artery interposition graft after resection of bilateral anterior cerebral arteries. Case report.

A patient with an olfactory neuroblastoma in the frontal skull base was treated with primary tumor excision and partial resection of the A2 segment of both anterior cerebral arteries (ACA's). Revascularization was carried out using a superficial temporal artery (STA) interposition graft between the proximal and distal stumps with A3 segment-to-A3 segment end-to-side anastomosis. This technique established flow to the peripheral territories of the ACA's through the interposed STA graft. The technical aspects and possible role of this procedure are presented.

Surgical treatment for abdominal neuroblastoma in the laparoscopic era.

BACKGROUND: The role of laparoscopy in children with cancer has not been fully defined. The aims of this study were to develop an optimal surgical procedure for the treatment of abdominal neuroblastoma in the laparoscopic era and to evaluate the advantages and disadvantages of laparoscopy in the pediatric population. METHODS: Since July 1997, 37 children were diagnosed with abdominal neuroblastoma at our center, and 44 surgical procedures were performed on them. Patients with advanced neuroblastoma underwent laparoscopic biopsy, open biopsy, and delayed primary or second-look excision, whereas early neuroblastoma cases had either laparoscopic or open excision. We compared the length of the operation, intraoperative blood loss, length of hospital stay, complications, and time to start postoperative feeding and chemotherapy for the laparoscopic and open surgery groups. RESULTS: Length of stay and time to postoperative feeding and chemotherapy were significantly lower in the laparoscopic group than the open surgery group. However, there were no significant differences between the two groups in length of operation and intraoperative blood loss. CONCLUSION: Laparoscopic biopsy and excision of abdominal neuroblastoma are effective and efficient surgical procedures in children.

[Anesthetic management of a patient with norepinephrine-secreting neuroblastoma by using prostaglandin E1].

The anesthetic management of a 5-month-old male with norepinephrine-secreting neuroblastoma was described. Partial excision of the tumor was carried out under general anesthesia induced with enflurane, fentanyl and succinylcholine, and maintained with enflurane, nitrous oxide and oxygen. In this case, hypertension was observed intraoperatively and prostaglandin E1 was continuously infused at a rate of 0.1-0.5 micrograms.kg-1.min-1 to control blood pressure. Severe hypotension after removal of the tumor was not observed. Continuous administration of prostaglandin E1 was useful in this patient with norepinephrine-secreting neuroblastoma.

Intraoperative phosphorus-32 brachytherapy plaque for multiply recurrent high-risk epidural neuroblastoma.

Achieving local control is a crucial component in the management of neuroblastoma, but this may be complicated in the setting of prior radiation treatment, especially when the therapeutic target is in proximity to critical structures such as the spinal cord. The authors describe a pediatric patient with multiply recurrent neuroblastoma and prior high-dose radiation therapy to the spine who presented with progressive epidural disease. The patient was managed with resection and intraoperative high-dose-rate brachytherapy using a phosphorus-32 ((32)P) plaque previously developed for the treatment of brain and spine lesions.