1/55. Artificial abdominal hernia for the treatment of hepatomegaly in a neonate with stage 4S neuroblastoma.Surgical widening of the abdomen by a silastic pouch has been used very rarely in the management of critically ill infants with hepatomegaly due to neuroblastoma stage 4S. A female newborn baby was referred on the second day of life because of local compressive effects of a massive hepatomegaly, which lead to multiorgan failure. An artificial abdominal hernia was created on the third day of life using a silastic pouch. During the operation oxygenation and ventilation improved and urinary output returned. After chemotherapeutic reduction of hepatic metastases and primary tumor the pouch was successfully removed on day 57 without local complications. The child has survived for more than 1 year and is in complete remission. An artificial abdominal hernia should be considered more often in the critically ill neonate with stage 4S neuroblastoma and massive hepatomegaly.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/55. Multifocal neuroblastoma: biologic behavior and surgical aspects.BACKGROUND: Although multifocal neuroblastoma is rare, its incidence has increased because of recent improvements in diagnostic tools and the introduction of mass screening. Among the 106 neuroblastoma cases treated at the authors' hospital between 1984 and 1998, 8 were multifocal neuroblastoma. methods: The authors examined clinicopathologic findings and biologic features, including MYCN amplification, NTRK1 and Ha-ras p21 expression, cellular dna content, and telomerase activity in these 8 multifocal neuroblastoma cases. Moreover, clinicopathologic findings were investigated with a review of 53 published cases of multiple neuroblastoma in the literature published in English between 1966 and 1999. RESULTS: Among these eight cases, five were detected by mass screening and three were incidental neuroblastomas. Histologically, all tumors were classified as ganglioneuroma or favorable neuroblastoma except one advanced case. All tumors lacked the MYCN gene amplification and expressed NTRAK1 mRNA and Ha-ras p21 protein. Cellular dna content showed that half of these tumors were near-triploid, and the proliferative index (%S-phase) of all tumors was less than 25%. High telomerase activity was detected in none of these cases. Four patients underwent multistage operation and five patients with bilateral adrenal neuroblastomas underwent tumor enucleation to preserve adrenal function. Currently, all patients are disease free and none have required corticosteroid replacement therapy. Among the previously reported 53 cases with multifocal neuroblastoma, 25 were incidentally detected, 18 had familiar history, and most patients without other major complications also had extremely good prognoses. CONCLUSIONS: These findings suggested that most multifocal neuroblastomas have favorable biologic features. Clinically, surgical approaches should be attempted to preserve organ function, especially adrenal function, and minimal invasive surgery should be performed. In cases of thoracoabdominal neuroblastoma, multistage surgery is effective and safe.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
3/55. Preservation of olfaction in anterior skull base surgery.OBJECTIVES/HYPOTHESIS: In selected unilateral tumors and defects of the anterior skull base, the preservation of contralateral olfaction is achievable through a localized subcranial approach without compromising surgical objectives of resection or repair. STUDY DESIGN: Description of a functional adaptation of anterior skull base surgical techniques through a retrospective patient series. methods: Nine patients underwent anterior skull base surgery for unilateral cribriform plate disease including four malignant and two benign tumors, two encephaloceles, and one iatrogenic cribriform injury with cerebrospinal rhinorrhea. All nine patients consented to a localized subcranial approach to the anterior skull base to preserve the contralateral olfactory nerves. In four patients with benign disease a portion of the ipsilateral nerves was additionally conserved. Postoperative olfaction was assessed objectively with a commercially available smell test. Indications, technique, results, and complications are reported and discussed. RESULTS: All patients had eradication of disease with preservation of functional olfaction CONCLUSIONS: Conservation of olfaction is possible in selected cases of anterior skull base surgery when the lesion is unilaterally confined.- - - - - - - - - - ranking = 25.480795389985keywords = nerve (Clic here for more details about this article) |
4/55. Myopathy with muscle spindle excess: A new congenital neuromuscular syndrome?An infant presented with congenital weakness, hypotonia, arthrogryposis, atrial tachycardia, and a left intra-abdominal neuroblastoma. Muscle biopsy revealed marked excess of muscle spindles with atrophy of extrafusal fibers. The patient expired at age 14 months from progressive cardiorespiratory failure. Postmortem examination demonstrated muscle-spindle excess in other muscles, along with hypertrophic obstructive cardiomyopathy and organomegaly. Muscle spindle excess has previously been reported in two patients with noonan syndrome and progressive hypertrophic cardiomyopathy. Muscle spindle excess with hypertrophic cardiomyopathy, organomegaly, and, possibly, congenital neuroblastoma suggests a syndromic association and may represent an unusual form of congenital myopathy.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
5/55. Congenital neuroblastoma mimicking early onset sepsis.A newborn girl presented with symptoms of severe early onset sepsis but also with systemic hypertension (SH) at age 3 h. plasma catecholamine (CAT) levels were extremely elevated, reflecting increased release of CAT from a congenital neuroblastoma (NB). Clinical symptoms at time of admission were: prolonged capillary refill (5 s), tachycardia, tachydyspnoea, metabolic acidosis (pH 7.17, lactate 11.8 mmol/l), fever (38.4 degrees C) and SH: 90/50/65 mmHg (systolic/diastolic/mean). The infant experienced organ failure (lung, heart, liver). A retroperitoneal dumbbell tumour was detected. plasma CAT levels at age 15 h were: noradrenaline 219 nmol/l; adrenaline 13 nmol/l; and dopamine 65.3 nmol/l. SH responded to intermittent alpha-adrenergic blockage. CAT-related symptoms ceased within 1 week. The intraspinal NB was surgically removed when cord compression became symptomatic. The neurological and developmental state is normal at age 17 months. The abdominal NB regressed spontaneously. CONCLUSION: A neuroblastoma should be considered in newborn infants presenting with a shock-like condition together with systemic hypertension.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/55. Second malignancies in children with neuroblastoma after combined treatment with 131I-metaiodobenzylguanidine.BACKGROUND: (131)I-metaiodobenzylguanidine ((131)I-MIBG) is selectively taken up by cells of neural crest origin, allowing targeted radiotherapy of tumors such as neuroblastoma (NB) and pheochromocytoma. radiotherapy may provide additional benefits in the treatment of NB, with moderate side effects such as hematologic and thyroid toxicity. However, with longer follow-up, other complications might occur. We describe our experience with second cancers occurring in children treated with (131)I-MIBG and chemotherapy. methods: The clinical records of 119 consecutive NB cases treated with (131)I-MIBG at a single institution between 1984 and 2001 were reviewed for the occurrence of a second malignant neoplasm (SMN). RESULTS: overall, five cases of SMN occurred in the study patients. In particular, two cases of myeloid leukemia, one of angiomatous fibrous histiocytoma, one of malignant schwannoma, and one case of rhabdomyosarcoma were detected. The schwannoma and the rhabdomyosarcoma developed within the residual neuroblastic mass after first-line therapy. CONCLUSIONS: Should (131)I-MIBG treatment become more broadly employed in the therapeutic strategy for neuroblastoma, the risk of second cancer will have to be taken into consideration. The organization of an international registry of subjects treated with (131)I-MIBG might better define the frequency and features of second malignancies following this radiometabolic approach.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/55. Primitive neuroectodermal tumor (neuroblastoma) arising in sciatic nerve of a child.A well-documented case of a primitive neuroectodermal tumor (neuroblastoma) arising in the sciatic nerve of a 6-year old boy is presented. After radical surgical excision of the tumor, followed by chemotherapy, the child remained clinically free of disease for 20 months. Tumor then recurred locally followed by widespread dissemination. He expired 2 years postoperatively. The literature on this type of rare and controversial tumor of peripheral nerves is reviewed. Only a very few reported cases are considered fully acceptable. Interest in this subject, and adequate documentation of future reported cases may lead to a better understanding of this class of malignant tumors.- - - - - - - - - - ranking = 76.442386169954keywords = nerve (Clic here for more details about this article) |
8/55. Metastatic neuroblastoma presenting with binocular blindness from intracranial compression of the optic nerves.A 2-year-old boy with blindness as an isolated symptom was found to have no light perception binocularly because of compression of both optic nerves by a neuroblastoma infiltrating the walls of the optic canals and medial sphenoid bone. Imaging disclosed a primary tumor near the kidney and multiple osseous metastases. Although neuroblastoma commonly causes blindness by metastasis to the orbit, it rarely causes bilateral blindness from intracranial compression of the optic nerves. This is the first report of bilateral blindness as the presenting feature.- - - - - - - - - - ranking = 76.442386169954keywords = nerve (Clic here for more details about this article) |
9/55. Cerebral neuroblastoma.A cerebral neuroblastoma removed surgically from a female child is presented. Electron microscopy showed numerous neuronal processes with growth cones which are a feature of the developing neurone. In addition there were some rosettes with distinct lumina. The luminal surfaces were covered with a smooth plasma membrane lacking any surface differentiation and the lateral surface of these cells had many cell junctions (terminal bars), reminiscent of a primitive neural tube. These features in a nerve cell tumor help to substantiate it as a neuroblastoma arising from immature rather than differentiated cells. The nature of this rare tumor is discussed.- - - - - - - - - - ranking = 12.740397694992keywords = nerve (Clic here for more details about this article) |
10/55. Superior mediastinal syndrome with Rowland-Payne syndrome: an unusual presentation of cervico-mediastinal neuroblastoma.A 5-month-old boy presented with superior mediastinal syndrome due to a large, unresectable cervico-mediastinal neuroblastoma. He had an unusual constellation of findings not previously reported in neuroblastoma: horner syndrome, phrenic nerve palsy, and palsy of the ipsilateral recurrent laryngeal nerve. This constellation of findings in adult malignancies is termed the Rowland-Payne syndrome. The child required prolonged mechanical ventilation but responded to chemotherapy and is now free of tumor and completely recovered.- - - - - - - - - - ranking = 25.480795389985keywords = nerve (Clic here for more details about this article) |
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