Cases reported "Neuroblastoma"

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1/18. Injuries of large vessels in high stage neuroblastoma surgery. A case report.

    Complete resection of the primary lesion in stage III neuroblastoma improves survival neuroblastoma has a tendency towards surrounding and infiltrating the large vessels, leading to injuries during tumor resection. We operated on a stage III neuroblastoma, which resulted in the right and left common iliac artery and vein damage. The right common iliac artery and, veins were repaired by end to end anastomosis. There was a long gap between the two ends of the left common iliac artery and it was repaired using a mesenteric vein (marginal vein of the colon) graft. Digital subtraction angiography performed 6 months after the operation did not reveal any stenosis or aneurysmatic changes in the anastomoses. We conclude that short segments of large vessels may be sacrificed during the resection of neuroblastomas invading the vessel wall, and the resulting defects may be repaired by end to end anastomosis, or even by substituting mesenteric vein grafts, for the purpose of total or near total removal
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2/18. jaw and pulpal metastasis of an adrenal neuroblastoma.

    Hematogenous spread of malignant tumors to the dental pulp is very rare. A case of adrenal neuroblastoma in a 71/2-year-old boy which metastasized to the mandible and dental pulp is described. Tumor cells were found within the pulpal blood vessels of a deciduous molar tooth.
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3/18. Cerebral ganglioglioma with long history and unusual prominence of the mesenchymal elements. Case report.

    A case of cerebral ganglioglioma, a relatively rare and controversial tumor of the central nervous system, is reported. The histological pattern of the tumor consisted of differentiated neuronal cells and glial elements displaying a various extent of cytologic abnormalities. Beside these two typical components for ganglioglioma, an abundance of collagen fibrils and numerous blood vessels were encountered. The long clinical course manifested by temporal epilepsy preceding clinical diagnosis of brain tumor and peculiar histological appearance seem to be of considerable interest.
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4/18. Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report.

    A 27-year old male patient underwent surgery for a cerebellar ganglioglioma in which a pleomorphic xanthoastrocytoma constituted the gliomatous element. Neither radiation nor chemotherapy was administered. The patient was well for 12 years and then he suffered a recurrence and underwent a gross total resection. Histological examination revealed a ganglioglioma with thrombosed blood vessels, areas of tumor necrosis, and scattered mitoses. These anaplastic changes, which were restricted to the glial component of the tumor, may herald a more aggressive clinical course. However, 11 months after the second operation, there have been no signs of tumor recurrence.
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5/18. Successful treatment of chemoresistant stage 3 neuroblastoma using irinotecan as a single agent.

    The authors describe a 1-year-old boy who was diagnosed with neuroblastoma by mass screening at age 6 months. The tumor originated from the left retroperitoneum and extended over the midline, involving major vessels and invading the spine with compression of the spinal cord. Although seven courses of chemotherapy consisting of vincristine sulfate, cyclophosphamide, pirarubicin hydrochloride, and cisplatin were administered, there was no reduction in tumor size or decrease in tumor markers. The patient received irinotecan 180 mg/m per day for 3 days. Approximately 3 weeks later the tumor had regressed remarkably, and tumor markers normalized after the second course of irinotecan. This therapy was given a total of four courses every 4 weeks, with the tumor shrinking successively in each session. Four years after treatment there is no sign of recurrence and the patient is doing well. This case may be the first report showing the dramatic efficacy of irinotecan in the treatment of chemoresistant neuroblastoma without the use of other antitumor agents. Irinotecan might be a promising drug in the management of patients with high-risk neuroblastoma.
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6/18. High-grade cerebral neuroblastoma: a case study.

    The case of a 48-year-old male patient is reported in whom a primary malignant cerebral neoplasm was cured by its neurosurgical removal and by postoperative radiotherapy and chemotherapy. Initially, from the results of the examination of frozen and paraffin section, the tumour was considered to be a glioblastoma multiforme. Electronmicroscopy, immunohistochemistry and review of the light microscopy of the original biopsy sample after his death by suicide four-and-a-half years later showed the neoplasm to have been a primary cerebral neuroblastoma rather than a glioblastoma. The diagnosis of glioblastoma multiforme, which depends upon multiple non-specific microscopic findings, such as necrosis, abnormal blood vessels, anaplasia and the pleomorphism of tumour cells, often is imprecise. Our experience underlines the need for comprehensive neuropathological studies of malignant cerebral neoplasms, including transmission electronmicroscopy and immunohistochemistry. This is of particular importance in view of the dismal prognosis of glioblastoma multiforme and of the palliative, rather than curative, treatment programmes that frequently are indicated for this tumour. The value of our report is to demonstrate that a cerebral neuroblastoma, which potentially is curable, may be mistaken easily for a glioblastoma-even by competent neuropathologists.
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7/18. Resection of advanced stage neuroblastoma with the cavitron ultrasonic surgical aspirator.

    Current protocols for the treatment of neuroblastoma emphasize total or near total resection of tumor to improve survival. This is preferentially performed as a primary procedure, or is attempted at a second-look operation. Unfortunately, this tumor often grows to large size with invasion of the spinal canal, or encasement of major vascular or other retroperitoneal structures. A primary attempt at complete removal may result in difficult-to-control hemorrhage or injury to, or loss of, vital organs. A second-look procedure carries other intrinsic risks. It often must be performed during a period of chemotherapeutically induced hematologic and immunologic suppression. The presence of adhesions and dense scar tissue increases the complexity of the dissection. The Cavitron Ultrasonic Surgical Aspirator (CUSA) combines continuous fragmentation, irrigation, and aspiration in one instrument. tissues high in water content are selectively fragmented and aspirated, while tissues high in collagen and elastin (such as blood vessels and pseudocapsular walls) are selectively spared. Five patients, two with large pelvic dumbell tumors, two with large intrathoracic tumors, and one with a seemingly unresectable large right adrenal tumor (crossing the midline with extensive aortocaval nodal involvement) had total or near-total resection accomplished using the CUSA. In these patients, initial resection of the relatively soft inner part of the tumor left a collapsed pseudocapsule, which was then removed under greatly improved exposure in a relatively small field. The constant aspiration virtually eliminated tumor spillage. Since most vessels were skeletonized without penetration, total blood loss was minimized. There were no intraoperative or postoperative complications.
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8/18. Primary differentiated neuroblastoma of the orbit.

    A 49-year-old woman had a 12-year history of a localized left orbital tumor that required five subtotal excisions, orbital radiotherapy, and finally an exenteration. The last procedure was performed after visual function had deteriorated and in order to prevent spread of the tumor into a surrounding compartment. The biopsy specimens from the first four surgeries showed a stroma-free spindle cell tumor with benign cytologic features and no mitotic activity, which exhibited palisading of nuclei, imbrication of delicate cytoplasmic processes (neuropil), true perivascular rosettes with cytoplasmic processes oriented perpendicular to vessel walls, and Wright rosettes. The biopsy after radiotherapy and the exenteration specimen contained more polyhedral (gemistocytoid) tumor cells with abundant eosinophilic cytoplasm and tapering cell processes; nuclear pleomorphism without mitotic activity was also seen. Electron microscopy showed the presence of neurosecretory dense-core granules in the perikaryon region of the tumor cells and in the myriad interweaving cytoplasmic processes (neurites); neither Nissl substance nor synapses were identified. Immunohistochemical staining for neuron-specific enolase was positive, but glial fibrillary acidic protein stained negative. This previously undescribed orbital tumor is interpreted as a primary differentiated neuroblastoma without evidence of ganglion cell differentiation that exhibited locally aggressive behavior. The distinctions between neuroblastic and neuroendocrine tumors are discussed.
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9/18. Mechanism of direct spread of abdominal neuroblastoma: CT demonstration and clinical implications.

    neuroblastoma, a malignant tumor of neural crest origin, is the most common extracranial solid tumor in children. In 1971 Evans et al. introduced a clinical staging for neuroblastoma. Over sixty percent of patients present with neuroblastoma beyond stage I. Despite more aggressive therapy there has been only minimal improvement in survival. Since 1978, all patients with neuroblastoma have had CT scanning as part of their initial evaluation at our institution. Children with abdominal neuroblastoma beyond stage I form the basis of this report. Selected cases illustrating the permeative nature of neuroblastoma and the mechanism of direct abdominal spread by CT scanning are presented. The tumor originates in the retroperitoneum and spreads to the abdominal aorta where it gains access to the subperitoneal space via the celiac axis and superior mesenteric artery. These vessels course from the aorta to their ultimate destination within their peritoneal folds. These folds form the interconnecting space (subperitoneal space) between the retroperitoneum and the peritoneal organs. Such scanning is extremely sensitive in detecting neuroblastoma with early infiltration into adjacent tissues and contiguous spread through abdominal spaces. The clinical implications of the permeative nature of neuroblastoma and the mechanism of contiguous abdominal spread are discussed.
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10/18. Primary cervical neuroblastoma in infants.

    We describe three cases of cervical neuroblastoma, each occurring in an infant less than one year old, and discuss the investigation and treatment of these patients. The prognosis of patients with advanced neuroblastoma is poor. By contrast, patients with stage I and II tumours often do well and it therefore seems appropriate for otolaryngologists to be familiar with the clinical features and management of localized cervical neuroblastoma. A history of upper aerodigestive compromise is typical, and in the evaluation of this complaint, the possibility of a cervical neuroblastoma must be borne in mind. A retropharyngeal firm, smooth mass is consistent with this diagnosis and supportive evidence can be obtained from ancillary studies. A biopsy is required for tissue diagnosis and, if possible, the entire tumour and sample local nodes should be excised. This will also relieve airway and pharyngeal obstruction. Complete tumour removal at the expense of major nerves and vessels is not necessary in view of the tendency to tumour regression. We conclude that prolonged disease-free survival can be achieved in infants by the use of limited surgery, with chemotherapy in some instances, and without the use of radiation therapy. Management decisions should be made jointly by paediatric otolaryngology and paediatric oncology teams.
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