1/89. Neuroblastoma of the urinary bladder, preclinically detected by mass screening.BACKGROUND: Since the introduction of mass screening of infants for neuroblastoma, the incidence of neuroblastoma has increased in japan. The reason for this increased incidence is the possible inclusion of many neuroblastomas that would have regressed spontaneously and would never have been detected clinically. An extremely rare tumor at the dome of the urinary bladder in a 7-month-old infant was detected by the mass screening. methods: A case of neuroblastoma of the urinary bladder is reported with a review of the literature. The data in the japan Children's Cancer Registry are also reviewed to analyze the incidence and the site of origin of neuroblastoma for evaluation of mass screening. RESULTS: A 7-month-old female infant was referred because of a positive urine vanillylmandelic acid screening test. ultrasonography showed a solid mass attached to the urinary bladder. At laparotomy a 35 x 30 x 25-mm egg-shaped tumor was found at the dome of the bladder, and a partial cystectomy was performed. During the operation no changes in blood pressure occurred when the tumor was manipulated. Histologic diagnosis was rosette-fibrillary neuroblastoma originating in the bladder wall, with a favorable Shimada histopathologic classification. N-myc was not amplified, which predicted a favorable prognosis, and no postoperative chemotherapy was given. The patient was free of symptoms and tumor after a follow-up period of 16 months. literature review revealed that this was the second case of neuroblastoma of the urinary bladder ever reported in the world, although several cases of pheochromocytoma originating in the bladder wall had been reported. Both neuroblastoma and pheochromocytoma derive from the neural crest. The sympathogonia from the neural crest, a common stem cell, differentiates into a ganglion cell or into a secretory cell known as a chromaffin cell, able to manufacture catecholamines. The first case in the world that was reported as neuroblastoma of the urinary bladder was in a 4-month-old infant who was noted to have a 4-cm lower abdominal mass on routine physical examination. A ganglioneuroblastoma of the dome of the bladder was excised and the patient was doing well. On reviewing the japan Children's Cancer Registry, the incidence of neuroblastomas in infants has increased as well as the number of stage 1, 2, and 4s (stage 4 special) neuroblastomas since the introduction of mass screening. However, there has been no significant change in the number of stage 3 or 4 diseases diagnosed in older children. According to the japan Children's Cancer Registry, pelvic origin neuroblastoma, which has been noted to have spontaneous regression, was more frequent in the primary tumors detected by mass screening when compared with those presenting clinically. During preparation of this manuscript another case of bladder dome neuroblastoma was detected by urinary vanillylmandelic acid screening of 6-month-old infants for neuroblastoma in japan. CONCLUSION: These extremely rare cases of neuroblastoma of the urinary bladder involved children younger than 1 year of age and were incidentally detected by routine physical examination or mass screening. This raises the question of whether these tumors might have regressed spontaneously had they gone undetected and untreated.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/89. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases.A series of 35 primary cerebral neuroblastoma is reported. These rare tumours occur most often in children in the first half of the first decade. Grossly the tumors are often massive, discrete, lobular, firm and cystic. Histologically three variants, largely determined by the extent and distribution of the fibrous connective tissue stroma, are recognized: (1) a classical variant, which most resembles the peripheral neuroblastoma and is characterized by a high frequency of Homer Wright rosettes and a relatively high frequency of ganglionic differentiation; (2) a desmoplastic variant, which is characterized by an intense connective tissue stroma; and (3) a transitional variant, in which both the classical and the desmoplastic features may be present within the same case, either concurrently or consecutively. Both the desmoplastic and the transitional forms are less likely to exhibit differentiation to mature ganglion cells, but the importance of identifying the primitive cell elements as neuroblasts is emphasized. With rare exceptions, this can be established only by specific silver impregnations on frozen material. Occasionally the direction of growth may be largely leptomeningeal. Seven illustrative clinical histories with pathological correlations are described. The over-all clinical behaviour of these tumours is that of malignant neuroepithelial neoplasms, characterized by a high recurrence rate. recurrence may, however, be a late development, in some cases occurring five or seven years after apparently successful surgical removal. The tumour shows shows a high incidence of metastatic spread, almost 40 per cent of the cases examined at autopsy having disseminated in the cerebrospinal pathways. Exceptionally, extraneural metastases may also develop. However, long post-operative survival occasionally occurs, and the subsequent clinical course is not always predictable in the individual case. The differential diagnosis is briefly discussed. The cellular nature of the tumour and its biological behaviour recall those of the cerebellar medulloblastoma. Post-operative radiation to the entire neuraxis should be considered for these neoplasms.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
3/89. Spontaneous regression of neuroblastoma.Case of spontaneous regression of neuroblastoma continue to occur in the present multimodal therapy era at institutions where physicians are prepared to withhold treatment on certain patients with residual primary or metastatic disease. From a survey of the 22 member institutions of Children's Cancer Study Group, seven hospitals submitted data on 24 neuroblastoma patients whose disease underwent regression after minimal, unusual, or no treatment. An analysis of these patients and of 33 patients form two large series in the literature shows that the majority of patients are infants with Stage II or Stage IVS disease. The spontaneous regression usually consists of complete disappearance of the disease, but in some neuroblastomas, maturation to ganglioneuroma takes place. The various factors that may influence regression are discussed.- - - - - - - - - - ranking = 0.5keywords = ganglion (Clic here for more details about this article) |
4/89. Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.- - - - - - - - - - ranking = 3.5keywords = ganglion (Clic here for more details about this article) |
5/89. 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 = 0.5keywords = ganglion (Clic here for more details about this article) |
6/89. Late recurrence of neuroblastoma stage 4S with unusual clinicopathologic findings.The authors present unusual clinicopathologic findings of a patient with neuroblastoma stage 4S that recurred 11 years after induction of complete remission with chemotherapy. A 12-year-old girl presented with recurrent tumor in the liver. Urinary catecholamine metabolites were within normal range in contrast to the increased values at initial presentation. She underwent left lateral segmentectomy and biopsy of the right lobe. Histologic analysis of the recurrent tumor showed undifferentiated neuroblastoma intermingled with mature ganglioneuromatous lesions. There also were scattered ganglioneuromatous lesions throughout nontumorous area of the liver. Although multimodal intensified treatments including autologous bone marrow transplantation were performed, the patient died of obstinate recurrent tumor at age 14 years. The clinicopathologic findings suggested dedifferentiation from the ganglioneuromatous lesion rather than ordinary recurrence of the primary tumor. The current case and the literature review may indicate that long-term follow-up would be necessary for neuroblastoma stage 4/4S cases. J Pediatr Surg 36:953-955.- - - - - - - - - - ranking = 1.5keywords = ganglion (Clic here for more details about this article) |
7/89. Neuroblastoma with concomitant giardiasis: report of a case with diagnosis by fine needle aspiration cytology.BACKGROUND: diagnosis of two pathologies, including a neoplasm and infectious condition, by fine needle aspiration (FNA) cytology in the same patient is rare. CASE: A 2-year-old, male child presented with fever, abdominal pain and abdominal mass. Imaging findings were strongly in favor of a neuroblastoma. FNA smears from the mass revealed fecal material containing numerous trophozoites of giardia lamblia. FNA was repeated in view of the imaging findings. Repeat smears showed a small round cell tumor with rosettes and background filamentous/fibrillar material consistent with a neuroblastoma. Chemotherapy reduced the mass considerably. Histopathology of the resected residual mass revealed a ganglioneuroma in addition to remnants of neuroblastoma. The patient was free of disease two years after the initiation of chemotherapy. CONCLUSION: When FNA cytology shows an infectious pathology in the clinical and imaging setting of a tumor, FNA should be repeated so that an important component of the diagnosis is not missed.- - - - - - - - - - ranking = 0.5keywords = ganglion (Clic here for more details about this article) |
8/89. Congenital central hypoventilation syndrome associated with Hirschsprung's disease and neuroblastoma: case of multiple neurocristopathies.We report on a male infant with the rare combined occurrence of congenital central hypoventilation syndrome (CCHS or Ondine's curse), Hirschsprung's disease (HD), and neuroblastoma. Current therapeutical options leave no doubt that children with isolated forms of CCHS, HD, or neuroblastoma must be treated, but management decisions and the ethical dilemma become more difficult with the presence of multiple neurocristopathies. Our patient was dependent on mechanical ventilation and total parenteral nutrition, when a neuroblastoma was diagnosed at age 5 months. We initiated an attempt at curative chemotherapy. The tumor failed to respond to recommended chemotherapeutic regimens, and the patient died at 11 months of age. We emphasize the importance of screening CCHS patients for associated illnesses such as neuroblastoma and ganglioneuroblastoma at time of diagnosis.- - - - - - - - - - ranking = 0.5keywords = ganglion (Clic here for more details about this article) |
9/89. Mediastinal neuroblastoma and ganglioneuroma. The differentiation between primary and secondary involvement on the chest roentgenogram.The roentgenologic diagnosis and differentiation of mediastinal neurogenic tumors are possible on the chest roentgenogram as a rule. The soft tissue mass may be ill-defined and the tumor "ghost-like" in the case of primary neuroblastoma, but it is usually obvious in ganglioneuroma and metastatic disease. The presence of calcifications differentiates neurogenic tumors from other posterior mediastinal tumors of childhood. They are common in primary and rare in secondary disease. Rib erosions and displacement are striking in neuroblastoma (after a few months of age), more subtle in ganglioneuroma, and absent with secondary involvement. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis and treatment were delayed, as the adjacent rib changes were not appreciated for some time. "Dumbbell" shaped tumors are usually associated with vertebral changes and myelography is indicated even in the absence of neurologic deficit. Thoracic deformity and disability subsequent to laminectomy, radiation therapy, or both, are present in all survivors.- - - - - - - - - - ranking = 3keywords = ganglion (Clic here for more details about this article) |
10/89. Central pancreatectomy for a pancreatic ganglioneuroma in a patient with previous neuroblastoma.This is a case presentation of a 28-year-old woman with a mass at the junction of the neck and the body of the pancreas and a childhood history of a resected adrenal neuroblastoma. A central pancreatectomy was performed in this patient. The pathology revealed a benign ganglioneuroma. Ganglioneuromas and their association with a neuroblastoma are briefly reviewed. The indications and contraindications for, as well as the advantages and disadvantages of, a central pancreatic resection are discussed.- - - - - - - - - - ranking = 2.5keywords = ganglion (Clic here for more details about this article) |
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