Cases reported "Neuroblastoma"

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1/106. Schwannoma with features mimicking neuroblastoma: report of two cases with immunohistochemical and ultrastructural findings.

    OBJECTIVE: A study of two cases of a rare variant of benign schwannoma showing areas mimicking neuroblastoma/peripheral primitive neuroectodermal tumour (PNET). methods: Sections of formalin fixed, paraffin embedded specimens were studied by tinctorial stains and immunohistochemistry, and the tissue retrieved from formalin was examined by electronmicroscopy in one case. RESULTS: The tumours were small and subcutaneous. Both showed features of benign schwannoma; one had a multinodular plexiform pattern. In addition, rosette-like structures consisting of collagenous cores surrounded by small round cells or slightly larger epithelioid cells were present. Tumour cells were positive for S100 protein, Leu7, and in one case GFAP, but were negative for neurofilament protein, synaptophysin, and MIC2. Type IV collagen surrounded individual cells. Electronmicroscopy in case 2 confirmed schwannian features (lamina, processes) and failed to show features of neuroblastoma (neuroendocrine granules). CONCLUSIONS: Benign schwannomas may contain rosette-like structures mimicking neuroblastoma/PNET. The techniques used confirmed schwannian differentiation only and eliminated neuroblastoma/PNET. These uncommon variants should be recognised by practising histopathologists to avoid erroneous diagnoses and inappropriate treatment.
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keywords = rosette
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2/106. 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.
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keywords = rosette
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3/106. 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.
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keywords = rosette
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4/106. SiMa, a new neuroblastoma cell line combining poor prognostic cytogenetic markers with high adrenergic differentiation.

    We describe the establishment and characterization of a new neuroblastoma (Nb) cell line, SiMa, carrying the major recurrent chromosome changes associated with poor prognosis Nb, including amplification of N-MYC by formation of double minutes (dmin), der(1)t(1;17)(p35;q12) and der(22)t(17;22)(q22;p13), and loss of chromosome 11, documented at both initiation and late passage. In contrast to these cytogenetic stigmata of poor prognosis, analysis of catecholamine synthesis by high pressure liquid chromatography (HPLC) measurement revealed an advanced degree of adrenergic differentiation with high rates of 3,4-dihydroxyphenylalanine (DOPA), noradrenaline, homovanillic acid (HVA), and vanillylmandelic acid (VMA) production. Contrastingly advanced differentiation and poor prognostic genetic markers combine to render SiMa a unique instrument for investigating the pathology and therapy of Nb.
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keywords = formation
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5/106. Hepatic cirrhosis and portal hypertension in neuroblastoma.

    Hepatic portal hypertension is an unusual complication of cancer therapy. The authors report 2 children under the age of 18 months with disseminated neuroblastoma who developed cavernous transformation of the portal vein during chemotherapy. While the older child remains well, the second patient succumbed from overwhelming variceal hemorrhage as a result of this condition. Full recovery of hepatic function is not invariable following the regression of extensive liver infiltration by neuroblastoma and consideration should be given to follow-up Doppler ultrasound imaging of the hepatic portal system in affected infants.
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keywords = formation
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6/106. Neuroblastomatous recurrence of ganglioglioma. Case report.

    This case is believed to be the first reported recurrent intracranial ganglioglioma with purely neuroblastomatous malignant transformation. A complete macroscopic resection of a right frontal lobe tumor in an 18-year-old woman revealed differentiated ganglioglioma. Seven years later a large, well-demarcated recurrent tumor was again macroscopically totally resected in the same patient. Histological analysis showed malignant transformation in only the neuronal component of the original tumor. A review of the literature on recurrent gangliogliomas and their malignant transformation is included.
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ranking = 0.00028172320494258
keywords = formation
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7/106. Neuroblastoma and bronchogenic cyst: a rare association.

    A few of the known associations between paediatric cancer and congenital anomalies are attributable to contiguous-gene syndromes. Neuroblastoma (NB) has been linked with an excess of gastrointestinal malformations, but there is a significant scarcity of associated respiratory anomalies. We report on two children having an abdominal NB and a bronchogenic cyst diagnosed simultaneously and in different order of appearance. Both masses were removed in separated procedures, taking into account the priority and the time sequence of chemotherapy. literature is reviewed, checking that the genetic basis for this association is supported by speculations about the oncogene RON.
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ranking = 9.390773498086E-5
keywords = formation
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8/106. Disaloganglioside GD2 loss following monoclonal antibody therapy is rare in neuroblastoma.

    BACKGROUND: Gangliosicle GD2 is abundant on human neuroblastoma (NB). Monoclonal antibody 3F8 targeted to GD2 may have imaging and therapeutic potential. Antigen-negative clones can escape immune-mediated attack leading to clinical resistance or recurrence. PROCEDURE: Among 95 evaluable patients treated intravenously with 3F8 (94 Stage 4, 1 Stage 3), 66 received nonradiolabeled 3F8, 11 received 131-iodine-labeled-3F8 (8-28 mCi/kg) with autologous bone marrow rescue, and 18 received both forms of treatment. Prior to treatment, 90 patients tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 68), tumor immunohistochemistry (n = 20), or diagnostic radioimmunoscintigraphy (n = 2). RESULTS: Of 62 patients who had refractory or recurrent neuroblastoma following 3F8 treatment, 61 (98%) tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 51) or tumor immunohistochemistry (n = 10). The sole tumor that lost GD2 expression underwent phenotypic transformation into a pheochromocytoma-like tumor. CONCLUSIONS: The persistence of GD2 expression in refractory or recurrent NB suggests that complete antigen loss is an uncommon event and cannot account for treatment failure.
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keywords = formation
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9/106. 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.
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keywords = rosette
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10/106. Congenital heart disease and neuroblastoma: just coincidence?

    The clinical history of a neonate with simple transposition of the great arteries in whom a metastatic neuroblastoma was diagnosed incidentally at autopsy is described, and the literature containing all 66 previously reported cases of neuroblastoma associated with congenital cardiac malformations is reviewed. One third of the described cases were classified as in situ neuroblastoma; neural crest derived cardiac lesions were present in 31%. Several possible aetiological mechanisms are discussed, and we conclude that the association of neuroblastoma with congenital cardiac malformations is multifactorial in origin. The described case represents the first reported example in which catecholamine release may have contributed to the fatal outcome of definitive congenital cardiac surgery.
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ranking = 0.00018781546996172
keywords = formation
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