Cases reported "Mediastinal Neoplasms"

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1/73. Posterior mediastinal capillary hemangioma with extradural extension resembling neuroblastoma.

    We present two patients with posterior mediastinal capillary hemangiomas that were paraspinal and had intraspinal extension. Computed tomography demonstrated the strikingly hypervascular nature of these tumors, distinguishing them from neuroblastoma.
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ranking = 1
keywords = neuroblastoma
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2/73. Competitive polymerase chain reaction for the determination of N-myc amplification in neuroblastoma: report of clinical cases.

    If an unfavorable prognosis is suspected in neuroblastoma, decision on a treatment protocol should be based on the N-myc copy number (12). We already demonstrated that the newly developed competitive polymerase chain reaction (competitive PCR) is a promising method for the determination of the N-myc copy number (6), and have started to use this competitive PCR procedure in neuroblastoma patients, together with fine-needle biopsy in selected cases. Seven children were studied. In one infant of 5 months of age whose tumor was diagnosed before undergoing mass screening for neuroblastoma, the competitive PCR procedure was performed with a fine-needle biopsy, and after obtaining a negative report on N-myc amplification within 48 hours, a regular protocol of treatment could be started without delay. We report that competitive PCR is a rapid and accurate method for the determination of the N-myc copy number, requiring only a small amount of material, and anticipate that competitive PCR will become the procedure of choice for the determination of N-myc copy number in neuroblastoma.
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ranking = 1.6
keywords = neuroblastoma
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3/73. False-negative scintigraphy with Tc-99m sestamibi in stage IV neuroblastoma.

    Tc-99m sestamibi, originally developed for myocardial studies, has been used as a tumor-seeking agent. Recently, the agent also was reported to be a functional tracer to predict multidrug resistance-related p-glycoprotein expression in tumor tissue. The current report presents the authors' experience with sestamibi tumor scintigraphy in a neuroblastoma. Although I-131 MIBG tumor imaging and Tc-99m MDP bone scanning accurately demonstrated the extent of the disease, Tc-99m sestamibi showed no accumulation in primary and metastatic foci. Lack of sestamibi uptake was initially thought to be suggestive of failure to respond to chemotherapy because of p-glycoprotein expression. However, the patient responded well to chemotherapy and complete remission was achieved. The failure of Tc-99m sestamibi to detect a neuroblastoma and the lack of sestamibi accumulation in the tumor may not always be related to chemotherapy resistance.
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ranking = 1.2
keywords = neuroblastoma
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4/73. shoulder pain in a child: a case presentation of ganglioneuroblastoma.

    A four and one-half year-old child presented with a several month history of shoulder pain. Her workup revealed a large, homogeneous tumor in the apex of the chest. Surgical resection was performed demonstrating ganglioneuroblastoma. This case illustrates an unusual cause of joint discomfort in children.
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ranking = 1
keywords = neuroblastoma
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5/73. Disseminated metastasis of neuroblastomatous component in immature mediastinal teratoma: a case report.

    A 17-year-old man with high levels of serum AFP and hCG was diagnosed as having primary mediastinal GCT. cisplatin-based chemotherapy decreased the biomarkers, but the mass showed further growth. Pathological examination of the resected mass revealed a mixture of immature and mature teratomas. Six months after the surgery, the patient died of a dissemination of neuroblastomatous cells, which were similar to those in the immature neural component of the primary tumor. A disseminated metastasis of neuroblastoma in immature mediastinal teratoma is a rare complication. serum NSE can be a useful marker in detecting the metastasis.
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ranking = 1.2
keywords = neuroblastoma
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6/73. 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.
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ranking = 1.4
keywords = neuroblastoma
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7/73. ganglioneuroblastoma in adults.

    ganglioneuroblastoma is a rare neoplasm of adults. Some information about the natural history and response to therapy can be obtained from two cases seen recently at our hospital and 17 cases in the literature. These tumors occur in adults of any age and are usually located in the retroperitoneum, mediastinum, or neck. Evidence of the tumor may be present years before diagnosis. Elevations of urinary catecholamines were documented in three cases and the levels correlated with progression or resection of the neoplasm. The tumor spreads either by local invasion or distant metastasis. Attempts at radiotherapy and chemotherapy in the treatment of unresectable or disseminated tumor has not resulted in objective improvement. This contrasts with the widely reported objective successes and cures of ganglioneuroblastomas occurring in childhood. Surgical excision of localized disease was the only curative therapeutic modality in the cases we reviewed.
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ranking = 1.2
keywords = neuroblastoma
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8/73. ganglioneuroma presenting as an asymptomatic huge posterior mediastinal and retroperitoneal tumor.

    ganglioneuroma is a rare, differentiated, benign and slow-growing tumor that commonly arises from sympathetic ganglion cells. Most of them are asymptomatic and found incidentally. We here report a quite rare case of silent huge ganglioneuroma growing in both posterior mediastinum and retroperitoneum occurring in a 3.5-year-old girl. The patient was relatively well before and incidentally found to have a huge chest mass by chest x-ray film at an episode of respiratory tract infection. Computed tomography showed a huge tumor extending from bilateral posterior mediastinum to the level of the adrenal gland in the retroperitoneum. Initially, neuroblastoma was highly suspected and 24-hour urine vanillyl mandelic acid was slightly elevated. Cytology by bone marrow aspiration revealed no tumor nests or clumps. biopsy and pathology proved it as ganglioneuroma (GN). Due to too extensive involvement of the tumor and compression of the vital vessels, surgical removal became difficult. The family of the patient refused surgery due to there being no significant symptoms. Because of the potential for growth of unresectable GN and because the component of neuroblasts could not be completely excluded, the patient was still in dangerous status. The only thing we can do is to keep the family alert and continue regular follow-up.
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ranking = 0.2
keywords = neuroblastoma
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9/73. Long-term survival in adult mediastinal neuroblastoma.

    Neuroblastoma rarely occurs in adults, in whom prognosis is poor. Here we report on the effective use of multimodal therapy to achieve long-term survival in adult mediastinal neuroblastoma. In a 33-year-old male with severe pain radiating from the left hypochondrium to the back area, no abnormal shadow was detected on first examination; the shadow appeared in chest X-ray only 10 months later when the severe pain recurred. The patient was then referred to our department for further examination. Chest x-rays and chest CT scans revealed a posterior mediastinal mass beside the thoracic vertebrae. diagnosis was confirmed after surgical resection, which was followed by outpatient adjuvant therapy through radiation and chemotherapy. The patient has survived 8 years and 8 months without recurrence or metastasis.
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ranking = 1
keywords = neuroblastoma
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10/73. Fine needle aspiration cytology of primitive neuroectodermal tumors. A report of these cases.

    Primitive neuroectodermal tumor (PNET) is a small round cell malignancy arising in soft tissue and bone, predominantly in older children and adolescents. We report the cytomorphologic features and findings of ancillary studies of eight fine needle aspiration (FNA) biopsies from three patients (7-year-old male, 12-year-old female, 9-year-old female). Two of the biopsies suggested the initial diagnosis of PNET of the chest wall, while the remaining six documented recurrent or metastatic disease. In one of these cases the primary diagnosis made by FNA biopsy enabled the pediatric oncologists to give specific therapy for the unresectable tumor and achieve remission. Local recurrences included the chest wall (two cases), pleura (one case) and pericardium (one case), while metastatic disease involved the supraclavicular lymph node and breast. All the cases consisted of small malignant cells with a high nuclear/cytoplasmic ratio and hyperchromatic nuclei without prominent nucleoli. Homer Wright rosettes were seen in only two of the aspirates, and neuropil and ganglion cells were not present. Ancillary studies, including electron microscopy (two cases), immunocytochemistry (four aspirates from two cases) and cytogenetics (11/22 translocation, one case) performed on the aspirated material were aids in making a specific diagnosis and excluded other small round cell tumors of childhood, such as malignant lymphoma, rhabdomyosarcoma and Ewing's sarcoma. The differential diagnosis between PNET and neuroblastoma can be difficult on the basis of an FNA biopsy alone, although light microscopic morphologic differences exist. Clinical features (e.g., age, primary site, metastatic patterns), catecholamine levels, electron microscopy and cytogenetics are necessary in establishing the correct diagnosis.
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ranking = 0.2
keywords = neuroblastoma
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