Cases reported "Retroperitoneal Neoplasms"

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1/73. Two cases of neuroblastoma presenting to the emergency department.

    Neuroblastoma is the second most common malignant solid tumor in the pediatric population. Recent advances in treatment options and identification of prognostic subsets have made early detection important. Early classification into a favorable stage and subset may carry a 10-year survival of about 90% (1). With newer treatment regimens, the probability of long-term survival in patients with metastatic disease has also increased about fourfold since 1979 (2). Emergency physicians can contribute to improved morbidity and mortality with appropriate evaluation and disposition of the patient presenting with neuroblastoma. Two patients, a 6-month-old and a 2-week-old, presented to the Emergency Department with weakness. Both had a presumptive diagnosis of neuroblastoma made by the emergency physician. A brief discussion of the incidence, presentation, and diagnosis of neuroblastoma follows.
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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 = 1.1428571428571
keywords = neuroblastoma
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3/73. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.

    Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.
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ranking = 0.85714285714286
keywords = neuroblastoma
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4/73. Involvement of chromosomes 1 and 17 in a case of neuroblastoma.

    We report here the cytogenetic analysis of a neuroblastoma from a 6-month-old male. Both conventional GTG banded analysis and fluorescence in situ hybridization were performed. The tumour was found to have a der(17)t(1;17)(p34;q21).
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ranking = 0.71428571428571
keywords = neuroblastoma
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5/73. Primary retroperitoneal ganglioneuroblastoma in an adult.

    A case of retroperitoneal ganglioneuroblastoma in a 60-year-old man is reported. This retroperitoneal tumor was surgically removed and pathologic diagnosis was ganglioneuroblastoma. ganglioneuroblastoma usually occurs in children and is extremely rare in adults. The characteristics are described of an unusual tumor based on the published reports.
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ranking = 1
keywords = neuroblastoma
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6/73. thallium-201 scintigraphy of neuroblastoma: different results for primary tumors and skeletal lesions.

    thallium-201 scintigraphy was performed in 8 children with neuroblastoma, and uptake by the tumors was evaluated in comparison with the results of 123I-MIBG scintigraphy. No primary tumors or metastatic lymph nodes showed 201Tl accumulation, but in 4 cases of bone marrow metastases accompanied by focal cortical invasion, the metastatic lesion was demonstrated more clearly on the early image than on the delayed image. In another case of bone metastases infiltrating cortical bone revealed by 123I-MIBG scintigraphy and biopsy before treatment, 201Tl scintigraphy performed after chemotherapy showed abnormal accumulation in the tibia, but the second 123I-MIBG scintigraphy performed 1 week after the 201Tl scintigraphy showed no abnormal uptake. 201Tl does not appear to have good affinity for neuroblastoma, but it accumulates in metastatic skeletal lesions. A reactive hypermetabolic bone marrow, and/or inflammatory process and periosteal reaction due to the presence of metastatic foci may have induced the 201Tl accumulation. It seems that 201Tl is not useful for the diagnosis. Nevertheless, the discordance between 201Tl uptake in primary tumors and skeletal lesions allows speculation on the mechanism of 201Tl accumulation in skeletons.
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ranking = 0.85714285714286
keywords = neuroblastoma
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7/73. Conformal proton radiation treatment for retroperitoneal neuroblastoma: introduction of a novel technique.

    BACKGROUND: Postoperative irradiation for locoregionally advanced neuroblastoma requires coverage of the paraspinal retroperitoneum. The proximity of both kidneys and the liver, and a more complex target configuration, can pose a dosimetric challenge for conventional X-ray treatment and intraoperative irradiation. We utilized proton radiation therapy (PRT) to reduce dose to uninvolved kidneys, liver, intestine, and spinal cord. PROCEDURE: A 4-year-old male underwent PRT for neuroblastoma of the right adrenal gland, following chemotherapy and delayed surgical resection. Clinical target volume (CTV), boost volume, and normal structures were outlined on the 3D treatment planning CT scan. The patient received 25.2 CGE (cobalt Gray equivalent) to the CTV and 34.2 CGE to the boost region, using 1.8 CGE per fraction, five treatments per week. Dose-volume histograms (DVHs) were obtained for target and nontarget structures. RESULTS: The 95% isodose volume enclosed CTV and boost volumes. The dose to 50% of the ipsilateral kidney, with tumor involvement of the medial renal surface, was < or = 16 CGE (47% of prescribed total dose). Doses to 50% and 20% of the contralateral kidney in close proximity to deep left-side, paraspinal soft tissue involvement were restricted to 1 CGE and 10 CGE, respectively. Eighty percent of the liver received 27 CGE (80% of prescribed dose). Using a patch technique, unique to charged particle therapy, the spinal cord was almost completely spared during boost volume irradiation. CONCLUSIONS: PRT can achieve excellent dose conformity for advanced retroperitoneal, paraspinal lesions, while respecting normal tissue tolerance levels.
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ranking = 0.85714285714286
keywords = neuroblastoma
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8/73. 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.
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ranking = 0.85714285714286
keywords = neuroblastoma
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9/73. adult-type neuroblastoma originated in retroperitoneum beginning with obstructive jaundice.

    Abdominal neuroblastoma in adults is a rare neoplasm and only 30 patients have been described in japan since 1985. The patient was a 43-year-old woman with jaundice. The tumor originated from retroperitoneum. The enlarged gall bladder and dilatation of intrahepatic bile ducts were noted by ultrasonography and computed tomography. We report the first adult-type neuroblastoma with obstructive jaundice.
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ranking = 0.85714285714286
keywords = neuroblastoma
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10/73. Oral etoposide following combination chemotherapy and radiation therapy in a patient with advanced adult neuroblastoma led to long survival.

    Although neuroblastoma is a common tumor of early childhood, it is rare in adults. In spite of the poor prognosis of advanced neuroblastoma in adults, there is still no effective treatment for this condition in adults. Here we report that oral etoposide salvage chemotherapy was able to prolong the survival of an adult patient with advanced neuroblastoma. A 26-year-old man had advanced neuroblastoma that was resistant to combination intravenous chemotherapy and radiation therapy. He was then treated with oral etoposide, at a dose of 50 mg daily for 5 days in a 21-day course. Toxicity was very mild and he was followed as an outpatient while he received 76 courses of this oral etoposide salvage chemotherapy. Seventy months after diagnosis, this patient is still alive and has no new distant metastases.
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ranking = 1.1428571428571
keywords = neuroblastoma
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