1/226. Neuroblastoma metastatic to the liver in infants.Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/226. Adrenal neuroblastoma in an adult with tumor thrombus in the inferior vena cava.We describe the appearance of an adrenal neuroblastoma associated with tumor thrombus occurring in an adult patient. The tumor measured 14 x 12 x 12 cm, showed heterogeneous signal intensity on both T1-weighted and T2-weighted images, and displayed peripheral nodular enhancement on early post-gadolinium images. Tumor thrombus was identified within the inferior vena cava, which extended into the right atrium.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
3/226. prenatal diagnosis of adrenal neuroblastoma by ultrasound: a report of two cases and review of the literature.prenatal diagnosis of neuroblastoma is a rare event. Two cases diagnosed at 32 and 34 weeks are reported together with a description of the various ultrasound appearances of this tumor. Both cases had a favorable outcome and surgery was necessary in only one case. The management options are discussed in the light of the current literature. The role of ultrasound in the detection and follow-up of this tumor in the third trimester of pregnancy is discussed.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
4/226. 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.- - - - - - - - - - ranking = 4keywords = neuroblastoma (Clic here for more details about this article) |
5/226. Cystic neuroblastoma: emphasis on gene expression, morphology, and pathogenesis.Cystic neuroblastoma (CN) is an unusual variant of neuroblastoma characterized by a grossly visible cyst(s) and almost always distinctive microcysts on light microscopy. Rarely, CN will appear solid grossly, but microcystification will be present. We examined the clinical, pathologic, and biologic features of 17 cases of CN. The majority of CN had been detected by prenatal ultrasound. The tumors were favorable stage, stroma-poor, but with low or intermediate mitotic-karyorhectic indices and had favorable biologic markers reflected by aneuploidy and by an absence of N-myc amplification and chromosome 1p deletions. However, the high trk expression typically identified in good risk tumors was absent. Although the complete natural history of CN is not fully defined, our experience suggests that some tumors progress in size, whereas others may spontaneously regress or mature. The clinical outcome is excellent, as is expected in localized and stage 4S neuroblastoma in infancy.- - - - - - - - - - ranking = 3.5keywords = neuroblastoma (Clic here for more details about this article) |
6/226. Rapid reversal of dilated cardiomyopathy following removal of neuroblastoma.Reported is a child with dilated cardiomyopathy, in whom medical therapy resulted in a mild improvement of cardiac function. Metabolic studies suggested the presence of a catecholamine-secreting tumour; and an adrenal neuroblastoma was identified and surgically removed. Following surgery, there was progressive and complete normalization of cardiac function. Although very rare, neurogenic tumours may be involved in the development of a dilated cardiomyopathy in the infant and child.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
7/226. pheochromocytoma associated with neuroendocrine carcinoma. A new type of composite pheochromocytoma.The coexistence of pheochromocytoma and other tumor types in a single adrenal gland has been rarely documented. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether the pheochromocytoma and the nonpheochromocytoma components show the same embryologic origin. The nonpheochromocytoma components reported in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The components found in the mixed pheochromocytoma include adrenal cortical neoplasms and spindle cell sarcoma. We report a unique case of composite pheochromocytoma in which the nonpheochromocytoma element is a neuroendocrine carcinoma. The histologic and the immunohistochemical profiles of the 2 distinct components of this tumor were typical for those of pheochromocytoma and neuroendocrine carcinoma. This dual differentiation was also supported by ultrastructural findings. This case not only broadens the morphologic spectrum of composite pheochromocytoma but also provides some additional insight into the histogenesis of this rare but fascinating type of tumor.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
8/226. Metastatic neuroblastoma of the mandible mimicking osteogenic sarcoma radiologically. Case report.This paper presents a case of a neuroblastoma of the adrenal gland metastasizing to the mandible of a 21-month-old infant, which presented radiographically as the so-called 'sun-ray' appearance, characteristic of osteogenic sarcoma.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
9/226. 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.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
10/226. Possible association of retinoic acid with bone marrow transplant nephropathy.bone marrow transplant (BMT) nephropathy is characterized by the acute onset of nephritis more than 100 days after BMT. The renal lesion in BMT nephropathy is similar to radiation nephritis, but BMT nephropathy occurs earlier and with lower radiation doses than radiation nephritis. The combined effects of chemotherapeutic agents and nephrotoxic drugs given before and after BMT appear to sensitize or unmask radiation nephritis. Reporting of drugs that may contribute to BMT nephropathy is critical for the development of optimal treatment regimens. Herein, we report two cases of BMT nephropathy that developed coincident with retinoic acid therapy. Both patients received autologous BMT for neuroblastoma after preparative therapy with total body irradiation/melphalan/carboplatin/etoposide. They were randomized to receive cis-retinoic acid as part of a clinical trial. Both patients developed acute nephritis during their second 2-week course of retinoic acid on post-BMT days 105 and day 139. The nephritis was associated with hypertension, anemia, thrombocytopenia, azotemia, hematuria, and proteinuria. Clinical features, laboratory evaluation, and renal biopsy indicated that these two patients developed radiation-induced BMT nephropathy. The fact that both patients developed nephritis concurrent with retinoic acid therapy raises a concern that retinoic acid may have unmasked radiation injury and triggered BMT nephropathy.- - - - - - - - - - ranking = 0.5keywords = neuroblastoma (Clic here for more details about this article) |
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