1/7. ganglioneuroma : primary tumor or maturation of a suspected neuroblastoma?ganglioneuroma is a benign neurogenic tumor. These tumors are originating from neuroepithelium along sympathetic ganglia. Main localization is the mediastinum in children older than 10 years. An association with malignant neuroblastoma is rarely observed and it still remains a topic under current discussion. We describe the clinical course of a 17 year-old female patient with a large presacral mass causing amenorrhoea and weight loss. Eleven years before presentation, an incidental urine test showed an elevation of vanillylmandelic acid (VMA) and homovanillic acid (HMA) and a neuroblastoma was suspected. However, further investigations showed no tumor and the test results turned out to be normal within 1 year. Now, a malignant neurogenic tumor was again suspected, but a CT-guided biopsy revealed a benign tumor. The mass was originating from the left sacral nerve roots. A tumor resection via a dorsal approach was performed. Final histology showed a differentiated ganglioneuroma. This is, to our knowledge, the first report describing a patient with elevated VMA/HMA and suspected neuroblastoma who developed a ganglioneuroma 11 years later. The association of ganglioneuroma and neuroblastoma and the abnormal urine tests pointing toward a neuroblastoma 11 years ago remains unclear and the possible answers are discussed in our report.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
2/7. Glioneurofibroma: renaming the pediatric "gliofibroma": a neoplasm composed of schwann cells and astrocytes.Three children had central nervous system tumors with histologic and ultrastructural features corresponding to those of tumors previously described as "gliofibromas." These features, which include a composite appearance with glial and mesenchymal elements, with glial fibrillary acidic protein (GFAP)-containing and GFAP-immunonegative cells, diffuse S-100 immunoreactivity, and basal lamina wrapping processes of both cell types, suggest that the "mesenchymal" cells are schwann cells, not fibroblasts. We therefore propose to rename this entity "glioneurofibroma." The clinical behavior of these lesions is uncertain but is more often indolent or benign.- - - - - - - - - - ranking = 0.5keywords = acid (Clic here for more details about this article) |
3/7. Detection of occult CNS involvement of follicular small cleaved lymphoma by the polymerase chain reaction.A patient with follicular small cleaved lymphoma presented with an unusual clinical relapse in the central nervous system (CNS) without morphologic evidence of lymphoma cells in the cerebral spinal fluid (CSF). Molecular genetic analysis of the small number of cells in the CSF after in vitro dna amplification by the polymerase chain reaction demonstrated the presence of an abnormal translocation sequence between chromosomes 14 and 18. A similar translocation could be detected from the original fixed archival lymph node biopsy and from a small proportion of circulating mononuclear cells. These results indicated that occult lymphoma cells were present in the CSF and peripheral blood. Secondary CNS lymphoma involvement was identified at autopsy. This case demonstrates the enhanced sensitivity of lymphoma diagnosis from poorly cellular specimens after in vitro dna amplification.- - - - - - - - - - ranking = 6850.9671952612keywords = amplification (Clic here for more details about this article) |
4/7. The value of tumor markers in a mixed tumor, mucinous and neuroendocrine carcinoma of the ovary.serum CA125 is frequently used to monitor the treatment response and to detect early recurrence during the follow-up of patients who have epithelial ovarian tumors. In this report, the level of serum CA125 and urinary 5-hydroxyindole acetic acid of a patient with a carcinoid tumor which occurred in association with a mucinous adenocarcinoma of the ovary is described. The level of serum CA125 decreased despite the presence of a clinically progressive tumor. There was a rise of the 5-hydroxyindole acetic acid level 16 weeks before pelvic recurrence was detected clinically. This indicates that it was the neuroendocrine component, and not the mucinous component of the tumor, that was not responsive to the chemotherapy. The problem of using tumor markers to monitor patients with mixed tumors is discussed.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
5/7. "Vascular neurofibromatosis" and infantile gangrene.An 11-year-old boy with slowly progressive gangrene caused by vasculopathy similar to that of neurofibromatosis (NF) type 1 (NF I; von Recklinghausen disease [NFvR]) and a newborn girl with idiopathic gangrene with vascular changes resembling those of NFvR prompted the analysis of all 105 propositi with NF (NF I and NF II) evaluated between January 2, 1982, and December 31, 1986, at the genetics clinic of University of South florida. They were analyzed for renal hypertension, symptomatic ischemia, and known vascular changes. One additional 27-month-old boy with NFvR was found to have extensive vascular changes with renal hypertension. The vasculopathy indicated asymmetric over/undergrowth of cellular and extracellular components of the vascular wall and implied dysregulation of the paracrine growth mechanism. Immunocytochemical studies of affected vessels were done only in the 11-year-old boy and showed positive neuron-specific enolase, S-100 protein, and glial fibrillary acidic protein (GFAP) reactions indicative of Schwann cell involvement. The vascular changes in children with NFvR are mostly asymptomatic; however hypertension secondary to renal artery stenosis and/or Moya-moya disease have been reported infrequently. Our patients with vasculopathies provoked thoughts in regard to the so-called vascular NF, its place in current NF nomenclature and classification, relationship to fibromuscular dysplasia (FMD), and possible role in infantile gangrene.- - - - - - - - - - ranking = 0.5keywords = acid (Clic here for more details about this article) |
6/7. Primary melanocytic schwannoma of the stomach. Evidence of dual melanocytic and schwannian differentiation in an extra-axial site in a patient without neurofibromatosis.Pigmented neoplasms of the peripheral nervous system are uncommon. Such lesions, while well documented in the para-axial region, are infrequently encountered in more peripheral extra-axial sites. The authors present a case of a melanocytic schwannoma arising in the gastric antrum of a 51-year-old woman. Ultrastructural features attesting to the nerve sheath origin of the neoplasm included the presence of a well-developed basal lamina, complex infoldings of the plasma membrane, and intercellular bundles of long spaced collagen ("Luse bodies"). Premelanosomes and melanosomes in all stages of development were identified within the cytoplasm of many of the neoplastic cells, providing morphologic support for a close relationship between schwann cells and melanocytes. Immunoperoxidase studies with S-100 protein disclosed intense cytoplasmic staining throughout the tumor. The S-100 protein antigen has been documented in both nerve sheath elements and melanocytes; this shared antigenicity lends further support to the close relationship between melanocytic and schwannian elements suggested by ultrastructural observations. Additional immunoperoxidase studies employing antibody to glial fibrillary acidic protein revealed focal cytoplasmic staining, an uncommon phenomenon in peripheral nerve sheath neoplasms. A review of the literature failed to provide any previous well documented cases of melanocytic schwannoma of the stomach. The patient was treated with en bloc resection of the tumor and gastric antrum, and was free of evidence of recurrent or metastatic disease 22 months postoperatively. Theories regarding the histogenesis of pigmented nerve sheath neoplasms and prognostic implications are discussed, based on experience with this and previously reported pigmented nerve sheath tumors.- - - - - - - - - - ranking = 0.5keywords = acid (Clic here for more details about this article) |
7/7. Amplification of N-myc gene and increase of urinary VMA and HVA in patients with neuroblastic tumors.Neuroblastic tumor cases in our departments were evaluated in terms of the stage of the tumor, N-myc amplification, urinary vanillylamndelic acid (VMA)/homovanillic acid (HVA) and survival rate. Two asymptomatic cases, diagnosed when under a year old by mass screening, had no amplifications of N-myc but showed more than one value of urinary VMA/HVA ratio. The patients are now doing well eight years after complete excision of the neuroblastoma which had originated in the sympathetic ganglion. On the other hand, two other symptomatic cases, operated on at the ages of 3 and 5 years, showed remarkable amplifications with less than one value of urinary VMA/HVA, and died from the tumor soon after partial resection of the neuroblastoma and ganglioneuroblastoma which had originated in the adrenal gland and the sympathetic ganglion, respectively. The present monograph reports our cases and discusses prognostic factors.- - - - - - - - - - ranking = 10277.450792892keywords = amplification, acid (Clic here for more details about this article) |