1/495. Third ventricular lesion masquerading as suprasellar disease. We discuss the case of a patient who presented with a bitemporal visual field disturbance thought to arise from chiasmatic compression secondary to a suprasellar mass. The patient was ultimately diagnosed with medulloblastoma with diffuse intraventricular disease. Careful review of magnetic resonance (MR) findings in this case demonstrate the apparent suprasellar mass to be within the suprachiasmatic recess of the third ventricle. The role of MR imaging in distinguishing between suprasellar disease involving the third ventricle and primary third ventricular lesions is discussed. ( info) |
2/495. Cicatricial fibromatosis mimics metastatic medulloblastoma. Cicatricial fibromatoses usually occur in the anterior abdominal wall or in the extremities, but rarely in the scalp or the soft tissues of the neck. We report a case of desmoid fibromatosis that developed in a 15-year-old boy 8 months after surgery for cerebellar medulloblastoma. ( info) |
3/495. Generalized osteoblastic bony metastases from medulloblastoma. Osteoblastic bony metastases were observed in a case of medulloblastoma three years after surgery and radiation treatment. There was clinical response to COP therapy (cytoxin, oncovin, prednisone). Radiographic and isotopic bone scan study showed uniform increase in bone density. serum calcium and phosphorous and acid phosphatase levels were normal. alkaline phosphatase was elevated and declined with therapy without change in the bone appearance. This suggested an increase in bone deposition activity. However, no increase in calcitonin level was detected either during active disease or following a chemotherapy-induced remission. ( info) |
4/495. Unexpected stomach uptake of technetium-99m-MDP. Two pediatric cases are described in which the results of each patient's bone scan demonstrated abnormal stomach uptake. There have been a number of reports in the literature describing stomach uptake of bone agents, however, it is an uncommon finding. ( info) |
5/495. Effects of puberty on bone age maturation in a girl after medulloblastoma therapy. BACKGROUND: Craniospinal radiotherapy for malignant brain tumors can result in a variety of neuroendocrine disturbances, among which are the development of growth hormone deficiency and early puberty, which can markedly reduce adult height. methods: The authors report the case of a girl who received craniospinal radiotherapy for a medulloblastoma at the age of 3.4 years. At 9.1 years, growth hormone therapy was started, and spontaneous onset of puberty (Tanner stage B2) occurred at age 10.3 years. Interval until menarche was short, at only 0.9 years. RESULTS: Although chronologic age at appearance of Tanner stages was within the normal range, the patient showed a rapid acceleration in skeletal maturation, resulting in adult short stature. CONCLUSION: Bone age seems to be a more precise parameter for biologic maturation in some patients after craniospinal irradiation than is clinical assessment of pubertal stages. Thus, if progression of bone age and decreasing final height predictions are noted, puberty should be stopped with gonadotropin-releasing hormone analogs, even if pubertal development seems to be adequate for chronologic age, because this increases the remaining time for growth hormone treatment. ( info) |
6/495. Medullomyoblastoma: A case report. Medullomyoblastoma is a rare tumour seen in childhood. We report a medullomyoblastoma occurring in the cerebellar vermis of a 4 year old boy. The light microscopic features, immunohistochemistry and histogenesis are described. ( info) |
| A 12-year-old boy presented with the classic CT and MRI findings of medulloblastoma and the unusual finding of increased signal on diffusion MRI. The small-cell histology of medulloblastoma may account for the increased signal seen on diffusion MRI. diffusion MRI with echoplanar technique may be useful in evaluation of these tumors and metastatic disease. ( info) |
| This report and the accompanying review of the literature address the challenges, when using surveillance magnetic resonance (MR) imaging, of establishing the origin of newly detected central nervous system lesions. Routine surveillance MR imaging in a 16-year-old boy, whose medulloblastoma had been successfully treated, demonstrated asymptomatic nodular leptomeningeal enhancement of the brain and spinal cord, which was consistent with recurrent disease. Examination of the cerebrospinal fluid, however, led to the diagnosis of bacterial meningitis. Two weeks after completion of antibiotic therapy, the original MR imaging findings were seen to have resolved. This case illustrates the importance of considering clinical and laboratory data, including results from a complete examination of the cerebrospinal fluid, when interpreting the origin of new lesions revealed by MR imaging. ( info) |
9/495. Secondary supratentorial anaplastic astrocytoma following treatment of medulloblastoma. The development of secondary tumours is a rare but well known late effect of radiation therapy of lesions in the central nervous system. Most radiation-induced tumours are of mesenchymal origin, but on rare occasions gliomas can occur. We describe a patient in whom a supratentorial anaplastic astrocytoma developed 15 years after surgery and radiotherapy for a childhood posterior fossa medulloblastoma. A concise review of the pertinent literature is given. ( info) |
| li-fraumeni syndrome is an autosomal dominant disorder that is characterized by various types of cancer in childhood and adult cases. Although hereditary TP53 mutation is very rare in different human cancers, it has been frequently reported in li-fraumeni syndrome. On the other hand, hereditary mutations of TP57KIP2, P15INK4B, and P16INK4A, which affect the cell cycle similar to TP53, were observed in some types of cancer. In a Turkish family with the diagnosis of li-fraumeni syndrome, we analyzed the mutation pattern of TP53, P57KIP2, P15INK4B, and P16INK4A in the peripheral blood, and loss of heterozygosity (homo/hemizygous deletion) pattern of TP53 and P15INK4B/P16INK4A in two tumor tissues. The propositus had a seminoma, his daughter a medulloblastoma, and one of his healthy cousins, a TP53 codon 292 missense point mutation (AAA-->ATA; Lys-->Ile) in the peripheral blood cells. Tumor tissue obtained from the propositus with the seminoma revealed loss of heterozygosity in the TP53 gene. In the analyses of tumor tissues from the propositus and his daughter, a P16INK4A codon 94 missense point mutation (GCG-->GAG; Ala-->Glu) was observed with the hereditary TP53 mutation. P16INK4A codon 94 mutation observed in our family is a novel mutation in li-fraumeni syndrome. No other gene alteration in TP53, P57KIP2, P15INK4B, and P16INK4A was observed. Existence of the P16INK4A mutation and the hereditary TP53 mutation with or without loss of heterozygosity in the TP53 gene (seminoma/medulloblastoma) may be evidence for a common mechanism involved in tumorogenesis. The gene alterations in TP53 and P16INK4A genes may be used as tumor markers in our family. ( info) |