11/1473. Dysplastic gangliocytoma (Lhermitte-Duclos disease) associated with Cowden disease: report of a case and review of the literature for the genetic relationship between the two diseases. We report a case of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease, LDD). The patient also had cutaneous and mucosal hamartomas, adenomatous goiter, bilateral breast tumors, and gastrointestinal polyposis, indicating the diagnosis of Cowden disease (CD), the familial hamartoma syndrome. This was a rare sporadic case without any family history of CD, though CD is considered to be an autosomal dominant hereditary disease. Based on a thorough review of the previously reported cases, it is reasonable to consider that CD is inherited in autosomal dominant fashion through a CD gene (PTEN) containing a germline mutation, and that the occurrence of LDD is predicted on an additional somatic hit on the remaining normal CD allele or another unknown gene. ( info) |
12/1473. 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) |
13/1473. 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) |
14/1473. Hemorrhagic cerebellar metastasis from papillary thyroid carcinoma. Papillary thyroid carcinoma has a low incidence of distant metastases. Brain metastasis is extremely rare with a frequency of 0.1-1.3%. In the present series, the rate was 1.5%, only two cases had cerebral metastases in 136 patients with papillary thyroid carcinoma from January 1988 to April 1998. Cerebellar metastasis is even rarer, and solitary cerebellar metastasis has not been reported to the best of our knowledge. Two cases of papillary thyroid carcinoma with cerebellar metastases presenting as tumor hemorrhage are reported. In one patient, the lesion was in the bilateral cerebellar hemispheres with obstructive hydrocephalus. After operation, the patient had an uneventful course with recovery of her consciousness. In the other, the solitary lesion was in the left cerebellar hemisphere without obstructive hydrocephalus. After surgical treatment, the patient had a smooth course with resolution of his neurological deficit. It shows the importance of surgery in the management of a hemorrhagic cerebellar metastasis from papillary thyroid carcinoma, not only in reducing acute aggravating cerebral symptoms, but also in prolonging survival time. ( info) |
| We report an unusual case of cerebellar metastasis from a cervical adenosquamous carcinoma in which molecular techniques assisted in establishing the correct diagnosis. The patient was a 43-year-old woman with surgically unresectable cervical carcinoma diagnosed 2 years before presenting with neurological symptoms. A magnetic resonance imaging scan showed a large, enhancing cerebellar lesion with significant brain stem compression. The excised cerebellar tumor resembled a small cell carcinoma and was initially not thought to be a metastasis from the cervical adenosquamous carcinoma. in situ hybridization with catalyzed signal amplification and polymerase chain reactions with primers specific for human papilloma virus (HPV) types 16 and 18 were used to determine the relationship between the cervical and the cerebellar neoplasms. A positive signal was present in the nuclei of both neoplasms by in situ hybridization using HPV16/18 dna probes. polymerase chain reaction revealed the presence of HPV-18 DNA sequences in the cervical and cerebellar neoplasms confirming that the cerebellar neoplasm was a metastasis from the cervical primary. ( info) |
16/1473. 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 case of choroid plexus papilloma resembling meningioma of cerebellopontine (CP) angle with its extension to foramen magnum is presented. Occurrence of this tumour in CP angle is very rare. Its extension towards foramen magnum is further rare. It was a real diagnostic enigma preoperatively as the tumour was resembling meningioma upto some extent on radiological study. Retromastoid craniectomy with microsurgical excision of tumour and its extension was achieved in toto. Tumour was attached to few rootlets of lower cranial nerves which were preserved. Attachment of the tumour with lower cranial nerves again caused diagnostic confusion with neurofibroma intraoperatively. ( info) |
18/1473. Brain metastasis of Merkel cell carcinoma. Case report and review of the literature. Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine tumor that is locally aggressive and has potential for metastatic spread. However, brain metastases are rare, and therapy for such tumors has never reported. The authors present a 48-year-old woman with MCC of the left elbow and a right cerebellar metastasis. After the right cerebellar mass was totally resected, radiation treatment and chemotherapy were performed. Eight cases of brain metastasis have been reported in the literature, but only 5 have been presented in sufficient detail for analysis. Therapy for brain metastases has always been palliative whole-brain irradiation and chemotherapy except for our patient, who underwent total removal of the tumor and survived for 11 months without neurological deficit. Except in the case of 1 with a particularly radiosensitive MCC, the patients with brain metastases died within 9 months after detection of the brain lesions. If possible, aggressive excision of brain metastases as well as of the primary lesion should be done. ( info) |
19/1473. Magnetic resonance cisternography using the fast spin echo method for the evaluation of vestibular schwannoma. neuroimaging of vestibular schwannoma was performed with the fat-suppression spoiled gradient recalled acquisition in the steady state (SPGR) method and magnetic resonance (MR) cisternography, which is a fast spin echo method using a long echo train length, for the preoperative evaluation of the lateral extension of the tumor in the internal auditory canal, and the anatomical identification of the posterior semicircular canal and the nerves in the canal distal to the tumor. The SPGR method overestimated the lateral extension in eight cases, probably because of enhancement of the nerves adjacent to the tumor in the canal. The posterior semicircular canal could not be clearly identified, and the cranial nerves in the canal were shown only as a nerve bundle. In contrast, MR cisternography showed clear images of the lateral extension of the tumor and the facial and cochlear nerves adjacent to the tumor in the internal auditory canal. The anatomical location of the posterior semicircular canal was also clearly shown. These preoperative findings are very useful to plan the extent to which the internal auditory canal can be opened, and for intraoperative identification of the nerves in the canal. MR cisternography is less invasive since no contrast material or radiation is required, as with thin-slice high-resolution computed tomography (CT). MR cisternography should replace high-resolution CT for the preoperative neuroradiological evaluation of vestibular schwannoma. ( info) |
| A case of cerebellar hemangioblastoma with a coexistent arterial aneurysm on the feeding artery of the tumor is reported. The patient presented with an acute onset of headache, loss of consciousness, and left-sided hemiparesis due to a posterior fossa hemorrhage found adjacent to a hemangioblastoma. Four-vessel angiography revealed an aneurysm on the anterior inferior cerebellar artery (AICA), which was the main feeding vessel of the hemangioblastoma. Successful total excision of the hemangioblastoma and clipping of the AICA aneurysm achieved in a one-stage operation was demonstrated on postoperative angiography. ( info) |