Matching of adjacent radiation beams for isocentric qadiotherapy.
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The case of a 25-year-old primipara in the second trimester of pregnancy, suffering from a peripheral primitive neuroectodermal tumour (pPNET) diagnosed by bone biopsy, is described. External irradiation was initially performed because of Jacksonian seizures due to a lesion in the right cerebral hemisphere. Appropriate shielding was used to reduce fetal exposure during brain radiotherapy. Caesarian delivery at the 27th week of gestation was performed because of tumour progression. The neonate had no evidence of disease and survived for 1 month. However, the placenta and ovaries showed metastases from the maternal pPNET. The patient died 14 months after initial diagnosis owing to the aggressiveness of the tumour, the rapid and extensive semination (bone marrow, lung, liver, craniospinal axis involvement) and the inability to adequately treat the patient with appropriate doses of chemotherapy.
Craniospinal irradiation is technically demanding due to the complex shape of the planning target volume (PTV). Radiotherapy treatment techniques have evolved over time as imaging and radiotherapy treatment technology have improved. However, most are variations on a class solution utilizing a prone patient position with two shaped lateral cranial portals and a matched posterior spinal portal with moving junctions. Major areas of difficulty remain with the accurate definition of the PTV and achieving a homogeneous dose within it, especially at the junctions. We describe a three-dimensionally (3D) planned craniospinal radiation technique that permits rapid image acquisition with reduced localization time, simplified spinal PTV definition and standardized cranial PTV definition. Improved dose homogeneity within the PTV is achieved by use of a segmented "field-in-field" technique (forward planned intensity-modulated radiotherapy (IMRT)) in place of customized compensators. This has negated the requirement for constructing physical compensators. Autosequencing for field delivery enables the junction to be "moved" during a single fraction and reduces the overall treatment time, an important consideration when treating very young patients.
There is agreement that the morbidity and mortality of CPA tumors are directly proportional to the size of the tumor. The diagnostic difficulty, however, is indirectly proportional to the size: the large tumors are diagnosed with little difficulty, the smaller ones with considerable challenge. In the search for these tumors, it is apparent that the incidence of all sizes is low. To arrive at the diagnosis, efforts must be pursued in any patient with unilateral sensorineural hearing loss, unilateral tinnitus, or vertigo. In the past 12 years, 19,000 new patients were seen in the office of the Pittsburgh Otological Association. In this new patient population, 120 tumors were proven by surgery. Six others (bilateral tumors) were diagnosed but not operated on, and four others were diagnosed but were operated on elsewhere for a total of 130 patients. An estimate of the cost of diagnosing these tumors is presented. Consideration is given to the cost-benefit ratio of diagnosing those diseases which must be considered also in the differential diagnosis when the above symptoms are present. These are: congenital changes, trauma, metabolic neuropathies, Meniere's disease, noise-induced hearing loss, lues, sudden hearing loss, and unilateral symptoms of undetermined etiology.
We present the results of the examination of prognostic markers in 40 children suffering from brain tumors. Prognostic markers such as amplification of the N-myc and c-myc, deletion of the 17p, and DNA ploidy are indispensable factors for the determination of diagnosis. An increased number of c-myc gene copies was found in malignant brain tumors, especially embryonal, more often than reported in the literature. N-myc amplification occurs in our group seldom, but it seems to be a sign of worse prognosis in glial and embryonal brain tumors. DNA aneuploidy was not found very frequently, but in high-grade tumors only.
We assessed the efficacy of GH treatment in 25 GH deficient patients irradiated for brain tumors (eight with glioma cranio-irradiated, eleven with medulloblastoma and six with ependymoma craniospinal-irradiated). We administered GH at doses of 0.6-0.9 IU/kg/week for one to three years at least two years after diagnosis of the tumor. We assessed the efficacy of the treatment each year by comparing the values of height velocity over bone age and change in the ratios progression of chronological age/progression of bone age and progression of statural age/progression of bone age. The treatment promoted satisfactory growth; better results were obtained in patients with glioma, who received cranial irradiation only, than in those with medulloblastoma or ependymoma, who received spinal irradiation as well. Moreover, the growth prognosis improved, especially in the cranio-irradiated patients. In our series of patients four presented tumor recurrence; these results did not differ significantly from those in irradiated patients with cerebral tumors who were not treated with GH.
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A case of metastasis of breast adenocarcinoma into the internal auditory canal (IAC) and cerebellopontine angle (CPA) is presented, which appeared 16 years after primary tumor had been treated by surgery and radiation therapy. The 66-year old patient was considered cured from the primary disease, when she started with a rapidly developing hearing loss and intermittent facial palsy. Magnetic resonance image (MRI) displayed an intra- and extracanalicular tumor mass, which radiologically resembled a vestibular schwannoma. Surgery was performed and histopathological studies showed an adenocarcinoma compatible with breast origin. Metastasis is a rare occurrence within the IAC and CPA. Clinical history of severe facial palsy will rise suspicion of malignant tumor in spite of the radiological findings.
A case of primary ocular and central nervous system malignant lymphoma was reported which was first manifested as bilateral uveitis ten months before the neurological symptoms appeared. The lesion of lymphoma in the central nervous system was clearly demonstrated as a heavy accumulation of N-isopropyl-123I-p-iodoamphetamine by single photon emission computed tomography. Since most brain tumors reportedly show a low uptake of N-isopropyl-123I-p-iodoamphetamine, single photon emission computed tomography with N-isopropyl-123I-p-iodoamphetamine could be helpful in making the diagnosis, as well as in visualizing primary central nervous system malignant lymphoma.
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A 15-year-old male was admitted because of diplopia and persistent headache. Postcontrast computed tomography (CT) revealed a homogeneously enhanced large mass, 3 x 4 cm in size, in the pineal region and moderate obstructive hydrocephalus. A right ventriculoperitoneal shunt was installed. At that time, the serum alpha-fetoprotein (AFP) level increased to 23,036 ng/ml, but the level of serum beta-subunit of human chorionic gonadotropin was less than 0.2 ng/ml. These data indicated the tumor to be a pure yolk sac tumor. Following cisplatin-vinblastine-bleomycin (PVB) therapy and whole-brain irradiation (50 Gy), the tumor disappeared on CT, although the AFP level did not return to normal. Eight months after the completion of initial therapy, he had lumbago. Spinal magnetic resonance imaging revealed a metastatic mass at the L5-S2 levels, which was subtotally removed and histologically diagnosed as yolk sac tumor. Postoperative local irradiation (30 Gy) was performed. Seven months after the operation, spinal dissemination at the Th7 level occurred and, 1 month later, intracranial dissemination in the left cerebellopontine angle was detected. He died 25 months after the first admission. PVB therapy did not prevent spinal dissemination in this case.
We report the rare association of a meningioma and an ependymoma in a young boy. A meningioma was detected in the left cerebellopontine angle at age 8 months, and an ependymoma in the left frontal lobe 2 years later. Chromosomal analysis was normal, and his family showed no signs of phacomatosis. Both tumors were totally removed, and he recovered well after both operations.
A cavernous angioma of the cerebellum occurred in a 55-year-old female presenting with a 3-year history of slowly progressive cerebellar signs and symptoms. Computed tomography and magnetic resonance imaging revealed a lesion in the left cerebellar nuclei and atrophic change of the affected cerebellar hemisphere. The final diagnosis was made at operation. The cerebellar nuclei and white matter were probably affected by the slowly expanding lesion, with repeated hemorrhage leading to degeneration of the afferent and efferent fibers, and subsequent atrophy of the affected cerebellar hemisphere.
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