Hyperdiploid Precursor B-acute Lymphoblastic Leukemia Presenting as a Cavernous Sinus Mass in a 4-Year-old Male.

Risk stratification and appropriate treatment selection for children with precursor B-acute lymphoblastic leukemia (B-ALL) have improved outcomes. We report the case of a 4-year-old male with a lymphomatous cavernous sinus mass, a previously undescribed presentation of newly diagnosed hyperdiploid B-ALL. Few case reports in the literature describe lymphomatous involvement in this region, but none are associated with pediatric B-ALL. This case presented unique treatment and risk assignment challenges given the intracranial location of this tumor and proximity to the central nervous system.

Nasopharyngeal carcinoma presenting as a sixth nerve palsy and Horner's syndrome.

The combination of a sixth nerve palsy and ipsilateral Horner's syndrome localises the disease process to the posterior cavernous sinus and can be a result of various pathologies in this region. A 74-year-old Chinese woman presented with a 9-month history of binocular horizontal diplopia worse when looking left. She was found to have a left sixth nerve palsy and Horner's syndrome and MRI revealed an enhancing soft tissue mass in the nasopharynx with involvement of the bones of the skull base and invasion of the left cavernous sinus. Endoscopic biopsy of the mass confirmed the diagnosis of non-keratinising squamous cell carcinoma, which was Epstein-Barr virus positive. She was treated with radiation therapy. Patients with a sixth nerve palsy and ipsilateral Horner's syndrome should have urgent neuroimaging with careful attention to the cavernous sinus since sympathetic fibres join the sixth nerve for a short distance in this location.

A Propensity Score-Matched Cohort Analysis of Outcomes After Stereotactic Radiosurgery in Older versus Younger Patients with Dural Arteriovenous Fistula: An International Multicenter Study.

OBJECTIVE: This study aims to evaluate the outcomes of Gamma Knife stereotactic radiosurgery (SRS) for dural arteriovenous fistulas (dAVFs) in older patients (≥65 years) compared with younger patients (age <65 years). METHODS: Two groups with a total of 96 patients were selected from a database of 133 patients with dAVF from 9 international medical centers with a minimum 6 months follow-up. A 1:2 propensity matching was performed by nearest-neighbor matching criteria based on sex, Borden grade, maximum radiation dose given, and location. The older cohort consisted of 32 patients and the younger cohort consisted of 64 patients. The mean overall follow-up in the combined cohort was 42.4 months (range, 6-210 months). RESULTS: In the older cohort, a transverse sinus location was found to significantly predict dAVF obliteration (P = 0.01). The post-SRS actuarial 3-year and 5-year obliteration rates were 47.7% and 78%, respectively. There were no cases of post-SRS hemorrhage. In the younger cohort, the cavernous sinus location was found to significantly predict obliteration (P = 0.005). The 3-year and 5-year actuarial obliteration rates were 56% and 70%, respectively. Five patients (7.8%) hemorrhaged after SRS. Margin dose ≥25 Gy was predictive of unfavorable outcome. The obliteration rate (P = 0.3), post-SRS hemorrhage rate (P = 0.16), and persistent symptoms after SRS (P = 0.83) were not statistically different between the 2 groups. CONCLUSIONS: SRS achieves obliteration in most older patients with dAVF, with an acceptable rate of complication. There was no increased risk of postradiosurgery complications in the older cohort compared with the younger patients.

Pathological considerations to irradiation of cavernous malformations.

Stereotactic radiosurgery is a controversial treatment modality in the management of cerebral cavernous malformations (CVMs). Systematic pathological studies of irradiated specimens probably could help to resolve the controversy. Light microscopic investigation of a surgically resected thalamic CVM 1 year after 40-Gy irradiation revealed endothelial cell destruction in the cavernous channels, and marked fibrosis with scar tissue formation in the connective stroma of the lesion. These histopathological findings were similar to those described in arteriovenous malformations after Gamma Knife surgery, and suggest that the ionizing effect of radiation energy evokes vascular and connective tissue stroma changes in CVMs as well.

Adenoid cystic carcinoma of the cavernous region. Case report.

A 62-year-old Japanese male patient presented with right oculomotor, abducens, and trigeminal nerve disturbances. Neuroimaging revealed a right middle cranial fossa mass lesion with the characteristics of trigeminal schwannoma. The patient underwent a right temporal craniotomy for gross total resection. Histological examination confirmed a metastasis of adenoid cystic carcinoma (ACC). After the operation, no evidence of the primary lesion could be found. The patient was treated with radiation therapy. ACC can be difficult to identify before histological confirmation. The incidence of intracranial invasion of ACC is 4-22%, but in this case no evidence of the primary lesion was found.

Multidisciplinary treatment of cavernous sinus dural arteriovenous fistulae with radiosurgery and embolization.

OBJECTIVE: With the advent of interventional neuroradiology and stereotactic radiosurgery, dural arteriovenous fistulae are less often managed with open surgery. We evaluated the outcome of dural arteriovenous fistulae of the cavernous sinus treated with a combination of radiosurgery and embolization. MATERIAL AND METHODS: Twenty dural arteriovenous fistulae located in the cavernous sinus were enrolled in our study. Fifteen patients received X-knife radiosurgery alone and 5 also required embolization, one before radiosurgery and 4 after radiosurgery. The mean volume of the lesions was 2.8 ml (range 0.2-12.6), the corresponding radiation volume was 6.5 ml (range 0.6-24.6), and the conformity index was 2.9 (range 1.8-5.3). The mean peripheral and maximum radiation dose was 17.8 Gy (range 17-20) and 28.3 Gy (range 19-37) Gy, respectively. The clinical and imaging data were analyzed. RESULTS: The mean follow up period was 29 months (23-39). Seventy-five percent (15/20) of patients receiving radiosurgery alone achieved a symptomatic cure and with additional embolization 90% (18/20) were cured. All patients achieved cure on imaging after radiosurgery alone or in combination with embolization. Abnormal imaging findings were observed in two patients after treatment, one had an intracerebral hemorrhage and the other radiation edema, but both were asymptomatic. CONCLUSION: With multidisciplinary treatment with combined radiosurgery and embolization, satisfactory results can be achieved for dural arteriovenous fistulae with a low complication rate. In patients with mild symptoms, radiosurgery is the initial treatment option. Embolization should be performed in patients with severe symptoms or who have failed radiosurgery.

Gamma knife radiosurgery in skull base meningiomas: a possible relationship between somatostatin receptor decrease and early neurological improvement without tumour shrinkage at short-term imaging follow-up.

BACKGROUND: This study investigates a possible relationship between the effects of gamma knife (GK) on meningioma somatostatin receptors (SRs) and the high rate of early neurological improvement without tumour reduction at short-term imaging follow-up. METHODS: From December 1997 to December 2002, somatostatin receptor scintigraphy (SRS) using an 111Indium-labelled somatostatin analogue, Octreotide, was performed both before and 7-12 months after radiosurgery in 20 patients with intracranial meningiomas. Semiquantitative data were calculated as an SRS index. FINDINGS: The pre-GK SRS index was always > 1, averaging 4.44 +/- 3.20. There were no statistically significant differences between the pre-GK average values of primary (4.80 +/- 3.65) and residual (3.75 +/- 1.93) meningiomas. At the first clinical/MRI follow-up, the neurological examination had improved in 15/20 (75%) and had not changed in 5/20 patients. A corresponding slight tumour shrinkage on high-resolution MRI was documented in 3/20 cases only. The post-GK average SRS index was lower than pre-GK values both in primary (3.87 +/- 3.19) and in adjuvant (2.52 +/- 1.14) treatments, but the differences were not significant. However, the subgroup of patients with early neurological improvement showed a higher pre-GK average SRS index (5.21 +/- 3.33) and a more substantial post-GK average SRS index decrease (3.86 +/- 3.00) than the patients whose clinical condition remained stable (2.10 +/- 0.59 and 1.99 +/- 0.55, respectively). The difference between the two subgroups of patients proved to be statistically significant (P < 0.05). CONCLUSIONS: Our preliminary findings suggest a possible relationship between a decrease in the concentration of SRs on meningioma cells at short-term functional imaging follow-up after radiosurgery and early neurological improvement.

Capillary hemangioma of the cavernous sinus. Report of two cases.

Capillary hemangiomas are rare benign vascular tumors that tend to occur in children. Whereas the majority of hemangiomas may regress spontaneously, those associated with functional sequelae or severe symptoms may require treatment. Two patients with capillary hemangiomas of the cavernous sinus that caused neurological symptoms were treated with fractionated stereotactic radiation therapy. Both hemangiomas had shown a progressive increase in size during observation before radiation therapy; both tumors regressed after radiotherapy. Up to the time of the last follow-up evaluation both patients experienced symptomatic relief after radiation. One patient's tumor remains in complete remission and the second tumor continues to demonstrate minor residual contrast enhancement without progression. The authors conclude that fractionated stereotactic radiation therapy is a useful treatment modality in the management of symptomatic capillary hemangiomas when these tumors arise in regions of the brain or skull base in which a complete resection cannot be accomplished.

Radiosurgery of angiographically occult vascular malformations.

Angiographically occult vascular lesions (AVOMs) are vascular lesions not visualized on standard cerebral angiography. The possibility of managing these difficult lesions with radiosurgery is discussed. The radiosurgical treatments and results of AVOMs are described and the strategies for managing AOVMs and cavernous malformations are discussed.

Dose-response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery.

UNLABELLED: As the number of patients treated with stereotactic radiosurgery increases, it becomes particularly important to define with precision adverse effects on distinct structures of the nervous system. OBJECT: This study was designed to assess the dose-response tolerance of the visual pathways and cranial nerves after exposure of the cavernous sinus to radiation. METHODS: A total of 66 sites in the visual system and 210 cranial nerves of the middle cranial fossa were investigated in 50 patients who had undergone gamma knife treatment for benign skull base tumors. The mean follow-up period was 40 months (range 24-60 months). Follow-up examinations consisted of neurological, neuroradiological, and neuroophthalmological evaluations. The actuarial incidence of optic neuropathy was zero for patients who received a radiation dose of less than 10 Gy, 26.7% for patients receiving a dose in the range of 10 to less than 15 Gy, and 77.8% for those who received doses of 15 Gy or more (p < 0.0001). Previously impaired vision improved in 25.8% and was unchanged in 51.5% of patients. No sign of neuropathy was seen in patients whose cranial nerves of the cavernous sinus received radiation doses of between 5 and 30 Gy. Because tumor control appeared to have been achieved in 98% of the patients, the deterioration in visual function cannot be attributed to tumor progression. CONCLUSIONS: The structures of the visual pathways (the optic nerve, chiasm, and tract) exhibit a much higher sensitivity to single-fraction radiation than other cranial nerves, and their particular dose-response characteristics can be defined. In contrast, the oculomotor and trigeminal nerves have a much higher dose tolerance.

Formation of intracerebral cavernous malformations after radiation treatment for central nervous system neoplasia in children.

OBJECT: Radiation is a common treatment modality for pediatric brain tumors. The authors present a retrospective review of six children who developed cerebral cavernous malformations after they underwent radiation treatment for central nervous system (CNS) neoplasia and propose two possible models to explain the formation of cavernous malformations. METHODS: Three boys, aged 13, 9, and 17 years, suffered intracerebral hemorrhages from cerebral cavernous malformations 87, 94, and 120 months, respectively, after they received whole-brain radiation therapy (WBRT) for acute lymphocytic leukemia. A 10-year-old girl and a 19-year-old man developed temporal lobe cavernous malformations 46 and 48 months, respectively, after they received radiation therapy for posterior fossa astrocytomas. A 12-year-old girl developed a temporal lobe cavernous malformation 45 months after WBRT was administered for a medulloblastoma. In all of these cases the cavernous malformation appeared in the irradiated field, was not known to be present prior to radiation therapy, and developed after a latency period following treatment. The incidence of cavernous malformations in these patients suggests that children who undergo radiation therapy of the brain may have an increased risk of hemorrhage. CONCLUSIONS: Two possible models may explain the formation of cavernous malformations following brain radiation in these patients. First, the cavernous malformations may form de novo in response to the radiation. Second, the cavernous malformations may have been present, but radiographically occult, at the time of radiation therapy and may have hemorrhaged in response to the radiation. The authors conclude that cavernous malformations may develop after brain radiation and propose a possible mechanism for this formation.

Acute bilateral ophthalmoplegia secondary to metastatic prostatic carcinoma. Demonstration on magnetic resonance imaging.

We report an unusual case of acute bilateral ophthalmoplegia secondary to bilateral cavernous sinus metastasis from prostatic adenocarcinoma. The lesion was demonstrated with magnetic resonance imaging.

Enhanced photorelaxation in aorta, pulmonary artery and corpus cavernosum produced by BAY K 8644 or N-nitro-L-arginine.

Segments of endothelium-denuded aorta, pulmonary arterial rings and strips of corpus cavernosum from rabbits were superfused with Krebs medium. Photorelaxation elicited by ultraviolet light (366 nm) was significantly enhanced by either BAY K 8644 (20 nM) or N-nitro-L-arginine (100 and 500 microM) and was associated with increased cyclic GMP. This action of both drugs was greater in pulmonary artery than aorta and corpus cavernosum and persisted in vascular rings for 90 min after drug removal. The effect was significantly attenuated by hemoglobin (10 microM) but was unaltered by superoxide dismutase (30 u/ml). The mechanism of such photosensitization is presently unclear.

[Effect of proton irradiation of Tolosa-Hunt syndrome]. / Effekt protonnogo oblucheniia pri sindrome Tolosy-Khanta.

The authors have analysed a case of Tolosa-Hunt syndrome with a persistent recurring course and a gradual development of resistance to steroid therapy. Computerized tomography revealed a high density zone in the affected cavernous sinus of the patient. Differential diagnosis between a tumor and Tolosa-Hunt syndrome was made. The patient received 3 fractions of proton beam irradiation by the "piercing" method (the beam being 15 mm in diameter) at a dose of 60 Gy. After irradiation computerized tomography showed complete recovery of disturbed functions and disappearance of the focus. Four-year remission was observed. In our experience, it was the first case of the use of radiation therapy in Tolosa-Hunt syndrome.