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Tadanori Tomita and Paolo Frassanito

Object

The superior medullary velum (SMV) is a thin lamina of white matter located between the superior cerebellar peduncles horizontally and between the midbrain and cerebellum vertically. The SMV has not previously been described as the primary location of a posterior fossa tumor, although it can be secondarily invaded by a tumor from the cerebellum or quadrigeminal plate. This paper aims to define clinical and radiological features of tumors primarily arising from the SMV during childhood.

Methods

The authors observed 6 infants and children harboring neoplasms of the SMV who were treated at Ann & Robert Lurie Children's Hospital of Chicago (formerly Children's Memorial Hospital) in Chicago, Illinois. Pathological diagnosis of the neoplasms was an atypical teratoid/rhabdoid tumor (ATRT) in 5 patients, and a juvenile pilocytic astrocytoma (JPA) in the remaining child. The tumors were diagnosed during infancy in all patients, with ages ranging from 3 months to 10 months, except for the patient with a JPA (diagnosed at 5 years old). All patients presented with signs and symptoms of increased intracranial pressure due to obstructive hydrocephalus.

Results

Characteristic MRI features were noted, consistent with a mass in both the fourth ventricle and the cerebellomesencephalic fissure and quadrigeminal cistern, resulting in the circumferential displacement of the neural structures surrounding the SMV. The tumor was removed effectively in gross-total fashion through the occipital transtentorial approach in all patients. This approach offers a wide exposure of the region. However, all infants with ATRT suffered tumor dissemination and died between 4 and 11 months after diagnosis, in spite of radical resection and oncological treatment. The 1 child with JPA is alive and well 30 months after tumor resection.

Conclusions

To the best of the authors' knowledge, this is the first description in the literature that focuses on tumors originating from the SMV. This entity must be promptly recognized on preoperative radiological studies to carefully plan the subsequent surgical and clinical management.

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Hydrocephalus and infratentorial tumors

Incidence, clinical picture, and treatment

Anthony J. Raimondi and Tadanori Tomita

✓ One may consider that the child with hydrocephalus complicating a primary brain tumor has two distinctly different diseases: 1) a neoplasm, and 2) hydrocephalus. The hydrocephalus may be obstructive, communicating, or (in the case of choroid plexus papilloma) hypersecretory. This paper presents the incidence, symptomatology, and management of hydrocephalus associated with infratentorial brain tumors in 156 children and with pineal tumors in 21 children, all treated by the first author during the period 1967 to 1979. Medulloblastoma was the most commonly encountered tumor. Of 117 children with cerebellar-fourth ventricle tumors, 110 had hydrocephalus, whereas 11 of 34 with brain-stem tumors and all 21 with pineal tumors presented with hydrocephalus. Of the total 143 patients with hydrocephalus, shunts were inserted before craniotomy in 123, with the interval between shunt insertion and craniotomy for tumor removal ranging from 1 to 21 days. Eighteen of these children required shunt revisions after craniotomy; 36 of 80 patients followed for 6 months or more had their shunts permanently removed, but it was necessary to reinsert the shunt from 5 days to 13 months later in 30% of these patients. Only 25 patients remained permanently shunt-free. It is considered that the placement of shunts before craniotomy is justified by the extraordinarily high incidence of papilledema and visual impairment associated with hydrocephalus. “Upward herniation” and dissemination of neoplastic cells through the shunting system are theoretical contraindications to precraniotomy shunt insertion, although the former is rare (3%), and there is no statistical evidence to support the latter.

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Tadanori Tomita and David G. McLone

✓ This review concerns 22 children who were treated from 1980 through 1983 for medulloblastoma in the posterior fossa. Treatment included attempts at radical resection of the tumor and postoperative craniospinal radiation therapy, with 5000 to 5500 rads directed to the posterior fossa and 2500 rads to the remaining craniospinal axis. This lower radiation dose to the neuraxis was used to avoid late adverse effects upon the growing central nervous system of the children. Gross confirmation of total resection was obtained in 13 patients (the “total resection group”); however, nine patients had a subtotal resection leaving a small portion of the tumor extending into the cerebellar peduncles or the cerebellopontine angle, or else encasing the posterior inferior cerebellar artery (the “subtotal resection group”). Six patients in the total resection group demonstrated tumor extension into the cerebellar peduncles, which was removed by means of a surgical carbon dioxide laser without neurological sequelae. Biopsy of the arachnoid membrane from the cisterna magna and cytological examination of the cerebrospinal fluid (CSF) prior to manipulation of the tumor werecarried out in 12 patients. All but one showed dissemination of medulloblastoma cells. Myelography and CSF cytological study were undertaken 2 months after radiation therapy in 12 patients and were positive in two. There were no case mortalities in the total resection group during the 24- to 67-month follow-up period, whereas the 1-year survival rate in the “subtotal resection group” was only 44.4%. This study suggests that medulloblastoma can be controlled with a low radiation dose to the neuraxis, should a grossly confirmed total resection be achieved at craniotomy.

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Tadanori Tomita, David G. McLone and Thomas P. Naidich

✓ A mycotic aneurysm of the intracavernous portion of the carotid artery produced total ophthalmoplegia in a 2-year-old boy. Serial angiography displayed progressive enlargement of the aneurysm which was successfully treated by carotid ligation. Angiography is mandatory to rule out carotid artery complications secondary to cavernous sinus thrombophlebitis.

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Adrian Caceres, Aaron J. Reitman and Tadanori Tomita

✓Craniopharyngioma is a common sellar region tumor occurring in children. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. Patients with craniopharyngioma commonly present with visual deficits and hydrocephalus.

The authors present the case of a child who presented with short stature and clinical evidence of Cushing disease (CD) associated with a suprasellar tumor. The patient underwent insertion of an Ommaya reservoir into the tumor's cystic portion. High adrenocorticotropic hormone (ACTH) levels were demonstrated within the cyst's fluid and in the serum. After adequate decompression of the tumor, the patient underwent total resection. The tumor pathology was compatible with an adamantinomatous craniopharyngioma and immunohistochemical studies failed to show staining for ACTH. Panhypopituitarism developed postoperatively in the patient and he received hormone substitution therapy with final adequate height and normal-high weight.

The neurosurgical implications of CD along with a possible mechanism for this patient's presentation are discussed in detail on the basis of the pertinent literature.

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Miguel Reyes-Mugica, Pauline Chou, Frank Gonzalez-Crussi and Tadanori Tomita

✓ A case of meningeal fibroma in a 5-year-old girl is described. The lesion presented as a benign intracranial tumor, eroding the frontal bone and protruding under the skin. It was composed of fibroblasts and collagen, embedded in a loose background with focal myxoid changes. The authors describe the patient's clinical presentation and the tumor's histological, immunohistochemical, and ultrastructural features, and discuss its differential diagnosis. It is concluded that fibromas of the meninges should be distinguished from fibroblastic meningiomas.

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Alexander Ksendzovsky, Roberta Glick, Manuel Utset, Tadanori Tomita and Gopa Srinivasan

Hemangiomas of infancy (HOIs) are among the most common benign tumors of childhood and classically appear as a vascular stain or small vascular papule at birth. They are unique tumors due to their propensity to proliferate, involute, and finally regress. These lesions can be associated with visceral malformations that have been shown to affect mainly the liver and the gastrointestinal tract, but rarely the spinal cord. The authors report a rare case of a spinal HOI in a 2-month-old infant presenting with quadriplegia due to intratumoral hemorrhage. Following resection of the lesion, the child regained function. This first reported case of spinal HOI suggests another location for hemangiomatosis. Spinal HOI should be included in the differential diagnosis of acute intraspinal hemorrhage in infants.

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Brian A. O'Shaughnessy, Kristian T. Schafernak, Arthur J. DiPatri Jr., Stewart Goldman and Tadanori Tomita

✓ Microfibrillar collagen hemostat (MCH), also known by its trade name Avitene, is commonly used to control hemorrhage during neurosurgery. Among the documented complications associated with this agent, a granulomatous foreign body reaction is rare, having been described in the central nervous system in only one previous clinical report. In the present study, the authors report the case of a 3-year-old boy who presented with a lesion which appeared to be the recurrence of a tumor 2 months after he had undergone gross-total resection for a medulloblastoma. The patient underwent resection of the presumed recurrent tumor, but histopathological analysis of the specimen revealed a granulomatous foreign body reaction to MCH and no tumor recurrence. In addition to describing the case, the authors review the surgical literature on foreign body reactions to MCH.

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Yannick Grenier, Tadanori Tomita, MaryAnne H. Marymont, Sharon Byrd and Delilah M. Burrowes

✓ The authors report two cases of ischemic stroke secondary to occlusive vasculopathy two decades after radiation therapy (RT) for medulloblastoma. Both patients underwent posterior fossa medulloblastoma partial resection, followed by craniospinal RT in which a cobalt 60 source was used; 40 Gy were given to the whole brain plus a 15-Gy boost to the posterior fossa. Both patients received multiagent chemotherapy, immediately following radiation therapy in the first case and after repeated craniotomy for recurrence 13 years after radiation in the second case. They experienced multiple sequelae from radiation and chemotherapy, including growth retardation and psychomotor delay. However, 20 years after treatment, they remained tumor free and able to work, until they presented with focal neurological deficits and seizures. Computerized tomography and magnetic resonance imaging of the brain in both cases showed no tumor recurrence, but did demonstrate ischemia in a posterior cerebral artery distribution. Cerebral angiography revealed multiple mid-sized arterial wall irregularities as well as focal stenoses consistent with a postirradiation vasculopathy. The pathophysiological mechanisms, radiological appearance, and incidence of this syndrome are reviewed from the literature.