✓ The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with symptomatic pineal cysts most often present with one of three syndromes: 1) paroxysmal headache with gaze paresis; 2) chronic headache, gaze paresis, papilledema, and hydrocephalus; or 3) pineal apoplexy with acute hydrocephalus. Surgical intervention with radical cyst removal is the treatment of choice for all symptomatic pineal cysts. Complete cyst removal is desirable; however, radical subtotal resection is appropriate if the cyst cannot be easily separated from the quadrigeminal plate. Ventricular shunting should be reserved for patients with persistent hydrocephalus after cyst resection.
Jeffrey H. Wisoff and Fred Epstein
The effect of patients' age and extent of disease on prognosis
Jeffrey C. Allen and Fred Epstein
✓ Studies to evaluate the extent of their disease were performed on 30 children with newly diagnosed malignant neuroectodermal tumors of the central nervous system, including 25 children with medulloblastomas. The studies consisted of postoperative computerized tomography scanning, cerebrospinal fluid (CSF) cytology, myelography, and bone marrow aspiration. In 11 patients (36%) one or more of these studies was positive, indicating disease dissemination (Group 1), and in 19 (64%) all four studies were negative (Group 2). The CSF cytology was positive for malignant cells in all 11 Group 1 patients; myelograms indicated subclinical filling defects in nine, and the bone marrow aspirate from two patients revealed extrinsic cells.
All patients received a conventional course of neuraxis radiation therapy as tolerated, and some also received chemotherapy. Patients with abnormal myelograms received additional radiation to areas of identifiable subarachnoid disease. The median survival time in Group 1 was 12 months, with five patients still surviving. The prognosis for Group 2 patients was considerably more favorable; only three of the 19 had died at the time of this report. Four of the six deaths in Group 1 were related to disease dissemination, the other two to chemotherapy toxicity. All three deaths in Group 2 were related to local tumor recurrence.
The mean age of Group 1 patients was 3.9 years compared to 11 years for Group 2. Of the 14 patients aged 5 years and under in both groups, nine presented with widely disseminated disease (Group 1), six of whom had a relatively short survival time. The performance of a postoperative extent-of-disease evaluation is especially important in young children with medulloblastoma. Knowledge of disease dissemination at diagnosis not only helps determine prognosis, but also assists in planning treatment. Aggressive multimodal therapy appears warranted in younger patients who present with widely disseminated disease.
Fred Epstein and Jeffrey Wisoff
The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.
Alan R. Cohen, Jeffrey H. Wisoff, Jeffrey C. Allen and Fred Epstein
✓ The authors review their experience with the operative management of 19 consecutive cases of malignant astrocytoma of the spinal cord. There was a male to female ratio of 1.1:1, and the median age of the population was 14 years (range 1 to 32 years). The median duration of symptoms prior to definitive diagnosis was 7 weeks. Radical excision was carried out in all cases, with 18 patients (95%) receiving radiotherapy and 10 patients (53%) receiving chemotherapy as well.
To date, 15 (79%) of the 19 patients in this series have died, with a median survival period of 6 months following surgery. No patient improved after operation. Hydrocephalus was present in 11 patients (58%), seven of whom underwent ventricular shunting procedures. Dissemination of disease was found in 11 patients (58%). Extraneural metastases did not occur in the absence of a ventricular shunt. The authors conclude that malignant astrocytomas of the spinal cord are heralded by a short history followed by rapid neurological deterioration and usually death. The rationale for operation is discussed, and an aggressive approach utilizing adjuvant therapy directed at the entire neuraxis is suggested.
Jeffrey H. Wisoff, Rick Abbott and Fred Epstein
✓ Sixteen children underwent 18 operations for radical resection of chiasmatic-hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with macrocephaly, failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had precocious puberty with mild visual deficits; and older children (> 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive tumors in spite of low-grade histology, and died from progressive tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of disease progression, 4 months to 4½ years following surgery. Six of these patients, with a follow-up period of 10 months to 4½ years (mean 27 months), have had no adjuvant therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-hypothalamic tumors of infancy are aggressive neoplasms that require multimodality therapy.
Barry M. Zide, Fred J. Epstein and Jeffrey Wisoff
✓ A technique of wound closure following tethered cord correction is presented that significantly reduces the incidence of cerebrospinal fluid collections in the subcutaneous space. In over 60 cases, the described method of fascia and skin closure has lessened wound problems to a minimal level. Patient hospitalization time has also been greatly diminished.
Barry M. Zide, Jeffrey H. Wisoff and Fred J. Epstein
✓ Fifty-eight patients with previously irradiated intramedullary spinal cord astrocytomas underwent laminectomy for radical excision of their tumors. A high incidence of postoperative cutaneous cerebrospinal fluid fistulas and large pseudomeningoceles following routine closure prompted the development of an alternative method of wound closure using mobilized musculofascial flaps. The authors describe the surgical techniques and pitfalls to be avoided during the closure of complicated laminectomy wounds.
An aid to the diagnosis and management of intracranial germ-cell tumors
Jeffrey C. Allen, Jerome Nisselbaum, Fred Epstein, Gerald Rosen and Morton K. Schwartz
✓ The cerebrospinal fluid (CSF) and serum of six patients with histologically verified intracranial germ-cell tumors were assayed serially for the presence of alphafetoprotein (AFP) and the beta subunit of human chorionic gonadotropin (HCG). Two patients had embryonal carcinomas, two had choriocarcinomas, and two had dysgerminomas. The marker profile for a given tumor in either CSF or serum correlated with the histological diagnosis; that is, embryonal carcinoma produced AFP and HCG, choriocarcinoma produced HCG, and dysgerminoma produced no markers. The marker levels in serum and CSF declined with therapy and rose usually prior to the development of overt clinical symptoms if the patient's tumor recurred. A CSF-to-serum gradient of the marker levels was present in three of four patients, and the serum levels were often normal when the CSF values were elevated. Ventricular marker levels were lower than the lumbar levels in two of two patients. The assay of these biological markers is a sensitive indicator of the success of therapy, and the presence of a CSF-to-serum gradient suggests that the major portion of the neoplasm rests within the central nervous system. A histological diagnosis can be inferred without the necessity of surgery in appropriate clinical contexts.
Shlomi Constantini, Douglas C. Miller, Jeffrey C. Allen, Lucy B. Rorke, Diana Freed and Fred J. Epstein
Object. The majority of intramedullary spinal cord tumors (IMSCT) in children and young adults are low-grade gliomas. Radical resection of similar tumors in the cerebral hemisphere or cerebellum is usually curative; however, the conventional management for IMSCTs remains partial resection followed by radiotherapy because of the concern for surgical morbidity. Nevertheless, radical resection of IMSCTs without routine adjuvant treatment has been the rule at our institution since 1980. In an attempt to resolve this controversy, the long-term morbidity and survival in a large series of children have been retrospectively reviewed.
Methods. The database records and current status of 164 patients 21 years of age and younger in whom an IMSCT was resected were reviewed. A gross-total resection (> 95%) was achieved in 76.8% of the surgical procedures. Subtotal resections (80–95%) were performed in 20.1%. The majority of patients (79.3%) had histologically low-grade lesions.
There were no deaths due to surgery. When comparing the preoperative and 3-month postoperative functional grades, 60.4% stayed the same, 15.8% improved, and 23.8% deteriorated. Only 13 patients deteriorated by more than one functional grade. Patients with either no deficits or only mild deficits before surgery were rarely injured by the procedure, reinforcing the importance of early diagnosis and treatment.
The major determinant of long-term patient survival was histological composition of the tumor. The 5-year progression-free survival rate was 78% for patients with low-grade gliomas and 30% for those with high-grade gliomas. Patients in whom an IMSCT was only partially resected (< 80%) fared significantly worse.
Conclusions. The long-term survival and quality of life for patients with low-grade gliomas treated by radical resection alone is comparable or superior to minimal resection and radiotherapy. The optimum therapy for patients with high-grade gliomas is yet to be determined.
Report of three cases
Karl F. Kothbauer, George I. Jallo, Joao Siffert, Elpidio Jimenez, Jeffrey C. Allen and Fred J. Epstein
✓ Chemical agents routinely used in neurosurgery to achieve intraoperative hemostasis can cause a foreign body reaction, which appears on magnetic resonance (MR) images to be indistinguishable from recurrent tumor. Clinical and/or imaging evidence of progression of disease early after surgical resection or during aggressive treatment may actually be distinct features of granuloma in these circumstances.
A series of three cases was retrospectively analyzed for clinical, imaging, surgical, and pathological findings, and the consequences they held for further disease management.
All patients were boys (3, 3, and 6 years of age, respectively) and all harbored primitive neuroectodermal tumors. Two tumors were located in the posterior fossa and one was located in the right parietal lobe. Two boys exhibited clinical symptoms, which were unexpected under the circumstances and prompted new imaging studies. One patient was asymptomatic and imaging was performed at planned routine time intervals. The MR images revealed circumscribed, streaky enhancement in the resection cavity that was suggestive of recurrent disease. This occurred 2 to 7 months after the first surgery. At repeated surgery, the resected material had the macroscopic appearance of gelatin sponge in one case and firm scar tissue in the other cases. Histological analysis revealed foreign body granulomas in the resected material, with Gelfoam or Surgicel as the underlying cause. No recurrent tumor was found and the second surgery resulted in imaging-confirmed complete resection in all three patients. Because recurrent disease was absent, the patients continued to participate in their original treatment protocols. All patients remain free from disease 34, 32, and 19 months after the first operation, respectively.
During or after treatment for a central nervous system neoplasm, if unexpected clinical or imaging evidence of recurrence is found, a second-look operation may be necessary to determine the true nature of the findings. If the resection yields recurrent tumor, additional appropriate oncological treatment is warranted, but if a foreign body reaction is found, potentially harmful therapy can be withheld or postponed.