✓ 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
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.
Eveline Teresa Hidalgo, Michelle W. McQuinn and Jeffrey H. Wisoff
Optic pathway gliomas (OPGs) are relatively common and benign lesions in children; however, in adults these lesions are nearly always malignant and hold a very poor prognosis. In this report the authors present the case of an adult patient with a benign OPG who underwent subtotal resection without adjuvant therapy and has had no tumor progression for more than 20 years. A 50-year-old woman presented with a 2-year history of personality changes, weight gain, and a few months of visual disturbances. Ophthalmological evaluation showed incomplete right homonymous hemianopsia. MRI demonstrated a 2.5 × 2.5 × 2.5–cm enhancing left-sided lesion involving the hypothalamus with extension into the suprasellar cistern, extending along the left optic tract and anterior to the level of the optic chiasm. A biopsy procedure revealed a juvenile pilocytic astrocytoma. A subtotal resection of approximately 80% of the tumor was performed. Postoperatively, the patient experienced complete resolution of her personality changes, and her weight decreased back to baseline. Ophthalmological examination showed increased right homonymous hemianopsia. In the years following her surgery, there was a spontaneous decrease in tumor size without adjuvant therapy. The patient continues to have an excellent quality of life despite a visual field defect, and no further tumor growth has been observed.
Robert E. Elliott and Jeffrey H. Wisoff
Fusiform dilation of the supraclinoid internal carotid artery (FDCA) is a reported occurrence following surgery for suprasellar tumors, in particular craniopharyngiomas. We report our experience of the incidence and natural history of FDCA following aggressive surgical resection of craniopharyngiomas in children.
Between 1986 and 2006, 86 patients under the age of 21 underwent radical resection of craniopharyngiomas at our institution. Ten cases with < 1 year of follow-up imaging (6), perioperative death (3), or nonsuprasellar tumors (1) were excluded. Data were retrospectively collected on the remaining 76 patients (43 male, 33 female; mean age 9.5 years; mean tumor size 3.3 cm) to determine the risk factors for and the rate and clinical significance of FDCA.
Fifty patients had primary tumors and 26 patients received treatment before referral to our center. Sixty-six children (87%) had gross-total resection. At a mean follow-up time of 9.9 years, FDCA had developed in 7 patients (9.2%), all of whom had primary tumors and gross-total resection. The mean time to onset of FDCA was 6.8 months (range 3–11 months) with stabilization occurring at mean of 17.7 months (range 9–29.5 months). The mean size of the aneurysms was 9.1 mm (range 7.1–12 mm). After arrest, no lesions showed continued growth on serial imaging or produced symptoms or required treatment. There were no significant differences in age, sex, tumor size, pre- or retrochiasmatic location, extent of resection, or surgical approach (p > 0.05) between patients with and without FDCA.
Fusiform dilation of the supraclinoid internal carotid artery occurred in almost 10% of children following radical resection of craniopharyngiomas. In agreement with other reports, the authors concluded that FDCA probably occurs as a result of surgical manipulation of the supraclinoid carotid artery and should be managed conservatively because very few patients exhibit continued symptoms or experience growth or rupture of the lesion.
Robert E. Elliott and Jeffrey H. Wisoff
Prior work by the authors' group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter).
Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment.
Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%.
In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.
Jeffrey H. Wisoff
Robert E. Elliott and Jeffrey H. Wisoff
Given the potential morbidity of cranial irradiation in young children, the risk-benefit analysis of limited surgery plus irradiation versus radical resection may favor the latter strategy. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes of patients 5 years of age and younger who underwent radical resection of craniopharyngiomas.
Between 1991 and 2008, 19 children age ≤ 5 years were diagnosed with a craniopharyngioma and underwent radical resection by the senior author (J.H.W.). Data were retrospectively collected on these 19 patients (11 males, 8 females; mean age 3.2 years) to assess the efficacy and impact of surgical treatment.
Eighteen (95%) of 19 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. There was no operative death and 18 of (95%) 19 patients were alive at a mean follow-up of 9.4 years (median 8.3 years). Six patients (31%) had a total of 7 tumor recurrences treated by repeat GTR in 5 patients and Gamma knife surgery in 1 patient. No patient required conventional, fractionated radiation therapy. Disease control was achieved surgically in 17 (89.5%) patients and with surgery and Gamma knife surgery in 1 patient, yielding an overall rate of disease control of 95% without the use of conventional radiotherapy. New-onset diabetes insipidus occurred in 50% of patients. Vision worsened in 1 patient, and there was no long-term neurological morbidity.
In this retrospective series, children aged ≤ 5 years with craniopharyngiomas can have excellent outcomes with minimal morbidity after radical resection by an experienced surgeon. Disease control in this population can be successfully achieved with GTR alone in the majority of cases, avoiding the detrimental effects of radiotherapy in this vulnerable population.
Harold L. Rekate
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.