Malignant transformation in benign cerebellar astrocytoma

1978 ◽  
Vol 49 (1) ◽  
pp. 111-118 ◽  
Author(s):  
George M. Kleinman ◽  
William C. Schoene ◽  
Thomas M. Walshe ◽  
Edward P. Richardson

✓ The authors give follow-up information on Case 59 of Cushing's 1931 series of cerebellar astrocytomas. The patient died with a malignant cerebellar astrocytoma 48 years after partial removal of a previously benign astrocytoma at the same site. Including the present one, there have been only five reported cases in which this has occurred. Ordinarily, juvenile pilocytic astrocytomas are of extremely benign character, and it is well established that even with incomplete resections patients have survived for years without progression of the tumor. Not all of the cases so reported can be wholly accepted as representing malignant transformation of the tumor, but may instead be instances of recurrence of an inherently benign glioma since the presence of features such as endothelial hyperplasia or nuclear atypicality in a juvenile pilocytic astrocytoma does not warrant its being classified as malignant. Features truly suggestive of malignancy are hypercellularity, frequent mitoses, necrosis, and, in some instances, a diffusely infiltrative growth pattern; all of these features were found in the present case.

2003 ◽  
Vol 98 (6) ◽  
pp. 1170-1174 ◽  
Author(s):  
Christoph Burkhard ◽  
Pier-Luigi Di Patre ◽  
Danielle Schüler ◽  
Georges Schüler ◽  
M. Gazi Yaşargil ◽  
...  

Object. The incidence of pilocytic astrocytomas and the rate of patient survival were analyzed in a population-based study in the canton of Zürich, Switzerland. Methods. Between 1980 and 1994, 987 astrocytic and oligodendroglial tumors were diagnosed, of which 55 (5.5%) were pilocytic astrocytomas. The incidence rate, adjusted to the World Standard Population, was 4.8 per 1 million per year. The mean age at clinical diagnosis was 19.6 ± 12.7 years, and the male/female ratio was 1.12. The most frequent tumor sites were the cerebellum (40%), followed by supratentorial locations (35%), the optic pathway and hypothalamus (11%), and the brainstem (9%). The mean follow-up period was 12 years. Observed survival rates were 100% at 5 years and 95.8% at 10 years after diagnosis (relative survival rate at 10 years: 96.8%). Seven patients (13%) received postoperative radiotherapy, but this did not significantly affect survival. In all patients the tumors were histologically classified as WHO Grade I, except in two patients who had anaplastic pilocytic astrocytoma (Grade III), one of whom died after 7 years, whereas the other was still alive after 10 years. Conclusions. This population-based study shows that, because of the benign biological behavior of pilocytic astrocytomas and advances in microneurosurgery, the survival rates for patients with these tumors are excellent, regardless of postoperative radiotherapy.


1991 ◽  
Vol 75 (1) ◽  
pp. 138-142 ◽  
Author(s):  
Dominique Figarella-Branger ◽  
Miguelina Perez-Castillo ◽  
Louise Garbe ◽  
François Grisoli ◽  
Danielle Gambarelli ◽  
...  

✓ What is apparently the first reported case of spontaneous malignant transformation of a benign osteoblastoma of the skull is described. The initial lesion was completely removed surgically and showed the histological features typical of a benign osteoblastoma. No radiotherapy was performed. Eleven years later the patient developed an osteosarcoma of the skull. Review of the literature showed that malignant transformation of benign osteoblastomas is extremely rare and could take place spontaneously. However, the risk of this occurring seems higher after inadequate initial treatment (curettage or partial excision). Follow-up monitoring of patients with osteoblastoma of the cranial vault is suggested.


1972 ◽  
Vol 37 (4) ◽  
pp. 470-474 ◽  
Author(s):  
William R. Bernell ◽  
John J. Kepes ◽  
Elson P. Seitz

✓ Two cases are presented in which childhood cerebellar astrocytomas recurred 20 and 23 years after gross total excision. Both recurrent tumors showed histological or clinical evidence of malignancy. The cases indicate that even two decades of symptom-free follow-up are not necessarily a guarantee of cure with this glioma.


2002 ◽  
Vol 96 (4) ◽  
pp. 704-712 ◽  
Author(s):  
Reizo Shirane ◽  
Ching-chan Su ◽  
Yasuko Kusaka ◽  
Hidefumi Jokura ◽  
Takashi Yoshimoto

Object. Craniopharyngiomas frequently grow from remnants of the Rathke pouch, which is located on the cisternal surface of the hypothalamic region. These lesions can also extend elsewhere in the infundibulohypophyseal axis. The aim of this study was to establish the usefulness of the frontobasal approach made through a relatively small craniotomy window for the removal of tumors protruding from the sellar—suprasellar region into the third and basal cistern. Methods. Thirty-one patients who were surgically treated for craniopharyngiomas extending outside the sellar—suprasellar region were evaluated. The diagnoses were established in all cases by using magnetic resonance and computerized tomography imaging. The initial symptoms and signs were increased intracranial pressure in eight, vision impairment or visual field defect in 16, hypopituitarism in 17, and psychological disturbances in three cases. All patients underwent surgery via the frontobasal interhemispheric approach, and the average follow-up period was 30 months. Total removal of the lesion was achieved in 22 cases, six patients underwent subtotal resection, and three underwent partial removal due to tumor recurrence after previous surgeries performed with or without adjunctive radiotherapy. Major complications, including impairment of the cranial nerves, were not observed in the immediate postoperative period. One patient exhibited transient memory disturbance due to infarction of the perforating vessels; after 3 months this symptom was ameliorated. None of the patients died during long-term follow up; however, four of the 22 who underwent total removal and six of the nine patients who underwent subtotal or partial removal suffered recurrence. Of the 10 patients with recurrence, six experienced a small recurrence of the lesion (average 3 months postsurgery); after gamma knife surgery (GKS), the size of two of the lesions was unchanged and in four reoperation was performed due to tumor enlargement during the follow-up period. Ultimately, a total of eight patients (four with recurrence and four who had been treated with GKS) underwent reoperation, with gross-total removal via the same approach or combined with the orbitozygomatic approach in patients with very short optic nerves. In no patient was deterioration of visual acuity and visual field observed after surgery. Although all patients except four children and one adult were receiving some form of hormone replacement therapy, their endocrine status was stably controllable. Conclusions. In the authors' experience, the frontobasal interhemispheric approach, even made through a small craniotomy window, is a valid choice for the removal of craniopharyngiomas extending outside the sellar—suprasellar region. Via this approach, tumors can be removed without significant sequelae related to the surgical method, due to ease of preservation of the pituitary stalk, hypothalamic structures, and perforating vessels. This approach offers a safe and minimally invasive means of treating craniopharyngiomas.


1981 ◽  
Vol 55 (2) ◽  
pp. 170-173 ◽  
Author(s):  
Sandro Mercuri ◽  
Antonio Russo ◽  
Lucio Palma

✓ In a series of 41 cases of hemispheric supratentorial astrocytoma (HSA) in children, 29 patients have been followed for periods ranging from 5 to 27 years. The follow-up data show that HSA's are relatively benign tumors. Twenty-two patients (76%) are still living: 12 in good, eight in fair, and two in poor neurological condition. Their mean survival is 13.3 years. Five patients died from tumor recurrence within 4 years of the operation. All five were operated on before the age of 8 years. The best results in terms of mean survival and quality of life were obtained in cases of cystic HSA, especially the pilocytic variety. In children, HSA tends to take the cystic form (often pilocytic), and its relatively benign course points up an analogy between this rare supratentorial tumor and the more common tumor of the cerebellum (the so-called “cerebellar astrocytoma”).


1982 ◽  
Vol 57 (4) ◽  
pp. 548-551 ◽  
Author(s):  
Charles E. Alpers ◽  
Richard L. Davis ◽  
Charles B. Wilson

✓ A 5-year-old girl with juvenile pilocytic astrocytoma subsequently had multiple episodes of symptomatic recurrence over the course of 21 years of follow-up review. This lesion underwent histological transformation to an anaplastic small-cell neoplasm 21 years after the initial resection and diagnosis. Late transformation of benign childhood cerebellar astrocytomas to malignant astrocytic tumors is very rare; transformation to an anaplastic small-cell neoplasm has not been reported previously. This case and other reported cases in which juvenile pilocytic astrocytoma recurred after a long interval indicate the requirement of long-term follow-up review of these patients before assuming either a cure or arrest of the tumor's growth.


2002 ◽  
Vol 97 ◽  
pp. 677-680 ◽  
Author(s):  
Jörgen Boëthius ◽  
Elvar Ulfarsson ◽  
Tiit Ráhn ◽  
Bodo Lippitz

Object. The authors report on the follow-up studies in patients treated at the Karolinska Hospital to evaluate the efficacy of gamma knife radiosurgery (GKS) for pilocytic astrocytoma. Methods. Twelve male and seven female patients were treated (mean age 10.6 years [range 2–60 years]). Sixteen of these patients were children in whom GKS was performed to treat residual tumor after surgery. Most tumors were treated with a prescription dose of 10 to 12 Gy (range 9–20 Gy). The corresponding maximum dose varied between 22 and 30 Gy (range 10–50 Gy). The median clinical follow-up time was 7 years and mean clinical follow-up time 8.5 years. Median radiological follow-up time was 4.7 years and the mean radiological follow-up time was 5.9 years. Tumor control was achieved in all patients. In 85% of the cases a moderate tumor volume reduction was observed after GKS. This result occurred despite the low prescription dose administered. The radiological follow-up studies showed adverse radiation effects in 25% of patients with increasing contrast enhancement and some edema. These effects generally appeared within 7 months and resolved later. Cyst development occurred in two patients, which may have been treatment related. Conclusions. It appears that small pilocytic astrocytomas may be treated with low-prescription-dose GKS, resulting in satisfactory clinical outcomes and only minor side effects. There were a certain number of radiologically demonstrated side effects that appeared relatively early but subsequently resolved. This study should be regarded as a preliminary one because the number of patients is small and the follow-up period is short compared with the natural time course of the disease.


2002 ◽  
Vol 97 (1) ◽  
pp. 56-64 ◽  
Author(s):  
Constantinos G. Hadjipanayis ◽  
Douglas Kondziolka ◽  
Paul Gardner ◽  
Ajay Niranjan ◽  
Shekhar Dagam ◽  
...  

Object. The goal of this study was to examine the role of stereotactic radiosurgery in the treatment of patients with recurrent or unresectable pilocytic astrocytomas. Methods. During a 13-year interval, 37 patients (median age 14 years) required multimodal treatment of recurrent or unresectable pilocytic astrocytomas. Tumors involved the brainstem in 18 patients, cerebellum in three, thalamus in five, temporal lobe in four, and parietal lobe in two, as well as the hypothalamus, optic tract, corpus callosum, insular cortex, and third ventricle in one patient each. Diagnosis was confirmed with the aid of stereotactic biopsy in 12 patients, open biopsy in five, partial resection in eight, and near-total resection in 12. Multimodal treatment included fractionated radiation therapy in 10 patients, stereotactic intracavitary irradiation of tumor in four, chemotherapy in two, cyst drainage in six, ventriculoperitoneal shunt placement in three, and additional cytoreductive surgery in four. Tumor volumes varied from 0.42 to 25 cm3. The median radiosurgical dose to the tumor margin was 15 Gy (range 9.6–22.5 Gy). After radiosurgery, serial imaging demonstrated complete tumor resolution in 10 patients, reduced tumor volume in eight, stable tumor volume in seven, and delayed tumor progression in 12. No procedure-related death was encountered. Thirty-three (89%) of 37 patients are alive at a median follow-up period of 28 months after radiosurgery and 59 months after diagnosis. Eight patients participated in follow-up review for more than 60 months. Three patients died of local tumor progression. Conclusions. Stereotactic radiosurgery is a valuable adjunctive strategy in the management of recurrent or unresectable pilocytic astrocytomas. Despite the favorable histological characteristics and prognosis usually associated with this neoplasm, an adverse location, recurrence, or progression of this disease requires alternative therapeutic approaches such as radiosurgery.


2004 ◽  
Vol 100 (4) ◽  
pp. 700-705 ◽  
Author(s):  
Peter Kan ◽  
Oren Gottfried ◽  
Deborah T. Blumenthal ◽  
Jeannette J. Townsend ◽  
Ela Drozd-Borysiuk ◽  
...  

✓ Multiple metastatic brain tumors and multifocal primary brain tumors of a single histological type are well described in the literature. The concurrent presence of multiple primary brain tumors with different histological characteristics, however, is very rare. The authors describe the first known case in which an oligodendroglioma and a juvenile pilocytic astrocytoma (JPA) presented as synchronous primary brain tumors in the same patient. This 43-year-old man presented with a 2-month history of progressive headaches, nausea, and vomiting. Magnetic resonance imaging demonstrated an enhancing heterogeneous right medial cerebellar lesion and a larger calcified, nonenhancing, heterogeneous right frontal lesion with surrounding edema and a mass effect. The results of a metastatic workup were unremarkable. The patient underwent an initial right frontotemporal craniotomy and a subsequent suboccipital craniectomy 2 years later for resection of the posterior fossa lesion. Histological examination revealed the frontal and cerebellar lesions to be an oligodendroglioma and JPA, respectively. A molecular analysis detected a deletion of chromosome 1p36 in the oligodendroglioma, but not in the JPA. After the initial operation, the patient received follow-up care for his oligodendroglioma, but eventually required temozolomide for tumor progression. His condition remains stable both neurologically and according to imaging studies. The authors describe the first known case in which a low-grade oligodendroglioma and a JPA presented as synchronous primary brain tumors. They review the literature on multiple primary brain tumors with different histological characteristics and discuss potential mechanisms for the development of these lesions.


1994 ◽  
Vol 81 (1) ◽  
pp. 24-30 ◽  
Author(s):  
Adam N. Mamelak ◽  
Michael D. Prados ◽  
William G. Obana ◽  
Philip H. Cogen ◽  
Michael S. B. Edwards

✓ Little is known about the risk of developing multicentric disease in patients with juvenile pilocytic astrocytoma (JPA), and even less about its prognosis. Only five cases have been reported. Between 1986 and 1992, the authors treated 90 patients with either primary or recurrent JPA, 11 of whom developed multicentric spread. Ten patients had primary tumors in the hypothalamic region, eight were under 4 years of age at initial diagnosis, all had initially undergone a subtotal resection or biopsy, and 10 received postoperative multiagent chemotherapy or irradiation for residual disease. Multicentric spread was discovered immediately to 108 months after initial diagnosis; nine patients were asymptomatic at the time. Most patients received chemotherapy for the multicentric disease, which was found throughout the craniospinal axis. During 21 to 148 months of follow-up monitoring, seven patients had stabilization or regression of multicentric disease and four died. Patients with hypothalamic region tumors were 23 times more likely to develop multicentric spread than were those with primary tumors located elsewhere (p < 0.001). Based on this review, it is concluded that multicentric spread of JPA occurs more frequently than was previously recognized. In patients with subtotally resected JPA and several years of follow-up review via magnetic resonance imaging, the incidence of recurrence in a site different from the original was 12%. Patients with subtotally resected JPA in the hypothalamic region should be considered to be at high risk for developing multicentric spread. Chemotherapy appears useful in stabilizing multicentric disease. Earlier detection and intervention may result in longer disease-free survival in patients with multicentric spread of JPA.


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