Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Neuroectodermal Tumors, Primitive”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,297 records · Page 72Linked to original sources
PubMed2004

[High-dose chemotherapy with autologous stem cell rescue in children with high-risk and recurrent brain tumors].

BACKGROUND: In the last few years, survival in children with central nervous system (CNS) tumors has slightly improved, especially in children with tumors such as medulloblastoma and those in which complete surgical resection is achieved. However, outcome remains poor in patients with incomplete surgical resection, neuroaxial dissemination, metastatic or recurrent tumors and in very young children. OBJECTIVES: To improve prognosis in patients with high-risk and recurrent tumors, new therapeutic strategies such as high-dose chemotherapy with autologous stem cell rescue (ASCR) have been developed. METHODS: We retrospectively studied patients with high-risk and recurrent CNS tumors who underwent ASCR between September 1995 and December 2002 in our unit. RESULTS: Thirty-five patients underwent ASCR. Seven patients died of treatment-related toxicities (20 %). Thirteen (37 %) are event-free survivors at a median post-ASCR follow-up of 18 months (range: 5-63 months). The 2-year Kaplan-Meier estimates of event-free survival was 37.64 +/- 8.7 % in all patients, 57 +/- 15 % in the group of patients with high-risk medulloblastoma/supratentorial primitive neuroectodermal tumor (stPNET) and 71.43 +/- 17 % in patients aged less than 4 years with medulloblastoma/stPNET. CONCLUSIONS: In our experience, ASCR may be effective in the treatment of malignant tumors of the central nervous system in patients with controlled disease, in certain histologic groups and chemosensitive tumors (medulloblastoma, malignant astrocytoma), as well as in very young children in whom cranial radiotherapy is contraindicated.

Adolescent↗
PubMed2006

Intensive therapy with growth factor support for patients with Ewing tumor metastatic at diagnosis: Pediatric Oncology Group/Children's Cancer Group Phase II Study 9457--a report from the Children's Oncology Group.

PURPOSE: Prognosis is poor for Ewing sarcoma patients with metastasis at diagnosis. We intensified a five-drug therapy (ifosfamide, etoposide alternated with vincristine, doxorubicin, and cyclophosphamide) using filgrastim but not stem-cell support. We studied topotecan alone and combined with cyclophosphamide in therapeutic windows before the five-drug therapy. A randomly assigned proportion of patients received amifostine as a cytoprotective agent. PATIENTS AND METHODS: Eligible patients were < or = 30 years old and had histologically proven Ewing sarcoma or primitive neuroectodermal tumor (PNET) and metastasis at diagnosis. Chemotherapeutic cycles began every 21 days, after recovery from toxicities. RESULTS: One hundred ten of the 117 patients enrolled were eligible. Thirty-six patients received initial topotecan. Three had partial responses (PRs), and 17 had progressive disease (PD). Thirty-seven patients were administered topotecan and cyclophosphamide; 21 of these patients achieved PR, and one patient had PD. In a randomly assigned group of 69 patients, amifostine did not provide myeloprotection, which was measured by absolute neutrophil count, platelet count, or cycle intervals. The best responses to the overall therapy included 45 complete responses, 41 PRs, stable disease in 14 patients, and PD in five patients. For all patients, the 2-year event-free survival (EFS) rate was 24% (+/- 4%), and the overall survival rate was 46% (+/- 5%). For the 39 patients with isolated pulmonary metastases, the 2-year EFS rate was 31% (+/- 7%) compared with 20% (+/- 5%) for patients with more widespread disease. CONCLUSION: Topotecan had limited activity in patients with Ewing sarcoma or PNET metastatic at diagnosis. The topotecan-cyclophosphamide combination was active. Amifostine was not myeloprotective. Overall results showed no improvement compared with previous studies.

Adolescent↗
PubMed1996

Hyperbaric oxygen therapy for the treatment of radiation-induced sequelae in children. The University of Pennsylvania experience.

BACKGROUND: The role of hyperbaric oxygen (HBO) therapy in the treatment of radiation-related sequelae in adults is well known. In contrast, its role in the management of radiation-related sequelae in children has not been well studied. In an effort to define its value better, the authors reviewed the University of Pennsylvania experience and hereby report the results of their analysis. METHODS: Between 1989 and 1994, ten patients who underwent radiation therapy for cancer as children were referred for HBO therapy. Six patients underwent HBO therapy as a prophylactic measure prior to maxillofacial procedures; dental extractions and/or root canals (four patients), bilateral coronoidectomies for mandibular ankylosis (one patient), and wound dehiscence (one patient). Therapeutic HBO was administered to four other patients; one patient for vasculitis resulting in acute seventh cranial nerve palsy and the other three after sequestrectomy for osteoradionecrosis (mastoid bone, temporal bone, and sacrum, respectively). Osteoradionecrosis was diagnosed both radiologically and histologically after exclusion of tumor recurrence. The number of treatments ranged between 9-40 "dives" (median, 30 dives). Treatments were given once daily at 2 atmosphere absolutes for 2 hours each. Adjunctive therapy in the form of debridement, antibiotics, and placement of tympanotomy tubes was administered to two patients. Ages at HBO treatment ranged from 3.5 to 26 years (median, 14 years). Six patients were male and four were female. The most commonly irradiated site was the head and neck region (eight patients; brain stem gliomas [one], posterior fossa primitive neuroectodermal tumor [one], rhabdomyosarcomas [three], nasopharyngeal cancer [one], carcinoma of the parotid gland [one], and Hodgkin's disease [one]). The remaining two patients received radiation therapy for pelvic tumors [Ewings's sarcoma and rhabdomyosarcoma). Radiation doses ranged between 4000 and 6660 centigray (cGy) (median, 5500 cGy). The interval between the end of radiation therapy and HBO treatment ranged between 2 months and 11 years (median, 15 years). The median follow-up interval after HBO therapy was 2.5 years (range, 2 months-4 years). RESULTS: Except for two patients who had initial anxiety, nausea, and vomiting, the HBO treatments were well tolerated. In all but one patient, the outcome was excellent. In the six patients who had prophylactic HBO, all continued to demonstrate complete healing of their orthodontal scars at last follow-up. In the four patients who received HBO as a therapeutic modality, all 4 had documented disappearance of signs and symptoms of radionecrosis and two patients demonstrated new bone growth on follow-up computed tomography scan. One patient with vasculitis and seventh cranial nerve palsy had transient improvement of hearing; however, subsequent audiograms returned to baseline. CONCLUSIONS: The use of hyperbaric oxygen for children with radiation-induced bone and soft tissue complications is safe and results in few significant adverse effects. It is a potentially valuable tool both in the prevention and treatment of radiation-related complications.

Adolescent↗
PubMed1984

Phase II evaluation of dibromodulcitol in the treatment of recurrent medulloblastoma, ependymoma, and malignant astrocytoma.

The authors have conducted a Phase II trial to evaluate orally administered dibromodulcitol in the treatment of 40 evaluable patients with recurrent medulloblastoma, ependymoma, and malignant astrocytoma. Ten of 20 patients harboring medulloblastoma responded to therapy with a median time to tumor progression (MTP) of 40 weeks, and four of 20 patients had no sign of progression of disease 4 years after treatment was begun. The MTP for all 12 patients with ependymoma was 30 weeks. Nine of these 12 patients had stabilization of their disease with an MTP of 67 weeks; three of these 12 patients had no signs of progression for 1 to 3 years after treatment was begun. Of six patients harboring supratentorial gliomas, none responded to dibromodulcitol. Two patients, one with a primitive neuroectodermal tumor and the other with a metastatic carcinoma of the breast, had stabilization of disease for more than 4 and 2 years, respectively.

Adolescent↗
PubMedDate not supplied

Stromal elements for tumor diagnosis: a brief review of diagnostic electron microscopic features.

Tumor diagnosis mainly depends on the appearance of the tumor cells in recapitulating the appearance of primordial cells from which they arise. However, certain tumors may present with specific stromal changes that may assist/enhance the diagnosis. In this presentation, diagnostic stromal features have been reviewed. The cytoplasm is enclosed by a unit membrane, which serves as a barrier to, as well as an interface with, surrounding structures. Epithelial cells usually show characteristic basal-apical orientation. In mesenchymal tissue, different types of interface can be found in different types of mesenchymal tissue. External lamina can be defined as an anatomic structure, which encloses anatomic functional units. In epithelial tissue, cells in a functional unit are enclosed within a well-defined external lamina (EL). In malignant epithelial tumors, EL can become increasingly indistinct as tumors become less differentiated, and one has to look for it diligently. Within the external lamina, epithelial cells are closely packed with closely apposed cell membranes and cell attachment junctions. In contrast to epithelial tissue, mesenchymal tissue is usually characterized by the stromal elements they produce. Individual cells are embedded in the stroma, and individual mesenchymal cells represent the functional unit. Vascular endothelial cells are an exception since their relationship to stroma resembles to that of epithelial cells. Thus, tumors deriving from mesenchymal cells known to have external lamina such as muscle cells and Schwann cells tend to show total enclosure of cells by external lamina. In malignant muscle tumors, external lamina production can be focally present and found only by diligent search. In Schwann cell tumors, the presence of EL is prominent in low-grade tumors and more irregular and variable in malignant tumors. In the latter, stromal aggregation of scrolls of external lamina can be characteristic. Similar features are seen in ossifying fibromyxoid tumors. Fibronexus junctions (composed of extracellular fibronectin fillements linking intracellular 5-nm filaments) is claimed to be typical of myofbroblasts. Finding them in spindle cell tumors justifies a diagnosis of myofibroblastomas. There have been several stromal changes diagnostic for certain tumors found only by electron microscopy. Fibrous long-spaced collagen (known as Luse bodies) is diagnostic for peripheral nerve sheath tumors, but they can rarely be found in other tumors. Luse bodies usually appear as focally as crystallized aggregates apart from the regular collagenous interstitial stroma. They should be distinguished from other nonspecific long-spaced collagen changes. The changes are diffusely stromal in contrast to Luse bodies. Spiny collagen and amianthoid fibers are interesting collagen fibrils and their diagnostic value is questionable. Skeinoid fibers (SF) are short-spaced collagen of 41- to 45-nm banding so-named because of their peculiar appearance by electron microscopy simulating skeins of yarn. They were originally described in neurogenic tumors and small intestinal stromal tumors with features of gastrointestinal autonomic nerve tumors (GANT). Although there have been a few sporadic case reports of the presence of skeinoid fibers in nonneurogenic tumors, the frequent presence of SF in spindle cell tumors signifies their neurogenic nature in this authors' experience. An exception to this is that SF can be a constant element of rare ciliary body tumors known as ciliary mesectodermal leiomyomas, in which tumor cells show some resemblance to smooth muscle as well as Schwann cells. In addition to SF, several other types of peculiar crystallized collagen were observed in GANT tumors, particularly those with multiple tumor syndromes such as neurofibromatosis and Carney's triad. They simulate the appearance of railroad tracks or centrosomes. The reason for this is not known. The authors speculate that such collagen crystallization may be caused by genetic alterations involving collagenosis. Further studies will be necessary to clarify their pathogenesis. Another peculiar stromal change is electron-dense stromal filamentous aggregates with extra-long banding of > 250-nm periodicity previously described in Ewing sarcomas. This stromal change simulating a tiger skin pattern is also seen in primitive neuroectodermal tumors and malignant melanomas. In view of continually new discoveries of stromal changes that can be used for the differential diagnosis of tumors, the importance of close evaluation of stromal elements of tumors, and diligent application of electron microscopy in tumor diagnosis cannot be overemphasized.

Cytoplasm↗
PubMed1998

Nephroblastoma-like tumors in patients with testicular germ cell tumors.

Nephroblastoma-like tumors (NLTs) developed in metastatic sites in nine men with testicular germ cell tumors (GCTs). These tumors had a characteristic "triphasic" admixture of primitive tubular structures, sometimes with a glomeruloid pattern, blastema and stroma. Skeletal muscle differentiation was apparent in two cases. Specific neuroendocrine markers (synaptophysin and chromogranin A) were negative. All patients were treated by surgical excision. Six patients were alive with no evidence of disease from 4 to 12 years after diagnosis of GCT and NLT. One patient was alive with disease 6 years after diagnosis of GCT and 3 years after diagnosis of NLT. One man who also had metastatic primitive neuroectodermal tumor (PNET) had short survival, and one patient died of postoperative infection. We conclude that patients with testicular GCTs in whom NLTs develop in metastatic sites often experience prolonged survival. Surgical excision appears to be adequate treatment for NLT arising in metastatic testicular GCT in most patients. It is important to distinguish NLTs from PNETs in metastatic GCTs because of the more aggressive course and the frequently fatal outcome of the latter.

Abdominal Neoplasms↗
PubMed1994

The surgical and natural morbidity of aggressive resection for posterior fossa tumors in childhood.

The morbidity associated with gross total removal of pediatric posterior fossa tumors is well recognized although it is rarely isolated from other factors that comprise the management morbidity for these tumors. This study reviews (1) the operative and postoperative complications in 105 patients and (2) the neurological morbidity in a subset of 91 patients undergoing gross total removal of their tumor between 1982 and 1992. Gross total removal was achieved in 102 patients with a single procedure. Two patients with residual tumor underwent early repeat craniotomy for excision and 1 is being followed without repeat resection. Intra- and postoperative complications occurred in 33 patients and included hematoma requiring craniotomy (3), gastrointestinal hemorrhage (2), hydrocephalus requiring shunt placement (9), wound problems (4), and pseudomeningocele formation requiring additional treatment (5). Delayed onset hydrocephalus requiring shunting occurred in 2 patients and spinal deformity in 4 patients. Worsening of preoperative deficit (new cranial nerve palsies, worsening ataxia, bulbar dysfunction including apnea, mutism and seizures) occurred in 41% of patients operated on for primitive neuroectodermal tumors (PNET) (14/34), 53% of ependymomas (10/19), and 30% of astrocytomas (15/50). No patient who had a choroid plexus tumor was worsened by the procedure. Complete recovery of new postoperative deficits occurred in 14% of PNET (2/14), 50% of ependymoma (5/10) and 47% of astrocytoma (7/15), most often within 6 months of the procedure. Residual neurological morbidity, due to persistence of preoperative symptoms or due to deficits that occurred as the result of the surgical procedure, was assessed in a subgroup of 91 patients followed for an average of 48 months (2-147 months). This assessment did not include morbidity due to adjuvant therapy. Sixty-two percent of patients continued to exhibit abnormal cerebellar or bulbar signs. Forty-three percent of the total population exhibited limitation in function due to residual deficit. Only 38% of patients were both functionally normal and had a normal neurological examination at last follow-up.

Adolescent↗
PubMedDate not supplied

Molecular abnormalities in pediatric embryonal brain tumors--analysis of loss of heterozygosity on chromosomes 1, 5, 9, 10, 11, 16, 17 and 22.

Embryonal tumors, the most common group of malignant brain tumors in childhood, are heterogeneous and have been associated with a large number of genetic abnormalities. The aim of this study was to comprehensively analyze loss of heterozygosity (LOH) on regions harboring suppressor genes (PTCH2, PTCH1, APC, PTEN, DMBT1, SUFU, AXIN1, hSNF5/INI1) and to study chromosomal regions in which deletions have been described most frequently (1p, 1q, 11p, 16p, 17p). Twenty-nine children (17 male and 12 female), aged from 1 year 13 years were included in this study. There were 24 medulloblastomas (MB) and 5 supratentorial primitive neuroectodermal tumors (sPNET). Tissue samples from 29 primary and 11 recurrent tumors were analyzed according to the LOH standard procedures, which were extended to include fluorescence in situ hybridization for detection of isochromosome 17q (i(17q)) and direct sequencing ofTP53 exon 4. LOH on 17p was found in 15 out of 29 tumors. FISH analysis identified the presence of i(17q) in 16 tumors. Comparison of LOH analysis and the FISH data indicated that alterations of 17p were related to be the introduction of an i(17q) formation. LOH on 10q and 9q was observed in 4 and 2 cases, respectively, and was associated with alterations of chromosome 17. These results indicated a connection between alterations of PTCH/SHH genes and abnormalities of chromosome 17. A deleted region on 22q, covering the hSNF5/INI1 locus, was observed in 3 tumors. Progression of the molecular changes occurred in 1 case of recurrent medulloblastoma. LOH on 10q and 17p was found in both primary and recurrent tumor, while losses on 11p, 16p, and 16q occurred only in the recurrent tumor. No evidence of alteration in TP53 exon 4 was identified.

Adolescent↗
PubMed1985

Shared antigenic determinants between human hemopoietic cells and nervous tissues and tumors.

A panel of nine monoclonal antibodies raised against human hemopoietic cells was used for immunohistological labeling of frozen sections of human nervous tissues and tumors. Three antibodies showed a remarkably consistent labeling pattern when tested on 18 samples of normal or reactive tissue, on 31 neurogenic and 17 non-neurogenic tumors in an indirect immunofluorescence technique. VIM C6, an antibody recognizing cells of the granulocyte series, showed surface labeling of normal and reactive glial cells and of all types of glioma regardless of the grade of malignancy. VIT 13, an antibody recognizing activated T-cells, labeled the processes of normal, reactive, and neoplastic glia in a manner very similar to but not identical with glial fibrillary acidic protein (GFAP). VIB C5, an antibody recognizing B cells and granulocytes, showed surface labeling restricted to malignant cells (malignant gliomas and primitive neuroectodermal tumors) and fetal brain, thus recognizing, within the nervous system, an oncofetal antigen. Due to this operational specificity within the nervous system, some of the antibodies described here might have a role as diagnostic markers for CNS tumors. This study confirms and expands previous data that sharing of antigenic determinants by hemopoietic cells and nervous tissue or neurogenic tumors is common. However, the significance of such cross-recognition is still obscure. It is tempting to speculate that cross-reacting auto-antibodies might contribute to tissue damage in some immune-mediated neurologic diseases (myasthenia gravis, multiple sclerosis, CNS involvement in systemic lupus erythematosus) or to impairment of immunoregulation in multiple sclerosis or glioma patients. Furthermore, sharing of surface determinants might be responsible for the dual tissue tropism of some viruses, including the lymphotrophic virus (HTLV) in the encephalopathy of the acquired immune deficiency syndrome (AIDS).

Antibodies, Monoclonal↗
PubMed2000

In vivo assessment of the window of barrier opening after osmotic blood-brain barrier disruption in humans.

OBJECT: Osmotic blood-brain barrier (BBB) disruption induced by intraarterial infusion of mannitol is used in conjunction with chemotherapy to treat human brain tumors. The time course to barrier closure, or the so-called therapeutic window, has been examined in animals but little information is available in humans. The authors, therefore assessed the time course to barrier closure after osmotic BBB disruption in humans. METHODS: Disruption of the BBB was demonstrated using 99mTc-glucoheptonate (TcGH) single-photon emission computerized tomography (SPECT) scanning in 12 patients who were treated monthly with combination chemotherapy in conjunction with BBB disruption. The primary diagnosis was primary central nervous system lymphoma in seven patients and primitive neuroectodermal tumors in five. The TcGH (20 mCi) was injected at 1- to 480-minute intervals after osmotic BBB disruption, and patients underwent SPECT scanning after 4 hours. A total of 38 studies was performed. Good-to-excellent BBB disruption was obtained in 29 procedures and poor-to-moderate disruption was seen in the other nine studies. The TcGH indices correlated with the degree of BBB disruption as measured postprocedure on contrast-enhanced CT scans (r = 0.852). Mean baseline TcGH indices were 1.02+/-0.07. For the group of patients with good-to-excellent disruptions the mean indices at 1 minute postdisruption measured 2.19+/-0.18. After 40 minutes no significant change was noted (mean index 2.13+/-0.2). Then the indices declined more steeply and at 120 minutes after the disruption the index was 1.36+/-0.02. A very slow decline was noted between 120 and 240 minutes after mannitol infusion. At 240 minutes the barrier was still open for all good-to-excellent disruptions (index 1.33+/-0.08) but at 480 minutes the mean indices had returned to the baseline level. CONCLUSIONS: Results of these in vivo human studies indicate that the time course to closure of the disrupted BBB for low-molecular-weight complexes is longer than previously estimated. The barrier is widely open during the first 40 minutes after osmotic BBB disruption and returns to baseline levels only after 6 to 8 hours following the induction of good or excellent disruption. These findings have important clinical implications for the design of therapeutic protocols.

Adult↗
PubMed2002

[Neuroradiological findings of relatively rare tumors of the brain].

In the publication by the World Health Organization of the histological classification of central nervous system tumors in 1993, several new tumor types were added, including pleomorphic xanthoastrocytoma (PXA), dysembryo-plastic neuroepithelial tumor (DNT), and desmoplastic infantile ganglioglioma (DIG). Referring to these newly classified tumors of the brain, the present article describes the neuroradiological findings of relatively rare tumors of the brain such as PXA, subependymoma, gangliocytoma, dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease), DIG, central neurocytoma (CN), neuroblastoma, desmoplastic medulloblastoma, atypical teratoid/rhabdoid tumor (ATRT), glossopharyngeal schwannoma, and malignant fibrous histiocytoma (MFH). PXA and DIG affect the cerebral hemisphere and appear to be cystic masses with a solid component on CT and MRI. Gangliocytomas commonly occur in the floor of the third ventricle and the temporal lobe. CT and MRI typically show a mass lesion with no vasogenic edema. In Lhermitte-Duclos disease, T2-weighted MR images reveal characteristic enlarged folia. Desmoplastic medulloblastomas tend to favor the cerebral hemisphere in the adolescent. ATRTs are rarely seen in infants or children and resemble medulloblastomas or primitive neuroectodermal tumors. Intracranial MFHs are also rare tumors and sometimes resemble the meningiomas.

Brain Neoplasms↗
PubMed1997

Specificity of radioiodinated MIBG for neural crest tumors in childhood.

UNLABELLED: The high sensitivity of metaiodobenzylguanidine (MIBG) scintigraphy for sympathomedullary tumors such as neuroblastoma and pheochromocytoma is well documented. The specificity of MIBG scintigraphy for these tumors is also high but has been incompletely characterized for other neural crest tumors and non-neural crest tumors of childhood. METHODS: The medical records and MIBG scans of all children who had undergone MIBG scintigraphy for known or suspected neuroblastoma or pheochromocytoma were retrospectively reviewed at five major referral centers. Those patients found to have pathologies other than neuroblastoma or pheochromocytoma form the basis of this study. RESULTS: One hundred children with a total of 110 lesions met the inclusion criteria. All had negative MIBG scans except 1 of 2 children with infantile myofibromatosis, 1 of 2 with neuroendocrine carcinomas, 1 of 2 with pancreaticoblastomas and 1 of 10 with primitive neuroectodermal tumors. CONCLUSION: MIBG scintigraphy is highly specific for neuroblastoma and pheochromocytoma. Only 4% (4/100) of nonsympathomedullary tumors (non-pheochromocytoma and non-neuroblastoma) in childhood showed MIBG uptake, of which only 2% (2/100) were of non-neural crest origin.

3-Iodobenzylguanidine↗
PubMed1991

The risks of metastases from shunting in children with primary central nervous system tumors.

The authors reviewed the hospital charts of 415 pediatric patients treated for benign or malignant primary brain tumors over the past 20 years at the Children's Hospital Medical Center, Seattle. Patients' ages ranged from the neonatal period to 18 years. A shunt was placed in 152 patients (37%), 45 before and 94 after surgery. Confirmation of extraneural metastases was based on clinical and diagnostic examination. Factors analyzed as possibly influencing the occurrence of extraneural metastases were: 1) the shunt: type, valve, location, filter, and revisions; 2) extent of resection; 3) pathology; and 4) treatment regimen. Eight of the 415 patients developed extraneural metastases during life. All eight patients had a medulloblastoma (cerebellar primitive neuroectodermal tumor). These eight patients were separated into Group A (without a shunt) and Group B (with a shunt). In Group A (five patients), the mean interval from primary diagnosis to metastasis was 15 months. Two children had gross total resection of the tumor. The predominant location of metastases in Group A was: bone (two cases); cervical lymph nodes (one); lung/bone (one); and retroperitoneal pelvic mass (one). Three Group A patients had a simultaneous central nervous system (CNS) recurrence. Of the three Group B patients, two had a ventriculoperitoneal (VP) shunt and one a ventriculoatrial (VA) shunt; all were placed postoperatively. One Group B patient had a simultaneous CNS recurrence. No shunt revisions were performed in these three patients. The mean time from primary diagnosis to metastasis was 25 months. One patient had a total tumor resection. The predominant location of metastases was bone (one case), retroperitoneal pelvic mass (one), and abdominal cavity with ascites (one case). Only one patient in the entire series had a filter placed; this resulted in shunt obstruction and was removed 1 month following placement. It is concluded that cerebrospinal fluid shunts, regardless of type, location, revision rate, or filter insertion, do not predispose pediatric patients with brain tumors to develop extraneural metastases. A diagnosis of shunt-related metastases should be based on the development of intra-abdominal (VP shunt) or pulmonary (VA shunt) dissemination primarily with or without additional sites. The diagnosis of medulloblastoma is an important factor related to metastasis occurrence while the extent of resection and postoperative therapy are not influential.

Adolescent↗
PubMed1996

Pilot study of high-dose thiotepa and etoposide with autologous bone marrow rescue in children and young adults with recurrent CNS tumors. The Children's Cancer Group.

PURPOSE: This study was designed to determine the toxicity, radiographic response rate, and outcome following high-dose thiotepa, etoposide, and autologous bone marrow rescue (ABMR) for young patients with recurrent malignant brain tumors. METHODS: Eligibility criteria required adequate renal, hepatic, and pulmonary function, and no bone marrow infiltration. Thiotepa 300 mg/m2 and etoposide 500 mg/ m2 were infused on 3 consecutive days, and autologous bone marrow was infused 72 hours following chemotherapy. RESULTS: Forty-five patients with recurrent high-grade brain tumors, aged 8 months to 36 years (median, 8 years), were treated. Seven patients (16%) died of treatment-related toxicities within 56 days of marrow reinfusion. Delayed platelet engraftment occurred in 44% of patients who survived beyond day 56. Of 35 patients with radiographically measurable disease who survived at least 28 days following ABMR, there were two complete responses (CRs) and six partial responses (PRs), for an overall response (CRs plus PRs) rate of 23% (SE = 7%). Objective responses were observed in four of 14 assessable patients with high-grade glioma and in two of six with primitive neuroectodermal tumors (PNETs)/ medulloblastoma. Survival was significantly improved in patients treated with minimal residual disease (P < .0005). Five of 18 patients (28%) with high-grade gliomas remain free of disease at 39+, 44+, 49+, 52+, and 59+ months post-ABMR. CONCLUSION: The combination of high-dose thiotepa and etoposide has activity against a variety of recurrent childhood brain tumors. These results merit further evaluation in children and young adults with both recurrent and newly diagnosed high-grade brain tumors.

Adolescent↗
PubMed1998

Blood-brain barrier disruption for the treatment of malignant brain tumors: The National Program.

Chemotherapy delivery for the treatment of malignant brain tumors is markedly enhanced when given in conjunction with osmotic opening of the blood-brain barrier. Osmotic opening or disruption of the blood-brain barrier is achieved while the patient is under general anesthesia, by the infusion of mannitol into the internal carotid or vertebral artery circulation. The mannitol infusion is followed by administration of intraarterial chemotherapy. A National Blood-Brain Barrier Program now exists and includes six universities. Within the National Program over 4200 blood-brain barrier disruption procedures have been performed in over 400 patients. Patients with primary central nervous system (CNS) lymphoma, glioma, primitive neuroectodermal tumor (PNET), germ cell and metastatic cancer are eligible for treatment. Results in patients with primary CNS lymphoma, recently reported in the Cancer Journal, include the first example of a durable response in a primary brain tumor without loss of cognitive function and without use of radiotherapy. Results with PNET and germ cell tumors are also very encouraging. Advanced practice nurses coordinate the care of blood-brain barrier disruption patients. Care includes patients selection, education, close neurological observation, maintenance of fluid and electrolyte balance and managing effects of high-dose chemotherapy. Both acute and long-term medical and psychological follow-up are an essential component of the program, as well as patient and family support.

Antineoplastic Agents↗
PubMed1999

Etoposide with or without mannitol for the treatment of recurrent or primarily unresponsive brain tumors: a Children's Cancer Group Study, CCG-9881.

PURPOSE: This study was undertaken to evaluate the response of recurrent brain tumors to intravenous etoposide and to evaluate the efficacy of mannitol in augmenting etoposide's tumoricidal effect. PATIENTS AND METHODS: Ninety-nine children between one and 21 years of age with recurrent brain tumors were randomly assigned to treatment with intravenous etoposide 150 mg/M2, with or without mannitol 15 gm/M2, daily for five days every three weeks for one year or until disease progression or death. Computerized tomographic (CT) or magnetic resonance image (MRI) scans, obtained after three cycles of therapy, were compared with pre-therapy scans. Scans were centrally reviewed. RESULTS: Of 87 evaluable patients, 12 (13.8%) were determined to have had an objective response by the institutional radiologist. On central review, 7/66 (10.6%) responses were documented. Responses in centrally reviewed patients were observed in 2/12 (16.7%) low grade astrocytomas, 4/26 (15.4%) medulloblastoma or primitive neuroectodermal tumors (PNET), 1/13 (7.7%) high grade astrocytomas and 0/15 (0%) brain stem gliomas. Survival at one year was 53% (SE 12%) for low grade astrocytomas, 38% (SE 7%) for medulloblastoma or PNET, 28% (SE 10%) for high grade astrocytomas and 9% (SE 5%) for brain stem gliomas. An effect of mannitol was not observed. CONCLUSION: Intravenous etoposide has a low level of activity in the treatment of recurrent low grade astrocytomas and medulloblastoma or PNET. The efficacy of this agent was not enhanced by the coincident intravenous administration of mannitol.

Adolescent↗
PubMed1982

Infantile small cell gliomas.

Fourteen juvenile patients with small cell gliomas were studied at two institutes. These tumors are believed to form a distinct entity. They arise mostly in the diencephalon or the brain stem and are composed of a poorly differentiated small cell component having a pronounced tendency to differentiate into a glioma. Signs of neuroblastic differentiation were also found with the electron microscope. Small cell gliomas disseminate early and profusely throughout the ventricular walls and the subarachnoid spaces including the spinal meninges. Prognosis is grave, most patients dying within 1 year of diagnosis or surgical intervention. The designation "infantile small cell glioma" overlaps with both the "metastasising gliomas in young subjects" of Eade and Urich (1971) and with the primitive neuroectodermal tumor of infancy of Hart and Earle (1973).

Adolescent↗
PubMed2003

Surgical outcome following resection of contrast-enhanced pediatric brainstem gliomas.

BACKGROUND: The role of surgery in the management of gadolinium-enhancing pediatric brainstem lesions on magnetic resonance imaging (MRI) has been a matter of open debate. This clinical series correlates radiological and pathological findings to assess the role of contrast enhancement as an indication for surgery with respect to clinical outcome. METHODS: We retrospectively reviewed the charts of all pediatric patients admitted to the Johns Hopkins Hospital with a diagnosis of a brainstem tumor between January 1985 and December 2000. RESULTS: There were a total of 89 patients who met the inclusion criteria. Fifty-seven patients (64.0%) underwent surgical resection while 32 (36%) were treated with radiation and/or chemotherapy. Of the surgical candidates, 57 (100%) had an accompanying MRI scan significant for an enhancing lesion in the midbrain, pons or the medulla. The pathology was consistent with juvenile pilocytic astrocytoma in 30 patients (52.6%) and glioblastoma multiforme in 12 patients (21.1%). The remaining cases consisted of 10 patients (17.5%) with fibrillary astrocytomas, 3 (5.3%) with gangliogliomas, 1 (1.8%) with an oligodendroglioma and 1 (1.8%) with a primitive neuroectodermal tumor. A total of 29 patients had a total surgical resection, 8 a near total resection (>90%), 15 a subtotal resection (50-90%) and 5 a partial resection (<50%). The progression-free survival of all patients was 71.9% at 3 years and 45.6% at 5 years. CONCLUSIONS: This case series illustrates that contrast-enhanced MRI has positive prognostic value in the management of pediatric brainstem gliomas. In our study, the majority of enhancing tumors were low grade and amenable to surgical intervention. Consequently, we recommend surgical resection and pathological diagnosis of all enhancing brainstem tumors with adjuvant therapy reserved for recurrent or unresectable cases.

Adolescent↗