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PubMed1991

Is treatment intensification by adding etoposide and carboplatin to fractionated total body irradiation and melphalan acceptable in children with solid tumors with respect to toxicity?

Conventional chemotherapy results in high mortality rates in patients with solid tumors involving the bones or the bone marrow. High dose melphalan (MEL) with or without total body irradiation followed by bone marrow transplantation (BMT) has prolonged survival, but curative potential has remained disappointing. In order to improve survival 20 children with generalized or relapsed solid tumors (neuroblastoma, peripheral neuroectodermal tumor, Ewing's sarcoma, rhabdomyosarcoma) underwent autologous (n = 16) or allogeneic (n = 4) BMT. The myeloablative regimen consisted of 12 Gy fractionated total body irradiation (FTBI) and high dose MEL. In 12 of these patients this regimen was intensified by giving 60 mg/kg etoposide (1800 mg/m2 VP), and 1.5 g/m2 carboplatin (CBDCA) was added in seven of these 12 patients. The intensification of FTBI and MEL by adding VP and CBDCA was followed by acceptable toxicity. Acute liver toxicity in 15/20 patients (75%) and acute renal toxicity in 17/20 patients (85%) did not exceed WHO grade 1. The use of the conditioning regimen FTBI-MEL-VP-CBDCA during first chemotherapy response is a promising approach in treatment of children suffering from generalized solid tumors.

Adolescent↗
PubMed1990

Positron emission tomography of fluorine-18-deoxyglucose and image-guided phosphorus-31 magnetic resonance spectroscopy in brain tumors.

Positron emission tomography (PET) of 2(18F)-fluoro-2-deoxy-D-glucose (FDG) and volume-selective phosphorus-31 magnetic resonance spectroscopy (31P-MRS) are methods used to assess the energy metabolism of the brain. Both methods were studied with respect to their contribution to differential diagnosis in 23 patients with various brain tumors. The various neuroectodermal tumors differed with respect to their metabolic rate for glucose (MRGL). Benign and malignant tumors could be better differentiated by using tumor metabolism relative to contralateral brain and by evaluating heterogeneities in tumors. Low-grade gliomas usually showed normal 31P-MR spectra; high-grade gliomas were characterized by reduced and often split phosphodiester peaks and alkaline pH. Meningiomas, which had variable MRGL, typically showed extremely low phosphocreatine levels, reduced phosphodiesters, and alkaline pH. We concluded that FDG-PET and 31P-MRS examine different aspects of tumor metabolism. Therefore, both can contribute independently and complementarily to the differential diagnosis of brain tumors.

Astrocytoma↗
PubMed1989

[Surgery of lung metastasis in childhood].

From 1975 to 1987, we have operated 20 cases with lung metastases. They were 12 females and 8 males with ages between 1,6 and 12 years old (average of 5 years). The histology of the primary neoplasia was: 13 cases with Wilms' Tumors, 3 cases with Ewing's Sarcoma and 1 case with Neuroblastoma, Rhabdomiosarcoma, Hepatoblastoma and Neuroectodermic Tumor of Askin. We found 38 metastases and there were performed through a Thoracotomy 28 wedge resection, 7 pulmonary lobectomies and 3 biopsies. The operative mortality was 0%. The most frequent location of the lesion were on medium and lower lobule of the right lung. The survival was 70% (14 cases) after 4 years of average of the follow up. The survival for Wilms Tumors was 61% (8 cases) and all patients with Ewing's Sarcoma, Rhabdomiosarcoma, Neuroblastoma and Neuroectodermic Tumor of Askin were alive. The Thoracotomy is a useful method for therapy of tumors in children with lung metastases associated to chemotherapy and radiotherapy.

Child↗
PubMed1985

Chromosome 1 abnormalities: a common feature of pediatric solid tumors.

Abnormalities of chromosome 1 were found in 32 of 46 pediatric solid tumors including Ewing's sarcoma, Wilms' tumor, rhabdomyosarcoma, primitive neuroectodermal tumor, and hepatoblastoma. Trisomy of 1q was the most common abnormality, and breakpoints were most frequent in the region 1cen to 1p22. Abnormalities of chromosome 1 are not specific to any type of tumor. However, their frequent occurrence indicates that they may endow a clonal advantage in the development of cancer.

Aneuploidy↗
PubMed1986

Computed tomographic characteristics of primary brain tumors in 50 dogs.

Fifty histologically identified primary brain tumors in the dog were analyzed by computed tomography to establish criteria for identifying tumor types by computed tomography characteristics. Meningiomas could be distinguished from tumors within the brain parenchyma because they usually were broad-based, peripherally located masses that were enhanced homogeneously with contrast material. Among parenchymal tumors, astrocytomas were not distinguished easily from oligodendrogliomas because both tumors had similar features of ring-like and nonuniform enhancement, and poorly defined tumor margins. Choroid plexus tumors were seen as well-defined, hyperdense masses that had marked, uniform contrast enhancement. Pituitary tumors were distinguished readily by their location, minimal peritumoral edema, uniform contrast enhancement, and well-defined margins. Distinguishing features of other less frequently seen tumors (ependymoma, primitive neuroectodermal tumor, glioma, and neoplastic reticulosis) were not identified.

Animals↗
PubMed1998

[Teratoid/rhabdoid tumor of the central nervous system].

Teratoid/rhabdoid tumor is a recently introduced infantile brain neoplasm of uncertain origin clinically characterized by a most aggressive course. We describe the case of a 2-year-old boy and a 7-year-old girl, the former presenting with central fever and hemiparesis, while vomiting and headache were symptoms in the latter. A brain tumor in the right fronto-parieto-occipital and the left fronto-medial parenchyma, respectively, were found and removed by surgery. On histology, both lesions consisted of sheets of ovoid cells with prominent cytoplasm, vesicular nuclei and macronucleoli. In the second case, a spindle-cell component and bone metaplasia were also noted. Ultrastructurally, tumor cells contained whorls of intermediate filaments. Immunohistochemistry revealed a polyphenotypic expression profile including positivity for epithelial membrane antigen, vimentin and a-actin. There was no evidence of a germ-cell origin. Chromosomal translocation involving 22q11 was established cytogenetically in the first case. The teratoid/rhabdoid tumor shares many common traits with renal rhabdoid tumors and primitive neuroectodermal tumors as well as germ cell neoplasms; therefore its being an entity is debated.

Brain Neoplasms↗
PubMed1993

Round cell tumours of bone.

Ewing's sarcoma is a very rare tumor which has, however, attracted much oncological interest since the dramatic improvement of its prognosis under chemotherapy. Its histogenesis has been discussed controversially for a long time, including a possible origin in immature reticulum, myogenous, endothelial and undifferentiated mesenchymal cells. Repeated reports have also suggested a possible neuroectodermal genesis. Convincing arguments, however, have only been brought forward during recent years, since it was found out that Ewing's sarcoma and malignant peripheral neuroectodermal tumor share a common chromosome translocation 11;22. In the meantime this hypothesis has been strengthened by numerous cell biological analyses. Histological differential diagnosis of Ewing's sarcoma has been improved by immunohistological methods. In most cases, they can be distinguished from lymphoma (leucocyte common antigen, B and T markers) and embryonal rhabdomyosarcoma (muscle specific actin, desmin). Apart from this, we now have an antibody specific for Ewing's sarcoma and malignant peripheral neuroectodermal tumors but not with neuroblastoma. Recent investigations regarding the prognosis under chemotherapy have shown that tumors with neural differentiation have a comparatively poor prognostic outcome. This is why in addition to the demonstration of Homer Wright pseudo-rosettes different neural markers such as neuron specific enolase, synaptophysin and chromogranin are to be analysed. Small cell osteosarcoma is a very rare sub-entity of osteosarcomas. Recent studies have shown that the tumor resembles Ewing's sarcoma with partial mesenchymal differentiation including osteoid formation.

Adolescent↗
PubMed1999

Immunohistochemical evaluation of small round cell tumors of childhood.

OBJECTIVE: This study was done to evaluate the pediatric undifferentiated small round cell tumors with immunohistochemical staining. SETTING: The present study included consecutive cases of small round cell tumors which were diagnosed in children (< 15 years) in the section of Histopathology at the Aga Khan University Hospital, Karachi during the period of two years. METHODS: The group of undifferentiated small round cell tumors were evaluated immunohistochemically by using a panel of antibodies on sections from routinely processed, formalin fixed, paraffin embedded tissue blocks. RESULTS: The category of undifferentiated small round cell tumors included rhabdomyosarcoma (23.2%), primitive neuroectodermal tumor (17.9%), non-Hodgkin's lymphoma (16.1%), neuroblastoma (14.2%), Ewing's sarcoma (10.7%) in order of frequency. Osteosarcoma (Small cell variant), retinoblastoma and medulloblastoma comprised 1.8% each. In seven cases (12.5%), the immunohistochemical analysis was inconclusive. CONCLUSION: Immunohistochemistry is a very valuable diagnostic tool which helps in distinguishing the undifferentiated tumors especially small round cell tumors. The immunohistochemical staining needs to be performed routinely for undifferentiated tumors in diagnostic histopathology.

Adolescent↗
PubMed1989

Biological aspects of brain tumors in infancy and childhood.

Notwithstanding the definitive systematization of the clinical features of childhood brain tumors, many biological laws governing this vast area of pathology still escape us. There are no sure explanations for the fickleness of supratentorial/subtentorial distribution in fetal life and in the first 15 years of life--something not found in later years. Another focus of discussion is the tendency of brain tumors in infancy to concentrate along the midline due to the fact that most infantile neuroectodermal tumors arise from the phylogenetically older structures of the CNS (periventricular regions, brainstem, cerebellum), in contrast to tumors of adulthood. Neuroepithelial tumors, much more frequent in childhood than in later years, exhibit substantial histological differences in infancy: mixed gliomas and primitive neuroectodermal tumors (not otherwise specified, or with astrocytic, ependymal, oligodendrocytic...cells) are the best examples. As to the question of whether a given oncotype exhibits different biological behavior according to patient age, there is no single answer: some malignant tumors (medulloblastomas, ependymomas, neuroblastomas) are more aggressive in infancy, in line with Collins' law, while others (optic gliomas) offer a better prognosis in younger patients. The most peculiar and disquieting aspect of brain tumors in infancy/childhood, however, is what emerged from a recent epidemiological survey. The survey was conducted on the close relatives of a boy with a brain tumor from point of view of a possible "second malignancy" in this boy. Since a brain tumor is a rare occurrence in the very young, it may signal heightened susceptibility to malignancy in the individual, extending to other tumors in the patient himself and even to other members of his family.

Brain↗
PubMed2000

Miscellaneous rare paratesticular tumors.

A few uncommon but distinctive tumors may preferentially involve the paratestis. The 3 unusual tumors that represent the focus of this discussion are the ovarian-type epithelial tumors (OTET), the desmoplastic small round cell tumor (DSRCT), and the melanotic neuroectodermal tumor of infancy (MNTI). The OTETs are testicular homologues of their more common namesake counterparts that arise in the ovary. Most frequent of these are serous tumors of borderline malignancy, with fewer cases of serous carcinomas or other forms of mullerian differentiation. DSRCT is an increasingly recognized, aggressive, "small blue cell" neoplasm with distinctive clinical and pathologic features. These polyphenotypic tumors characteristically, but not invariably, arise in intimate association with the serosal membrane of the peritoneal cavity and harbor a signature translocation-t(11;22)(p13,q12). In the paratestis they often involve the surface of the epididymis. The MNTI is an enigmatic, histologically distinctive, low-grade neoplasm occasionally encountered in the epididymis. Recognition of its features is essential to avoid misdiagnosis as a more aggressive "small blue cell" neoplasm and consequent therapeutic mismanagement. Primary hematopoietic tumors of the paratesticular structures are rare. There appears to be a tendency for young men to have low-grade lymphomas with an indolent course and older patients to develop higher-grade tumors. Plasmacytoma and granulocytic sarcoma of the paratestis are even more rare and are often susceptible to misinterpretation. Finally, metastatic tumors and a variety of other very rare neoplasms are discussed.

Adolescent↗
PubMedDate not supplied

Primary intracranial peripheral primitive neuroectodermal tumor/Ewing's sarcoma presenting with acute intracerebral hemorrhage.

OBJECTIVE: To report two cases of intracerebral hemorrhage due to primary intracranial peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) and review of related literatures. MATERIAL: Two cases of 17-year-old patients presented with acute increased intracranial pressure one of which also had left hemiparesis. METHOD: On neuroimaging studies, the first patient had an intraparenchymal hematoma with a size of 4 cm at the right fronto-parietal junction adjacent to tumor infiltrating the superior sagittal sinus. The second patient had a large left temporal tumor with intraventricular hemorrhage. Both patients underwent craniotomy with complete removal of tumor and hematoma. RESULTS: Pathological examination in both cases revealed numerous small round tumor cells with stippled chromatin pattern and scanty cytoplasm. Tumor cells strongly expressed CD99. Vimentin immunoreactivity was observed. The final diagnosis of pPNET/ES was rendered. There was no evidence of extracranial disease in both cases. Both patients were doing well without evidence of recurrent disease at 12 and 24-month follow-up respectively. CONCLUSIONS: Peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) is a malignant small round cell tumor, commonly arising in soft tissue of the trunk and lower extremity. Those occurring in the intracranium are rare, and most patients present with progressively increased intracranial pressure and/or cranial nerve deficit. The occurrence of intracerebral hemorrhage due to primary intracranial pPNET/ES is exceedingly rare. The role of adjuvant therapy in this condition is yet to be investigated.

Acute Disease↗
PubMed1998

Phase II trial of topotecan administered as 72-hour continuous infusion in children with refractory solid tumors: a collaborative Pediatric Branch, National Cancer Institute, and Children's Cancer Group Study.

The antitumor activity of topotecan administered as a 72-h continuous i.v. infusion was evaluated in children with refractory neuroblastoma and sarcomas of soft tissue and bone. We also attempted to increase the dose intensity of topotecan by including an intrapatient dose escalation in the trial design. Ninety-three children (85 eligible and evaluable for response) with recurrent or refractory neuroblastoma, osteosarcoma, Ewing's sarcoma/peripheral neuroectodermal tumor, rhabdomyosarcoma, or other soft-tissue sarcomas received topotecan administered as a 72-h i.v. infusion every 21 days. The initial dose was 1.0 mg/m2/day, with subsequent intrapatient dose escalation to 1.3 mg/m2/day for those patients who did not experience dose-limiting toxicity after their first cycle of topotecan. There was one complete response in a patient with neuroblastoma (n = 26) and one partial response in a patient with Ewing's sarcoma/peripheral neuroectodermal tumor (n = 25). No complete or partial responses were observed in 17 patients with osteosarcoma, 15 patients with rhabdomyosarcoma, or 2 patients with other soft-tissue sarcomas; however, 8 patients had prolonged (15-48 weeks) stable disease while receiving topotecan. Topotecan was well tolerated. The most commonly observed toxicities were myelosuppression (dose-limiting) and nausea and vomiting. Intrapatient dose escalations were performed in 68% of the patients who received more than one cycle of topotecan, and 1.3 mg/m2/day was tolerated by 79% of the patients who received the higher dose and were evaluable for hematological toxicity. In conclusion, topotecan administered as a 72-h continuous infusion every 21 days is inactive (objective response rate, < 20%) in children with refractory or recurrent neuroblastoma and sarcomas of soft tissue or bone.

Adolescent↗
PubMed2006

Central nervous system tumors in patients under three years of age: treatment results of a single institute.

Eighty-six patients under 3 years of age with central nervous system tumors were retrospectively analyzed between 1972 and 2003. Surgical resection was done in all patients except for those with optic glioma, pons glioma and pineal tumor. Three different chemotherapy regimens were used in different time periods. In 48 patients, the tumor was located in the posterior fossa, and 29 patients had a supratentorial tumor. We had 32 (37.2%) patients with embryonic tumors (21 medulloblastoma, 4 ependymoblastoma, 5 with atypical teratoid rhabdoid and 2 with supratentorial primitive neuroectodermal tumors), 21 (24.4%) with ependymoma, 14 (16.3%) with optic glioma, 10 (11.6%) with astrocytoma, 3 (3.5%) with pons glioma and 6 (7.0%) with others. Overall (OS) and event-free survival rates were 49.5 and 40.9%. OS rates according to the tumor localizations were 40.9 and 68.1% in the posterior fossa and supratentorial localizations, respectively (p=0.001). OS rates were 33.7, 41.3 and 88.8% for the medulloblastoma+primitive neuroectodermal tumor groups, ependymoma and astrocytoma, respectively (p=0.0001). Most of the patients had primitive embryonic tumors (37.2%). The best prognostic factors were tumor localization and histology.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

[Epidemiology of primary tumors of the central nervous system in the 1st year of life].

148 children under the age of 5 have been identified from the files of the National Cancer Registry of the GDR. They all had primary tumors of the central nervous system (CNS), which were histopathologically diagnosed in the period 1975 to 1980. Additional information concerning their health status and diseases was collected from birth history, postnatal care-unit files, out- and in-patient medical records and other sources. Based on these materials the time of clinical onset of the disease has been elucidated. In 12 of the 148 patients the onset was in the perinatal period and in another 36 during the first year of life. 1,322,450 births took place in the GDR in the index period. The incidence of primary tumors of the CNS with onset of signs and symptoms prior to the end of the first year of life was 3.6 per 100,000 births. The most common type of tumor was astrocytoma, with an incidence of 0.9/100,000. It is followed in frequency by other neuroectodermal tumors and by intracranial teratomas. No meningiomas or pituitary adenomas had their onset in infancy. The conclusion is drawn that the incidence of neuroectodermal tumors is almost the same in newborns and infants as it is in older age groups. The findings contradict the genera view, that CNS tumors in infants are very rare.

Astrocytoma↗
PubMed1997

Primary intracerebral malignant fibrous histiocytoma: immunohistochemical findings and etiopathogenetic considerations.

We report a case of malignant fibrous histiocytoma (MFH) arising as a second malignancy in a 13-year-old girl with a previous primitive neuroectodermal tumor of the cerebellum (medulloblastoma). The earlier tumor had been resected and treated with chemotherapy. Because of the age of the patient, no radiation therapy was used. The second neoplasm (an MFH), which presented almost 12 years later, was located within the left frontal region and had discrete meningeal attachment. Histologically, it showed the characteristic storiform pattern and pleomorphism of an MFH and displayed characteristic immunohistochemical reactivity. Careful clinical and radiologic investigation failed to reveal any other neoplasm in the body. This case is unique in that the MFH occurred in a young child after an earlier primary primitive neuroectodermal tumor, although at a distant site, and, importantly, without prior radiation therapy. In this report, we briefly review the literature with an emphasis on immunohistochemistry and histogenesis, raising the possibility that the MFH was induced by chemotherapy.

Adolescent↗
PubMed2000

Tumor-derived p53 mutant C174Y is a gain-of-function mutant which activates the fos promoter and enhances colony formation.

SV40 large T antigen-induced primitive neuroectodermal tumors of the rat provide a model system to study induction and progression of primitive neuroectodermal tumors at the molecular level. A cell line derived from such a tumor reproducibly gave rise to malignant derivatives that ceased large T-antigen expression but harbored a mutant p53 allele with a common mutation at Cys(174) to Tyr (C174Y). To determine whether this p53 mutation contributes to tumor progression, we analyzed mutant C174Y functionally. Co-transfection experiments in Saos-2 cells with mutant or wild-type p53 and reporter genes linked to various p53-responsive promoters revealed that mutant C174Y failed to transcriptionally transactivate the Mdm2, Waf1, Cyclin G and Bax promoters. Loss of transcriptional activation correlated with loss of DNA-binding activity. Moreover, mutant C174Y exhibited a dominant negative effect on co-expressed wild-type p53. The ability of mutant p53 to repress the viral RSV, LTR or SV40 early promoters or the cellular fos promoter was likewise impaired. In contrast, it showed even induction of the fos promoter. Consistent with these observations, mutant C174Y was non-functional in the suppression of Saos-2 cell growth and even conferred a growth advantage to the cells. Surprisingly, mutant C174Y was also impaired in nuclear transport, as revealed by immunofluorescence analyses. Taken together, our results demonstrate that mutant C174Y possesses features that can positively contribute to cancer progression.

Amino Acid Substitution↗
PubMed1997

[Pathologic diagnosis on bone and soft tissue tumors by molecular biological methods].

Characteristic chromosome aberrations and the rearranged genes resulting in chimeric fusion genes have been reported in some bone and soft tissue tumors; t(X; 18) in synovial sarcoma, t(11; 22) in Ewing's sarcoma and primitive neuroectodermal tumor, and t(2; 13) in alveolar rhabdomyosarcoma. We practically used the chromosome analysis and the reverse transcription-polymerase chain reaction (PCR) method as a tool for diagnosis and follow up. All of 10 cases of synovial sarcoma had a chimeric product of SYT/SSX gene. Eleven cases of Ewing's sarcoma and primitive neuroectodermal tumor showed 6 variants of chimeric products between EWS gene and Fli1 gene in the PCR-directed sequence analysis. Although PAX3/FKHD or PAX7/FKHD transcripts were amplified in alveolar rhabdomyosarcoma cases, MyoD1 and myogenin gene which are myogenic transcription factor were also expressed in most rhabdomyosarcomas. These findings indicate that molecular biological analysis may be a useful supplementary method for pathologic diagnosis of bone and soft tissue tumors.

Adolescent↗
PubMed1998

Farm and animal exposures and pediatric brain tumors: results from the United States West Coast Childhood Brain Tumor Study.

Nineteen counties from San Francisco and Los Angeles, California and Seattle, Washington were the United States sites for a large population-based case-control study of childhood brain tumors (CBTs), sponsored by the National Cancer Institute. CBT patients who were < 20 years of age and were diagnosed between 1984 and 1991 were reported to each region's cancer registry. The 801 control subjects were obtained by random digit dial and were frequency-matched to the 540 CBT patients in San Francisco and Seattle (one patient to two controls) and in Los Angeles (one patient to one control). Data collected by in-person interview with subjects' mothers were analyzed to investigate an association between risk for CBTs and life on a farm, exposure to farm animals (dairy cattle, beef cattle, pigs, sheep/goats, poultry, and horses), and some cat and non-farm horse exposures. Elevated risks for CBTs were observed in association with mothers' exposure to pigs [odds ratio (OR) = 3.8, 95% confidence interval (CI) = 1.2-12] and horses (OR = 2.2, 95% CI = 1.0-4.8) on a farm during the index pregnancy. Children diagnosed with primitive neuroectodermal tumors showed elevated risks for CBTs with personal and maternal prenatal exposure to pigs (child, OR = 4.0, 95% CI = 1.2-13; mother, OR = 11.9, 95% CI = 2.8-51) and poultry (child, OR = 3.0, 95% CI = 1.1-8.0; mother, OR = 4.0, 95% CI = 1.2-14). No other animal exposures of children or mothers were found to be consistently related to CBTs. Children diagnosed with primitive neuroectodermal tumors who were on a farm for > 1 year and were first on a farm when they were < 6 months of age also had increased risk for CBTs (OR = 3.9, 95% CI = 1.2-13). A somewhat increased risk for CBTs was found for children of mothers who ever had worked on livestock farms compared with mothers who never had worked on a farm (OR = 7.4, 95% CI = 0.86-64, based on five case mothers and one control mother who worked on livestock farms during the 5 years preceding the birth of the index child). The associations are consistent with those of two previous studies in Norway (P. Kristensen et al., Int. J. Cancer, 65: 39-50, 1996) and the United States and Canada (G. R. Bunin et al., Cancer Epidemiol. Biomark. Prev., 3: 197-204, 1994) that investigated the role of farm-related exposures in the etiology of CBTs.

Adolescent↗