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PubMed1984

Malignant peripheral nerve tumors: a clinicopathological and electron microscopic study.

Sixteen cases of malignant peripheral nerve tumors that were recorded in the files of the Department of Orthopedics, National Cancer Center Hospital, Tokyo, between 1972-July 1983 were studied clinicopathologically. The patients' ages ranged from 24-51 years, and both sexes were affected equally. Histologically, in 13 cases of nerve sheath tumors the tumors were spindle-cell type, two of these patients had manifestation of multiple neurofibromatosis (von Recklinghausen's disease). One malignant epithelioid schwannoma was found to arise from the tibial nerve. Other two cases were of primitive neuroectodermal tumors (primary malignant peripheral neuroblastoma) which showed rosette formation. The common primary symptoms in all patients were a noticeable mass which increased in size over a variable period of time, with or without associated pain and tenderness. Ultrastructural findings of spindle-cell type (in 7 tumors examined) and epithelioid type (1 tumor) showed evidence of Schwann cell differentiation of the tumors in all cases. Immunohistochemically, by the PAP method (Sternberger), staining for S-100 protein was positive in 3 of 14 tumors. Ultrastructural findings in two S-100 protein-positive cases showed evidence of Schwann cell differentiation better than the S-100 protein-negative cases, such as pronounced interdigitation of cytoplasmic processes, presence of fibrous long-spacing collagen and well-developed basal lamina. Local recurrence occurred in nine patients, and metastasis was found in five. The total 5-year survival rate was 58.5%. Tumors associated with multiple neurofibromatosis and primary peripheral neuroblastomas had the worst prognosis. Complete removal of the tumor by means of wide excision as primary treatment seemed to be the most important factor in decreasing the morbidity and mortality rates.

Adult↗
PubMed1996

Trilateral tumors in four different lines of transgenic mice expressing SV40 T-antigen.

PURPOSE: A line of transgenic mice containing the simian virus (SV) 40 T-antigen (T-ag) gene driven by the beta-luteinizing hormone (BLH) promoter developed bilateral retinoblastoma and primitive neuroectodermal tumors (PNET) of the midbrain. Midbrain tumors arose from the subependymal layer of the cerebral aqueduct. Bilateral ocular and brain tumors ("trilateral") were found in three other SV40 T-ag transgenic murine lines containing different promoters (murine interphotoreceptor retinoid-binding protein (IRBP), human IRBP, and alpha A-crystallin). To gain insight into the regulatory mechanisms involved in central nervous system tumorigenesis, the authors examined brain tumors from four lines of SV40 T-ag mice with different promoters. METHODS: Formalin-fixed brain tumors were examined from four lines of transgenic mice containing different promoters linked to the protein coding region of the enhancerless SV40 T-ag oncogene. Transgenes contained the following promoters: BLH, mouse 1.8-kb IRBP, human 1.3-kb IRBP, and alpha A-crystallin. RESULTS: Mice with a 1.8-kb IRBP promoter develop retinal photoreceptor and pineal tumors. Intracranial tumors arising from the subependymal layer of the third ventricle also were observed. Mice with a 1.3-kb IRBP promoter exhibit bilateral retinal PNET and PNET originating from the subependymal layer of the third ventricle. Mice with the alpha A-crystallin promoter exhibit bilateral lens tumors and PNET of the midbrain. CONCLUSIONS: Ocular tumors in these mice may be ascribed to the promoter-driven, tissue-specific expression of SV40 T-ag. The common finding of PNET arising from the subependymal layer of the diencephalon is unlikely to be promoter related. These findings indicate that a regulatory region specific to the subependymal layer of the cerebral aqueduct and third ventricle resides in the structural region of the SV40 T-ag gene.

Animals↗
PubMed1989

Embryonal neuroepithelial tumors induced by human adenovirus type 12 in rodents. 1. Tumor induction in the peripheral nervous system.

Human adenovirus type 12 (Ad 12) was inoculated intraperitoneally, intrapleurally, intramuscularly or subcutaneously into newborn rodents. Tumors developed preferentially in the peritoneal cavities in 93.9% of the hamsters and 82.6% of the mice, but none in rats; in contrast to the high incidence of brain tumors in rats when the virus is injected intracranially. Serial section of peritoneal tissues and muscle of hamsters revealed multicentric microtumors with a close relation to peripheral nerve fibers 10 to 35 days after virus inoculation. Histologically, most tumors consisted of closely packed, irregularly arranged, small spindle or tadpole-shaped cells. However, divergent morphological differentiation showing palisade arrangement of spongioblastic tumor cells forming trabeculae, pseudorosettes with or without central blood vessel, and true rosettes of immature ependymal (ependymoblastic) or medulloepithelial type were observed. No further differentiation was detected on immunohistochemical or electron microscopical examination of the tumor cells. The immature neuroepithelial phenotypes and the early stages of tumor development indicated that Ad 12 had a definite affinity for embryonic neuroepithelial elements that have migrated along the peripheral nerve fibers of newborn hamsters and mice, perhaps with cells of neural crest origin, and had induced primitive neuroectodermal tumors as observed in human peripheral neuroepithelioma and medulloepithelioma.

Adenoviridae Infections↗
PubMed2005

Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.

Adult medulloblastoma is a rare tumor with few retrospective studies published so far. The role of adjuvant chemotherapy or chemotherapy at relapse is unclear. This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002. The median age was 24.5 years (range 16-76). After resection, 16 patients were treated with craniospinal radiotherapy alone, 20 patients also received adjuvant chemotherapy (8 vincristine, CCNU, cisplatin; 7 methotrexate alone or methotrexate/vincristine-based polychemotherapy; 5 other protocols). Median survival in the whole cohort was 126 months (2+ - 200+months). Five-year and 10-year survival rates were 79 % and 56%. Adjuvant chemotherapy was associated with a non-significant trend to prolonged survival (relative risk (RR) 1.89; p=0.068). The median progression-free survival (PFS) after primary therapy was 83 months. At relapse, 10 of 12 evaluable patients achieved a complete response upon second-line therapy. The median survival times from first (n=17) and second relapse (n=9) were 21 months (0-67+ months; 5/17 without second relapse) and 20 months (1-29 months). Cox regression analysis revealed the infiltration of the floor of the 4(th) ventricle at diagnosis as the only therapy-independent prognostic factor (RR 0.48; p=0.03). In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients. Moreover, second-line therapy may be beneficial for these patients. As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.

Adolescent↗
PubMed2002

II. Perinatal brain tumors: a review of 250 cases.

Central nervous system tumors occur considerably less often in the fetus and neonate than in the older child. They are not entirely the same as those present later in life. Their location, biologic behavior, response to therapy, and histologic types are different. Fetal and neonatal brain tumors (n = 250) were collected from the literature and studied for this review. The overall survival rate was 28%. The entire cranial cavity may be filled with tumor, and stillbirth is not uncommon. Macrocephaly was the most frequent presentation regardless of histology. Outcome is related to the size and location of the tumor, the histologic type, surgical resectability, and the condition of the infant at the time of diagnosis. Neonates with choroid plexus papillomas, gangliogliomas, and low-grade astrocytomas have the best prognosis, whereas those with teratomas and primitive neuroectodermal tumors have the worst prognosis.

Brain Neoplasms↗
PubMed2002

I. Perinatal brain tumors: a review of 250 cases.

Central nervous system tumors occur considerably less often in the fetus and neonate than in the older child. These tumors are not entirely the same as those found later in life. Their location, biologic behavior, response to therapy, and histologic types are different. Reports of 250 fetal and neonatal brain tumors were collected from the literature and studied for this review. The overall survival rate was 28%. The entire cranial cavity may be filled with tumor, and stillbirth is not uncommon. Macrocephaly was the most frequent presentation regardless of histology. Outcome is related to the size and location of the tumor, the histologic type, surgical resectability, and the condition of the infant at the time of diagnosis. Neonates with choroid plexus papillomas, gangliogliomas, and low-grade astrocytomas have the best prognosis, whereas those with teratomas and primitive neuroectodermal tumors have the worst prognosis.

Brain Neoplasms↗
PubMed1987

Immature cerebellar astrocytoma in an infant.

A case of immature cerebellar astrocytoma in a 1 year and 4 months-old girl was described. The tumor consisted mainly of closely packed relatively uniform round cells with prominent intervening capillaries. Although tumor cells showed negative GFA (glial fibrillary acidic protein) immunoreactivity, demonstration of occasional bundles of 10 nm intermediate filaments (glial filaments) in cytoplasm on electron microscopy confirmed the diagnosis of astrocytoma. Differential diagnosis to hemangioblastoma, astroblastoma, and primitive neuroectodermal tumor was discussed.

Astrocytoma↗
PubMed2002

Neuropathology of genetically engineered mice: consensus report and recommendations from an international forum.

The Mouse Models of Cancer Consortium of the NCI sponsored a meeting of neuropathologists and veterinary pathologists in New York City in November of 2000. A rapidly growing number of genetically engineered mice (GEM) predisposed to tumors of the nervous system have led to a concomitant need for neuropathological evaluation and validation of these models. A panel of 13 pathologists reviewed material representing most of the available published and unpublished GEM models of medulloblastoma, primitive neuroectodermal tumor, astrocytoma, oligodendroglioma, mixed glioma, and tumors of the peripheral nerve. The GEM tumors were found to have many similarities and some distinct differences with respect to human disease. After review of the biology and pathology for all models presented, participants were split into groups reflective of clinical expertise in human pathology, tumor biology, neuroimaging, or treatment/intervention. Recommendations were made detailing an extensive and complete neuropathological characterization of animals. Importance was placed on including information on strains, tumor clonality, and examination for genetic mutation or altered gene expression characteristics of the corresponding human malignancy. Specific proposals were made to incorporate GEM models in emerging neuroradiological modalities. Recommendations were also made for preclinical validation of these models in cancer therapeutics, and for incorporation of surrogate markers of tumor burden to facilitate preclinical evaluation of new therapies.

Animals↗
PubMed2005

Atypical teratoid rhabdoid tumors of childhood: diagnosis, treatment and challenges.

Atypical teratoid rhabdoid tumor of the brain was described as a unique entity in the late 1980s. It occurs primarily in early childhood but the true incidence of the disease is not yet known. At presentation, the differential diagnosis includes medulloblastoma, primitive neuroectodermal tumor, ependymoma and choroid plexus carcinoma. Atypical teratoid rhabdoid tumor behaves in a very aggressive manner and while cure is possible for a small minority of patients, no standard or effective therapy has been defined for most patients. Since its first description, considerable pathologic, cytogenetic and molecular characterizations, as described in this review, have been accomplished that provide insight into the possible molecular etiology of the disease and of malignant rhabdoid tumors that occur outside the CNS. Co-operative group clinical trials that focus solely on atypical teratoid rhabdoid tumor are needed that incorporate biologic studies along with evaluations of aggressive treatment approaches. The goal of these trials should be to increase the cure rate for children with atypical teratoid rhabdoid tumor and further increase our understanding not only of atypical teratoid rhabdoid tumor, but also of other pediatric brain tumors.

Brain Neoplasms↗
PubMed1992

[Expression of adhesion molecules N.CAM, L1 and HNK1 epitope by medulloblastoma].

Twelve medulloblastomas were screened for their expression of adhesion molecules L1, N.CAM isoforms and HNK1 epitope by Western blotting and immunohistochemistry. Highly sialylated N.CAM isoforms were distinguished from total N.CAMs by using a monoclonal antibody (anti-MenB) specifically recognizing high polymers of 2-8 linked neuraminic acid. All tumors expressed HNK1 epitope, N.CAM and its highly sialylated isoforms on their surface membrane. L1 adhesion molecule was detected by immunohistochemistry in only one medulloblastoma. This spectrum of expression of cell surface adhesion molecules distinguishes medulloblastomas from other primitive neuroectodermal tumors. Medulloblastomas share some immunological features with post-mitotic cells forming the external granular layer of the cerebellum. Western blotting analysis of cerebrospinal fluid (CSF) samples with anti-MenB antibody enabled us to detect highly sialylated N.CAM in some samples. The presence of this antigen in CSF appears to correlate with meningeal spreading of medulloblastomas and could help monitoring chemotherapeutic treatment.

Antibodies, Monoclonal↗
PubMed2006

Arsenic trioxide concentration determines the fate of Ewing's sarcoma family tumors and neuroblastoma cells in vitro.

Arsenic trioxide (As(2)O(3)) induces both the differentiation and apoptosis of acute promyelocytic leukemia cells in a concentration dependent manner. We assessed the effects of As(2)O(3) in CADO-ES Ewing's sarcoma (ES), JK-GMS peripheral primitive neuroectodermal tumor (PNET), and SH-SY5Y neuroblastoma cells, as they share common histogenetic backgrounds. As(2)O(3) at low concentrations (0.1-1 microM) induced SH-SY5Y differentiation, and whereas PNET cells acquired a slightly differentiated phenotype, change was minimal in ES cells. Extracellular signal-regulated kinase 2 (ERK2) was activated at low As(2)O(3) concentrations, and PD98059, an inhibitor of MEK-1, blocked SH-SY5Y cell differentiation by As(2)O(3). High concentrations (2-10 microM) of As(2)O(3) induced the apoptosis in all three cell lines, and this was accompanied by the activation of c-jun N-terminal kinase. The generation of H(2)O(2) and activation of caspase 3 were identified as critical components of As(2)O(3)-induced apoptosis in all of the above cell lines. Fibroblast growth factor 2 enhanced As(2)O(3)-induced apoptosis in JK-GMS cells. The overall effects of As(2)O(3) strongly suggest that it has therapeutic potential for the treatment of ES/PNET.

Antineoplastic Agents↗
PubMed2000

Analysis of PTCH/SMO/SHH pathway genes in medulloblastoma.

Inactivation of the PTCH tumor suppressor gene occurs in a subset of sporadic medulloblastomas, suggesting that alterations in the PTCH pathway may be important in the development of this tumor. In order to address the frequency of genetic alterations affecting genes in this pathway, we used a combination of loss of heterozygosity (LOH) analysis, single-stranded conformational polymorphism (SSCP) analysis, and direct sequencing of DNA samples from sporadic primitive neuroectodermal tumors (PNETs). To identify alterations in the PTCH gene, we performed LOH analysis on 37 tumor DNA samples. Of those with matched constitutional DNA samples, one demonstrated LOH. Of those without matched constitutional DNA, six were homozygous with all markers. All exons of the PTCH gene were sequenced in these seven tumors, and three mutations were found. To identify alterations in the SHH and SMO genes, we analyzed all exons of both genes in 24 tumors with SSCP and sequenced any exons that showed aberrant band patterns. No mutations were found in either SHH or SMO in any tumor. We also identified the following genes as candidate tumor suppressors based on their roles in controlling hh/ptc signaling in Drosophila: EN-1 and EN-2, deletion of which results in a lack of cerebellar development in mice; SMAD family members 1-7, and protein kinase A subunits RIalpha, RIbeta, RIIbeta, Calpha, and Cbeta. Each of these genes was investigated in a panel of 24 matched constitutional and tumor DNA samples. Our search revealed no mutations in any of these genes. Thus, PTCH is the only gene in this complex pathway that is mutated with notable frequency in PNET. Genes Chromosomes Cancer 27:44-51, 2000.

Cerebellar Neoplasms↗
PubMed2003

Non-germ cell malignancy in residual or recurrent mass after chemotherapy for nonseminomatous testicular germ cell tumor.

BACKGROUND: After chemotherapy for nonseminomatous testicular germ cell tumor (NSTGCT), residual masses or recurrent disease may contain a non-germ cell malignancy (NGCM). METHODS: Over 20 years, 369 patients with disseminated NSTGCT were treated with cisplatin-based polychemotherapy at the University Medical Center Groningen. Residual tumor masses were resected in 244 patients and recurrent tumor masses in 37 patients. Histology was reviewed, focusing on the presence of NGCM. RESULTS: Nine patients developed an NGCM. Four patients had an NGCM in the resected residual tumor mass after chemotherapy: three patients had a sarcoma, and one patient had both a sarcoma and an adenocarcinoma. Five patients developed a late recurrence with an NGCM after 39, 40, 72, 72, and 84 months. One patient had a primitive neuroectodermal tumor, one had a sarcoma, and three had an adenocarcinoma in the resected recurrent tumor mass. A complete surgical resection was achieved in five (56%) of the nine patients. After a median follow-up of 48 months (range, 3-271 months), five patients had no evidence of disease (56%), three patients were dead of disease (33%), and one patient was alive with disease (11%). CONCLUSIONS: Sarcoma, adenocarcinoma, or both in residual or recurrent tumor masses after combined-modality NSTGCT treatment are rare. Complete surgical resection of the tumor mass is the only curative treatment option.

Adenocarcinoma↗
PubMed1996

Value of postoperative surveillance imaging in the management of children with some common brain tumors.

The rationale for obtaining surveillance computerized tomography (CT) scans or magnetic resonance (MR) images in pediatric patients with brain tumors is that early detection of recurrence may result in timely treatment and better outcome. The purpose of this study was to investigate the value of surveillance cranial images in a variety of common pediatric brain tumors managed at a tertiary care pediatric hospital. A retrospective chart review was performed of children with astrocytoma of the cerebral hemisphere, cerebellum, optic chiasm/hypothalamus, or thalamus; cerebellar or supratentorial high-grade glioma; supratentorial ganglioglioma; posterior fossa or supratentorial primitive neuroectodermal tumor (PNET); and posterior fossa ependymoma. Data were analyzed to determine the frequency with which recurrences were identified on a surveillance image and how the type of image at which recurrence was identified related to outcome. In 159 children, 17 of 44 recurrences were diagnosed by surveillance imaging. The percentage of recurrences identified by surveillance imaging was 64% for ependymoma, 50% for supratentorial PNET, 43% for optic/hypothalamic astrocytoma, and less than 30% for other tumors. The rate of diagnosis of recurrence per surveillance image varied from 0% to 11.8% for different tumor types. Only for ependymomas did there appear to be an improved outcome when recurrence was identified prior to symptoms. Our results indicate that, using the protocols outlined in this study, surveillance imaging was not valuable in identifying recurrence of cerebellar astrocytoma or supratentorial ganglioglioma during the study period, but was probably worthwhile in identifying recurrence of posterior fossa ependymoma and optic/hypothalamic astrocytoma and, possibly, medulloblastoma. Surveillance protocols could be made more effective by individualizing them for each type of tumor, based on current data on the patterns of recurrence.

Astrocytoma↗
PubMed1998

Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group.

OBJECT: Ependymomas in children continue to generate controversy regarding their histological diagnosis and grading. optimal management, and possible prognostic factors. To increase our knowledge of these tumors the authors addressed these issues in a cohort of children with prospectively staged ependymomas treated with radiotherapy and chemotherapy. METHODS: Children between the ages of 2 and 17.3 years harboring an intracranial ependymoma confirmed by a central review of the tumor's pathological characteristics were treated according to Children's Cancer Group Protocol 921 from 1986 to 1992. Treatment following surgery and postoperative tumor staging (including brain computerized tomography or magnetic resonance [MR] imaging, spinal MR imaging or myelography, and cerebrospinal fluid cytological investigation) included craniospinal irradiation with a local boost to the primary tumor and patient randomization to receive adjuvant chemotherapy with either 1) CCNU, vincristine, and prednisone, or 2) the eight-drugs-in-1-day regimen. Centralized review of the tumor pathological characteristics revealed 20 ependymomas and 12 anaplastic ependymomas in the 32 children included in the study. Diagnoses made at the individual institutions included anaplastic (malignant) ependymoma (15 patients), ependymoma (four patients), ependymoblastoma (nine patients), ependymoastrocytoma (one patient), and primitive neuroectodermal tumor (three patients), which were discordant with the centralized review diagnosis in 22 of 32 cases. Only three of the 32 patients had metastatic disease (two with M and one with M3 stages). At surgery, 47% of tumors were estimated to be totally resected. Among the 14 of 17 patients who suffered a relapse and were evaluated for site of relapse, 10 (71%) had an isolated local relapse, three (21%) had concurrent local and metastatic relapse, and only one (7%) had an isolated metastatic relapse. Kaplan-Meier estimates of 5-year progression-free survival (PFS) and overall survival rates were 50 +/- 10% and 64 +/- 9%, respectively. CONCLUSIONS: Predictors of PFS duration included an estimate of the extent of resection made at surgery (total compared with less than total, p = 0.0001) and the amount of residual tumor on postoperative imaging as verified by centralized radiological review (< or = 1.5 cm2 compared with > 1.5 cm2, p < 0.0001). No other factors, including centrally reviewed tumor histopathological type, location, metastasis and tumor (M and T) stages, patient age, race, gender, or chemotherapy treatment regimen significantly correlated with PFS duration. The pattern of predominantly local relapse and the important influence of residual tumor or the extent of resection on PFS duration confirms a prevailing impression that local disease control is the major factor in the prediction of outcome of ependymoma. Survival rates were comparable with those reported by other investigators who have treated patients with similar doses of radiation and no chemotherapy.

Adolescent↗
PubMed2003

Genomic structure and mutational analysis of the human KIF1Balpha gene located at 1p36.2 in neuroblastoma.

KIF1a is a member of the kinesin superfamily proteins that are microtubule-dependent molecular motors involved in important intracellular functions such as organelle transport and cell division. We previously determined the structure of the human KIF1Bbeta gene, which was found to be a homologue of the murine Kif1bbeta, and demonstrated that the human KIF1Bbeta is a causative gene of Charcot-Marie-Tooth disease type 2A although we did not prove that it is a tumor suppressor gene of neuroblastoma. Here, we identified another isoform of the human KIF1B gene, KIF1Balpha. The KIF1Balpha and KIF1Bbeta are alternative splicing products of the KIF1B gene located on 1p36.2. The KIF1Balpha is distinct from KIF1Bbeta in the C-terminal cargo-binding domain; however, they have the same N-terminal motor domain. We found that the transcript of approximately 7.8 kb of KIF1Balpha was expressed in several tissues, especially in skeletal muscle, by Northern blot analysis. To determine whether this gene is one of the candidate tumor suppressor genes for neuroblastoma (NB) or other pediatric solid tumors, we performed mutational screening of KIF1Balpha in 25 NB, 9 rhabdomyosarcoma, 12 Ewing sarcoma and 24 other pediatric solid tumor cell lines. Using RT-PCR single-strand conformation polymorphism analysis and direct sequencing we detected a missense mutation (M807I) in 1 NB cell line (SK-N-SH), 3 silent mutations in 2 NB cell lines and 1 primitive neuroectodermal tumor cell line, respectively. RT-PCR analysis revealed that KIF1Balpha was obviously expressed in almost all of the tumor cell lines examined except NB-1. Furthermore, real-time quantitative RT-PCR showed that there was no significant difference in KIF1Balpha expression between 14 early-stage (stage I and II) and 14 advanced-stage (stage III and IV) NB fresh tumor specimens. These results suggest that KIF1Ba in addition to KIF1Bbeta may not be a candidate tumor suppressor gene for NB.

Alternative Splicing↗
PubMed2003

Identification of prognostic markers in bone sarcomas using proton-decoupled phosphorus magnetic resonance spectroscopy.

It has been hypothesized that the (31)Phosphorus ((31)P) nuclear magnetic resonance spectrum from certain tumors may provide prognostic information. The goal of the present study was to identify prognostic metabolic markers by using proton-decoupled phosphorus magnetic resonance spectroscopic imaging ((31)P MRSI). Twenty patients with bone [osteogenic (OS) and Ewing's (ES) and/or primitive neuroectodermal tumor (PNET)] sarcoma, treated with chemotherapy and surgery or with chemotherapy alone, underwent (31)P MRSI studies pre- and post-therapy. The studies were performed on a 1.5 Tesla General Electric (GE) clinical scanner equipped with a stand-alone proton decoupler and a dual (1)H/(31)P surface coil pair. The limited sensitivity of the (31)P nucleus required that a large soft tissue component of the disease be located within 10 cm (maximum distance) of the body surface and the use of a highly sensitive coil placed near the skin surface. Proton decoupling and nuclear Overhauser enhancement were used to improve the spectral resolution and signal:noise ratio. Baseline (31)P spectral features and metabolic changes with treatment were compared with treatment outcome. The patients were categorized depending on survival as event-free survivors or those who died. The pretreatment nucleoside triphosphate:inorganic phosphate (NTP:P(i)) ratio, an index of tumor bioenergetic status, was significant (P = 0.003) in differentiating event-free survivors versus those who died. The pretreatment NTP:P(i) was higher in patients who were destined to undergo a durable event-free survival compared with those who died. The results are promising, although a prospective study is necessary for confirmation. (31)P MRSI appears to be a useful tool for the prediction of survival before therapy in bone sarcomas.

Adolescent↗
PubMed1987

Leu-7 in small cell neoplasms. An immunohistochemical study with ultrastructural correlations.

In an effort to delineate the distribution of the Leu-7 antigen in small cell neoplasms, 283 paraffin-embedded examples of such tumors were studied immunohistochemically. These included 125 malignant lymphomas, 94 neuroendocrine carcinomas, 13 adenocarcinomas, 14 squamous carcinomas, four malignant melanomas, six neuroblastomas, four nephroblastomas (Wilms' tumors), six primitive neuroectodermal neoplasms, three "Askin" tumors, ten Ewing's sarcomas, and four embryonal rhabdomyosarcomas. Histologic diagnoses were verified by the use of electron microscopic study or independent immunostains. Overall, 44% of small cell neuroendocrine carcinomas expressed Leu-7, whereas nonendocrine carcinomas were uniformly nonreactive for this determinant. All neuroblastomas yielded immunopositivity, as did three primitive neuroectodermal tumors, three rhabdomyosarcomas, two "Askin" tumors, one nephroblastoma, one Ewing's sarcoma, and one malignant melanoma. None of the small cell lymphomas were Leu-7 positive. These results suggest that Leu-7 is a specific neuroendocrine marker in cases of small cell carcinoma, but its sensitivity is lower than that of other "endocrine" determinants. Reactivity patterns for Leu-7 in other small cell tumors demonstrate no specificity for any given line of cellular differentiation. However, the shared expression of this antigen by neuroblastomas, neuroectodermal tumors, Ewing's sarcomas, and Wilm's tumors contributes further to the hypothesis that these neoplasms may be related histogenetically.

Antigens, Differentiation, T-Lymphocyte↗