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PubMed2001

[Atypical teratoid and rhabdoid tumor. Report of a congenital case].

The atypical teratoid rhabdoid tumor is a rare brain tumor of childhood. We report a congenital case, revealed by peripheral facial palsy. This polymorphous tumor consisted of rhabdoid cells associated with areas of primitive neuroectodermal tumor. The immunoreactivity for the three proteins vimentin, epithelial membrane antigen and smooth-muscle actin was suggestive of rhabdoid tumor. Genetic study showed a homozygous mutation in the tumoral DNA and a constitutional heterozygous mutation, as it was demonstrated in atypical teratoid rhabdoid tumors and in renal and extra-renal rhabdoid tumors.

Actins↗
PubMed2005

Pineal tumors: analysis of treatment results in 20 patients.

OBJECT: The authors evaluate their results when using gamma knife surgery (GKS) in the management of patients with tumors in the pineal region. METHODS: This is a retrospective clinical evaluation of 20 patients with primary tumors of the pineal region treated with GKS from November 1994 through August 2003. There were 13 germ cell tumors, two pineoblastomas, two low-grade gliomas, one primitive neuroectodermal tumor, one teratoma, and one pineocytoma. There were 10 male and 10 female patients. Their median age was 15.5 years (range 5-71 years). The median margin dose was 11 Gy (range 8-20 Gy). The median target volume was 3.1 cm3 (range 0.1-49.9 cm3). Five patients received sequential systemic chemotherapy and four underwent adjuvant conventional radiation therapy. Seventeen (85%) of 20 patients are alive with a median survival of 30.4 months (range 0-85.7 months). Two patients required retreatment. Three patients died: one of unrelated causes, one who presented with extensive local disease, and the other of meningeal carcinomatosis with local control of the primary tumor. No complications from GKS were noted. CONCLUSIONS: This initial experience suggests that GKS is a valuable treatment modality for the management of pineal region tumors. This technique offers excellent local tumor control and minimal patient morbidity, allowing for immediate use of systemic chemotherapy and/or conventional radiation if indicated.

Adolescent↗
PubMed1996

Pediatric brain tumors express multiple receptor tyrosine kinases including novel cell adhesion kinases.

We have used the polymerase chain reaction to clone and characterize growth factor receptor tyrosine kinases (RTKs) expressed in 3 pathologically distinct pediatric brain tumors, an anaplastic ependymoma, a glioblastoma multiforme and a primitive neuroectodermal tumor (PNET). These neoplasms are presumed to be derived from embryonic neuroepithelial precursor cells of the central nervous system. This cloning demonstrated expression of 24 distinct kinase genes: 16 receptor type kinases and 8 nonreceptor type kinases. The expression of 6 receptors, including Hek2, IRR, Ryk, FGFR3, and 2 members of the newly identified cell adhesion kinase receptor family, DDR and TKT, in such tumors has not been reported previously. Northern analysis of mRNA levels revealed DDR expression in 6 of 7 pediatric brain tumors including an ependymoma, PNET, glioblastoma and astrocytoma, and also in an adult pheochromocytoma. Thus, the DDR cell adhesion kinase may be widely expressed in pediatric brain tumors. Also, PCR cloning may be an effective procedure for characterizing RTKs in clinical tissue samples and revealing the expression of novel RTK species.

Adrenal Gland Neoplasms↗
PubMed2000

Treatment of progressive or recurrent pediatric malignant supratentorial brain tumors with herpes simplex virus thymidine kinase gene vector-producer cells followed by intravenous ganciclovir administration.

OBJECT: The outcome for children with recurrent malignant brain tumors is poor. The majority of patients die of progressive disease within months of relapse, and other therapeutic options are needed. The goal of this Phase I study was to evaluate the safety of in vivo suicide gene therapy in 12 children with recurrent, malignant, supratentorial brain tumors. METHODS: After optimal repeated tumor resection, multiple injections of murine vector-producing cells shedding murine replication-defective retroviral vectors coding the herpes simplex virus thymidine kinase type 1 (HSV-Tk1) gene were made into the rim of the resection cavity. Fourteen days after the vector-producing cells were injected, ganciclovir was administered for 14 days. The retroviral vector that was used only integrated and expressed HSV-Tk1 in proliferating cells, which are killed after a series of metabolic events lead to cell death. The median age of the patients was 11 years (range 2-15 years). Treated brain tumors included seven malignant gliomas, two ependyminomas, and three primitive neuroectodermal tumors. The patients were treated with one of three escalating dose concentrations of vector-producer cells. Four transient central nervous system adverse effects were considered possibly related to the vector-producing cells. In no child did permanent neurological worsening or ventricular irritation develop, and tests for replication-competent retroviruses yielded negative findings. CONCLUSIONS: This Phase I study demonstrates that in vivo gene therapy in which a replication-defective retroviral vector in murine vector-producing cells is delivered by brain injections can be performed with satisfactory safety in a select group of children with localized supratentorial brain tumors.

Adolescent↗
PubMed2004

Fine-needle aspiration of adult small-round-cell tumors studied with flow cytometry.

Immunophenotypic study is critical for the diagnosis of adult small-round-cell tumors (SRCTs). We describe three patients with Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) and one patient with neuroblastoma in which flow cytometry immunophenotyping (FCI) on the fine-needle aspirate (FNA) and bone marrow aspirate (BMA) demonstrated an abnormal population of cells that were CD45(-) and CD16/CD56(+). Four patients with mean age of 30 years, three male and one female, clinically suspicious for a lymphoma or SRCT are described. FNA, BMA, and biopsy specimens were obtained for routine cytologic and histologic evaluation. Fresh tissue was studied by FCI. In all cases, the cytology smears showed small cells with round nuclei, slightly irregular nuclear membranes, fine chromatin, and scant cytoplasm. FCI showed CD16/56(+) and CD45(-) neoplastic cells in all cases. In one case, 76% of these cells were CD99(+). The diagnoses of ES/PNET were confirmed by immunohistochemical, ultrastructural, and cytogenetic studies. ES/PNET in FNA and BMA can be efficiently and rapidly diagnosed by combining cytologic examination with FCI using a panel including CD45, CD16/56, and CD99.

12E7 Antigen↗
PubMed2006

High histologic and overall response to dose intensification of ifosfamide, carboplatin, and etoposide with cyclophosphamide, doxorubicin, and vincristine in patients with high-risk Ewing sarcoma family tumors: the Bambino Gesù Children's Hospital experience.

BACKGROUND: Ewing sarcoma (ES) and extraosseous ES/primitive neuroectodermal tumors (PNET) share histopathologic features of the ES family of tumors (ESFT). The authors report on their results from a regimen of ifosfamide, carboplatin, and etoposide (ICE) with cyclophosphamide, doxorubicin, and vincristine (CAV) dose intensification in patients with high-risk ESFT. METHODS: Since 1990, patients with ESFT and with 1 or more of the following risk factors were reviewed: tumor volume > 200 mL, tumor site with a poor prognosis, and pulmonary and/or bone marrow metastases. RESULTS: Thirty-six patients with ESFT who were involved in the study were divided into 2 arms of 18 patients each. One group received treatment with various regimens, and the other group received treatment with ICE plus CAV. The disease was brought under control more rapidly in the latter patients, for whom surgery was more easily feasible, and up to 90% of patients achieved a major response, with an estimated 3-year overall survival rate of 67% +/- 12%. CONCLUSIONS: The current results showed that ICE plus CAV was tolerated well and was effective in the studied subset of tumors, indicating that dose intensification correlates with better disease control, a high percentage of necrosis, and conservative surgery in patients with high-risk ESFT.

Adolescent↗
PubMed2002

Prognostic factors and survival in late adolescent and adult patients with small round cell tumors.

The primary objective of this study is to review the clinical characteristics of 25 patients in the adult and late adolescent age group, diagnosed and treated with small round cell tumors involving soft tissues (extraosseous Ewing sarcoma, rhabdo-myosarcoma, primitive neuroectodermal tumor, and undiffer-entiated small round cell tumors). Additionally, survival and prognostic factors influencing the outcome with multimodality treatment are evaluated. There were 19 males (76%) and 6 females (24%). The median age was 26 years (range: 15-56 years). In 9 patients (36%), the tumor was located at an extremity, whereas 16 patients (64%) had central localizations. Tumor size was larger than 10 cm in 7 patients (29.2%). Six patients (24%) had metastatic disease. Twelve patients (48%) received radiation and 16 patients (64%) underwent surgery. Among the resected tumors, 2 were resected with contaminated margins (12.5%), whereas 2 were radically resected and 12 (75%) were resected with wide margins. All patients were given a median of 4 cycles of multiagent chemotherapy (1-14 cycles). With preoperative chemotherapy, complete regression (CR) of the tumor was achieved in 6 patients (24%). In 4 patients (16%), a partial response was obtained. After the completion of multimodality treatment, 12 patients (48%) had a CR. Progression-free (PFS) and overall survival (OS) for the entire group was 25.0 +/- 10.8% at 1 year and 30.5 +/- 15.5% at 3 years, respectively. Nonmetastatic disease, wide and radical resection, and presence of CR to multimodality treatment were associated with a significantly longer PFS and OS by univariate analysis. By multivariate analysis, CR to multimodality treat-ment was the only independent predictive factor for a longer OS (p: 0.0036, relative risk [RR]: 23.6, 95% CI: 2.8; 198.7) and metastatic presentation was the only independent factor predic-tive for a shorter PFS (p: 0.017, RR. 15, 95% CI: 1.6; 141.2). Large-scale, multicenter studies are required for a better eval-uation of the nonpediatric age group with small round cell tumors.

Adolescent↗
PubMedDate not supplied

Fine needle aspiration cytology of the Ewing's sarcoma family of tumors.

OBJECTIVE: To study the cytomorphologic features of the Ewing's sarcoma (ES) family of tumors. STUDY DESIGN: During a period of eight years (1990-1997), 123 soft tissue tumors and 65 bone tumors were evaluated by fine needle aspiration cytology (FNAC); 14 cases were diagnosed as in the ES family of tumors. The ages of the patients ranged from 8 to 30 years. All the cases were histologically confirmed. RESULTS: Of 14 cases of the ES family of tumors, 7 were ES, 3 extraosseous ES (EOE), 2 peripheral primitive neuroectodermal tumor (PPNET) and 2 Askin tumor. Cytologically, smears from all the cases showed round tumor cells with a high nuclear/cytoplasmic ratio. On detailed examination, subtle differentiating features were observed. The cells in ES had finer nuclear chromatin in comparison to those of PPNET and Askin tumor, and punched-out clear cytoplasmic vacuoles were present. PPNET showed nuclear molding, unipolar cytoplasmic tags and Homer-Wright rosettes. Histologically, all cases of ES and EOE and one case of Askin tumor showed periodic acid-Schiff-positive inclusions. CONCLUSION: FNAC features coupled with clinical findings enable a rapid diagnosis of the ES family of tumors, from which treatment modalities can be determined.

Adolescent↗
PubMed2003

Peripheral medulloepithelioma: an immunohistochemical, ultrastructural, and cytogenetic study of a rare, chemotherapy-sensitive, pediatric tumor.

A case of peripheral medulloepithelioma, a rapidly growing tumor involving the pelvic cavity of a 12-year-old girl, is presented. The diagnosis was supported by expression of vimentin, nestin, alpha-internexin, neurofilaments, and microtubule-associated protein 5 and by characteristic ultrastructure that included absence of cilia or microvilli. Trisomy of chromosomes 2 and 8 was the only detectable chromosomal abnormality. Combination chemotherapy resulted in complete remission. Because some of these rare tumors are sensitive to chemotherapy, their recognition and separation from other neuroectodermal tumors are advisable for better understanding of their biology and determination of optimal treatment.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Primary intracranial neoplasms of infancy and early childhood.

We investigated the age-related location, gender distribution, and histology of 107 brain tumors in children under 4 years of age seen in our department between 1984 and 1997. The male-to-female ratio was 1.4 (62/45 cases) with a prevalence of supratentorial tumors (60/47 = 1.3); the main histological entity was astrocytoma (33.6%), followed by ependymoma (14.0%). In the 1st year of life 22 cerebral neoplasms became clinically apparent. A higher ratio for supratentorial tumors was revealed (17/5 = 3.4), but without gender preference, and primitive neuroectodermal tumors (PNET) were the most frequent (5/22). In the 2nd year 25 tumors were found. The male-to-female ratio was 1.5 (15/10) and the supratentorial-to-infratentorial ratio, 1.1 (13/12). The two most common entities were astrocytoma and ependymoma (6 cases each). In addition, a survey of previously published investigations into this subject was performed and a compilation of data on 1960, 545 and 1084 tumors in children below the age of 1, 2 and 4 years, respectively, was prepared, which makes it the most extensive review of brain tumors of infancy and early childhood yet undertaken.

Age Factors↗
PubMed2006

Topotecan by 21-day continuous infusion in children with relapsed or refractory solid tumors: a Children's Oncology Group study.

PURPOSE: The Children's Oncology Group conducted a phase II trial of 21-day continuous infusion topotecan to determine the response rate in pediatric patients with recurrent or refractory malignant solid tumors. PROCEDURE: Patients with Ewing sarcoma family of tumors (ESFT), osteosarcoma (OS), soft tissue sarcomas (STS), medulloblastoma (MB)/primitive neuroectodermal tumor (PNET), astrocytoma, or neuroblastoma (NB) recurrent or refractory to conventional therapy, measurable disease, and adequate organ function were treated with topotecan 0.3 mg/m2/day by continuous intravenous infusion for 21 consecutive days, followed by 7 days without therapy prior to response assessment. RESULTS: Fifty-five patients were enrolled; two were ineligible, two were removed from protocol therapy prior to evaluation for response, and one was inevaluable for response, leaving 53 and 50 patients evaluable for toxicity and response, respectively. Objective responses were seen in 2/20 patients with ESFT (both partial responses, 4 and 19 courses), 0/10 OS patients, and 0/12 STS patients. There were insufficient patients enrolled to determine the response rate for the MB/PNET, astrocytoma, and NB strata. The most common Grade 3 or 4 toxicities during the first course of therapy were thrombocytopenia (12/53), neutropenia (8/53), and fatigue (7/53). CONCLUSION: Intravenous topotecan by 21-day continuous infusion is tolerable in pediatric patients with recurrent or refractory solid tumors. Limited activity was seen in ESFT and further development of this topotecan schedule as a single agent is not warranted.

Adolescent↗
PubMed2000

Overexpression of the hepatocyte growth factor (HGF) receptor (Met) and presence of a truncated and activated intracellular HGF receptor fragment in locally aggressive/malignant human musculoskeletal tumors.

Enhanced hepatocyte growth factor (HGF) receptor (Met) signaling has been suggested to play an important role in the development and progression of various epithelial and nonepithelial tumors. N-terminally truncated forms of the HGF receptor have been shown to be constitutively activated and tumorigenic in animal experiments. In the present study, 102 benign and malignant human musculoskeletal tumors were examined for expression of the HGF receptor by Western blotting and/or immunohistochemistry. A clear predominance of HGF receptor expression was seen in malignant as compared to benign tumors (Western blotting, P < 0.001; immunohistochemistry, P < 0.02). For the first time we show HGF receptor expression in the following four tumor types: dermatofibrosarcoma protuberans, clear cell sarcoma of tendons, malignant primitive neuroectodermal tumor, and benign fibrous histiocytoma. In three cases of sarcoma with high HGF receptor expression by Western blotting, we found indications of a short 85-kd N-terminally truncated HGF receptor that was tyrosine phosphorylated and located in the cytoplasm. Although fragments of this length were seen in 18 of 65 tumors, most were not tyrosine-phosphorylated. Northern blotting revealed only the 7.5-kb full-length HGF receptor transcript, suggesting that the 85-kd fragment is generated by an alternative initiation of translation or by proteolytic cleavage. Southern blotting detected no amplification of the Hgfr/Met gene in the 35 tumors examined, in contrast to our recent report of Hgfr/Met gene amplification in 7, 12-dimethylbenz(a)anthracene (DMBA)-induced rat sarcomas. The present data suggest that the locally aggressive and malignant properties of human mesenchymal tumors maybe related, in part, to high levels of full-length HGF receptors, and in some cases to the occurrence of N-terminally truncated HGF receptors, activated independently of HGF.

Blotting, Western↗
PubMed2000

Expression of protein gene product 9.5 and tyrosine hydroxylase in childhood small round cell tumors.

Small round cell tumors of childhood can be histologically ambiguous, can require tumor markers for an accurate diagnosis, and include neuroblastoma, peripheral primitive neuroectodermal tumor (pPNET), Ewing's sarcoma (ES), lymphoma, and rhabdomyosarcoma. Because the cell type of origin for ES remains controversial, characterizing gene expression in ES can provide diagnostic markers and lead to better understanding of tumor biology. We studied RNA expression of the neuronal genes protein gene product 9.5 (PGP 9.5) and tyrosine hydroxylase (TH) by Northern analysis in cell lines and tissue from small round cell tumors. PGP 9.5 showed strong expression in 17 of 17 neuroblastoma cell lines, 9 of 9 pPNET cell lines, and 11 of 11 ES cell lines. PGP 9.5 was weakly expressed in 1 of 1 alveolar rhabdomyosarcoma cell lines but not in 1 of 1 embryonal rhabdomyosarcomas, and weak expression was seen in 1 of 7 leukemia cell lines. In tumor tissue, all 12 neuroblastomas expressed PGP 9.5, as did all 7 pPNET and all 7 ES. PGP 9.5 was very weakly expressed in 6 of 9 rhabdomyosarcomas and 1 of 9 lymphomas. TH was expressed only in neuroblastomas, and no TH expression was seen in cell lines or tissue from other tumors. As high expression of PGP 9.5 was only found in neural tumors; PGP 9.5 expression by ES provides further evidence for a neural origin of this tumor, whereas TH expression is highly specific for neuroblastomas. PGP 9.5 expression should allow sensitive detection of minimal residual disease for ES and pPNET using reverse transcription-PCR, and the variability in TH and PGP 9.5 expression levels in neuroblastomas indicates that expression of both genes should be used for monitoring minimal residual disease by reverse transcription-PCR.

Adolescent↗
PubMedDate not supplied

Unique intracerebral tumor with divergent differentiation in a patient presenting as NF2: report of a case with features of astrocytoma, ependymoma, and PNET.

Patients with neurofibromatosis 2 (NF2) are predisposed to a variety of neoplastic and dysplastic lesions, including schwannomas, neurofibromas, meningiomas, astrocytomas, and ependymomas, as well as entities such as meningioangiomatosis, schwannosis, and hamartomas. This study reports a unique intracerebral frontotemporal tumor in a 6-year-old boy with presumed NF2, on the basis of bilateral cerebellopontine tumors consistent with acoustic neuromas. The intracerebral tumor revealed a variety of histological patterns, including foci of primitive neuroectodermal tumor (PNET), low-grade astrocytoma and ependymoma, as well as neuroepithelial rests with immature ganglion cells and hamartomatous areas. The MIB-1 labeling index ranged from 63% in the foci of PNET to 4-7% in other foci. The PNET component revealed immunopositivity for synaptophysin and neurofilament and showed cells with delicate intercellular junctions, profiles of rough endoplasmic reticulum, mitochondria, and dense core granules, and cell processes with microtubules and neurofilaments. The glial and ependymal components showed bundles of glial filaments and prominent cell junctions, cilia, and microvilli. The hamartomatous component also included aggregates of cells with hyaline eosinophilic cytoplasm. By EM these cells contained abundant amorphous flocculent material. This constellation of pathologic findings, especially the finding of PNET, is unique and not previously reported in the setting of NF2.

Astrocytoma↗
PubMed1997

[Malignant non-epithelial tumors of the pancreas].

Nonepithelial malignant tumors of the pancreas are extremely rare neoplasms with a frequency of approximately 0.6%. They are always explored because of a suspected diagnosis of pancreatic carcinoma. Amongst the more than 600 primary pancreatic neoplasms in our pancreatic tumor archive only 5 neoplasms were of nonepithelial origin (one was a malignant peripheral nerve sheath tumor [MPNST], one a leiomyosarcoma, one a malignant mesothelioma, and two were peripheral neuroectodermal tumors [PNET]. The differential diagnosis includes secondary infiltration of the pancreas by mesenchymal tumors of the retroperitoneum, undifferentiated pancreatic carcinoma and, especially in the case of PNET, malignant lymphoma. Preoperative chemotherapy and down-staging can improve the operability and prognosis, especially in PNET.

Adolescent↗
PubMed1983

Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study.

Neuroepithelial neoplasms of childhood were examined immunohistochemically using antibodies against a neurofilament polypeptide and glial fibrillary acidic protein. Ninety-one cases, including 11 controls, were examined. Positively reacting cells, indicating neuronal and glial differentiation, were found in 59 of the 80 tumors. The study supports a neuroepithelial origin for medulloblastomas, central neuroblastomas, and primitive neuroectodermal tumors of childhood. The results also indicate that only a small number of the tumor cells differentiate along either neuronal or glial cell lines.

Astrocytoma↗
PubMedDate not supplied

Intra-abdominal polyphenotypic tumor.

The presence of t(11;22)(q24;q12) is often considered diagnostic of Ewing sarcoma and peripheral primitive neuroectodermal tumor (pPNET). We report a case of a polyphenotypic tumor that possessed this translocation as detected by reverse transcriptase polymerase chain reaction (RT-PCR). This tumor was positive for vimentin, desmin, low-molecular-weight keratin, neuron-specific enolase, S-100 protein, and CD57 by immunohistochemistry. Of note, the tumor was negative for MIC2. The tumor had double-minute chromosomes with > 100 copies of the MDM2 gene. Thus, the presence of the t(11;22)(q24;q12) translocation should not be considered diagnostic of Ewing sarcoma and pPNET in the absence of supporting histologic evidence such as positive staining for MIC2. The presence of this translocation in Ewing sarcoma and pPNET has been taken as evidence that these two tumors are related. Rather than extending this relationship to include some polyphenotypic tumors, other tumors may acquire this genetic change during tumor progression. Treatment regimens for tumors may be better based on phenotype rather than genotype when these two profiles are seemingly in conflict.

Abdominal Neoplasms↗
PubMed1994

Intracranial vascular tumors.

The study of intracranial vascular tumors is of significant importance because these tumors may stimulate vascular malformations, infarctions, or infections. This article describes vascular tumors and discusses their appearance on MR imaging studies. Choroid plexus neoplasms, primitive neuroectodermal tumors, hemangioblastoma and other disorders are discussed.

Adult↗