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PubMedDate not supplied

Enrichment of residual tumor cells in bone marrow or peripheral blood cells in a neuroectodermal tumor model.

To enrich a small population of malignant cells contaminating human bone marrow or peripheral blood, a method of immunomagnetic depletion of the normal nucleated cells was developed. In a model using neuroectodermal cell lines, contaminations between 0.1% and 1.4% of tumor cells could be increased by a factor of 7.5 (median; range, 3.8 to 18.0; n = 10). Because incubation with antibodies is restricted to the magnetic beads in this method, the cell population after removal of the beads has not been incubated with antibodies. The risk of unspecific staining of the tumor cells during the enrichment procedure is minimal. This simple method is therefore well suited to the study of the characteristics of a minimal residual disease population.

Blood Cells↗
PubMed1995

Is esthesioneuroblastoma a peripheral neuroectodermal tumor?

Esthesioneuroblastoma (ENB; olfactory neuroblastoma) is a rare, locally aggressive neoplasm of the sinonasal area and anterior cranial fossa. The histogenesis of the lesion is not clearly delineated, and the broad histological spectrum of ENB has confounded the issue. The location, histological features (neuropil, Homer Wright, and olfactory rosettes), and reported immunocytochemical reactions (neuron-specific enolase (NSE) and chromogranin (CHR) positivity) suggest that ENB may be a neural or neuroendocrine neoplasm derived from the olfactory membrane. Recent demonstration in two of three metastatic putative ENB cell lines of the 11;22 chromosomal translocation, seen in Ewing's sarcoma (ES) of bone and peripheral neuroectodermal tumors (PNET) of bone and soft tissue, has led to the conclusion that ENB may be closely related histogenetically to PNET. The overwhelming majority of cases of ES and PNET express the protein product of MIC-2, a gene located on the pseudoautosomal region of the X and Y chromosomes. This protein can be identified immunocytochemically by antibodies 12E7, HBA71, and ON13. We studied the expression of MIC-2 using the 12E7 antibody as well as multiple neural markers in 18 ENB samples obtained from the files of The Johns Hopkins Hospital. The patients ranged in age from 19 to 90 years (mean, 47.5; median, 47) and included five men and 13 women. None of the 18 specimens reacted with antibody 12E7, but 16 were positive for NSE, nine reacted to synaptophysin (SYN), and 13 showed antibodies to chromogranin (CHR). Our studies agree with the previous suggestions that ENB is a primitive neural tumor but fail to support the hypothesis that it is a member of the PNET family.

12E7 Antigen↗
PubMed1995

Primary cutaneous Ewing's sarcoma/peripheral primitive neuroectodermal tumors in childhood. A molecular, cytogenetic, and immunohistochemical study.

Childhood cutaneous and subcutaneous malignancies are rare and include metastatic tumors of diverse histogenesis as well as primary lesions, such as sweat gland carcinomas. Some cutaneous malignancies exhibit a small round cell tumor morphology with few definitive differentiating features; they can thus pose a significant diagnostic problem. We describe two primary malignancies of the skin and superficial subcutis, which were originally diagnosed as sweat gland carcinomas on the basis of their morphological features. A cytogenetic analysis performed on one of these lesions showed the t(11;22)(q24;q12) rearrangement, believed to be unique to the Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) group of neoplasms. In view of this unexpected result, reverse transcriptase-polymerase chain reaction analysis was performed on both lesions and showed that they expressed EWS/FLI-1 fusion gene mRNA transcripts, the molecular equivalent of t(11;22)(q24;q12). The two tumors also had an immunohistochemical profile suggesting ES/pPNET, including strong expression of the MIC2 antigen. Both patients were treated with wide local excision, and one was given a course of chemotherapy. Neither patient showed evidence of tumor elsewhere after follow-up periods of 2 years and 16 years. These findings suggest that these tumors are indeed a form of primary ES/pPNET arising in the skin or superficial subcutis, which may be of low-grade malignancy and curable by local surgery.

Base Sequence↗
PubMed1991

Extracranial primitive neuroectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience.

The clinical data of 54 patients (57% males) with extracranial primitive neuroectodermal tumors (PNET) seen over a 20-year period at Memorial Sloan-Kettering Cancer Center were reviewed. The age at diagnosis ranged from 1 month to 81 years (median, 17 years). One PNET arose in a previously irradiated site. One patient had an unaffected identical twin. Primary sites were thoracopulmonary (n = 25), pelvis (n = 12), retroperitoneum or abdomen (n = 10), limbs (n = 5), neck (n = 1), and unknown (n = 1). At diagnosis, epidural extension was present in 13 patients with truncal primaries, and 11 patients had distant metastases. All of the latter died with disease. Progression-free survival (PFS) among the 43 patients with localized tumors (all greater than 5 cm) was 25% at 24 months. Two of 13 patients who relapsed after more than 12 months without therapy were long-term survivors. Patients with localized PNET who had resection of all gross disease within 3 months of diagnosis had a significantly longer PFS (P = 0.0003). Radiation therapy caused tumor shrinkage but was not curative of measurable disease. A dose-response effect was evident with the most commonly used drug, cyclophosphamide. Myeloablative regimens using melphalan (n = 8) or thiotepa (n = 1) with autologous bone marrow rescue were not clearly beneficial. The treatment results favor: (1) early surgical removal, (2) dose-intensive use of drugs active against PNET (especially cyclophosphamide), and (3) radiation therapy to ablate residual microscopic disease.

Adolescent↗
PubMed1980

Pigmented neuroectodermal tumor of infancy. An example of rarely expressed malignant behavior.

A 4-month-old patient presented with a melanotic neuroectodermal tumor. The tumor continued to progress and recur following each surgical intervention and the patient died at the age of 38 months, 36 months following discovery of the tumor. This article describes the clinical and pathologic features of the disease. At autopsy, the tumor resembled that of a neuroblastoma.

Child, Preschool↗
PubMed1995

Primitive neuroectodermal tumor (PNET). A case report.

The authors present a case of relatively rare tumor of the central nervous system (CNS) in a 19-year-old female, who died 18 months after the first manifestation of meningismus, increased intracranial pressure and secondary hydrocephalus. Brain autopsy revealed abundant neoplastic infiltrations, which spread through the subarachnoid space. Neoplastic infiltrations were also present in the third ventricle and in a form of small subependymal nodules along the whole ventricular system. The microscopical examination showed that neoplasm consisted of small cells, which formed neuroblastic Homer Wright rosettes. Immunohistochemical studies (for synaptophysin, chromogranin A, GFAP, vimentin) together with morphology and localization of neoplasm suggested diagnosis of primitive neuroectodermal tumor (PNET) that spread mainly in the leptomeninges and caused obliteration of subarachnoid space.

Adult↗
PubMed1978

Methylcholanthrene induced murine primitive neuroectodermal tumor: ultrastructure and nuclear RNA polymerase activity.

The histology, ultrastructure, and nuclear RNA polymerase activity are described in a murine primitive neuroectodermal tumor derived by serial transplantation from a tumor originally induced with methylcholanthrene and classified as an ependymoblastoma. The light microscope and ultrastructural studies show that this tumor does not contain the distinguishing morphological features of differentiated ependymal cells which are also commonly seen in human ependymomas. One outstanding feature is the size and number of the nucleoli. The mean number of nucleoli/nucleus is 4 which is two to four times that of the normal neuroglial cell. The nucleolar diameter is about twice that found in normal neuroglial cells. The nucleolar diameter is about twice that found in normal neuroglial cells. The nuclear RNA synthesizing activity is the highest of the chemically induced animal tumors we have studied. The alpha amanitin inhibition is the lowest seen in any of these tumors which suggests that RNA polymerases inhibited by alpha amanitin contribute less to the total nuclear RNA synthesis. Adriamycin significantly inhibits the nuclear RNA polymerase activity of this tumor.

Animals↗
PubMed1994

Cerebellar dysplasias in humans: development and possible relationship to glial and primitive neuroectodermal tumors of the cerebellar vermis.

Cerebellar dysplasias are commonly found in the white matter and nodulus of the vermis in newborns and are particularly prominent in infants with trisomy 13-15 and trisomy 18 syndromes. Little is known of the developmental biology of these structures. We have studied the development of cerebellar dysplasias in human fetuses ranging from 15 to 32 weeks gestational age and from 11 days to 15 months postnatal. The expression of developmentally regulated neuronal and glial polypeptides was investigated by immunohistochemistry using a panel of extensively characterized monoclonal antibodies. Dysplasias were first observed at 15 weeks gestation as irregularly distributed, parenchymal or perivascular clusters of primitive cells in the inferior vermis. These cell clusters resembled primitive neuroepithelial cells or cells of the cerebellar external granule cell layer and they persisted into postnatal life. They retained the capacity to undergo cell division and were weakly reactive for the low affinity nerve growth factor receptor but were negative for all other neuronal or glial proteins at all gestational ages. At about 20 weeks gestation, cerebellar dysplasias become more complex with the appearance of ganglion cells which matured histologically and phenotypically in parallel with normal dentate neurons and Purkinje cells. These dysplasias often contained a prominent glial component which was identified by immunostaining for glial fibrillary acidic protein. Our findings suggest that midline cerebellar dysplasias are normal variants of development. Whether the mitotically active cells comprising these dysplasias are targets for neoplastic transformation into cerebellar primitive neuroectodermal tumors or other types of childhood tumors such as pilocytic astrocytomas or atypical teratoid/rhabdoid tumors remains speculative.

Abortion, Therapeutic↗
PubMed1994

Increased risk of neuroectodermal tumors and stomach cancer in relatives of patients with Ewing's sarcoma family of tumors.

BACKGROUND: Previous studies of the genetic epidemiology of Ewing's sarcoma have shown neither an increased incidence nor a distinct pattern of cancers in family members of Ewing's sarcoma patients. PURPOSE: Because of a new biologic and cytogenetic classification of Ewing's sarcoma family of tumors, we wanted to reinvestigate the incidence and distribution of cancers in relatives of probands with Ewing's sarcoma family of tumors. METHODS: Patients treated at the Pediatric Branch and the Radiation Oncology Branch of the National Cancer Institute between 1965 and December 1992, or their next of kin, were asked to complete a questionnaire on the history of cancer in all first- and second-degree relatives. The incidence of cancer in family members was compared with Connecticut Tumor Registry rates specific for sex, age, and 5-year calendar-year intervals. Observed/expected (O/E) ratios, 95% confidence intervals (CIs), and tests of homogeneity were calculated. RESULTS: Four thousand six hundred seventy-eight family members with 196,640 person-years at risk entered the analysis. Overall, there was no increased risk of cancer (observed 472; O/E = 0.9; 95% CI = 0.8-1.0). However, several tumor types were found in significant excess. These tumors included stomach cancer (observed 34; O/E = 2.0; 95% CI = 1.4-2.8), melanoma (observed 23; O/E = 1.9; 95% CI = 1.2-2.8), brain tumor (observed 18; O/E = 1.9; 95% CI = 1.1-3.0), and bone cancer (observed 7; O/E = 4.2; 95% CI = 1.7-8.6). Risks of these cancers were higher among maternal than paternal relatives, but these differences were not statistically significant. There was a significant deficit of bladder cancer (observed 5; O/E = 0.2; 95% CI = 0.1-0.5) and rectal cancer (observed 0; O/E = 0.0; 95% CI = 0.0-0.1). Second-degree relatives had a significant cancer deficit (observed 389; O/E = 0.9; 95% CI = 0.8-0.95). This deficit was accounted for by the observed deficit of bladder and rectal cancer and is probably related to under-reporting or misclassification of cancer in second-degree relatives. Family members of 10 probands with second malignancies did not have an increased risk of all cancers (observed 20; O/E = 1.2; 95% CI = 0.7-1.8) but had an increased risk of both melanoma (observed 3; O/E = 7.3; 95% CI = 1.5-21.0) and breast cancer (observed 8; O/E = 3.2; 95% CI = 1.4-6.3). CONCLUSION: Finding an increased risk of neuroectodermal tumors and stomach cancer in families of patients with Ewing's sarcoma family of tumors suggests that these tumors might share a common etiology. Further studies should try to confirm this hypothesis and to examine if genetic factors may have a role in these families by assessing the mode of inheritance and examining families with multiple affected members.

Adolescent↗
PubMed2003

Involvement of p53 in specific anti-neuroectodermal tumor activity of aloe-emodin.

Previously, we have identified aloe-emodin (AE) as a new type of anticancer agent, with activity that is based on apoptotic cell death promoted by a neuroectodermal tumor-specific drug uptake. We attempt to clarify the intracellular target of AE and the apoptosis-signaling pathway activated by AE in neuroblastoma cell lines. Two-photon excitation microscopy and spectroscopic titrations documented that AE is highly concentrated in susceptible cells and binds to DNA. One of the most important mediators of apoptotic response to genotoxic stimuli, such as anticancer agents, is the p53 tumor suppressor gene. To evaluate the role played by p53 in AE-induced apoptosis a p53 mutant cell line, which lacks transcriptional activity of p53 targeted genes, was tested. AE displayed a reduced growth inhibitory and pro-apoptotic activity in p53 mutant cells (SK-N-BE(2c)) with respect to the p53 wild-type line (SJ-N-KP). This effect was not caused by a reduced drug uptake in the mutant neuroblastoma cell line but was related to a different apoptotic cell phenotype. Whereas SJ-N-KP cells were susceptible to a p53 transcription-dependent pathway of apoptosis, SK-N-BE(2c) cells underwent apoptosis with up-regulation of p53 expression but not of p53-target genes. After AE treatment p53 translocates to the mitochondria inter-membrane space in both neuroblastoma cell lines. Due to its high accumulation in neuroectodermal tumor cells AE could also kill tumor cells harboring p53 mutant genes. This property would further contribute to AE specific anti-tumor activity and might be exploitable in the clinic.

Anthraquinones↗
PubMed1986

Tumor of the testicle: a case of melanotic neuroectodermal tumor of infancy.

We describe an 8-month-old boy with a tumor involving the right testicle and epididymis. Anatomical and pathological examination of this tumor revealed embryological tissue and melanin granules derived from the neural crest. This is an uncommon neoplasm, which is especially rare in the testicles. The patient was free of disease after 30 months.

Epididymis↗
PubMed1992

Primitive neuroectodermal tumor arising in the skin. Differentiation from neuroendocrine carcinoma of the skin.

We report a 25-year-old male patient with primitive neuroectodermal tumor presenting as a subcutaneous tumor on the right buttock, which was initially diagnosed as neuroendocrine carcinoma of the skin. Subsequently, local recurrence and metastatic spread to the lung occurred. The tumor was resistant to highly aggressive chemotherapy. The patient died 6 months later with severe pulmonary and lymph node involvement.

Adult↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy occurring in the left thigh of a 6-month-old female infant.

We report an exceptional case of melanotic neuroectodermal tumor of infancy (MNTI) occurring in the soft tissue of the left thigh of a 6-month-old female infant. The tumor consisted mainly of small round cells (neuroblasts) arranged in cords and nests that were separated by broad fibrovascular areas. In addition, there were a few medium-sized tumor cells containing melanin pigment (melanocytic cells) that in electron microscopy contained melanosomes as well as tonofilaments. Both tumor cell types immunostained for neuron-specific enolase (NSE) and vimentin, and the melanocytic cells reacted additionally with the antikeratin antibody KL1. Within the tumor stroma, neurofilament- and S-100-protein-positive neural cells and vimentin- and desmin-positive myofibroblasts were seen. Although dense-core granules were demonstrated ultrastructurally in some neuroblasts, no immunostaining for chromogranin A, Leu-7, serotonin, or regulatory peptides was found. MNTI located in an extremity can be confused with malignant small round and blue cell tumors of childhood. The distinction between MNTI and these tumors is of clinical significance because MNTI, in most cases, is a benign tumor that, in contrast to the latter, can be cured by complete excision. The presence of a biphasic cell population with neuroblasts and melanocytic cells must be considered the main diagnostic feature of MNTI.

Female↗
PubMed1988

Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients.

The clinical presentation of the disease and the results of treatment in 42 patients with malignant peripheral neuroectodermal tumors (MPNT) entered into the Cooperative Ewing's, soft tissue, and neuroblastoma trials of the German Society of Pediatric Oncology were retrospectively analyzed. Within the Ewing's sarcoma trial, patients with chest wall lesions were particularly analysed for MPNT features. The period of observation ranged from 15 to 86 months; the median relapse-free time was 24 months. There were 28 male and 14 female patients, the median age of patients was 15 years (range, 9 months-23 years). Thirty-two patients had localized disease (M0), and ten patients presented with primary metastases (M1). The predominant location of the tumors was the thoracopulmonary region, followed by the extremities, the abdominal/pelvic, and head and neck region. Thirty-one of 42 tumors involved the adjacent bone. The disease-free survival according to Kaplan-Meier life-table analysis was 56% +/- 11% for Stage M0 patients at 3 years. Nine of ten patients with M1 disease showed progression of their disease. Most patients had combined modality treatment with surgery, chemotherapy and radiation therapy. Best results were obtained with extensive surgery. Radiation doses ranged from 20 to 60 Gy and could not be correlated with the outcome of the disease. Most recurrences occurred at the site of the primary tumor. In patients with primary chemotherapy after biopsy-proven diagnosis, the responsiveness of this disease to chemotherapy could be demonstrated. Combination chemotherapy containing anthracyclines and high doses of alkylating agents appeared superior.

Actuarial Analysis↗
PubMed1997

Chromosome arm 17p deletion analysis reveals molecular genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumors of the central nervous system.

The current World Health Organization (WHO) classification groups together both infratentorial neoplasms (medulloblastomas) and their supratentorial counterparts as primitive neuroectodermal tumors (PNETs), implying a common origin. Previous analyses of medulloblastoma have shown loss of chromosome arm 17p as the most frequent genetic abnormality: the molecular genetic constitution of supratentorial PNETS has not been systematically studied. We therefore examined 8 hemispheric PNETs and 35 medulloblastomas with 17p restriction fragment length polymorphism (RFLP) and microsatellite markers. We also examined the TP53 tumor suppressor gene by a combined polymerase chain reaction-denaturing gradient gel (PCR-DGGE) technique. Our results showed that all of the 17p markers tested were preserved in all of the supratentorial PNET specimens. In contrast, loss of distal chromosome arm 17p was detected in 37% of the medulloblastomas. Analysis of the TP53 gene showed 2 mutations in the medulloblastomas and no mutations in the supratentorial tumors. These results show that the most common molecular genetic abnormality in infratentorial PNETS is absent in their supratentorial counterparts and suggests that alternative pathways and genetic events may be involved in their etiology.

Adolescent↗
PubMedDate not supplied

Primitive neuroectodermal tumors of the cerebrum: a histological and immunohistochemical study of 10 cases.

Four males and 6 females, 5-66 (mean 27) years, with supratentorial primitive neuroectodermal tumors (PNETs) have been treated in one center in a 5-year period. Symptoms and signs had been present from 4 days to 8 years and were highly variable. The frontal hemisphere was involved in 6 cases. Seven patients have died but with a combination of surgery and radiotherapy 3 patients have survived 20 months or longer. All tumors were composed of rounded, hyperchromatic undifferentiated cells but there were focal areas of astrocytic (5 cases), mesenchymal (4 cases) or neuronal differentiation (1 case). In 9 of the 10 cases a proportion of the undifferentiated round cells stained with antibodies to GFAP. Seven of these also showed nuclear staining for S100 protein and 6 of these, plus the tumor that did not stain for GFAP, had cytoplasmic staining for neuron specific enolase. Six tumors stained with antibodies to vimentin but there was no staining for common leukocyte antibody, cytokeratin or myoglobin. These results, in particular those with GFAP, indicate that PNETs undergo differentiation even in areas of apparent morphological uniformity. Furthermore, immunohistochemistry is useful in distinguishing PNETs from other "small blue cell tumors" such as malignant lymphoma and undifferentiated secondary carcinoma.

Adolescent↗