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PubMed1985

Immunohistochemical demonstration of neuronal and astrocytic differentiation in retinoblastoma.

Sections of 51 surgically enucleated eyes from cases of retinoblastomas were examined immunohistochemically to delineate patterns of cellular differentiation. Employing the avidin-biotin immunoperoxidase technique, antibodies were used against glial fibrillary acidic protein (GFAP), S-100 protein and neuron specific enolase (NSE). Areas of uninvolved retina and/or optic nerve were used as built-in positive control. Most of the tumors showed GFAP and S-100 protein-positive perivascular glial cells that were interpreted as reactive astrocytes. In three well-differentiated retinoblastomas, glial cells were found to be interspersed randomly among tumor cells and not associated with blood vessels. These glial cells were interpreted as neoplastic based on their distribution pattern and cytologic features. In about half of the tumors, the retinoblastoma cells stained positively for NSE, indicating their neuronal tumor cells that stained positively for NSE but failed to show any evidence of photoreceptor differentiation. Based on these observations and similar differentiation patterns described in other primitive neuroectodermal tumors of the brain, it is suggested that retinoblastoma cells can differentiate not only into photoreceptor cells but also along other neuronal cell lines and rarely into glial cells.

Astrocytes↗
PubMed1999

Comparative genomic hybridization reveals novel chromosome deletions in 90 primary soft tissue tumors.

Comparative genomic hybridization (CGH) was used to detect chromosomal gains and losses in a series of 90 frozen soft tissue primary tumors (STTs), all untreated. The material consisted of 69 malignant sarcomas, including 20 malignant fibrous histiocytomas (MFH), 23 liposarcomas (LPS), 6 leiomyosarcomas (LMS), 4 synovial sarcomas, 4 primitive neuroectodermal tumors (PNETs), and various others subtypes, in addition to 21 benign tumors. Within the benign tumors, only 2 of the 3 schwannomas showed genetic changes. In malignant sarcomas, genetic changes were detected in 64 of the 69 samples analyzed (92%), with a mean of 4.5 per sample (range 0-10). Gains and losses on chromosome 13 were observed in 32% of the sarcomas with genomic imbalance. Recurring low-level copy number increases were found at new sites on chromosomes 7 (6 MFH samples, 30%) and 8 (10 LPS samples, 43%), the minimal common regions being 7p15-pter and 8q24. No new recurring high-level amplifications were found. Surprisingly, losses of DNA sequences were more frequent than gains; particularly, losses were the main feature in LMS, with highly recurrent common minimal losses at 11q14-qter and 13q21-q22 (4 samples, 66%, and 5 samples, 83%, respectively). Losses of chromosome 2 sequences (minimal common regions at 2p24-pter and 2q32-qter) were observed in 50% of the MFH analyzed. New recurrent losses of whole or part of chromosome 14 were found in 57% of the pleomorphic LPS (PLPS) analyzed. This study uncovers new clues for the diagnosis of malignant STTs and shows the importance of deletions as events in the early steps involved in the tumorigenesis of STTs.

Chromosome Deletion↗
PubMed2000

Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors".

We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass. The patient had been well from birth and had shown no signs of immunodeficiency. She was negative for human immunodeficiency virus. Blood examination revealed elevated serum neuron specific enolase (NSE). Histologically, the tumor was comprised of solid growths of round or polygonal cells with vesicular nuclei and often vacuolated cytoplasm rich in glycogen. The tumor cells were positive for vimentin, NSE, and MIC2, and were negative for desmin and neurofilament. The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor. Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells. An expanded panel of immunostains showed that the tumor cells were intensely positive for smooth muscle actin, and ultrastructural study revealed abundant fine cytoplasmic filaments with focal subsarcolemmal densities, various amounts of glycogen, and irregularly arranged, thick basal lamina. The diagnosis of epithelioid leiomyosarcoma was made. Following reduction in tumor size by chemotherapy, the serum NSE level was normalized. From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome. Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors."

Abdominal Neoplasms↗
PubMed1987

[S-100 protein in non-pigmented melanocytic and neuro-ectodermal tumors of the conjunctiva].

Using an immunoperoxidase method, S-100 protein was localized in 51 nonpigmented or slightly pigmented nevi, eight malignant melanomas, three neurofibromas, and one neurilemmoma of the conjunctiva. Clinico-immunohistochemical correlations are presented. Cells of these neuroectodermal tumors contain S-100 protein. Epithelial invaginations arising from the conjunctival surface and lying between melanocytes are S-100 protein-negative. This facilitates the differential diagnosis of particularly amelanotic lesions of the conjunctiva. Immunohistochemical identification of the S-100 protein represents an improvement in current histochemical differential diagnosis of these tumors.

Conjunctiva↗
PubMedDate not supplied

Effect of P-glycoprotein expression on outcome in the Ewing family of tumors.

This study was designed to determine the prognostic significance of multidrug resistance, mediated by P-glycoprotein (Pgp) expression, in Ewing sarcoma. The clinical and laboratory features, treatment protocol, and outcome of 75 patients with Ewing sarcoma or peripheral neuroectodermal tumor treated between 1972 and 1997 were reviewed. Pgp expression was tested with the monoclonal antibody JSB-1. Thirty-four (64%) of the 53 tissue samples from untreated patients stained positive for Pgp. Progression-free and overall survival were 44 and 59%, respectively, in patients with negative findings, and 28 and 41% in those with positive findings; neither difference was significant. Of the 12 relapsed patients, 6 (50%) expressed more Pgp after chemotherapy than at diagnosis and 4 (33%) expressed less. Within these subgroups, 5 out of 6 and 3 out of 4 died from the disease. No correlation was found between Pgp and known prognostic factors of Ewing tumors. Pgp expression is probably an intrinsic factor of Ewing tumors but has no correlation to prognosis.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1995

Association of Wilms' tumor with primary brain tumor in siblings.

The cases of two young male siblings independently developing unilateral Wilms' tumors and brain tumors are reported. The renal tumors were resected; the first child was treated with chemotherapy and the second child was given additional radiotherapy. Five years after treatment, both children developed a second primary neuroectodermal tumor. All four tumors showed a high proliferative activity, and rapidly progressing disease led to the death of the first child. Histopathological and molecular studies were carried out on all four neoplasms. No functionally relevant mutation was found in selected exons of the p53, K-ras and WT1 gene loci of tumor and germ line DNA. Since additional family members had developed brain tumors and carcinomas, this peculiar association of neoplasms may be due to germ line mutation of a hitherto unidentified oncogene acting in a recessive or weakly dominant fashion.

Base Sequence↗
PubMed1997

Chromosomal translocations in human soft tissue sarcomas by interphase fluorescence in situ hybridization.

In soft tissue sarcomas, clonal rearrangement of chromosomes has been shown by cytogenetic analysis to be unique and specific for tumor types. The development of fluorescence in situ hybridization (FISH) has allowed detection of chromosomal rearrangements in the interphase nuclei isolated from paraffin-embedded tissues. Three kinds of translocations in the interphase nuclei that were isolated from 47 cases of soft tissue sarcomas were examined by FISH with chromosome-specific DNA probes of centromeric and total probes. Of 47 soft tissue sarcomas 42 (89.4%) revealed tumor-specific translocations by retrospective cytogenetic analysis. Translocation t(X;18) was detected in 25/28 synovial sarcomas; translocation t(11;22) in 5/6 Ewing's sarcomas and primitive neuroectodermal tumors (PNET); and translocation t(12;16) was found in 12/13 liposarcomas, including 10 myxoid and two round cell types as clonal chromosomal aberrations specific for both subtypes. Based on the cytogenetic analysis, Ewing's sarcoma is related closely with PNET as shown by MIC2-protein reactivity. Other cytogenetic findings of translocation t(12;16) indicate that round cell liposarcomas share chromosomal changes with myxoid liposarcomas, and further suggest that both tumor subtypes of liposarcoma may possess common precursor cells. FISH is a useful aid in determining the tumor type of soft tissue sarcomas with regard to histogenetic origin.

Adolescent↗
PubMedDate not supplied

Primary thoracic sarcomas.

Primary sarcomas of the thorax are rare. The diagnosis is established only after sarcomalike primary lung malignancies and metastatic disease have been excluded. Primary sarcomas of the thorax are classified according to their histologic features and constitute a large group of tumors that occur in the lung, mediastinum, pleura, and chest wall. Angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and mesothelioma (sarcomatoid variant) are the most common primary intrathoracic sarcomas. Ewing sarcoma, primitive neuroectodermal tumor, chondrosarcoma, malignant fibrous histiocytoma, osteosarcoma, synovial sarcoma, and fibrosarcoma usually arise in the chest wall. Although primary thoracic sarcomas commonly manifest as large, heterogeneous masses, they have a wide spectrum of radiologic manifestations, including solitary pulmonary nodules, central endobronchial tumors, and intraluminal masses within the pulmonary arteries. The different histologic types of sarcomas are frequently indistinguishable at radiologic analysis. However, differences in clinical presentation and the location of the tumor, as well as morphologic features such as calcification within the mass and rib involvement, can be useful in suggesting the appropriate diagnosis. For example, a large rib mass in a child with fever and malaise indicates a Ewing sarcoma, a mass with a calcified matrix is likely a chondrosarcoma or osteosarcoma, and a pulmonary artery mass is likely a leiomyosarcoma.

Adult↗
PubMed1990

Soft tissue tumors in first year of life: a report of 190 cases.

A comprehensive review of soft tissue tumors in children and adolescents disclosed the presence of 190 neoplasms in 183 patients, which were diagnosed in infants between birth and 12 months of age; these infants represented approximately 20% of our entire pediatric soft tissue tumor series. In excess of 75% of cases were pathologically benign with the hemangioendothelioma, lymphangioma, and fibromatosis-myofibromatosis constituting the majority of cases in this category. Fibrous histiocytoma and lipoblastoma were the other two benign entities. Congenital-infantile fibrosarcoma was considered a borderline tumor because of its infrequently manifested potential for metastasis; none of the 13 cases in the present study behaved in a malignant fashion. Embryonal rhabdomyosarcoma and peripheral primitive neuroectodermal tumor were the two principal types (17 of 27 cases) of malignant soft tissue tumors. In contrast to soft tissue tumors in the first two decades, those in the first year of life were more often benign despite their cellularity and presence of mitotic activity. Fibroblastic-myofibroblastic tumors were more frequent in this young age group, whereas neurogenic and myogenic tumors were relatively more common in children older than 1 year of age. The trunk and head and neck region were the preferred topographic sites rather than the extremities, which was the case in children beyond the first year of life.

Female↗
PubMed1994

[Immunohistochemical and ultrastructural studies of intracranial immature teratoma--a comparative observation on neuroblastoma, PNET and ependymal tumor, with a special reference to rosette structures].

Immature teratoma occurs occasionally in the brain of children and contains a large amount of immature neuronal tissue. These primitive neuronal components are a good target for studying the morphology of primitive neuroectodermal tumors, including neuroblastoma, ependymoblastoma, medulloepithelioma and so on. Primitive neural tubes are immunohistochemically and ultrastructurally studied in two cases of primary immature teratoma of the child brains, compared to true rosettes in a case of neuroblastoma, primitive neural tube in the fetal rat brain (9 to 13 days of gestational age). The study also extends to the pathology of PNET. Ultrastructurally the primitive neural tube like structures in two teratomas were virtually identical those of developing fetal rat brains and true rosettes in a neuroblastoma. However, these tubular structures are different from each other in immunohistochemistry. These differences are considered to reflect the different developmental lineage of the tumor cells, that is, neuroblastoma produces only neuroblastic cells, and primitive neural tubes in teratoma both neuroblastic and glioblastic cells. Rejuvenation of neuroblastoma cells seems to render a VIM-positivity of the tumor cells.

Animals↗
PubMed1998

Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors.

Two hundred fifty-five well-characterized formaldehyde-fixed and paraffin-embedded small round cell tumors mainly from children and young adults including 105 neuroblastomas were immunohistochemically analyzed with the NB84 monoclonal antibody raised to neuroblastoma cells. Most of the undifferentiated neuroblastomas (21 of 22) and all 83 differentiated neuroblastomas reacted with NB84, but none of these tumors were CD99 positive. Compared with synaptophysin, NB84 was more sensitive, although less specific, in the identification of neuroblastoma in formaldehyde-fixed tissue. In addition to neuroblastoma, skeletal and extraskeletal Ewing's sarcoma and medulloblastoma showed NB84 reactivity in approximately 20% of cases, and 50% of desmoplastic small round cell tumors showed positive cells, usually in smaller numbers than the neuroblastomas. The NB84 reactivity was seen slightly more commonly in morphologically defined (rosette-positive) cases of peripheral primitive neuroectodermal tumors than in Ewing's sarcoma. However, the NB84 positivity did not correlate with the expression of other neural markers (neurofilament proteins, CD57, and synaptophysin) in these tumors. All other small round cell tumors including rhabdomyosarcomas, Wilms' tumors, and lymphomas were NB84 negative. In the case of NB84-positive tumors other than neuroblastoma, their specific reactivity for other markers was useful (Ewing's sarcoma CD99 positive, desmoplastic small round cell tumor desmin and keratin positive). The NB84 monoclonal antibody is a useful reagent to separate neuroblastoma from other small round cell tumors. In problem cases it is best used in a panel together with other markers that address the significant differential diagnostic alternatives.

Adolescent↗
PubMed1996

Cutaneous neural heterotopias and related tumors relevant for the dermatopathologist.

Heterotopic neural tumors are rare in the skin; however, when encountered, they often pose a diagnostic problem. While the clinicopathologic features of most neuroaxis abnormalities are well-documented in the neuropathologic literature, their significance in cutaneous pathology as well as the associated nonneural dermatopathologic findings have not received enough attention. This report offers a comprehensive review of the most common cutaneous neural heterotopic abnormalities and their related tumors. The clinicopathologic features of the following entities along with their histogenetic considerations are discussed: classical and rudimentary meningocele, primary cutaneous meningioma, heterotopic brain tissue (nasal glioma), peripheral primitive neuroectodermal tumor, metastatic neuroblastoma, and ganglioneuroma. Familiarity with the associated dermatopathologic changes and with the differential diagnosis should assist in arriving at the correct diagnosis even without special training in dermatopathology or neuropathology.

Choristoma↗
PubMed2005

Epidemiology and etiology of gliomas.

Gliomas of astrocytic, oligodendroglial and ependymal origin account for more than 70% of all brain tumors. The most frequent (65%) and most malignant histological type is the glioblastoma. Since the introduction of computerized tomography and magnetic resonance imaging, the incidence rates of brain tumors have been rather stable, with a tendency of higher rates in highly developed, industrialized countries. Some reports indicate that Caucasians have higher incidence than black or Asian populations, but to some extent, this may reflect socio-economic differences and under-ascertainment in some regions, rather than a significant difference in genetic susceptibility. With the exception of pilocytic astrocytomas, the prognosis of glioma patients is still poor. Less than 3% of glioblastoma patients are still alive at 5 years after diagnosis, higher age being the most significant predictor of poor outcome. Brain tumors are a component of several inherited tumor syndromes, but the prevalence of these syndromes is very low. Several occupations, environmental carcinogens, and diet (N-nitroso compounds) have been reported to be associated with an elevated glioma risk, but the only environmental factor unequivocally associated with an increased risk of brain tumors, including gliomas, is therapeutic X-irradiation. In particular, children treated with X-irradiation for acute lymphoblastic leukemia show a significantly elevated risk of developing gliomas and primitive neuroectodermal tumor (PNET), often within 10 years after therapy. TP53 mutations are frequent in low-grade gliomas and secondary glioblastomas derived therefrom. Approximately 60% of mutations are located in the hot spot codons 248 and 273, and the majority of these are G:C-->A:T transitions at CpG sites. TP53 mutations are significantly more frequent in low-grade astrocytomas with promoter methylation of the O(6)-methylguanine-DNA methyltransferase repair gene, suggesting that, in addition to deamination of 5-methylcytosine, exogenous or endogenous alkylation in the O(6) position of guanine may contribute to the formation of these mutations.

Age Factors↗
PubMed2003

Intra-epithelial neuroendocrine carcinoma of the nasal cavity.

We describe a distinctive neuroendocrine carcinoma (NEC) that proliferated intra-epithelially. The tumor was 35 mm in diameter and arose from the right superior turbinate of a 46-year-old woman. Histologically, the tumor exhibited papillary growth and the tumor cells were localized in the thickened mucosal epithelium. The tumor cells had round to oval and vesicular or hyperchromatic nuclei, and cohered without any specific structure such as a fibrillary background, rosette or glandular structure. No stromal invasion by the tumor was observed. Immunohistochemically, the tumor cells were positive for neuron-specific enolase focally. In addition, many tumor cells expressed cytokeratin (AE1/AE3 and CAM 5.2), mostly with characteristic perinuclear dot-like patterns. Electron microscopy revealed focal but well-eveloped cytoplasmic processes containing arrays of microtubules and a few dense core granules. The tumor was considered to be a poorly differentiated neuroendocrine carcinomas (NEC) that exhibited exceptional intra-epithelial proliferation. The tumor completely disappeared after the stereotactic radiosurgery and has not recurred for 40 months. It might be difficult to distinguish a poorly differentiated NEC in the sinonasal region from other neuroectodermal tumors, including olfactory neuroblastoma, but the differential diagnosis is important because each tumor has different clinicopathological characteristics.

Biomarkers, Tumor↗
PubMed1984

Malignant peripheral nerve sheath tumors arising from ganglioneuromas.

Two typical malignant peripheral nerve sheath tumors (PNST) arising in preexisting ganglioneuromas are described. To the best of our knowledge, this association of tumors has not been reported in detail previously. Neither patient had the stigmata nor family history of Von Recklinghausen's neurofibromatosis. In both cases, the ganglioneuromas evolved from more primitive neuroectodermal tumors (one neuroblastoma, one ganglioneuroblastoma) and both patients developed their malignant PNST at previously irradiated sites. Both patients died within 2 years of the diagnosis of their malignant PNST. The origin of these malignant PNSTs from Schwann cells is supported by an ultrastructural analysis of 27 neuroblastomas, ganglioneuroblastomas, and ganglioneuromas.

Adolescent↗
PubMed1989

[Cell kinetic analysis of human brain tumors by bivariate flow cytometric measurement of cellular DNA content and amount of incorporated bromodeoxyuridine].

Cell kinetics of 91 human brain tumors obtained from 88 patients were analyzed with the following two methods, 1) bivariate (two-color) flow cytometric measurement of cellular DNA content and amount of bromodeoxyuridine (BrdU) incorporated into cellular DNA, in 66 specimens, 2) immunohistochemical detection of BrdU incorporated S-phase cells, in 34 specimens. Patients were given an intravenous 1 hour infusion of 200 mg/sq. m. of BrdU 1-2 hours before the surgical removal. The excised tumor specimen was divided into several portions. One was fixed with 70% ethanol and embedded in paraffin, and another was digested mechanically and/or chemically to obtain a single cell suspension, and fixed in 70% ethanol. Paraffin-embedded tissue sections were stained by the peroxidase-antiperoxidase immunohistochemical method using anti-BrdU monoclonal antibody (MoAb). Single cell suspensions were reacted with fluorescein isothiocyanate (FITC) conjugated anti-BrdU MoAb, or anti-BrdU MoAb and FITC-conjugated second antibody successively by the staining with propidium iodide, for flow cytometry (FCM). Rates of S-phase fraction in single cell suspensions calculated by bivariate FCM were correlated well with labeling indexes (LI, i.e. the percentage of BrdU incorporated cells) calculated in tissue sections, but not with the result of analysis of DNA histogram by Dean's method. This discrepancy is probably due to large coefficient value in several samples. Histological malignancy of the tumors was reflected both in the proliferating index (PI, i.e. % S+G2M phase) calculated by bivariate FCM and the LI by immunohistochemical method. PI tended to be high in primitive neuroectodermal tumors and metastatic carcinomas, moderately high in gliomas, and low in benign tumor groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Monoclonal↗
PubMed2004

Expression of PAX 3 alternatively spliced transcripts and identification of two new isoforms in human tumors of neural crest origin.

The developmental gene PAX 3 is expressed in the early embryo in developing muscle and elements of the nervous system, including the brain. Since no one has investigated the expression of the isoforms of PAX 3 in the neuroectodermal tumors melanoma and small cell lung cancer (SCLC), we have carried out a comprehensive screening for the expression of the isoforms PAX 3a-e using RT-PCR in human melanoma cell lines, primary human ocular and secondary cutaneous melanomas. We have identified 2 new isoforms of PAX 3, g and h, which we have isolated, cloned and sequenced. Sets of primers for each isoform were designed and their specificity was confirmed by sequence analysis of the products. The isoforms PAX 3a-e were detected in all human cutaneous melanoma cell lines (8/8), but only PAX 3c (1/2) and PAX 3d (2/2) in ocular melanoma cell lines. The same PAX 3 isoforms were detected in more than 80% of human cutaneous melanomas: PAX 3a and b (15/17), PAX 3c (14/17), PAX 3d (16/17) and PAX 3e (15/17). In contrast the results for 7 SCLC cell lines were PAX 3a (0/7), PAX 3b (1/7), PAX 3c (3/7), PAX 3d (6/7), PAX 3e (2/7); 8/8 cutaneous melanoma cell lines and 8/8 ocular melanoma tissues, together with 14/17 cutaneous melanoma tissues screened, expressed the new isoform PAX 3g. All 8 cutaneous melanoma cell lines expressed PAX 3h, but it was not detectable in any of the tumor tissues (0/20). Neither of the 2 ocular melanoma cell lines expressed the 2 new isoforms. Comparison of the different amplicon staining intensities on a gel suggests that PAX 3c and PAX 3d are the predominant transcripts expressed, with relatively low expression of PAX 3e and PAX 3h. We propose that these and the 2 new isoforms we have discovered may be important in oncogenesis and differential diagnosis of melanomas or SCLC.

Alternative Splicing↗
PubMed2006

Metalloproteinase disintegrins ADAM8 and ADAM19 are highly regulated in human primary brain tumors and their expression levels and activities are associated with invasiveness.

Patients with primary brain tumors have bleak prognoses and there is an urgent desire to identify new markers for sensitive diagnosis and new therapeutic targets for effective treatment. A family of proteins, the disintegrin and metalloproteinases (ADAMs or adamalysins), are cell surface and extracellular multidomain proteins implicated in cell-cell signaling, cell adhesion, and cell migration. Their putative biological and pathological roles make them candidates for promoting tumor growth and malignancy. We investigated the expression levels of 12 cerebrally expressed ADAM genes in human primary brain tumors (astrocytoma WHO grade I-III, glioblastoma WHO grade IV, oligoastrocytoma WHO grade II and III, oligodendroglioma WHO grade II and III, ependymoma WHO grade II and III, and primitive neuroectodermal tumor WHO grade IV) using real-time PCR. The mRNAs of the five ADAMs 8, 12, 15, 17, and 19 were significantly upregulated. The ADAM8 and ADAM19 proteins were mainly located in tumor cells and in some tumors in endothelia of blood vessels. In brain tumor tissue, ADAM8 and ADAM19 undergo activation by prodomain removal resulting in active proteases. By using specific peptide substrates for ADAM8 and ADAM19, respectively, we demonstrated that the proteases exert enhanced proteolytic activity in those tumor specimens with the highest expression levels. In addition, expression levels and the protease activities of ADAM8 and ADAM19 correlated with invasive activity of glioma cells, indicating that ADAM8 and ADAM19 may play a significant role in tumor invasion that may be detrimental to patients survival.

ADAM Proteins↗