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PubMed1995

Primitive neuroectodermal tumor of maxilla in an adult.

Primitive neuroectodermal tumor is primarily a central nervous system tumor. These tumors are generally manifest in infancy or early childhood. The following article reports a rare case of primitive neuroectodermal tumor in posterior maxilla in an adult. Treatment for primitive neuroectodermal tumor in extracranial sites in adults is not clearly defined in the literature. This case was treated by combined chemotherapy followed by radiotherapy, which failed to cause regression of the lesion.

Adult↗

PubMed1999

Cell cycle-associated proteins in melanotic neuroectodermal tumor of infancy.

OBJECTIVE: The purpose of the present study was to compare the immunohistochemical expression of cell cycle-associated proteins in neuroblastic and melanocytic cell populations of melanotic neuroectodermal tumor of infancy. STUDY DESIGN: Three cases of melanotic neuroectodermal tumor of infancy were selected. The immunohistochemical expression of MDM-2, p53, proliferating cell nuclear antigen, cyclin D1, and cyclin A was assessed through use of the streptavidin-biotin-peroxidase complex technique. RESULTS: Positive immunostaining for MDM-2, proliferating cell nuclear antigen, cyclin D1, and cyclin A was occasionally observed in the large melanin-containing epithelioid cells. CONCLUSIONS: These data suggest that MDM-2 expression may be important for the development of melanotic neuroectodermal tumor of infancy and that the melanocytic cell population, not the neuroblastic one, is the proliferative component of the tumor.

Cell Cycle Proteins↗

PubMed1996

Carcinosarcoma of the uterus with extensive neuroectodermal differentiation.

A case of uterine carcinosarcoma with extensive neuroectodermal differentiation occurring in a 54-year-old woman is described. Microscopically, the endometrial tumour was predominantly composed of a sheet-like proliferation of small- and medium-sized cells. These cells were characterized by fibrillary cytoplasmic processes, rosette-like formations, perivascular palisading patterns, and immunoreactivity for glial fibrillary acidic protein, synaptophysin, leu 7 and neuron-specific enolase. In limited areas, features of conventional carcinosarcoma, including squamous cell carcinoma, leiomyosarcoma and islands of the above mentioned small- to medium-sized cells that were intermingled, were observed. The tumour was aneuploid by flow cytometry. The patient is alive with tumour for three months. It is suggested that at least some cases of uterine primitive neuroectodermal tumour may be explained by one-sided neuroectodermal development within a carcinosarcoma.

Biomarkers↗

PubMed2001

Teratoid carcinosarcoma of the ovary with prominent neuroectodermal differentiation.

We present what we believe to be only the second report of ovarian teratoid carcinosarcoma. The patient, a 59-year-old woman, was admitted to hospital complaining of a pelvic mass and of abdominal fullness. Advanced ovarian cancer was diagnosed, and a tumorectomy was done. The tumor occupied the pelvis, and metastasis was found in the liver and spleen. The solid tumor was composed of chondrosarcoma, squamous cell carcinoma, adenocarcinoma and malignant neuroectodermal components, which contained ganglioneuroblastoma-like and medulloepithelioma-like areas. Immunohistochemically, the neuroectodermal cells were positive for both neural and epithelial markers. This ovarian tumor consisted of frankly malignant components, with prominent neuroectodermal elements mixed with epithelial and mesenchymal elements in an organoid fashion; a quite rare tumor.

Biomarkers, Tumor↗

PubMed2002

Primitive neuroectodermal tumours of the uterus: a case report with cytological correlation and review of the literature.

A case of primitive neuroectodermal tumour (PNET) co-existing with endometrial adenocarcinoma of the uterus is reported in a 48-year-old female. Histologically, the neoplastic cells formed neuroectodermal rosettes and displayed ependymal differentiation. Focal positive immunohistochemical staining for synaptophysin, chromogranin A, CD57 and cytokeratins was noted. Merging imperceptibly with the neuroectodermal component were areas of endometrial adenocarcinoma and, to the best of our knowledge, this report represents the second case of a uterine PNET with an admixed müllerian component. The patient was disease-free 6 months following surgical resection of the tumour. The literature on this uncommon entity is critically reviewed and the histogenesis, differential diagnoses and cytomorphological features of PNET are also discussed.

Adenocarcinoma↗

PubMed1992

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.

Abdominal Neoplasms↗

PubMed1993

Primary neuroectodermal tumors of the ovary. A report of 25 cases.

Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories--differentiated, primitive, and anaplastic--with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.

Adolescent↗

PubMed1998

Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors.

Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other soft-tissue sarcomas; the poorly differentiated variant (PDSS), in particular, may be histologically indistinguishable from other small, blue, round cell tumors, including primitive neuroectodermal tumors (PNETs). Detection of the synovial sarcoma-associated t(X;18) by either cytogenetic or molecular genetic approaches may be necessary to confirm the diagnosis of synovial sarcoma in difficult cases. We evaluated 10 cases of PDSS from eight patients using a panel of antibodies (including those to intermediate filament proteins, nerve-sheath associated markers, and neuronal and neuroectodermal associated markers) in order to better establish the immunophenotype of this tumor and to help distinguish it from the tumors with which it may be confused, particularly PNETs and high-grade malignant peripheral nerve sheath tumors (MPNSTs). Our results showed PDSS to have significant immunophenotypic overlap with both PNETs and MPNSTs. In most instances these three entities may be differentiated by a panel of antibodies that should include those to both low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, CD99, CD56, and S-100 protein. Our results also suggest that synovial sarcoma may be a tumor showing combined neuroectodermal and nerve sheath differentiation--perhaps because of translocation-associated expression of specific proteins--rather than a carcinosarcoma of soft tissues or a tumor of specialized arthrogenous mesenchyme.

12E7 Antigen↗

PubMed1995

Ewing's sarcoma and extracranial peripheral neuroectodermal tumors.

Ewing's sarcoma and extracranial primitive neuroectodermal tumors of bone and soft tissue are small round-cell tumors that comprise a family of neoplasms distinguished by a cytogenetic marker that can be detected by reverse transcription and polymerase chain reaction. Based on molecular and immunohistochemical studies, it is generally acknowledged that this is a family of neuroectodermal tumors. Molecular methods allow accurate diagnosis with minimally invasive surgical options, provide a manner to detect minimal residual disease, and begin to shed light on the pathogenesis of these entities. Combined modality treatment regimens continue to improve the outlook for nonmetastatic Ewing's sarcoma and primitive neuroectodermal tumors patients. Patients who present with metastatic disease may benefit from intensive therapy as facilitated by peripheral stem cell and autologous bone marrow rescue programs.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2000

Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.

Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.

12E7 Antigen↗

PubMed2000

Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.

This study describes a new case of Ewing sarcoma (ES)-peripheral primitive neuroectodermal tumor (pPNET) with unusual phenotype and fusion gene structure. The tumor located in the inguinal area of a 15-year-old boy showed a highly aggressive behavior with hematogenous metastases after intensive chemotherapy and bone marrow transplant, causing death 28 months after diagnosis. The tumor displayed a clear cell pattern, and several neuroectodermal markers proved positive both in the original tumor and in xenografts. This neuroectodermal character was confirmed by electron microscopy. Moreover, cytogenetically the tumor has an unusual chromosomal rearrangement, t(2;22)(q13;q22,t(3;18)(p21;q23); representing a new EWS-FEV fusion type in which exon 7 of EWS gene is fused with exon 2 of FEV gene. This is the third published study of an ES-pPNET showing EWS-FEV fusion described, but it is the first study of a tumor with the aforementioned fusion points. These findings support the genetic and morphologic heterogeneity existing within the group of ES-pPNET tumors.

Adolescent↗

PubMed2003

Peripheral primitive neuroectodermal tumor after radiotherapy.

A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis. This type of sarcoma usually is classified into the heterogeneic group of small round-cell bone tumors as a subdivision of Ewing's sarcomas. The immuno-staining positivity of the neoplastic cells for the neuron-specific enolase allowed the authors to make the diagnosis of a tumor with neuroectodermal origin. When the histologic study confirmed the diagnosis, the patient was treated with chemotherapy, surgical excision of the tumor, and adjuvant radiotherapy. Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy.

Adult↗

PubMed2006

Primitive neuroectodermal tumor of the uterus: a case report and review of literature.

A case of primitive neuroectodermal tumor arising in the uterine corpus of a 43-year-old woman is presented. The tumor mass was 13.3 cm and extended to the uterine serosa, endocervical stroma, and left adnexa. Histologically, the tumor was composed of small blue cells with scant cytoplasm, indistinct cell borders, hyperchromatic round nuclei, and inconspicuous nucleoli focally forming pseudorosettes, suggestive of neuroectodermal origin. The tumor cells displayed strong immunoreactivity for CD99 and FLI1. Cytogenetic fluorescence in situ hybridization study revealed presence of an EWS-FLI1 fusion gene. To the best of our knowledge, this is the first case of primitive neuroectodermal tumor of the uterus with diagnosis confirmed by FLI1 immunohistochemical labeling and demonstration of t (11; 22) by fluorescence in situ hybridization.

12E7 Antigen↗

PubMed1991

Atypical primitive neuroectodermal tumors. Comparative light and electron microscopic and immunohistochemical studies on peripheral neuroepitheliomas and Ewing's sarcomas.

Recently, primitive neuroectodermal tumors (PNETs) have been shown to cover a wide spectrum of small round cell sarcomas, probably including some Ewing's sarcomas (ESs) and extraskeletal Ewing's sarcomas (EESs), in addition to classical peripheral neuroepitheliomas (PNs). In studies of small cell sarcomas, we found a group of undifferentiated tumors resembling PNETs with some features of neuroectodermal differentiation, but possessing areas of relatively large, pleomorphic cells. To clarify the nature of these tumors and their relationship to PNETs, we examined the variety of histological, immunohistochemical and ultrastructural features of 11 small cell sarcomas. Five of these tumors were composed of uniform, small round cells and were classified as PNs because of the presence of definite Homer-Wright rosettes and fibrillary processes. The presence of well developed neurite-like processes containing neurosecretory granules and immunoreactivities for various neural markers suggested that these PNs showed more advanced neuronal differentiation. Two tumors, with the classical features of ES, showed no ultrastructural evidence of neuronal differentiation, although only gamma-gamma neuron-specific enolase (NSE) positivity was detected. Four undifferentiated tumors with atypical features, included in this study as an atypical PNET group, showed certain neuroectodermal characteristics, such as ganglion cell differentiation, perivascular pseudorosettes, and gamma-gamma NSE reactivity. It is concluded from this study that PNETs may include small round cell tumors showing different degrees of neuro-ectodermal differentiation and some histological variations.

Adolescent↗

PubMed2006

Primitive neuroectodermal tumor of the adrenal gland.

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.

12E7 Antigen↗

PubMed2003

Does sophisticated diagnostic workup on neuroectodermal tumors have an impact on the treatment of esthesioneuroblastoma?

BACKGROUND: The diagnostic workup on esthesioneuroblastoma is more extensive than ever before. We have investigated whether improvements in diagnosis of sinonasal neuroectodermal tumors, including esthesioneuroblastomas (ENB), sinonasal neuroendocrine carcinomas (SNEC) and sinonasal undifferentiated carcinomas (SNUC), have had an impact on treatment and outcome. PATIENTS AND METHODS: 11 ENB, 7 SNEC and 1 SNUC in 13 men and 6 women (average age 52.9 years (range 26-82)), diagnosed between 1986 and 2001, were analyzed with regard to histopathologic and clinical diagnosis as well as outcome. Our results were compared with the available literature. RESULTS: According to the Morita classification considering endoscopy, CT and MRI scans, 2 tumors were staged D, 14 were found to be stage C, 2 were stage B and 1 was stage A. Lightmicroscopically only 4 of 19 showed higher differentiation and rosette-like structures, the others were poorly differentiated. 18 of 19 tumors were examined immunohistochemically. Neuronal markers (NSE, synaptophysin, chromogranin, S-100 and neurofilaments) were heterogeneously expressed in both ENB and NEC, only NSE stained all but 2 tumors. Coexpression of neuronal markers and cytokeratins was proven in all NEC and 5 of 11 ENB. Some tumors expressed atypical markers. Despite extensive diagnostic steps it was not possible to exclude a different histopathological diagnosis in 10 of 19 cases. CONCLUSION: For sinonasal neuroectodermal tumors no pathognomonic antigenic profiles are known. Immunohistochemical markers lack specificity and sensitivity. Nevertheless, in many sinonasal neuroectodermal tumors a panel of differentiation markers allows to specify the light-microscopic diagnosis. Until now no therapeutic consequence arises from a more extensive diagnostic workup. However, the histopathologic identification of subtypes (SNUC) and proliferation markers may help to identify patients with poor prognosis.

Adult↗

PubMed1997

EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells.

The translocation t(11;22) is a common chromosomal abnormality detected both in Ewing's sarcoma and in primitive neuroectodermal tumor cells. The translocation results in an EWS-Fli1 fusion gene, made up of the 5' half of the EWS gene on chromosome 22 fused to the 3' half of the Fli1 gene on chromosome 11. Recent studies have evaluated possible roles of the fusion gene products. However, the biological significance of EWS-Fli1 is still unknown. Using a competitive polymerase chain reaction technique, we show here that there might be a correlation between the expression levels of the EWS-Fli1 fusion gene and the proliferative activities of Ewing's sarcoma and primitive neuroectodermal tumor cells. When the EWS-Fli1 expression is inhibited by antisense oligodeoxynucleotides against the fusion RNA, the growth of the tumor cells is significantly reduced both in vitro and in vivo. The data further indicate the growth inhibition of the cells by the antisense sequence might be mediated by G0/G1 block in the cell cycle progression. These results suggest that EWS-Fli1 may play an important role in the proliferation of the tumor cells, and the EWS-Fli1 fusion RNA could be used as a target to inhibit the growth of Ewing's sarcoma and primitive neuroectodermal tumor with the specific antisense oligonucleotide.

Cell Cycle↗

PubMed1996

Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report.

The primitive neuroectoderm and its progeny seemingly give rise to an ever-increasing number of clinicopathologic entities. Ewing's sarcoma and peripheral primitive neuroectodermal tumor have recently been united by relatively unique antigens expressed by the MIC-2 gene, commonly coexpressed neural markers, and cytogenetic and molecular genetic abnormalities. Because of these factors, the current thinking is that Ewing's sarcoma and the peripheral primitive neuroectodermal tumor are parts of a phenotypic spectrum. We present an "interim report" on this group of neoplasms, emphasizing their presentation in the head and neck.

Female↗