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Results for “Neuroectodermal Tumors, Primitive”

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PubMed2005

Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies.

Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a "small round blue cell tumour", and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed.

Adult↗

PubMed2001

Peripheral primitive neuroectodermal tumour (pPNET) of the cerebellopontine angle presenting in adult life.

We report a case of a peripheral neuroectodermal tumour (pPNET) of the cerebellopontine angle of a 67-year-old woman. The patient's age at presentation was highly unusual. This case highlights the difficulties encountered, both clinically and pathologically, in securing the correct diagnosis of such a rare condition presenting in this relatively inaccessible area. The development of the nomenclature and classification of neuroectodermal tumours is traced. Recent advances in immunohistochemistry and genetic typing have shown the close relationship between pPNET and the previously difficult to classify Ewing's sarcoma and Askin's tumour.

12E7 Antigen↗

PubMed2006

Extracranial metastases of a supratentorial primitive neuroectodermal tumour.

Extracranial metastases from primary central nervous system (CNS) tumours have rarely been reported in the literature, and glioblastomas and medulloblastomas constitute the majority of these. The tendency of supratentorial primitive neuroectodermal tumours (PNET) to spread within the CNS is well-known, but few cases of extracranial metastases of supratentorial PNET have been reported. We report a 29-year-old man with a supratentorial PNET, which metastasized to his vertebral bodies and lung.

Adult↗

PubMed1998

Desmoplastic small round cell tumour of unknown primary origin with lymph node and lung metastases: histological, cytological, ultrastructural, cytogenetic and molecular findings.

Desmoplastic small round cell tumour (DSRCT) is an extremely aggressive neoplasm belonging to the family of "small round blue cell tumours", which includes primitive neuroectodermal tumour (PNET), Wilms' tumour and Ewing's sarcoma. DSRCT is considered to be a neoplasm derived from a primitive cell. It is immunohistochemically reactive with epithelial, neuronal and mesenchymal cell markers, demonstrating divergent differentiation along three cell lines. Originally thought to arise from serosal surfaces, the tumour has recently been reported in the central nervous system and ovary. The tumour in this case is a neoplasm that meets the histological, immunohistochemical, cytological and cytogenetic criteria of DSRCT; it is not associated with serosal membranes, and it has supraclavicular and axillary lymph node deposits and multiple pulmonary and brain metastases. Tumour cells from our case show cytogenetic similarities with Ewing's sarcoma and PNET. Electron microscopic findings suggest similarities between DSRCT and Wilms' tumour. Cloning and sequencing of PCR products showed in-frame fusion of EWS exon 7 to WT1 exon 8.

Adult↗

PubMed1992

Peripheral neuroepithelioma: a light microscopic, immunocytochemical, and ultrastructural study.

Forty-two cases of peripheral neuroepithelioma (PN) retrieved from the files of the National Cancer Institute (Bethesda, MD) and the Pathology Department of Padua University, Italy, were reviewed. No sex predilection was observed (25M/17F) and ages ranged from 7 to 54 yr (median 22 yr). Roughly a third of the tumors were thoracopulmonary ("Askin tumor"), a third were axial, and a third were in extremities. A lobular pattern with rosettes or pseudo-rosettes characterized PN. Seventeen cases showed a strong diastase-sensitive PAS positivity. Transitional areas with an Ewing's-like appearance and, in one case, transition to malignant nerve sheath tumor have been documented. The presence of neuron specific enolase (NSE), S-100 protein, HNK-1, neurofilaments, vimentin, keratin (AE1-AE3), beta 2-microglobulin, chromogranin A, and synaptophysin was investigated using the avidin-biotin technique. Immunocytochemically, NSE (95% of cases), beta 2-microglobulin (77.5%), synaptophysin (73.3%), and S-100 protein (67.5%) were the most consistently positive markers. Ultrastructurally, PN is characterized by a primitive appearance, although it was routinely possible to recognize neural features such as primitive neuritic extensions and dense core granules, either in the cytoplasm or in the cellular processes. In our experience, a light microscopic picture of a primitive round cell tumor with a lobular pattern, and particularly with rosettes when present, with NSE and beta 2-microglobulin positivity by immunocytochemistry, ideally with positive synaptophysin, along with supportive electron microscopy, is required for the diagnosis of PN. Conversely, no one feature alone is generally sufficient for diagnosis, but does allow distinction from extraosseous Ewing's, which (like osseous Ewing's) lacks features of neural differentiation.

Adolescent↗

PubMed2005

A 7-year-old boy with midline cerebellar mass.

January 2005. A boy aged 7 years was found to have a tumor arising from the roof of the fourth ventricle. Histopathologically, part of the tumor appeared as a PNET, while neuroglial tissue, striated and smooth muscle, cartilage and small glandular structures were present in other regions. Tumor cells in both primitive and mature elements showed a loss of chromosome 17p accompanied by a gain of 17q, a pattern consistent with the presence of an isochromosome 17q. This abnormality is not characteristic of intracranial germ cell tumors, but is present in over 30% of medulloblastomas. On the basis of the histologic and genetic abnormalities, we propose a diagnosis of PNET with multilineal differentiation.

Cerebellar Neoplasms↗

PubMed1990

Expression of the functional cone phototransduction cascade in retinoblastoma.

Retinoblastoma is a malignant intraocular tumor that primarily affects small children. These tumors are primitive neuroectodermal malignancies, however some of them show morphologic evidence of differentiation into photoreceptors. Phototransduction cascades are a series of biochemical reactions that convert a photon of light into a neural impulse in rods and cones. The components of these cascades are uniquely expressed in photoreceptors and, although functionally similar, distinct components of these cascades are expressed in rods and cones. Using HPLC anion exchange chromatography, Western blot analysis, and specific monoclonal and polyclonal antibodies, we found that the cone but not the rod cGMP phosphodiesterase is functionally expressed in all six primary retinoblastomas examined and in three continuous retinoblastoma cell lines. Morphologic evidence of differentiation did not correlate with the expression of the enzyme. Furthermore, GTP analogues could activate the phosphodiesterase activity suggesting that an intact phototransduction cascade is present in the tumors. The presence of the cone phototransduction cascade in retinoblastoma confirms that this tumor has biochemically differentiated along the cone cell lineage.

3',5'-Cyclic-GMP Phosphodiesterases↗

PubMed1999

Glioblastoma multiforme in a mature ovarian teratoma with recurring brain tumours.

AIMS: We report a case study to highlight the occurrence of glioblastoma multiforme in an ovarian teratoma. METHODS AND RESULTS: A 10-year-old girl presented with a left frontal lobe primitive neuroectodermal tumour which was successfully treated. After 6 uneventful years, she developed glioblastoma multiforme located posterior to the site of the initial tumour. Six years later, she presented with a mature cystic teratoma containing glioblastoma multiforme. CONCLUSIONS: Glioblastoma in an ovarian teratoma is an exceptional event, which might have an initial clinical presentation as a metastatic brain tumour. Alternatively, recurring glial tumours may occur in a genetically predisposed person; the role of radiation and chemotherapy in this context remains to be elucidated.

Biomarkers, Tumor↗

PubMed1993

Intracerebral primitive neuro-ectodermal tumor (PNET).

A case of intracerebral primitive neuro-ectodermal tumor (PNET) is presented. PNETs are rare, undifferentiated neoplasms occurring primarily in children and young adults. These tumors exhibit a highly malignant behaviour with a tendency to disseminate along the cerebrospinal fluid (CSF) pathways. Preoperative diagnosis is difficult, owing to the non-specific CT and MR appearances and to the rarity of this tumor. Our patient was 33 years old, which is uncommon. CT findings initially suggested an intracerebral hematoma. Later on, CT and MR findings were specific for a brain tumor and showed a solid, non-calcified invasive mass, with poor perilesional edema.

Adult↗

PubMed2003

Primary Ewing's sarcoma of the suodenum: a case report.

We report a case of Ewing's sarcoma arising from the duodenum in a 20-year-old woman who presented with a rapidly progressive ulcerative lesion. The surgical specimen obtained via Whipple's operation revealed a small round-cell tumor (SRCT) in the first and second portion of the duodenum. The tumor cells revealed strong immunore-activity for CD 99 and vimentin and focal paranuclear dot-like immunoreactivity for cytokeratin. Electron microscopy showed primitive tumor cells with few cytoplasmic organelles, but neither neurosecretory granules nor specific cell junctions were present. On Western blot study, 68-kDa EWS/FLI1 fusion protein was detected. The occurrence of Ewing's sarcoma in the gastrointestinal hollow viscus has recently been recognized, and this case expands the known anatomic sites that can harbor Ewing's sarcoma by demonstrating primary duodenal involvement.

Adult↗

PubMedDate not supplied

Intracerebral medulloepithelioma with a long survival.

This report concerns a 3-month-old boy where neuroimaging examination showed a large, well-circumscribed, mildly heterogeneous tumor arising in the left ventricle. Pathological findings were compatible with a medulloepithelioma. A survey of published cases of medulloepitheliomas showed this tumor to be highly malignant, possibly displaying the entire range of differentiation from embryonal primitive neuroepithelium to mature cells and usually involving the cerebral hemispheres with a very poor prognosis in this location. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis. Curiously, the patient reported here is doing well 7 years after the resection without any postoperative treatment. The exclusive intraventricular location of the tumor and its gross-total resection clearly seems to have contributed to this unusual recovery.

Brain Neoplasms↗

PubMed2005

An ovary in luteal phase mimicking common iliac lymph node metastasis from a primary cutaneous peripheral primitive neuroectodermal tumour as revealed by 18-fluoro-2-deoxyglucose positron emission tomography.

A 30-year-old female underwent surgical removal of a primary cutaneous peripheral primitive neuroectodermal tumour (PNET) of the left thigh. A subsequent 18-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) scan showed abnormal accumulation of FDG in the left upper pelvic region, consistent with metastasis to a left common iliac node. A series of follow-up imaging studies revealed that a cyst of the corpus luteum of ovary was responsible for the abnormal FDG accumulation.

Adult↗

PubMed2008

Familial Currarino syndrome presenting with peripheral primitive neuroectodermal tumour arising with a sacral teratoma.

This report illustrates a rare genetic disorder, Currarino syndrome, in association with an unusual malignant transformation to a peripheral primitive neuroectodermal tumour within a sacral teratoma. The triad of features consists of a presacral mass, partial sacral agenesis and anorectal anomalies. The most common presentation is constipation. In this case there was a history of constipation, teratomas and spinal abnormalities in many of the family members over three generations. Detailed family history taken at time of initial presentation may have prevented delay in diagnosis and averted the need for intensive treatment, which may well cause late sequelae.

Child, Preschool↗

PubMedDate not supplied

Immune cell infiltration of intrinsic and metastatic intracranial tumours.

BACKGROUND: Immune escape is one prerequisite for the formation of neoplasms that is reflected by the pattern of immune cell infiltration. Abundant monocytic infiltration without apparent phagocytic activity is well known in human gliomas, while other types of human intracranial tumours have not yet been investigated. MATERIALS AND METHODS: We analysed LCA-positive lymphocytes and CD68-positive macrophages/microglia by immunohistochemistry in 67 intracranial neoplasms: 18 glioblastomas (GBM), 14 primitive neuroectodermal tumours and medulloblastomas (PNET), metastases of 9 adenocarcinomas and of 8 malignant melanomas, and 18 benign meningiomas. RESULTS: Levels of monocytic infiltration in GBM and adenocarcinomas were higher than in PNET and meningiomas. Lymphocytes were rare in all tested tumours. No differences were found between all malignant neoplasms and benign meningiomas and between primary intracranial and metastatic tumours. CONCLUSION: Malignancy or primary intracranial origin seem not to be major determinants of immune cell infiltration. Different patterns of cytokine production may explain the differences in single tumour entities.

Adenocarcinoma↗

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↗

PubMed2000

Paediatric brain tumours: an embryological perspective.

Primitive neuroectodermal tumours are amongst the most common paediatric tumours of the central nervous system. These tumours are composed of undifferentiated cells and a variable component of more differentiated cell types. Most analysis of these tumours has focused on molecules normally found in the differentiated cells or those found in all primitive neuronal precursors. In this article we describe recent advances in understanding of the molecular processes involved in normal neurogenesis. We discuss the relevance of these data to the biology of neuronal tumours and describe strategies we and others have adopted to investigate the usefulness of molecules found in undifferentiated neuronal tissues in understanding the events which underlie oncogenesis in this tumour type.

Brain Neoplasms↗

PubMed1999

[Primitive carcinoid tumor of the middle ear: a case report and review of literature].

Carcinoid tumors are neuroendocrine neoplasms. Their prognosis is nearly always favorable and they rarely affect the middle ear. An accurate review of the literature has shown that since 1980 only 37 cases of primary carcinoid tumor of the middle ear have been described. Diagnostic precision has increasingly improved over the years thanks to the use of modern immunohistochemical techniques and electron microscopy. The treatment of choice is surgical exeresis through a radical tympanomastoidectomy. Distant metastases have never been indicated. The authors present a case of primary tympanic carcinoid tumor, basically extended to the external auditory duct. They underline the clinical aspects, the difficulties in histopathological diagnosis and the treatment strategies applied.

Adult↗

PubMed2001

Late recurrence of a primitive neuro-ectodermal tumor.

Late recurrences after therapy are rare in primitive neuro-ectodermal tumor (PNET). Most recurrences occur within the first 2 years after therapy, although a small number of recurrences may occur up to 5 years after therapy. We present a rare case of a recurrence of PNET in a 31-year-old woman 17 years after her initial presentation. The potential biological implications of this late recurrence as well as responses to subsequent therapy, including temozolomide, are discussed.

Adult↗