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PubMed2004

[Supratentorial primitive neuroectodermal tumor: report of a surgical case].

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.

Adolescent↗
PubMed1998

Exophytic primitive neuroectodermal tumor of the spinal cord.

We present a case of an exophytic spinal primitive neuroectodermal tumor that, radiologically, simulated an extramedullary nerve sheath tumor, meningioma, or metastatic tumor deposit. MR imaging provided discrete anatomic localization of the tumor, enabling exclusion of multicentricity in the brain and spinal cord.

Adult↗
PubMed1988

Cytogenetic abnormalities in a primitive neuroectodermal tumor.

Two distinct karyotypically abnormal cell lines were observed in cultures of a primitive neuroectodermal tumor. One involved multiple chromosome rearrangements, and the other an inversion of chromosome #7 and a t(3;10) showing an interstitial deletion of 3p; del(3)(p1?4p1?2). None of these rearrangements have been reported in previous descriptions of primitive neuroectodermal tumor karyotypes.

Child↗
PubMed1992

Peripheral primitive neuroectodermal tumors. A flow cytometric analysis with immunohistochemical and ultrastructural observations.

Flow cytometry of classical neuroblastoma has provided provocative evidence that cell cycle and ploidy analysis generate prognostically useful information. To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. An acceptable histogram was obtained in 16 cases. Of these, nine neoplasms were diploid and seven contained aneuploid DNA. Among patients with diploid lesions, four were free of disease, whereas three had persistent or recurrent disease, and two had died of tumor. Among patients with aneuploid neoplasms, four were free of disease, one had recurrence, and two had died. There was no apparent correlation between immunophenotype and proliferative activity with the clinical outcome. Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system.

Aneuploidy↗
PubMed2004

Valproic acid induces differentiation of a supratentorial primitive neuroectodermal tumor.

In preclinical models the antiepileptic drug valproic acid induces differentiation of neoplastic cells, representing an evolving anticancer approach that takes into account that malignant cells resemble immature progenitor cells capable of terminal differentiation. The authors report on a child suffering from a relapsing supratentorial primitive neuroectodermal tumor that received valproic acid for epilepsy treatment over 7 months before the relapse. In contrast to the initial tumor, the relapsing tumor showed glial differentiation and low proliferation index. This is the first report of a relapsed supratentorial primitive neuroectodermal tumor that shows histologically confirmed signs of tumor cell differentiation induction.

Cell Differentiation↗
PubMed1990

Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases.

A cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases in a 28-day-old infant is reported. The infant presented with a progressively enlarged head, cutaneous lesions in the neck, and enlarged cervical lymph nodes. A computed tomography brain scan demonstrated a giant thalamic tumor with subarachnoid dissemination and hydrocephalus. Biopsy material from the cervical lesions showed a picture of glioma with anaplastic astrocytes. The patient received a ventriculoperitoneal shunt operation and palliative chemotherapy, but died at 3 months of age. Autopsy was performed. Histological studies, which included immunohistochemical stains of the thalamic tumor, showed small, round, primitive, neoplastic cells with focal astrocytic differentiation.

Astrocytes↗
PubMed1994

Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney.

We describe a 61-year-old patient with primitive neuroectodermal tumor (PNET) arising from the kidney. Despite intensive treatment including surgery, combination chemotherapy and radiotherapy, rapid progression of the tumor was encountered and the patient died within six months with widespread disease. This appears to be the first recorded case of PNET of the kidney.

Fatal Outcome↗
PubMed2005

Primitive neuroectodermal tumor of the cervix uteri: a case report -- changing concepts in therapy.

BACKGROUND: Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Between 1987 and 2002, there have been eight cases described in the English literature. The treatment policies in these eight cases differed considerably, partly due to the rarity of the disease and to differing time periods of diagnosis and treatment. CASE: At the end of 2002, a 21-year-old woman presented with a PNET of the cervix uteri at our institute, the Erasmus Medical Center. For the appropriate treatment in this case, we reviewed the literature and decided that the treatment should be different from the local surgical treatment followed by additional treatments as most of the earlier reports describe. CONCLUSION: In view of the current knowledge of PNET belonging to the family of Ewing's sarcoma, and the improvement of treatment outcome in these tumors due to dose-intensive neo-adjuvant chemotherapy, patients with PNET of the cervix should be treated in accordance to the protocol for bony Ewing's sarcoma with multimodality therapy by means of induction chemotherapy, surgery, and consolidation chemotherapy.

Adult↗
PubMed2002

Supratentorial primitive neuroectodermal tumors in adults.

A retrospective clinical analysis was made of 12 patients with supratentorial primitive neuroectodermal tumor (PNET) who ranged in age from 20 to 62 years (median 24) and were managed at Seoul National University Hospital between January 1987 and December 1997. Six patients were male and six were female. Most presented with symptoms of increased intracranial pressure and mean duration of symptoms was four months (range: 1-12 months). The tumors were located in the posterior parieto-occipital area in six cases and the mean diameter of mass of these tumors was 5.3 cm. The characteristic magnetic resonance image finding was a large well-demarcated lobulating mass with intratumoral cyst, necrosis, and/or hemorrhage. Calcification was seen in five out of six patients who underwent computed tomography scan. All patients underwent craniotomy and three of them received subsequent operations due to local recurrence. Ten patients received postoperative whole neuraxis radiation therapy and five patients received additional chemotherapy. Mean survival after diagnosis was 86 months. The patients having intratumoral calcifications are all alive and two out of three showing a Ki-67 labelling index greater than 30% died at eight and 20 months after operation, respectively. In conclusion, supratentorial PNET must be included, even in adults, in the differential diagnoses if a tumor has characteristic radiological features. The adult supratentorial PNET seemed similar to that of children in the clinical features and the prognosis. Intratumoral calcifications and the Ki-67 labelling index might be prognostic factors, however, it should be considered that the sample size is too small and not all patients were evaluated.

Adult↗
PubMed2002

Medulloblastomas and primitive neuroectodermal tumors rarely contain polyomavirus DNA sequences.

To address the hypothesis that medulloblastoma or supratentorial primitive neuroectodermal tumor (sPNET) can arise through infection by polyomaviruses, we examined genomic DNA isolated from 15 primary medulloblastoma and 5 sPNET biopsy specimens and from 2 medulloblastoma cell lines for the presence of DNA sequences from the polyomaviruses simian virus 40 (SV40), JC virus, and BK virus. These polyomaviruses have oncogenic potential in animals, and their DNA sequences have been detected in other surveys of various solid tumors, including childhood brain tumors. The tumor DNA samples were analyzed by Southern blot hybridization of polymerase chain reaction products that employed probes designed to detect specific polyomavirus sequences. Neither JC virus nor BK virus DNA sequences were detected in any of the specimens. None of the primary medulloblastoma or sPNET specimens contained SV40 sequences. However, SV40 DNA coding and noncoding sequences were detected in the D283-Med (medulloblastoma) cell line. Immunocytochemical studies of D283-Med revealed nuclear expression of SV40 large T antigen. In contrast to childhood ependymomas and choroid plexus tumors, medulloblastomas and sPNETs infrequently express evidence of polyomavirus infection.

Antigens, Neoplasm↗
PubMed1994

[Supratentorial primitive neuroectodermal tumor. Study of 4 cases].

The clinicopathological and immunohistochemical features of four cases of primitive neuroectodermal tumors of the central nervous system were investigated. Three patients died. All cases showed vimentin-positive cells with the morphology of neoplastic cells and one case showed glial fibrillary acidic protein, neuron-specific enolase and neurofilament protein-positive cell. The present study indicates that this group of tumors have bad prognostics and may show immunocytochemical features indicating glial and/or neuronal differentiation.

Adolescent↗
PubMed1998

Primitive neuroectodermal tumor of the lingual nerve: a case report.

This is a report of a primitive neuroectodermal tumor (PNET) arising from the lingual nerve of a 5-year-old girl. Twelve months after surgical excision and postoperative chemotherapy, the patient remained alive without any evidence of recurrence or metastasis. The occurrence of PNETs in the head and neck region in children is rare. This is the first case report in the literature of a PNET originating from a cranial nerve in a child. Because of its aggressive nature and poor prognosis, aggressive surgical and medical treatment regimens are needed. Surgeons should be mindful to include PNET in the differential diagnosis of head and neck tumors.

Child, Preschool↗
PubMedDate not supplied

Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation.

We report the case of an 11-year-old girl with a retroperitoneal tumor in the left upper quadrant. The girl was admitted to hospital with weight loss and a painless abdominal mass that on biopsy was diagnosed as a peripheral primitive neuroectodermal tumor/Ewing sarcoma (pPNET/EWS) of the soft tissue. The patient underwent chemotherapy followed by surgical resection of the tumor 5 months after diagnosis. The posttreatment residual viable tumor showed a morphologic appearance resembling a neuroblastoma. Interphase and metaphase fluorescent in situ hybridization (FISH) studies performed on the pretreatment and posttreatment samples showed the presence of a t(11;22) rearrangement resulting in EWSR1/FLI1 gene fusion consistent with pPNET/EWS in both specimens. This case is unusual in the sense of showing the typical gene fusion for pPNET/EWS both in the pretherapy sample with the typical morphological appearance of this tumor and in the posttherapy specimen showing neural differentiation suggestive of a neuroblastoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Peripheral Primitive Neuroectodermal Tumor (Peripheral Neuroepithelioma) in a Dog.

A peripherally located primitive neuroectodermal tumor (peripheral PNET) originating in subcutaneous tissues was observed in an adult Beagle. The highly aggressive tumor spread rapidly and metastasized to various organs. The neoplasm was diagnosed as a peripheral PNET on the basis of morphologic and immunohistochemical characteristics such as small round tumor cells, rosette formations, many interdigitating cytoplasmic processes, neurosecretory granules and microtubules, and positive immunohistochemical reactions to neurogenic markers. We describe here the first report of a peripheral PNET in a dog. Peripheral PNET should be included in the list of differential diagnoses for soft-tissue tumors in dogs.

Journal Article↗
PubMedDate not supplied

Primitive neuroectodermal tumor of the kidney. Report of a case initially diagnosed by fine needle aspiration cytology.

BACKGROUND: Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor that exhibits neuroepithelial differentiation, most often presenting as a bone or soft tissue mass in the trunk or axial skeleton in adolescents and young adults. Isolated cases of PNET have been observed at visceral sites, such as the ovary, testis, uterus, bladder and pancreas. We present a case of PNET in the kidney initially diagnosed by fine needle aspiration (FNA). CASE: A 21-year-old woman presented with microhematuria. Urine cytology was negative for malignant cells. Physical examination was without abnormal findings. Computerized tomography revealed a partially cystic tumor in the left kidney. FNA showed monotonous-appearing small round tumor cells with occasional rosette formation. The differential diagnoses include other primitive small round cell tumors. The tumor cells were immunoreactive for neuron specific enolase and O13 (CD99). A cytologic diagnosis of PNET was suggested and subsequently confirmed on histopathology. CONCLUSION: To our knowledge, this is the first description of PNET of the kidney initially diagnosed by FNA. This nerve tumor must be included in the differential diagnosis of small cell malignant tumors of the kidney and adjacent organs.

12E7 Antigen↗
PubMed1997

Mutation analysis and loss of heterozygosity of PEDF in central nervous system primitive neuroectodermal tumors.

Deletion of 17p is the most frequent abnormality observed in central nervous system (CNS) primitive neuroectodermal tumors (PNETs), implicating the presence of a tumor suppressor gene which maps to 17p. The gene for pigment epithelium-derived factor (PEDF) has been cloned and mapped to 17p13. PEDF belongs to the serine protease inhibitor (SERPIN) gene family. The PEDF protein has neurotrophic and neuronal-survival activities and is expressed in the CNS. Twenty tumor and matched normal DNA samples from patients with PNETs were screened by single-strand conformation polymorphism (SSCP) analysis to determine loss of heterozygosity (LOH) and to identify potential mutations within the 8 exons of the PEDF gene. Ten of the 20 tumors demonstrated LOH, consistent with the deletion status of 17p determined by cytogenetic or fluorescence in situ hybridization studies. SSCP analysis of the genomic DNA from the 10 cases with LOH demonstrated several polymorphisms in exons 4 and 7, but no mutations. Our results are consistent with a loss of alleles on 17p in 50% of CNS PNETs, but do not suggest that PEDF is a candidate for the PNET suppressor gene in 17p13.

Adolescent↗
PubMedDate not supplied

Primitive neuroectodermal tumors of the biliary and gastrointestinal tracts: clinicopathologic and molecular diagnostic study of two cases.

Primitive neuroectodermal tumor (PNET) is a prototypic malignant small round cell tumor of childhood that is characterized in most cases by t(11;22) resulting in an EWS-FLI1 gene fusion. Once thought to be uncommon, PNET now accounts for almost 20% of malignant soft tissue tumors in children. Increased recognition of PNET is partly due to advances in immunohistochemistry and molecular diagnostics, which have led to the identification of the tumor in non-classical sites. We report the clinical, histologic, immunohistochemical, and molecular findings of two visceral PNETs of the digestive system--one involving the small intestine and the other involving the hepatic duct. Histologically, each tumor was composed of malignant small cells growing in sheets, nests, and lobules; the tumor cells of both cases showed characteristic immunoreactivity for vimentin and O13 (CD99). Reverse transcription-polymerase chain reaction (RT-PCR) analysis for t(11;22) using nested primers was performed with RNA extracted from paraffin-embedded, formalin-fixed tissue and demonstrated an EWS exon 7 to FLI1 exon 5 fusion in both cases, confirmed by Southern blot hybridization and DNA sequence analysis. These results illustrate the expanded clinicopathologic profile of PNET, and demonstrate that visceral PNETs, despite their unusual sites of presentation, maintain the characteristic immunohistochemical and genetic features of PNETs at more conventional sites.

Adolescent↗