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At least 343 records · Page 19Linked to original sources
PubMed1998

Mutant p53 may selectively suppress glial specific proteins in pluripotential human neuroectodermal tumor cells.

The p53 gene is mutated in pluripotential human neuroectodermal tumor DAOY cells which express both glial and neuronal markers. In most cells, nuclear m-p53 immunostaining was intense while cytoplasmic glial specific proteins (GSPs) were present at low levels. Conversely, glial fibrillary acidic protein (GFAP) and glutamine synthetase (GS) were expressed in the few cells devoid of nuclear m-p53 immunoreactivity. The level of neuron specific enolase (NSE) staining was low and not different between p53 positive and p53 negative cells. Therefore, a selective, mutually exclusive expression relationship exists between cytoplasmic GSPs and nuclear m-p53. Upon treatment with epidermal growth factor (EGF) and dibutyrylcyclic AMP, overall cytoplasmic GFAP and GS levels were increased while nuclear p53 was suppressed but a mutually exclusive expression pattern between these proteins was maintained. In cells which also express NSE, GFAP was selectively stimulated suggesting that nuclear expression of m-p53 and cytoplasmic expression of GSPs may be functionally related.

Cytoplasm↗
PubMed1988

So-called primitive neuroectodermal tumor in macerated fetuses: a confusing artifact.

We studied 11 macerated fetuses with so-called primitive neuroectodermal tumors. Because we doubled the tumorous nature of this disorder, we produced a similar lesion in a comparable 12th fetus. Experimental compression of the skull of the macerated fetus resulted in expulsion of the nervous tissue by way of the vertebral canal and into the retroperitoneal space along the peripheral nerves, with spreading into the adjacent tissues and in blood vessels. The macroscopic and microscopic picture that was induced was essentially identical to that of the 11 spontaneous cases. This lesion, which has been called primitive neuroectodermal tumor in macerated fetuses, must therefore be considered an artifact.

Diagnosis, Differential↗
PubMed1994

Melanotic neuroectodermal tumor of infancy in the skull associated with high serum levels of catecholamine. Case report.

The case of a 5-month-old boy with a left retromastoid melanotic neuroectodermal tumor of infancy is presented. The tumor extended from the subcutaneous tissue of the occiput to the cerebellar hemisphere. Histologically, the epidural part of the tumor was composed of undifferentiated neuroblasts, dense connective tissue, and glandular structures lined by melanin-containing cuboidal cells, whereas the subdural part contained differentiated neuroblasts and melanin-containing cells. The preoperative high serum levels of adrenaline, noradrenaline, vanillylmandelic acid, and neuron-specific enolase returned to normal after two operations and two cycles of chemotherapy; however, the dopamine level was mildly elevated. These data and immunohistochemical and ultrastructural findings strongly suggest that melanotic neuroectodermal tumor of infancy is derived from the neural crest.

Catecholamines↗
PubMed1998

Peripheral primitive neuroectodermal tumor presenting with diffuse cutaneous involvement and 7;22 translocation.

We report an unusual case of peripheral primitive neuroectodermal tumor (pPNET) in an infant presenting with congenital cutaneous nodules and a t(7;22)(p21;q11.2). The biologic behavior of the tumor diverged over time from a slowly growing tumor with multiple cutaneous nodules to a more aggressive neoplasm characterized by pulmonary metastases and a soft tissue mass showing additional cytogenetic alterations.

Chromosomes, Human, Pair 22↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy. Report of a case.

A case of melanotic neuroectodermal tumor of infancy involving the right maxillary alveolar ridge region of a 4 month old girl is reported. Clinical, histopathological and laboratory findings supported the diagnosis. This case had high levels of urinary excretion of vanilmandelic acid (2.8 mg/24 hrs.) and serum alpha-fetoprotein (210 mg/ml); which are characteristic of this tumor.

Alveolar Process↗
PubMed2006

Primary intramedullary primitive neuroectodermal tumor of the cervical spinal cord. Case report.

Primary intramedullary primitive neuroectodermal tumors (PNETs) of the spinal cord are rare. Only six cases have previously been reported, all involving tumors in the thoracic or lumbar spine. The authors report the case of a 54-year-old woman who presented with quadriplegia and bladder and bowel dysfunction. The patient had suffered symptoms of neck pain for 1 month and left shoulder weakness for 10 days. Magnetic resonance imaging of the cervical spine revealed an intramedullary mass extending from C-2 to C-5 with an exophytic component in the adjacent left subarachnoid space. Multiple biopsy specimens were obtained, and a partial excision was performed. Histological examination revealed nodular growth and neuronal differentiation, with a striking resemblance to desmoplastic medulloblastoma. A positron emission tomography scan did not reveal uptake at any site. These findings confirmed the diagnosis of a primary intramedullary PNET. Postoperatively, the patient was given craniospinal radiotherapy with a radiation boost to the tumor bed.

Cervical Vertebrae↗
PubMed1992

Chondrocytic differentiation of peripheral neuroectodermal tumor cell line in nude mouse xenograft.

We have established a cell line (KU-SN) from a peripheral neuroectodermal tumor originating in the left scapula of a 4-year-old girl. The original tumor was immunoreactive with antibodies for neurofilament proteins, neuron-specific enolase, vimentin, S100 protein, and beta 2-microglobulin. Dense core granules, 50-150 nm in diameter, were identified by electron microscopy. The cell line was established from tumor cells in metastatic lung fluid. KU-SN cells were immunoreactive with the antibodies for neurofilament proteins, vimentin, neuron-specific enolase, S100 protein, glial fibrillary acidic protein, cytokeratin, and carcinoembryonic antigen. Besides these neuronal features, KU-SN cells express type 2 collagen and insulin-like growth factor 1 receptor. The addition of insulin-like growth factor 1 (100 ng/ml) increased the growth rate of KU-SN cells 2.1-fold over control. Some cells were positive for Alcian blue and alkaline phosphatase staining. Cytogenetic analysis of KU-SN cells disclosed a reciprocal chromosomal translocation [t(11,22)]. Northern blot analysis of KU-SN cells demonstrated amplified expression of the c-myc gene but not the N-myc gene. When tumor cells were transplanted into nude mice, cartilage was formed. The cartilage was immunoreactive with the antibody for HLA-ABC, indicating that it was derived from the tumor cells, not from mouse tissue. Chondrocytic differentiation was not observed in xenografts of Ewing's sarcoma cell lines SK-ES or RD-ES or the peripheral neuroectodermal tumor cell line SK-N-MC. These results indicate that KU-SN cells represent primitive neural crest cells having the potential for chondrocytic differentiation.

Animals↗
PubMedDate not supplied

Pigmented neuroectodermal tumor of infancy in the epididymis. A case report.

BACKGROUND: Pigmented neuroectodermal tumor of infancy (PNTI) is a rare pigmentary tumor. Cytologic study of this tumor had not been performed before. In the present study, cytology of such a tumor was done in addition to histology, electron microscopy and immunohistochemistry. CASE: A 5-month-old infant had a scrotal tumor. After tumorectomy, touch smear for cytology revealed small and large tumor cells. The small cells showed a high nuclear/cytoplasmic ratio, and the large cells contained pigment in their cytoplasm. Histologically, the small cells and large, pigmented cells showed a typical alveolar and tubular pattern, respectively. The small cells neuroblastlike and the large cells showed melanocytelike features ultrastructurally and immunohistochemically. CONCLUSION: Cytology has not been applied to the diagnoses of PNTI. In our opinion, fine needle aspiration cytology could be helpful in the preoperative diagnosis of this tumor.

Biomarkers↗
PubMed1999

Primary primitive neuroectodermal tumor (PNET) of the kidney: a case report.

OBJECTIVES: Primary primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare entity and the case is presented to highlight the histological diagnostic problems. METHODS AND RESULTS: A 23-year-old female presented with a lump in the left hypochondrium that had appeared 3 months, earlier, which suggested a malignancy on radiological investigation. The nephrectomy specimen revealed a large tumor mass with only a small portion of normal renal parenchyma. Histology showed a small cell malignant tumor and the diagnosis of PNET (primary of kidney) and small cell carcinoma were suspected. The tumor showed NSE and vimentin positivity and cytokeratin negativity, which conformed with the diagnosis of PNET. CONCLUSION: The biological behaviour of PNET is more aggressive, with rapid progression and infiltration into the surrounding tissues and distant metastases. Thus it differs from the small renal cell malignancies and hence recognition of this tumor as a distinct entity is essential.

Adult↗
PubMed2007

Primary spinal primitive neuroectodermal tumor: case series and review of the literature.

Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.

Adolescent↗
PubMed2006

Primitive neuroectodermal tumor arising in the colon: report of a case.

Peripheral primitive neuroectodermal tumors (pPNETs) are usually found in the soft tissue of the extremities, paravertebral region, and chest wall. We report a rare case of a pPNET arising in the colon. A 59-year-old man underwent left hemicolectomy for an infiltrative ulcerating tumor, 11 cm long, in the descending colon. Histological examination of the resected specimen revealed small, round cell proliferation with rosette-like structures, and confirmed regional lymph node involvement and peritoneal dissemination near the primary tumor. Immunohistochemically, the tumor cells were positive for synaptophysin and MIC2 (CD 99). ESW-FLI1 chimeric mRNA was detected in the tumor by reverse transcriptase-polymerase chain reaction. The patient underwent resection of recurrence in the retroperitoneum 3 months later, but metastasis rapidly developed and he died of the disease 7 months after his first operation.

Base Sequence↗
PubMed1994

Primitive neuroectodermal tumor of the retroperitoneal cavity.

We report a case of primitive neuroectodermal tumor (PNET) arising from the retroperitoneal cavity. Because of infiltration, the tumor was excised en bloc with the left kidney. Despite postoperative adjuvant chemotherapy and local radiation therapy, the tumor recurred and the patient died of the disease 3 months postoperatively. Serum levels of neuron-specific enolase (NSE) correlated with those of lactate dehydrogenase (LDH), and it is suggested that monitoring NSE and LDH is useful in evaluating tumor recurrence. The clinical course and the treatment of this aggressive tumor are reviewed.

Adult↗
PubMed1997

Congenital primitive neuroectodermal tumor of the hand: a case report.

A congenital peripheral primitive neuroectodermal tumor of the hand demonstrating aggressive behavior by rapid growth and ulceration, as well as early diffuse metastasis is presented. Management consisted of below-elbow amputation and chemotherapy. Despite the tumor's initial response, intracranial metastases occurred 7 months later. The patient died shortly thereafter, 15 months after presentation.

Female↗
PubMed2003

Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature.

BACKGROUND: Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas. Involvement of the reproductive tract is unusual. CASE: A 35-year-old woman had a pelvic mass and omental cake. Frozen-section examination at laparotomy revealed small round cell tumor confirmed as pPNET. Chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide with mesna yielded complete response. The patient's mother died of a similar tumor at age 52 years, and the patient's husband had adult Ewing sarcoma, constituting an unusual cluster of related tumors. CONCLUSIONS: Genetic recombination resulting in a chimeric transcript of the Ewing sarcoma gene and the Friend leukemia virus integration site is characteristic of these tumors. Surgical resection and multiagent chemotherapy may enhance survival.

Adult↗
PubMed1996

[Peripheral neuroectodermal tumor of the thoracic wall. Experiences with surgical treatment].

The peripheral neuroectodermal tumor (PNET) is seen nowadays as a more specified form of Ewing's Sarcoma with a worse prognosis. We report our experiences of three children (10-14 years old) with PNET of the right thoracic wall, all treated surgically within 1 year. Complete local resection-the main aspect of PNET therapie-of the initially voluminous tumors was not difficult due to chemotherapy and radiotherapy for 5 months prior to surgery.

Adolescent↗
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↗
PubMed2006

Primary intramedullary primitive neuroectodermal tumor (PNET)--case report and review of the literature.

Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.

Adolescent↗