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PubMed2005

Mouth metastasis of peripheral primitive neuroectodermal tumor.

We describe a case of oral metastasis of peripheral primitive neuroectodermal tumor (pPNET) in a 68-year-old man, who presented the primary lesion in the chest. Oral metastasis of pPNETs is very rare and we have not found any similar case reported in the English literature. Clinical examination showed an extensive and ulcerated fleshy mass measuring 3.0 x 3.5 cm in the right lower gingivae. Microscopic examination showed sheets of proliferating small, hyperchromatic, round cells. Tumor cells were reactive to neuron-specific enolase (NSE), vimentin and MIC-2 gene by immunohistochemistry, consistent with PNET. The patient died 3 weeks later because of respiratory insufficiency.

Aged↗
PubMed1998

Identification of CD56 and CD57 by flow cytometry in Ewing's sarcoma or primitive neuroectodermal tumor.

CD56 and CD57 are commonly considered as natural killer and neuroectodermal markers, but their expression has been identified in a wide spectrum of neoplasms including some cases of Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET). We report two cases of small, round blue cell tumor (SRBCT), in which flow cytometry immunophenotyping (FCI) detected strong expression of CD56 and CD57 (one case). Immunohistochemical staining with Leu-19 and Leu-7 confirmed the FI results. Although CD56 and CD57 expression is consistent with ES/PNET, it can be potentially misleading if results of FCI are interpreted in the absence of other findings. These cases suggest the utility of FCI in undifferentiated SRBCT. The literature on CD56 and CD57 expression in ES/PNET is reviewed and discussed.

Adolescent↗
PubMed2006

Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report.

OBJECTIVE: Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET family) are small, round, blue cell tumors that have a decided predilection for young patients and commonly arise in bone and soft tissue. We are reporting a rare case of cavernous sinus pPNET in a 48-year-old woman. CLINICAL PRESENTATION: A 48-year-old woman presented with headache, ipsilateral maxillary, and ophthalmic and oculomotor nerve palsies. Neuroimaging revealed a cavernous sinus lesion. INTERVENTION: The patient underwent debulking of the tumor, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings. Bone scans, bone marrow aspiration, and biopsy and chest computed tomographic scans showed no evidence of systemic involvement. The patient had adjuvant treatment with radiotherapy and chemotherapy. After 14 months, the patient had no neurological deficits, and neuroimaging showed stable disease, although some chemotherapy complications occurred. CONCLUSION: This is a case of cavernous sinus pPNET in a 48-year-old woman, in whom the diagnosis is supported by the presence of EWS-FLI1 fusion gene. This seems to be the first reported case of a cavernous sinus pPNET confirmed by molecular genetic analysis.

Cavernous Sinus↗
PubMed1992

Secretogranin II expression in Ewing's sarcomas and primitive neuroectodermal tumors.

Production of chromogranins, the acidic components of the chromaffin granules regarded as specific neuroendocrine markers, was analyzed by immunocytochemistry and hybridization (Northern blotting and in situ hybridization) in primary lesions and cell lines of Ewing's sarcomas, primitive neuroectodermal tumors (PNETs), and neuroblastomas. Antibodies and probes specific for chromogranin A (CgA), chromogranin B (CgB), and secretogranin II (SgII) were used. Ewing's sarcomas and PNETs, unlike neuroblastomas, were negative for CgA and CgB. Two primary Ewing's sarcomas, one primary PNET (an Askin tumor), and one PNET cell line (TC32) were found to strongly express the SgII gene, as shown by the presence of specific mRNA. This result supports the hypothesis that some Ewing's sarcomas represent a most primitive form of neuroectodermal tumor; in addition, it indicates a diagnostic role of SgII in cases of Ewing's sarcomas and PNETs.

Adolescent↗
PubMed1996

Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant.

Primary spinal cord primitive neuroectodermal tumor (PNET) is a rare entity. In all 13 cases have been reported in the literature, including 3 with intracranial seeding. A 3-month-old girl with involvement of the spinal cord below the mid-thoracic level is described. The brain MRI revealed findings indicative of seeding along the intracranial subarachnoid space. Biopsy, duraplasty and removal of laminotomy flap were done. In spite of a good response to the first cycle of postoperative "8-drugs-in-a-day' chemotherapy, further treatment was refused. She died 21 days after the onset of leg weakness, which reveals the rapid progression of untreated cases. To our knowledge, this is the first case of spinal cord PNET with parenchymal involvement that has been described in an infant.

Fatal Outcome↗
PubMed1994

The impact of perioperative factors on subsequent intelligence quotient deficits in children treated for medulloblastoma/posterior fossa primitive neuroectodermal tumors.

BACKGROUND: Despite success in treating children with medulloblastoma/posterior fossa primitive neuroectodermal tumor (PF PNET), some children survive with significant neurocognitive sequelae. This study was performed to understand better the significance of perioperative factors on subsequent full scale intelligence quotient (FSIQ) deterioration in these children. METHODS: Twenty-eight children who underwent prospective and serial neurocognitive testing were studied. All children underwent surgery followed by radiotherapy with or without chemotherapy between 1983 and 1987 for medulloblastoma/PF PNET and were disease free when this study was conducted. IQ testing was performed before surgery and after the completion of radiation therapy. The clinical courses of the patients were correlated with changes in the corresponding intelligence quotients of each child. Factors correlating with neurocognitive declines were examined by chi-square or Fisher exact test analysis. Differences in mean IQs were examined by the t test. Factors found to be significant were analyzed by exact logistic regression analysis. RESULTS: The presence of adverse factors such as neurologic deficits, meningitis, or shunt infections, or the need for repeat surgery was correlated significantly with IQ deficits after treatment. Of the subset of children with one or more of these factors, 13 of 16 (81%) sustained decreases in FSIQ; 7 of 16 (43.8%) had decreases of 20 points of more. In contrast, only 3 of 12 (25%) of the children without the factors sustained FSIQ decreases, and no child sustained a decrease of more than 13 points. The mean FSIQ change after treatment in the group with factors was -15.7 (95% confidence interval [CI]: -24.0, -8.4), and the median was -18. The mean FSIQ change in the group without factors was 4.8 (95% CI: -0.5, 10.1), and the median was 5. The difference in mean FSIQ change between the two groups was significant (P < 0.0001). On univariate analysis, both the presence of adverse factors and an age less than six years correlated with neurocognitive deficit. On regression analysis, only the presence of adverse factors was significant (odds ratio 11.53; 95% CI, 1.65-116.58; P = 0.009), whereas age was not (P = 0.27). CONCLUSIONS: Perioperative events or complications may account for some of the neurocognitive deterioration seen in these children after treatment, especially in the very young. The occurrence of these factors is associated with a significantly greater risk of IQ deterioration. Studies of the neurocognitive effects of treatment for children with medulloblastoma/PF PNET should include an analysis of these postoperative factors.

Adolescent↗
PubMedDate not supplied

Prenatal imaging of congenital cerebral primitive neuroectodermal tumor.

A case of fetal brain tumor, which appeared after 32 weeks' gestation, is presented. Prenatal ultrasonography and magnetic resonance imaging demonstrated a large heterogeneous mass in the right supratentorial region and left enlarged ventricle. A male fetus weighing 2,616 g was delivered at 34 weeks' gestation by cesarean section and died on the 37th day of life due to rapid growth of the tumor. Following autopsy, the pathohistological examination revealed primitive neuroectodermal tumor. Magnetic resonance imaging in the prenatal management of the congenital brain tumor is efficient in evaluating the expansion and margin of the tumor and intratumoral bleeding, which are not demonstrated by ultrasonography.

Adult↗
PubMed1993

Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings.

OBJECTIVE: The objective of this study was to describe the CT and MR appearances of primitive neuroectodermal tumors of the thoracopulmonary region (Askin tumor), which typically manifest as large tumors involving the chest wall and pleura. This rare malignant neoplasm, seen predominantly in children and young adults, originates in soft tissues of the chest wall, occasionally in bone, or, rarely, in the periphery of the lung. SUBJECTS AND METHODS: The study group included two boys and six girls, 7-18 years old, with thoracic primitive neuroectodermal tumors who had had CT and MR studies at presentation. In seven of these patients, the preoperative imaging interpretation was compared with surgical observations. RESULTS: Seven tumors were large. Unilateral chest wall involvement was seen in all eight patients, pleural involvement in seven, and rib destruction in five. All tumors were heterogeneous on CT scans and T2-weighted MR images, and seven were heterogenous on T1-weighted MR images. The smallest tumor was homogeneous on T1-weighted MR images. Seven tumors had a signal intensity greater than that of skeletal muscle on T1-weighted MR images, and seven showed hemorrhage or necrosis. Invasion of muscle in the chest wall was noted on MR images in seven patients and on CT scans in four patients. CT scans and MR images showed extension of tumor into adjacent lung in four patients but were indeterminate in three patients. One patient had no extension of tumor into adjacent lung. Pulmonary metastases were noted on MR images in one patient and on CT scans in three patients. CT and MR studies correctly showed pleural (n = 6), rib (n = 4), pericardial (n = 1), diaphragmatic (n = 1), and vertebral/spinal cord (n = 1) involvement, confirmed during surgery. CT and MR scans of five patients imaged before surgery were indeterminate for adjacent lung invasion. CONCLUSION: The described radiologic features of Askin tumors may be useful in deciding when to include this entity in the differential diagnosis. Areas of hemorrhage and necrosis in the large tumors are responsible for their heterogeneous appearance on CT scans and MR images. CT scans and MR images are complementary for determining the extent of disease. MR imaging is informative for determining invasion of chest wall muscle, whereas CT scanning is preferred for detecting small pulmonary metastases. Neither technique is adequate for predicting invasion of adjacent lung.

Adolescent↗
PubMed1996

Two primitive neuroectodermal tumor cell lines require an activated insulin-like growth factor I receptor for growth in vitro.

OBJECTIVE: To determine the expression of the insulin-like growth factors (IGFs) and the IGF-I receptor in primitive neuroectodermal tumor cell lines and to assess the importance of these proteins in the growth of cell lines in vitro. METHODS: Ribonucleic acid blotting and reverse transcriptase-polymerase chain reaction were used for detection of IGF and IGF-I expression. Ribonucleic acid blotting was used for detection of up-regulation of c-fos in the presence of exogenous growth factor. Immunoprecipitation was used to demonstrate autophosphorylation of the receptor in the presence of exogenous growth factor. Ligand binding analysis was used to determine the binding affinity of the receptor and the number of receptors per cell. Growth of curves in the presence of monoclonal antibody that blocks binding of ligand to receptor was measured to determine the requirement for an activated receptor during growth. RESULTS: Expression of IGF-II was identified in one cell line. No expression of IGF-I was seen in any cell line. Expression of IGF-I receptor was detected in all three cell lines. Immunoprecipitation experiments demonstrated autophosphorylation of the receptor after addition of IGF-I to growing cells. Ligand binding analysis revealed 9.2 x 10(4) and 4 x 10(4) receptors per cell in the Daoy and PFSK cell lines, respectively. Addition of either IGF alone or in combination to serum-starved cells was not able to restore growth of the cell lines. A blocking monoclonal antireceptor antibody decreased growth of Daoy and PFSK cells in a dose-dependent fashion. Complete arrest of growth occurred at 1 microgram/ml antibody in both cell lines. CONCLUSION: The IGF-I receptor is expressed by primitive neuroectodermal tumor cell lines in vitro. An activated receptor is important for cell proliferation in vitro. Additional work will establish the importance of these findings for tumors in vivo.

Antibodies↗
PubMed2006

Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report.

OBJECTIVE: In adults, supratentorial primitive neuroectodermal tumor (sPNET) is a very rare undifferentiated embryoblastic neoplasm. Prognosis is worse in comparison to infratentorial medulloblastoma. Older age appears to be prognostically favorable. At present, 5-year survival rates remain below 50% in all age groups. Survival longer than 15 years in an adult has only been reported once so far. CASE REPORT: In 1987, a 33-year-old-male patient presented with seizures following a six-month's history of dizziness. CT- and MRI-scans revealed a right occipital tumor with moderate contrast enhancement. The tumor was completely removed. The original histological diagnosis was that of an undifferentiated sarcoma, malignant hemangioendothelioma, grade III. The patient was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy). Admission for another reason in 2003 led to a re-evaluation of the original diagnosis. Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component. Up to 20% of tumor nuclei were labeled for Ki-67. Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent. According to these findings, the diagnosis was changed to a sPNET (WHO IVdegrees ). Other tumor entities were excluded by immunohistochemistry. CONCLUSIONS: Although the prognosis of sPNET is reported to be poor, a small fraction with a rather benign biological and clinical behavior exists. Parameters determining long-term-survival in sPNET are not yet known. Whenever possible, complete surgical resection should be attempted followed by postoperative radiotherapy. The value of chemotherapy is an issue of continuous investigation.

Adult↗
PubMed2006

Peripheral primitive neuroectodermal tumor of the cauda equina in an elderly patient. Case report.

Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification remains disputed, PNETs are recognized as primary tumors in both the central and peripheral nervous systems. These rare tumors usually occur in children or young adults and are typically metastatic to the spinal cord. The authors report the case of a 70-year-old man with no significant medical history, in whom a peripheral PNET was demonstrated that, based on clinical and imaging findings, manifested as a nonspecific intrathecal tumoral process arising from the cauda equina. Although this lesion is rare in the elderly, the authors' case illustrates the importance of making this diagnosis as early as possible so that a treatment plan may be devised. The use of chemotherapy in association with radiotherapy in relation to the resection of the tumor should be reconsidered.

Aged↗
PubMed2006

Primitive neuroectodermal tumor (PNET) as second malignancy after treatment of Hodgkin's disease.

Hodgkin's disease survivors are at an increased risk of developing second malignant neoplasms including secondary bone tumors. Common secondary bone tumors are osteogenic sarcoma and fibrosarcoma. Secondary primitive neuroectodermal tumor is extremely rare in this group. We present below, a rare case of secondary PNET in an 8-year-old child with Hodgkin's disease which developed unusually early outside the radiation portal and discuss potential factors responsible for its causation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Peripheral neuroectodermal tumor presenting pleural effusion.

Pleural effusion is a common finding of peripheral neuroectodermal tumor (PNET) of the chest wall (Askin's tumor), but little is known about the characteristics. A case of Askin's tumor with pleural effusion is reported. Repeated cytologies were negative for malignancy, but levels of lactic dehydrogenase (LDH) and neuron-specific enolase (NSE) in the pleural effusion were increased. Surgical biopsy was performed and immunohistochemical study of the tumor revealed the diagnosis.

Adult↗
PubMed1997

Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization.

Peripheral primitive neuroectodermal tumors (PNETs) are rare lesions that form part of the Ewing family of tumors, which includes osseous and extraosseous Ewing's sarcoma and Askins tumor of the thorax. All are characterized by translocations involving the EWS gene at 22q12, usually the translocation t(11;22)(q24;12). PNETs usually occur in soft tissues but occasionally arise within a visceral organ. We describe a PNET of the kidney that showed characteristic microscopic and immunohistochemical appearances of a small, round, dark blue cell tumor with focal rosette formation and strong membrane positivity for the MIC2 gene product. Interphase fluorescence in situ hybridization on touch imprints prepared from frozen tissue using cosmid probes flanking the EWS gene at 22q12 and the FLI1 gene at 11q24 indicated the presence of t(11; = +22)(q24; = +q12), confirming the diagnosis of PNET. This is the first reported PNET of the kidney supported by cytogenetic analysis. We also review the literature on this fascinating tumor in this unusual location.

Female↗
PubMed2006

Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies.

OBJECTIVES: Peripheral primitive neuroectodermal tumor (PNET) is a highly aggressive neoplasm belonging to the Ewing family of tumors. It is characterized by the expression of MIC2 and neural markers (eg, neuron-specific enolase, synaptophysin, S-100, vimentin, Leu-7), and the presence of the EWS-FLI1 translocation. METHODS: We performed a MEDLINE search for PNET in urologic malignancies. Additionally, we report on 2 cases of renal and 1 case of bladder PNET. The data obtained by reviewing patients with renal PNET were analyzed using Kaplan-Meier analysis. RESULTS: Renal PNET is diagnosed in young adults (median age 24 years). In contrast, the incidence of bladder PNET seems to be dependent on a defective immune mechanism. Patients often present with pain (84%), palpable tumor (60%), and hematuria (38%). The radiologic findings are uncharacteristic; therefore, the diagnosis should be based on the histologic and immunohistochemistry findings. Renal and bladder PNET are both often diagnosed at an advanced stage and, therefore, the prognosis is poor, despite aggressive multimodal treatment (surgery, polychemotherapy, radiotherapy). We identified palpable tumor masses (log-rank test, P = 0.0027) and synaptophysin expression (log-rank test, P = 0.0422) as prognostic unfavorable markers for renal PNET. CONCLUSIONS: Renal PNET should be considered in young patients who present with the classic triad of renal cancer, hematuria, and pain and palpable tumor. Once PNET is diagnosed, multimodal treatment (radical surgery, multidrug chemotherapy, radiotherapy) must be initiated. Despite this, the prognosis is poor if distant metastases are present. Furthermore, palpable tumor masses and synaptophysin expression are associated with a shorter cancer-specific survival.

Adult↗
PubMed1991

Melanotic neuroectodermal tumor of infancy. A reexamination of a histogenetic problem based on immunohistochemical, flow cytometric, and ultrastructural study of 10 cases.

Ten cases of melanotic neuroectodermal tumor of infancy (MNTI) were studied. There were nine males and one female ranging in age from 2 weeks to 10 months; one patient was 8 years old. Sites of origin were the maxilla (five), epididymis (two), mandible (one), skull (one), and soft tissues of the cheek (one). Six tumors recurred from 1 to 18 months after diagnosis. One patient had widespread dissemination. Electron microscopic study of four cases showed cells with melanosomes at various stages of maturation, and cells with neuroblastic features, including neurosecretory granules and cytoplasmic processes. Nine cases of MNTI were studied immunohistochemically. Small neuroblastic cells and large cells in all cases were reactive for neuron-specific enolase (NSE), synaptophysin, HMB45, and dopamine-beta-hydroxylase, large cells in all cases and few small cells were reactive for cytokeratin (CK) and vimentin (VIM). Epithelial membrane antigen was observed in large cells in three cases, four cases expressed Leu 7 antigen, three were focally positive for glial fibrillary acidic protein, one for desmin, and one for chromogranin. All cases were nonreactive for retinol-binding protein, neurofilaments, alpha-fetoprotein, S-100 protein, and carcinoembryonic antigen. Five normal adult retinas were studied similarly; the pigmented epithelium of the retina was reactive for CK, VIM, HMB45, NSE, and S-100. DNA study, performed in eight tumors, revealed aneuploidy in two (DNA index = 1.7 and 1.8); these cases recurred within 1 month. No differences were observed according to site or behavior. MNTI is a primitive neuroectodermal tumor with polyphenotypic expression of neural and epithelial markers, melanin production, occasional glial, and rhabdomyoblastic differentiation, and no photoreceptor differentiation. It probably represents a dysembryogenetic neoplasm that recapitulates the retina at 5 weeks of gestation.

Child↗
PubMed1994

Ultrastructure of the primitive neuroectodermal tumors (PNET).

We report a prospective series of consecutive cases of primitive neuroectodermal tumor (PNET) studied by electron microscopy. Virtually all specimens showed a differentiation along neuroblastic lines as evidenced by the presence of neurites, dense-cored vesicles, microtubules and adhesive plaque junctions. We observed also numerous intracytoplasmic cilia and autophagic vacuoles. Synaptic specializations were only rarely seen. We conclude that PNET is a tumor category which is not undifferentiated ("primitive") and clearly exhibits features of neuroblastic differentiation.

Adolescent↗