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PubMed1992

Melanotic neuroectodermal tumor of infancy. A case report of paratesticular primary with lymph node involvement.

A 17-month-old boy had a melanotic neuroectodermal tumor of infancy in the left paratesticular region affecting the retroperitoneal lymph nodes. Immunohistochemical and ultrastructural study showed phenotypical diversity of the proliferating cells within a spectrum of neuroectodermal differentiation. Urinary catecholamine levels were initially elevated but returned to normal values after complete eradication of the tumor. The patient received chemotherapy and is now well, without evidence of disease 28 months after surgery.

Biomarkers, Tumor↗
PubMed2001

Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study.

PURPOSE: This study was designed to determine the feasibility and safety of delivering four consecutive cycles of high-dose cyclophosphamide, cisplatin, and vincristine, each followed by stem-cell rescue, every 4 weeks, after completion of risk-adapted craniospinal irradiation to children with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor (PNET). PATIENTS AND METHODS: Fifty-three patients, 19 with high-risk disease and 34 with average-risk disease, were enrolled onto this study. After surgical resection, high-risk patients were treated with topotecan in a 6-week phase II window followed by craniospinal radiation therapy and four cycles of high-dose cyclophosphamide (4,000 mg/m2 per cycle), with cisplatin (75 mg/m2 per cycle), and vincristine (two 1.5-mg/m2 doses per cycle). Support with peripheral blood stem cells or bone marrow and with granulocyte colony-stimulating factor was administered after each cycle of high-dose chemotherapy. Treatment of average-risk patients consisted of surgical resection and craniospinal irradiation, followed by the same chemotherapy given to patients with high-risk disease. The expected duration of the chemotherapy was 16 weeks, with a cumulative cyclophosphamide dose of 16,000 mg/m2 and a planned dose-intensity of 1,000 mg/m2/wk. RESULTS: Fifty of the 53 patients commenced high-dose chemotherapy, and 49 patients completed all four cycles. The median length of chemotherapy cycles one through four was 28, 27, 29, and 28 days, respectively. Engraftment occurred at a median of 14 to 15 days after infusion of stem cells or autologous bone marrow. The intended dose-intensity of cyclophosphamide was 1,000 mg/m2/wk; the median delivered dose-intensity was 1,014, 1,023, 974, and 991 mg/m2/wk for cycles 1 through 4, respectively; associated median relative dose-intensity was 101%, 102%, 97%, and 99%. No deaths were attributable to the toxic effects of high-dose chemotherapy. Early outcome analysis indicates a 2-year progression-free survival of 93.6% +/- 4.7% for the average-risk patients. For the high-risk patients, the 2-year progression-free survival is 73.7% +/- 10.5% from the start of therapy and 84.2% +/- 8.6% from the start of radiation therapy. CONCLUSION: Administering four consecutive cycles of high-dose chemotherapy with stem-cell support after surgical resection and craniospinal irradiation is feasible in newly diagnosed patients with medulloblastoma/supratentorial PNET with aggressive supportive care. The early outcome results of this approach are very encouraging.

Adolescent↗
PubMed1987

The spatial and morphological assessment of cerebral neuroectodermal tumors through stereotactic biopsy.

Stereotactic biopsies were carried out in an attempt to define the grading of 99 neuroectodermal tumors with respect to their modalities of growth and their degree of malignancy. Valuable data has been obtained in all explored tumors. The reliability of the information provided by stereotactic biopsy depends on the careful planning of the procedure by exploring different parts of the tumor as well as of the surrounding brain tissue.

Biopsy↗
PubMed2000

Melanotic Neuroectodermal Tumor of Infancy: Clinicopathologic Study of a Case, with Emphasis on the Chemotherapeutic Effects.

A case of melanotic neuroectodermal tumor of infancy (MNTI) in a 6-months-old white female child is presented. The tumor was located in the left half of the superior maxillary bone and affected the maxillary sinus, inducing large facial asymmetry. The histologic aspects of the tumor were typical for the entity: two cell populations, one pigmented melanocyte-like cell, the other small neuroblastic-like cell. After adjuvant chemotherapeutic treatment with a strong reduction of the tumor, the melanocyte-like cell predominated, allowing a better result of the surgery and probably a better prognosis. Int J Surg Pathol 8(3):247-251, 2000

Journal Article↗
PubMedDate not supplied

Ultrastructural study of nuclear evagination in neoplastic cells of a cerebral primitive neuroectodermal tumor.

Evaginations of the nuclear envelope containing nuclear material were observed in neoplastic cells of a cerebral primitive neuroectodermal tumor. The possible sequential development of nuclear evaginations included: 1) formation of a segmental naked inner membrane of the nuclear envelope due to loss of the underlying marginal heterochromatin, 2) elevation of the overlying outer nuclear membrane with focal expansion of the perinuclear cistern, 3) outpouching of the inner nuclear membrane forming a small bleb, and 4) protrusion of electron-lucent nuclear material into the bleb forming a nuclear evagination. Some nuclear evaginations were isolated in the cytoplasm and some protruded into the intercellular space. The inner nuclear membrane of the nuclear evagination was devoid of the marginal heterochromatin. Nuclear pores and rupture of membranes with release of the contents of nuclear evaginations into the cytoplasm were not observed. Instead, the nuclear evaginations isolated from the cytoplasm often exhibited degenerative changes with disintegration of their contents and inner nuclear membrane. It is suggested that the nuclear evaginations of the neoplastic cells in the present study may represent a means to eliminate excess nuclear material.

Brain Neoplasms↗
PubMedDate not supplied

Matrix metalloproteinase expression in childhood medulloblastomas/primitive neuroectodermal tumors.

The matrix metalloproteinases (MMPs) are a family of enzymes that degrade the extracellular matrix (ECM) and are considered to be important in neoplastic cell invasion and metastasis. Structural changes in the extracellular matrix are necessary for cell migration during tissue remodeling and neoplastic invasion. Expression of MMP-2, -3, -9, -10, and -13 was investigated in human childhood medulloblastomas (MEDs)/primitive neuroectodermal tumors (PNETs) employing an indirect alkaline phosphatase conjugated immunohistochemical antigen detection technique. Evaluation of the results was based on (a) the percent of neoplastically transformed tissue that reacted positively and (b) a measure of immunoreactivity or staining intensity [graded from A (highest) to D (negative)]. Strong overall expression of MMP-3 and -10 was found in MEDs/PNETs, especially in the ECM adjacent to blood vessels. Positive immunoreactivity was identified for these two MMPs in the ECM surrounding over 90% of the neoplastically transformed cells with the staining intensity being also the strongest possible (A,B). These two forms of stromelysin (SL), types 1 (MMP-3) and 2 (MMP-10), share 82% sequence homology, but exhibit differences in cellular synthesis and inducibility by cytokines and growth factors in vitro. Focal (surrounding less than 10% of the neoplastically transformed cells) but strong (A,B) immunoreactivity was determined for collagenase-3 (MMP-13), an endopeptidase characterized by a potent degrading activity against a wide spectrum of substrates. Weak (surrounding anywhere between 10% and 90% of the neoplastically transformed cells, and of B and B,C intensity) expression of MMP-2 (gelatinase A) and MMP-9 (gelatinase B), two cytokine-induced MMPs, was also observed. It is clear that the activation of MMPs and their inhibitors occurs in a very well orchestrated manner. The necessity of these same enzymes for the extravasation and infiltration of lymphocytes into regions of chronic local inflammation, as associated with neoplastically transformed masses of cells, may aid the transformed cells which have already acquired a more aggressive, metastatic immunophenotype (IP) to enter the peripheral circulation. Further characterization of the expression and utilization of MMPs and their inhibitors in the progression of solid human malignancies should lead to the development of novel anti-cancer therapies.

Alkaline Phosphatase↗
PubMed2005

Intracranial hypertension as an initial manifestation of spinal neuroectodermal tumor.

A 19-year-old girl had headaches, blurred vision and vomiting for 2 weeks. Neurological examination revealed only bilateral papilloedema and left abducens palsy. Neuroimaging of the brain was normal. Cerebrospinal fluid study showed intracranial hypertension (IH), hypoglycorrhachia, hyperproteinorrhachia, and a negative cytology study. Eight months after the onset, paraparesis occurred. Spinal magnetic resonance imaging showed intramedullary masses at the cervical and thoracic cords with extensive seeding. Biopsy of the mass showed primitive neuroectodermal tumor (PNET). IH rarely occurs in patients with spinal cord neoplasms. Its incidence is low and the condition is always associated with signs of myelopathy. We report a patient whose initial manifestation of spinal PNET was IH only. Spinal tumor should be considered in IH patients whose intracranial examinations are negative.

Adult↗
PubMed1998

[Primitive neuroectodermal tumors: difficult tumors versus modern oncology].

INTRODUCTION: Primitive peripheral neuroectodermal tumours (PNET) are rare masses and form part of the group of round small cell tumours which include a wide range of highly aggressive neoplasias such as Ewing's sarcoma, neuroblastoma, lymphoma and rhabdomyosarcoma. PNET present the same cell line as the tumours presented by F. Askin in 1979, both located in the thoracic-pulmonary region. MATERIAL AND METHODS: Of the last 26 thoracic neuroblastomas and 11 mediastinal-thoracic sarcomas treated at our centre, we observed 5 PNET in children with a mean age of 12 years (range: 9-14 years). These patients presented a thoracic mass infiltrating sternum, clavicle, supraspinal muscle or, in two cases, a left lateral or paravertebral intrathoracic mass. The time elapsed between clinical observation and diagnosis was 6 weeks. Diagnosis was established by chest X-Ray, CT, bone scintigraphy, immunocytochemistry and cytology. Aggressive local treatment associated with stage IV SIOP chemotherapy for rhabdomyosarcoma was applied in all cases to prevent metastasis. RESULTS: Of the five PNET treated, one 16-year-old patient died (4 y 5 m post-diagnosis) from bone marrow infiltration which had evolved badly from the beginning. The remaining patients are disease-free. One patient who did not undergo surgery relapsed 1 year and the half after completing chemotherapy. He then underwent resection of the cranial portion of the sternum and substitution with iliac graft from the tissue bank. CONCLUSION: PNET manifest clearly some of the characteristics of current paediatric oncology. These tumours are easily misdiagnosed and at present may be differentiated by new diagnostic methods (immunohistochemistry, cytogenetics, hybridomas, molecular genetics), with the aim of selecting the most adequate treatment and consequently improving the prognosis of these aggressive embryonary tumours.

Adolescent↗
PubMed2001

A case of canine primitive neuroectodermal tumor (PNET).

A 2-year and 6 month-old, female, Golden Retriever showed circling behavior and seizure. By magnetic resonance imaging (MRI) examination, a mass was found on the surface of the left cerebral hemisphere, invading to the left temporal muscle. The skull bone between them was destroyed. The dog was euthanized and necropsied. Histologically, the mass contained a lot of undifferentiated anaplastic cells, forming Homer-Wright rosettes and pseudopalisading patterns. Thus, the case was diagnosed as primitive neuroectodermal tumor (PNET).

Animals↗
PubMed1988

In vitro cytolysis of primitive neuroectodermal tumors of the posterior fossa (medulloblastoma) by lymphokine-activated killer cells.

Short-term stimulation of nonantigen-primed peripheral blood mononuclear leukocytes with interleukin-2 generates a population of oncolytic effectors designated "lymphokine-activated killer" (LAK) cells. These LAK cells express potent lytic activity against a wide spectrum of fresh or cultured autochthonous (patient's own) and allogeneic (unrelated) tumors, yet specifically spare normal tissues. In this study, cells derived from primitive neuroectodermal tumors of the posterior fossa (PNET-PF) were examined for their sensitivity to LAK cytolysis utilizing an in vitro 4-hour chromium-51-release assay. Five early-passage cell lines, derived from primary PNET-PF, demonstrated significant sensitivity to LAK cell cytolysis. Lysis was equally effective in culture medium and cerebrospinal fluid. Three freshly excised PNET-PF exhibited similar susceptibility to lysis by autochthonous LAK cells. Greatly increased expansion of LAK cell cultures could be achieved by short-term stimulation with monoclonal anti-CD3 antibodies in addition to interleukin-2 activation. These findings constitute the preliminary in vitro foundations for potential intrathecal adoptive immunotherapy of PNET-PF with LAK cells.

Adult↗
PubMed2001

[Establishment of human neuroectodermal tumor cell line (PFSK) with stable expression of xanthine oxidoreductase].

Xanthine Oxidoreductase (XOR) is the key enzyme in purine metabolism and also produces oxygen free radicals. As a continuation of our previous work, in this study, we constructed a retrovirus expression vector (pLNCX2-XOR) containing full length of XOR cDNA. Retrovirus was produced by the virus package cell line PT-67 following the transfection of PT-67 with pLNCX2-XOR and used to infect the human primitive neuroectodermal tumor cell line (PFSK). Infected PFSK cells were selected by G418 to establish cell line with stable expression of XOR. The expression of XOR in the cell line we established was confirmed by RT-PCR, Immunocytochemistry and XOR activity assay.

Genetic Vectors↗
PubMed1997

Mutations in the human homologue of the Drosophila segment polarity gene patched (PTCH) in sporadic basal cell carcinomas of the skin and primitive neuroectodermal tumors of the central nervous system.

The human homologue of the Drosophila segment polarity gene patched (PTCH) has recently been identified as the tumor suppressor gene responsible for the nevoid basal cell carcinoma (BCC) syndrome (H. Hahn et al., Cell, 85: 841-851, 1996; R. L. Johnson et al., Science (Washington DC), 272: 1668-1671, 1996). In addition to multiple BCCs, patients with nevoid BCC syndrome have a predisposition for the development of primitive neuroectodermal tumors (PNETs) of the central nervous system. We have analyzed 9 sporadic BCCs and 37 PNETs for mutation and expression of the PTCH gene. PTCH mutations were found in 3 BCCs (33.3%) and in 5 PNETs (14%), including 1 of 5 cerebral PNETs, 2 of 15 medulloblastomas, and 2 of 17 desmoplastic medulloblastomas. The sequence changes in six of these tumors (four PNETs, two BCCs) were mutations predicted to result in truncated proteins. Missense mutations were detected in one PNET and one BCC each. In addition, novel sequence polymorphisms were found in exon 2, intron 5, intron 10, and intron 14 of PTCH. Reverse transcription-PCR analysis revealed increased PTCH expression levels compared to nonneoplastic brain tissue and normal skin in the majority of PNETs and BCCs investigated. Our data suggest that genetic alterations of PTCH are not only of significance in hereditary and sporadic BCCs but are also involved in the molecular pathogenesis of a subset of sporadic central nervous system PNETs.

Adolescent↗
PubMed1996

High-dose melphalan +/- total body irradiation and autologous hematopoietic stem cell rescue for adult patients with Ewing's sarcoma or peripheral neuroectodermal tumor.

The role of high-dose therapy and autologous stem cell transplantation (ASCT) in the treatment of patients with Ewing's sarcoma (EWS) remains uncertain. From November 1985 to September 1994, 13 patients aged 16-30 years (median 20.5) received high-dose melphalan (HDM) 140-200 mg/m2 +/- 500 cGy TBI followed by ASCT for relapsed/refractory (n = 4), metastatic (n = 2), or non-metastatic (n = 6) EWS, or for peripheral neuroectodermal tumor (PNET) (n = 1). This regimen was well tolerated with no transplant-related mortality and no toxicity requiring life sustaining measures. Three of the four patients treated for relapsed/refractory EWS had progression-free survivals (PFS) less than 5 months. The only long-term survivor of these four patients received HDM while in complete remission following pulmonary irradiation. Both patients with pulmonary metastases at presentation died just 5 and 6 months post-ASCT. All four patients with non-metastatic, bulky (> 8 cm) osseous EWS progressed at a median of 11 months (range 7-22 months) while the two patients with non-bulky EWS remain progression-free 25+ and 28+ months post-HDM/TBI + ASCT. The 19-year-old patient with a PNET of the thoracoabdominal wall relapsed 4 months post-ASCT. Overall, only three of these 13 patients remain progression-free at 25+, 28+, and 108+ months following HDM +/- TBI and ASCT. In conclusion, HDM +/- TBI did not obviously improve the outcome of these 13 patients relative to that expected following conventional dose therapy alone.

Adolescent↗
PubMed1987

Pigmented primitive neuroectodermal tumor with multipotential differentiation in cerebellum (pigmented medullomyoblastoma). A case with light- and electron-microscopic, and immunohistochemical analysis.

A 5-year-old girl had a midline cerebellar primitive neuroectodermal tumor (PNET) with neuronal, astrocytic, rhabdomyoblastic and melanocytic differentiation (a variant of pigmented medullomyoblastoma). The tumor recurred following surgery and radiation therapy and the child died within 6 months. Autopsy revealed spread into cerebellar hemispheres, subarachnoidal space and brain stem. Neuronal differentiation was verified by electron microscopy and antiserum against neurofilament protein. Neoplastic astrocytes were identified by use of antisera to glial fibrillary acidic and S-100 proteins. Rhabdomyoblasts were identified by the use of antisera against skeletal muscle type 2 myosin and myoglobin, as well as by electron microscopy. This tumor is compared with 20 previously reported cases of cerebellar PNETs containing muscle, 2 of which showed a similar pattern of multipotential differentiation. The oncogenesis of these tumors is in dispute although origin of both the neuroectodermal and mesenchymal components from the neural crest (mesectodermal differentiation) is most widely accepted.

Cerebellar Neoplasms↗
PubMed2000

Are clinical parameters valuable prognostic factors in childhood primitive neuroectodermal tumors? A multivariate analysis of 105 cases.

BACKGROUND: Medulloblastoma is one of the most frequent brain tumors in children. Long-term survivors are often confronted with serious late sequelae, caused by the therapy. Therefore, prognostic markers must be identified that allow the children to be assigned to different treatment schedules according to their predicted outcome. PATIENTS AND METHODS: The medical data of 110 children with a medulloblastoma or central primitive neuroectodermal tumor (PNET), that were admitted to the Emma Kinderziekenhuis in Amsterdam were analyzed by univariate and multivariate analyses. RESULTS: In univariate analysis the following characteristics had a significant influence on progression free survival (PFS): (a) presence of meningeal metastases at the time of diagnosis, (b) presence of tumor cells in the cerebrospinal fluid before or after surgery, (c) extent of resection, (d) necessity for permanent cerebrospinal fluid (CSF) shunting and (e) radiation dose to the posterior fossa. On multivariate analysis only the presence of metastases and the radiation dose to the posterior fossa retained significance. CONCLUSION: At the time of diagnosis, no reliable clinical prognostic markers are available for the majority of patients. Further molecular studies must be undertaken to identify such prognostic factors.

Adolescent↗
PubMed1991

Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing's sarcoma. A report from the Kiel Pediatric Tumor Registry.

A new classification scheme is proposed for the differential diagnosis of Ewing's sarcoma and malignant peripheral neuroectodermal tumor (MPNT) based on conventional light microscopic and immunohistochemical findings. The presence of Homer-Wright rosettes and/or the expression of at least two neural markers is diagnostic of MPNT Ewing's sarcoma. Ewing's sarcoma was diagnosed in cases lacking Homer-Wright rosettes and expressing no neural marker or only one in immunohistochemistry. Using this "new" approach considerable differences were found between both tumor types. Although most MPNT were located in the thoracopulmonary region, Ewing's sarcoma was located predominantly in the pelvis and extremities. The mean age of MPNT patients was greater than that of Ewing's sarcoma patients. Most importantly, however, was a statistically significant difference in prognosis: disease-free survival in Ewing's sarcoma patients at 7.5 years follow-up was 60% compared with 45% MPNT patients (P = 0.026). The detection of HNK-1 in MPNT indicated a more aggressive biologic behavior, and the expression of protein S-100 appeared to be correlated with a more favorable clinical course.

Adolescent↗
PubMed1998

Primitive neuroectodermal tumor in a two-month-old black and white Colobus monkey.

A 2-month-old male black and white Colobus monkey (Colobus guereza kikuyuensis) was euthanatized because of progressive physical deterioration, rear limb paralysis, lymphadenopathy, and the presence of facial and retroperitoneal lumbar masses. At necropsy, soft white masses were present in and around lumbar vertebrae, the subcutis of the face, multiple lymph nodes, and the fourth ventricle of the brain. Histologic and immunohistochemical analysis of these masses revealed a primitive neoplasm with both neuronal and glial differentiation, consistent with a primitive neuroectodermal tumor (PNET) with bipotential differentiation. The extracranial tumors were synaptophysin (SYN)-positive, glial fibrillary acidic protein (GFAP)-negative, and neurofilament protein (NFP)-negative, while the intracranial tumor was SYN-positive, GFAP-positive, and NFP-negative.

Animals↗
PubMed1992

Cutaneous angiosarcoma as a second malignant neoplasm after peripheral primitive neuroectodermal tumor.

Second malignant neoplasms (SMN) in late childhood or young adulthood in individuals who have been successfully treated for an initial malignancy have emerged as a late effect of therapy in survivors of childhood cancer. Although radiation therapy is frequently implicated, chemotherapy with alkylating agents and antimetabolites has also been associated with SMN. Soft tissue sarcomas are among the most frequent primary malignancies complicated by a SMN and account for a majority of nonhematolymphoid SMN. We present the clinical and pathologic findings in a patient who had a peripheral neuroepithelioma (primitive neuroectodermal tumor, PNET) of the soft tissues diagnosed at 17 years of age, was treated with high-dose irradiation and multidrug chemotherapy, and developed an angiosarcoma 14 years later. This case represents an uncommon combination of mesenchymal malignancies in a young patient with an unusually favorable clinical course following the diagnosis of PNET.

Adolescent↗