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PubMed1991

[Primitive neuroectodermal tumor with Wilms' tumor. Case report].

Occurrence of embryonal kidney tumors in patients with primitive neuroectodermal tumors, so-called central nervous system-renal neoplasia has been reported. An infant who presented with masses in the right lateral ventricle and the cerebellar vermis is reported. Histological examination showed primitive neuroectodermal tumors. Further investigation revealed tumors in the bilateral kidneys, which were removed subtotally and pathologically shown to be Wilms' tumors. The patient was then treated with anticancer drugs and irradiation. However, he developed lung metastases from the renal tumors and expired. At autopsy, a small tumor was found in the inferior horn of the left lateral ventricle. Histological finding showed a primitive neuroectodermal tumor. Also, bilateral large masses of recurrent Wilms' tumors, multiple metastases to the lungs and peritoneal dissemination were found. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. Several reports demonstrate the presence of embryonal cells in the nervous tissue which could imply a neuroepithelial origin for Wilms' tumors.

Brain Neoplasms↗
PubMed1976

Histochemistry of oxidoreductases, enzymatic polymorphism and anaplasia of neuroectodermal tumors.

Oxidoreductases were studied histochemically in 162 cases of neuroectodermal tumors. In order of decreasing activity in the cytoplasma these enzymes could be arranged as follows: NADH diaphorase, lactate dehydrogenase, NADPH diaphorase, glutamate dehydrogenase, glucose-6-phosphate dehydrogenase, isocitrate dehydrogenase, succinate dehydrogenase, malate dehydrogenase. The weak activity of Krebs cycle enzymes and the relatively strong activity of other oxidoreductases, particularly of lactate dehydrogenase, permits to conclude that glycolysis prevails over oxidative processes in neuroectodermal tumor cells. But this should not be interpreted as a decrease of the Krebs cycle enzymes in astrocytoma and oligodendroglioma cells as compared with their parent cells because the latter themselves display a weak activity of these enzymes. A real decrease of Krebs cycle enzyme activity was established only for tumors, the parent cells of which are characterized by a strong (in choroid-papillomas) or moderate (in ependymomas) activity of these enzymes. Many neuroectodermal tumors, in particular those of astrocytic origin, demonstrate a certain correlation between the amount of cytoplasm and oxidoreductase activity. This results in enzymatic polymorphism of the tumor tissue. A certain similarity was established of the oxidoreductase activity in tumor cells and in reactive hypertophic astrocytes. This indicates that both tumor cells and reactive astrocytes may in certain conditions utilize similar mechanisms of increased metabolism. The oxidoreductase activity correlates not with the grade of anaplasia but with different directions of anaplasia reflected in different variants of neuroectodermal tumors. The concept "anaplasia" includes not only certain degrees of dedifferentiation of tumor cells but, as it has been shown histochemically, also an increase of metabolic processes in the tumor cell cytoplasma.

Anaplasia↗
PubMedDate not supplied

Pathology consultation. Melanotic neuroectodermal tumor of infancy.

The melanotic neuroectodermal tumor of infancy is a usually benign neuroectodermally derived tumor with a predilection for the anterior maxilla of children who are less than 1 year of age. The lesion is considered to be readily treated by conservative excision, but it should be regarded as potentially malignant, even though there is infrequent expression of such malignancy.

Humans↗
PubMed1982

Congenital primitive neuroectodermal tumor (neuroepithelioma) of the chest wall.

Primitive neuroectodermal tumor (neuroepithelioma) is a relatively common central nervous system tumor in children. Those arising from a peripheral nerve are extremely rare in childhood. There is only one reported case in 6-year-old where the tumor arose from the sciatic nerve. A case of neuroectodermal tumor of the chest wall, arising from the intercostal nerve, in a newborn is presented. The tumor metastasized to the brain. Prominent Homer-Wright rosettes, with central eosinophilic fibrillar substance similar to that seen in neuroepithelioma of the central nervous system, were present in the primary tumor and brain metastases. Ultrastructure, as revealed by transmission electron microscopy, is also described.

Brain Neoplasms↗
PubMed2002

Growth-inhibiting effects of intralesional docetaxel and paclitaxel on an experimental model of malignant neuroectodermal tumor.

Taxoids are effective agents against a wide variety of tumors, but their effectiveness on neuroectodermal tumors is not well-known. For this reason we have carried out an experimental study on the effect of intratumoral administration of docetaxel (DC) and paclitaxel (PC) on tumor growth, using a murine model of malignant neuroectodermal tumor. The results showed a delay in tumor growth in animals treated with PC compared with controls (dose of 5 mg/kg, three times per week every other day, forming a cycle; three cycles were completed with a break of a week between each cycle). The total dose administered to each animal was 45 mg/kg. When DC was administered, the results showed distinct tumor growth inhibition and tumor regression in 80% of animals (intratumoral administration at a dose of 5 mg/kg, three times per week every other day forming a cycle; three cycles were completed with a break of a week between each cycle). The total dose administered to each animal was 20 mg/kg. These results suggest the utility of taxoids, mainly DC, by intralesional administration, on malignant tumors of the nervous system.

Animals↗
PubMedDate not supplied

Melanotic neuroectodermal tumors of the brain and skull.

A case of melanotic neuroectodermal tumor in the cerebellum of a 21-year-old man is presented. Melanin was found mainly in small neoplastic cells rather than in large epithelioid cells. The tumor also contained neurons, as well as neoplastic tissue of ependyma, choroid plexus; and astrocytes. We propose that this neuroectodermal tumor resulted from a combination of cells originating in the neural crest and in the neural tube. The small cells have been described as medulloblasts, but they may be poorly differentiated ependymal cells, or, sympathicoblasts. The presence of pigment in these cells is compatible with an origin in the neural crest. The so-called melanotic progonoma, occurring most often in the maxilla of infants, is reviewed in relation to the melanotic neuroectodermal tumor of the brain. The tumor in facial bone occurs in adults as well as infants, in locations other than the maxilla, and has malignant forms. Melanotic neuroectodermal tumors, whether in brain or bone, are therefore similar in behavior as well as histologic appearance. The finding of similar tumors in these different locations is readily explained embryologically.

Adult↗
PubMed2006

[Peripheral primitive neuroectodermal tumor -- PNET].

The authors describe three cases of peripheral primitive neuroectodermal tumor. The tumor was found in soft tissues of the crus, shoulder girdle and perineum, and was also located paravertebrally and epidurally at the level of L1-L2 vertebrae. Radiological findings were not specific for this disease. The results of imaging methods (sonography, CT, MRI, DSA) were important for the assessment of tumor size, its boundary and invasion of the surrounding tissues, and for the evaluation of tumor response to therapy and detection of recurrent disease. The PNET diagnosis was based on immunohistochemical, biochemical and cytogenetic examinations. One patient died 5 months after the first clinical signs were manifested; the two patients surviving for 2 and 1 3/4 years after first sign manifestation, respectively, remained in the care of cancer specialists. Key words: skeletal Ewing's sarcoma, extra-skeletal Ewing's sarcoma, Ewing's sarcoma family of tumors, peripheral primitive neuroectodermal tumor.

Adolescent↗
PubMed1987

Malignant peripheral primitive neuroectodermal tumor of the uterus.

Peripheral primitive neuroectodermal tumor (PPNET) is rare, occurring most often in young adults. Approximately 50 cases have been reported, with only four cases involving the female genital tract. We report the fifth patient. The term "PPNET" should be used only for tumors with neuroectodermal elements exclusively that occur at sites outside the central and sympathetic nervous system. The pathologic differential diagnoses include rhabdomyosarcoma, immature malignant teratoma, small cell carcinoma of the cervix, and ganglioneuroma. Therapy for this tumor has varied, and no effective regimen has been established.

Adolescent↗
PubMed1996

Comparison of soft tissue Ewing's sarcoma and peripheral neuroectodermal tumor.

Thirty-four cases with either extraskeletal Ewing's sarcoma (21 patients) or malignant peripheral neuroectodermal tumor of the soft tissues (13 patients) were reviewed retrospectively. The patients were treated between 1964 and 1991. Followup periods averaged 7.2 years for patients with peripheral neuroectodermal tumors and 10.4 years for patients with Ewing's sarcoma (minimal followup of 2 years). There were no significant differences in patient's age, gender, tumor location, and stages on presentation between patients with Ewing's sarcoma and those with malignant peripheral neuroectodermal tumor of the soft tissue. All but 2 patients underwent surgery for tumor resection. Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. The 5-year overall survival was 50% for Ewing's sarcoma and 44% for peripheral neuroectodermal tumor. The 5-year disease free survival was 33% for both types of tumors. Survival rates were higher for Stage III compared with Stage IV disease for both types of tumors. There was a tendency for better outcome after complete surgical tumor resection. Survivors tended to be of a younger age at the time of tumor diagnosis. The results suggest that from a clinical perspective, these 2 tumors are quite similar.

Adolescent↗
PubMed1984

CNS primitive neuroectodermal tumors of childhood.

Primitive neuroectodermal tumors ( PNET ) of the central nervous system in children are rare, highly malignant, cystic, and sometimes calcific, and hemorrhagic tumors sharply demarcated from adjacent brain. Microscopically they consist of small predominantly undifferentiated dark cells with neuronal, glial, and mesenchymal elements. We have recently diagnosed and treated five children with PNET . We will review their case histories as well as summarize the previous 133 reported cases with reference to presenting complaints, tumor location, neurodiagnostic studies, and the retrospective results of four treatment programs: 1) surgery alone; 2) surgery and radiation therapy; 3) surgery, radiation therapy, and chemotherapy at time of recurrence; and 4) surgery, radiation therapy, and chemotherapy at onset.

Brain Neoplasms↗
PubMed1978

Histological and fine structural studies on pigmented neuroectodermal tumor of infancy.

Pigmented neuroectodermal tumor of infancy originating from the anterior maxilla in a two-month-old male has been studied by light and electron microscopy. The tumor is characterized histologically by two types of neoplastic cells embedded in considerable amounts of fibrous stromal tissue. The first type of cell is cuboidal to columnar in shape with an epithelial appearance having abundant cytoplasm; either scanty or a heavy accumulation of melanin pigment is observed in the cytoplasm. These cells are aligned along the cleft-like space or arranged in small ductal structures. Electron microscopy shows the characteristic features of melanocytic cells having a varying degree of asynchronous maturation of melanosomes. The second type of cells is small and round in shape and has a hyperchromatic nucleus and scanty cytoplasm, resembling neuroblastic cells or lymphocytes. Electron microscopy reveals cytoplasmic processes resembling immature neuritis which protrude into the intracellular space; a small number of secretory granules having a central core surrounded by a single limiting membrane are observed in the cytoplasm and cytoplasmic processes.

Humans↗
PubMed1985

Melanotic neuroectodermal tumor of infancy.

Melanotic neuroectodermal tumor of infancy is a specific but unusual tumor of infancy for which only sporadic cases have been reported in the literature. This paper presents a case in an infrequent site, the epididymis, and summarizes the literature on the subject.

Adult↗
PubMed1999

Treatment strategies for medulloblastoma and primitive neuroectodermal tumors.

Medulloblastoma and primitive neuroectodermal tumors (PNETs) are the most common malignant brain tumors in children. The concern for late sequelae of neuraxis irradiation and the obligation to improve disease-free survival in children who harbor malignant brain tumors has led to the additional provision of systemic chemotherapy to standard- and reduced-dose radiotherapy, as well as to the evaluation of alternate modes of radiotherapy delivery. Analysis of evidence has suggested that chemotherapy has an impact on length of survival in children with medulloblastoma and PNETs. The question remains as to whether chemotherapy combined with reduced-dose radiotherapy provides greater benefit than standard-dose radiotherapy alone, and which subset of children the treatment most benefits. Also unanswered is the question of whether chemotherapy can serve as the primary treatment in infants with these lesions. In an attempt to help answer these questions, the authors review the major chemotherapy and radiotherapy trials for newly diagnosed patients and those with recurrent medulloblastoma and PNETs.

Journal Article↗
PubMed2000

Betulinic acid induces apoptosis through a direct effect on mitochondria in neuroectodermal tumors.

BACKGROUND AND PROCEDURE: We identified BetA as a new cytotoxic agent active against neuroectodermal tumor cells including neuroblastoma, medulloblastoma, glioblastoma and Ewing sarcoma cells, representing the most common solid tumors of childhood. RESULTS: BetA induced apoptosis by a direct effect on mitochondria independent of accumulation of wild-type p53 protein and independent of death-inducing ligand/receptor systems such as CD95. Mitochondrial perturbations on treatment with BetA resulted in the release of soluble apoptogenic factors such as cytochrome c or AIF from mitochondria into the cytosol, where they induced activation of caspases. Overexpression of the anti-apoptotic proteins Bcl-2 or Bcl-X(L) that blocked loss of the mitochondrial membrane potential and cytochrome c release from mitochondria also conferred resistance to BetA. Most importantly, BetA exhibited potent antitumor activity on neuroblastoma cells resistant to CD95- or doxorubicin-triggered apoptosis and on primary tumor cells from patients with neuroectodermal tumors. CONCLUSIONS: Thus, BetA may be a promising new agent in the treatment of neuroectodermal tumors including neuroblastoma in vivo.

Antineoplastic Agents, Phytogenic↗
PubMedDate not supplied

A case of peripheral primitive neuroectodermal tumor of the ovary.

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycin, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.

Adolescent↗
PubMed2003

A unique occurrence of a cerebral atypical teratoid/rhabdoid tumor in an infant and a spinal canal primitive neuroectodermal tumor in her father.

This report describes the clinical, pathological, immunohistochemical and genetic data of two rare malignant neoplasms of the central nervous system (CNS)--a cerebral atypical teratoid/rhabdoid tumor (AT/RT) in a 5-month-old girl and a spinal canal primitive neuroectodermal tumor (PNET) in her father. Despite aggressive treatment, both tumors were fatal, displaying extensive local recurrence and diffuse neoplastic dissemination. The paraffin-embedded tumor tissue samples were analyzed using a dual-color FISH with a locus specific LSI22q (BCR) probe. In the AT/RT tissue, a loss of BCR locus was observed in a significant proportion of the cells in contrast to the PNET specimen where the majority of nuclei did not reveal any loss of the BCR region. No mutations in exon 5 and no changes in SNP of intron 5 of hSNF/INI1 gene were found. In addition, analysis of loss of heterozygosity (LOH) was performed using a panel of 15 microsatellite markers of chromosome 22. No LOH were found in both tumor tissues. In both cases no constitutional mutations of gene TP53 were found. Analysis of the TP53 mutations in the tumor tissues revealed that the PNET, not the AT/RT tumor, was homozygous for a missense mutation at codon 175 (CGC ==> CAC). Thus, our findings emphasize the genetic differences between the two specimens and suggest that the occurrence of these two aggressive tumors of CNS in one family could be coincidental.

Adult↗
PubMed2006

[Primitive neuroectodermal tumor of the kidney].

Primitive neuroectodermal Tumor (PNET) is a rare malignant tumor of young adult. Patients often present with the classical trias of renal cancer (pain, haematuria, palpable tumor). Specific radiological signs are missing, and therefore a PNET is often diagnosed postoperatively. It is characterized by the expression of MIC2, neural markers (Vimentin, S-100, Synaptophysin) and EWS/FLI1-translocation. The tumor is often diagnosed in advanced stage, and prognosis is poor despite of multimodal treatment including radical nephrectomy, polychemotherapy (vincristine, adriamycin, cyclophosphamid, etoposide, ifosfamide) and radiation in case of metastases or incomplete resection of primary tumor. We represent two cases of metastatic renal PNET in a 28- and 39-year-old patient. Due to the multimodal treatment a partial and a complete remission was achieved, respectively.

Adult↗
PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.

Five pathologically proved melanotic neuroectodermal tumors of infancy are reported. These rare neoplasms of infancy exhibit a distinct predilection for the maxillary bone. Three tumors originated in the maxilla, one in the calvaria, and one in the cerebellar vermis. Those occurring in bone did not metastasize but were locally invasive, as reflected in their radiologic appearance. Bone erosion, expansion, hyperostosis, and osteogenesis can occur in the same neoplasm and were appreciated best on CT. MR imaging showed the soft-tissue component and extent of the neoplasm better than CT did. The pathologic findings from all five cases (and one possibly related melanotic tumor of the face) revealed abundant melanin. MR imaging of two melanotic tumors showed isointense T1-weighted and slightly hyperintense T2-weighted signals. This appearance is contrary to that of most melanin-containing tumors, which exhibit enhanced T1 and T2 relaxation, and indicates that variables other than the absolute amount of melanin may determine the MR signal. Clinically, rapid neoplastic growth and excessive melanin production by the tumor cells caused facial disfigurement and visible blue black discoloration. All five melanotic neuroectodermal tumors were resected and the vermian tumor was also irradiated. Four of five children were well and free from disease 1 month to 7 years after resection. The calvarial tumor was incompletely resected and involved the underlying brain, eventually causing death. The clinical, radiologic, and pathologic features of melanotic neuroectodermal tumors of infancy are reviewed. Melanotic neuroectodermal tumors of infancy that involve bone can be diagnosed from the clinical and radiologic findings. Prompt diagnosis and surgical resection are essential for cure.

Cerebellar Neoplasms↗