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PubMedDate not supplied

Melanotic neuroectodermal tumor of infancy initially diagnosed by fine needle aspiration cytology.

A five-month-old male child presented with a tumor of the maxilla, which was clinically diagnosed as an eruption cyst or a rhabdomyosarcoma. Fine needle aspiration smears showed two types of cells: neuroblastlike cells and cells containing melanin pigment. A cytologic diagnosis of melanotic neuroectodermal tumor of infancy was made. This diagnosis was confirmed by histopathologic examination of the subsequently excised mass.

Biopsy, Needle↗
PubMedDate not supplied

Primitive neuroectodermal tumors including the medulloblastoma: glial differentiation signaled by immunoreactivity for GFAP is restricted to the pure desmoplastic medulloblastoma ("arachnoidal sarcoma of the cerebellum").

Immunoreactivity of tumor cells for glial fibrillary acidic protein (GFAP) is usually regarded as sign of astrocytic histogenesis and/or differentiation. The present study aimed at a systematic evaluation of the significance of GFAP-containing cells in primitive neuroectodermal tumors (PNETs) with special reference to the controversial entity of desmoplastic medulloblastoma (so-called "circumscribed arachnoidal sarcoma of the cerebellum"). Fifty-three PNETs, including 17 pure desmoplastic medulloblastomas were investigated, using GFAP antisera and the peroxidase-antiperoxidase (PAP) technique. Seventy percent of the pure desmoplastic medulloblastomas showed GFAP immunoreactive cells, in 47% indistinguishable from adjacent nonreacting tumor cells. Most immunoreacting cells were found in the reticulin free islands, showing in 6 cases a gradual transition of immunoreacting cells from tumor cells to larger cells shaped like astrocytes. The classical medulloblastomas showed only larger immunoreacting cells which were interpreted as reactive astrocytes. Therefore, the so-called circumscribed arachnoidal cerebellar sarcoma or pure desmoplastic medulloblastoma merits a separate place in the group of PNETs as a tumor with frequent signs of astroglial differentiation; this interpretation appears to be clinically correlated by a difference in age incidence and prognosis of that special tumor-type in comparison with classical medulloblastoma.

Adult↗
PubMed1991

Neuroectodermal tumors of the peripheral and the central nervous system share neuroendocrine N-CAM-related antigens with small cell lung carcinomas.

The current study describes the presence of neuroendocrine antigens of peripheral and central neural tumors using eight monoclonal antibodies raised to small cell lung carcinoma (SCLC), which recognize "neural/neuroendocrine" or "neural" antigens, as defined by their reaction pattern in normal tissues and tumors. At least five of them recognize different epitopes of the neural cell adhesion molecule (N-CAM). It was found that all of 12 neuroblastomas, 2 ganglioneuroblastomas and 4 ganglioneuromas as well as 23 central primitive neuroectodermal tumors, 13 astrocytomas and 4 ependymomas share "neural/neuroendocrine" antigens (as defined by the anti-N-CAM antibodies Moc-1, -21, -32, -52 and -191) with SCLC. The "neural/neuroendocrine" antigen defined by Moc-171 was also found in all peripheral tumors, but only in further differentiated central tumors. Non-N-CAM related "neural" antigens (as defined by Moc-51 and -172) were found only in better-differentiated peripheral and central tumors, but they could be demonstrated in all three medulloblastoma cell lines studied. In addition, the antigen defined by Moc-51 was demonstrated in an immunoblot of a neuroblastoma cell line. Antibodies recognizing "epithelial" antigens of SCLC and other epithelia and their tumors (Moc-31 and -181) were non-reactive. It was concluded that these findings give further support for a relation between neural and neuroendocrine tumors and that some of the antibodies may be useful for the detection of differentiation in neural tumors. Antibodies with an "epithelial" recognition pattern may serve to distinguish neural from neuroendocrine tumors.

Adolescent↗
PubMedDate not supplied

Peripheral neuroectodermal tumor of the chest wall in a 19-year-old female.

A small cell round tumor involving the chest wall of a 19-year-old female had features that by light microscopy were considered consistent with Ewing's tumor. At the ultrastructural level the cells contained small quantities of glycogen and had irregular dendritic processes with rare dense core granules and microtubules. Immunostaining for neuron-specific enolase was positive. The neoplasm is similar to reported cases of peripheral neuroectodermal tumor involving the chest wall of young patients.

Adult↗
PubMed1993

Primitive neuroectodermal tumor of the chest wall in a patient with Jeune's syndrome and renal transplant.

Jeune's syndrome is a rare autosomal disorder characterized by osseous dysplasia, fetal respiratory distress, and renal failure in later life. We describe a 27-year-old man with Jeune's syndrome who underwent renal transplantation and 6 years later developed a sarcoma (primitive neuroectodermal tumor [PNET]) in the soft tissue of the chest wall, a principal site of dysplasia in this disorder.

Adult↗
PubMed1984

Extracranial primitive neuroectodermal tumor.

An 11-year-old white girl was admitted to The Children's Hospital of Philadelphia in July 1983 for evaluation of metastatic tumor. She had been well until July 1982, when a mass developed over the right scapula. Treatment with warm compresses and antibiotics resulted in no improvement, and incision and drainage were unproductive. A biopsy at another hospital was interpreted as showing a primitive neuroectodermal tumor. There was no evidence of metastatic disease. She then underwent excision of the tumor with the underlying scapula. No further treatment was administered. She remained well until February 1983, when she began to complain of occasional lower back and thigh pains. Her symptoms worsened over the succeeding 3 months, despite treatment with analgesics and physical therapy. By May 1983, she was no longer able to attend school because of weakness and pain, and had sustained a 10% weight loss during the previous 2 months. She was admitted to her original hospital, where bone scan and bone marrow biopsy showed disseminated tumor; she then came to The Children's Hospital of Philadelphia. On examination, she appeared acutely and chronically ill. It was very uncomfortable for her to move, and she walked with a slow, stooped, shuffling gait. She complained of tenderness in the lower back and both sides. There were no other abnormalities on examination. The hemoglobin level was 9.7 gm/dl, following transfusion at the other hospital; white blood cell count was 6,900/mm3 with a normal differential, and the platelet count was 480,000/mm3. A 24-hr urine test for VMA excretion was normal. She underwent bone marrow aspiration and biopsy, and the radiographs and pathology slides from the other hospital were reviewed.

Adult↗
PubMed1981

Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome.

Fetal hydantoin syndrome (FHS), a characteristic pattern of altered growth and development, has been well described in recent years in offsprings of epileptic mothers taking phenytoin or other hydantoin anticonvulsants during the gestational period. Recent reports of neuroblastoma in three patients with the FHS further raise the questions of the "oncogenic effect " of hydantoin compounds. A case of melanotic neuroectodermal tumor of infancy (MNTI) has been studied clinically and pathologically including light microscopy, histochemistry, and electron microscopy. This case strengthens the evidence for the teratogenic and oncogenic effects of hydantoin compounds and we believe that it represents the first reported case of FHS associated with MNTI. It would be most important from a clinical standpoint to carefully scrutinize individuals with the FHS for neoplasias. Furthermore, detailed gestational drug history in children with neuroblastoma and other neoplasias should be carefully searched for, with the hope of clarifying the definitive oncogenic effect of hydantoin compounds.

Abnormalities, Drug-Induced↗
PubMed1989

Disseminated primitive neuroectodermal tumor: diagnosis using immunocytochemistry, electron microscopy, and molecular probes.

A patient with a disseminated small cell tumor presented with hyperuricemia, gingival hypertrophy, lymphadenopathy, and bone marrow replacement with tumor cells. Initial histologic examination and clinical presentation were consistent with presumed marker silent lymphoma/leukemia. Despite initial treatment with and response to lymphoma/leukemia therapy the patient relapsed in the testis, bone marrow, pancreas, and skin whereupon subsequent and retrospective immunocytochemical, ultrastructural, cytogenetic, and molecular analysis led to the diagnosis of primitive neuroectodermal tumor (PNET). Despite extensive investigation and autopsy no primary site of tumor could be found demonstrating that PNET should be considered in the differential diagnosis of disseminated small cell tumors without an apparent primary.

Adolescent↗
PubMed2004

Primary primitive neuroectodermal tumor of the uterus: a report of two cases and review of the literature.

BACKGROUND: Primary primitive neuroectodermal (pPNET) tumors rarely occur in adults, and they very rarely present as primary tumors of the uterus. Only 12 reported cases of pPNET of the uterus have been published in the English literature. We report two additional cases treated at the Roswell Park Cancer Institute, Buffalo, NY, between 1999 and 2002. CASES: Two postmenopausal patients presenting with abnormal uterine bleeding underwent endometrial biopsy, and subsequently staging laparotomy. The diagnosis of pPNET in both cases was confirmed only by extensive immunohistochemical analysis of the tumors. One patient with disease confined to an endometrial polyp received no adjuvant therapy, while the second patient with extrauterine disease received adjuvant pelvic radiation followed by chemotherapy. CONCLUSIONS: The diagnosis of pPNET of the uterus may be a challenge. Features of diagnostic significance include positive staining with neuron-specific enolase, presence of neurosecretory granules, and positive staining with the MIC-2 gene. Currently, there is no uniformity in the treatment of these cases since the majority of the patients reported to date have had surgery, chemotherapy, and/or radiation therapy.

Aged↗
PubMed1983

Primitive neuroectodermal tumor of the testis. Report of a case.

A 30-year-old man died of a testicular tumor with widespread metastases. Both the right testis and the adjoining epididymis were replaced by a mass that consisted mainly of small cells, but differentiated focally into medullary tubules, ependymal rosettes, and glia in which glial fibrillary acidic protein was demonstrated; rare foci of cartilage and cellular mesenchyme established the teratomatous nature of the neoplasm. We believe this is the first reported case of a primitive neuroectodermal tumor of the testis.

Adult↗
PubMed1991

Clinically effective monoclonal antibody 3F8 mediates nonoxidative lysis of human neuroectodermal tumor cells by polymorphonuclear leukocytes.

Most studies of antibody-dependent cellular cytotoxicity (ADCC) by polymorphonuclear leukocytes (PMN) have supported oxidative lytic processes. This may be because the studies used nonhuman or nonneoplastic cells that were highly sensitive to reactive oxygen species or were small enough to be phagocytosed by PMN. We therefore investigated whether oxygen radicals participate in PMN cytotoxicity toward human neuroectodermal solid tumor cells sensitized by 3F8, which is an anti-ganglioside GD2 murine IgG3 monoclonal antibody with documented anticancer activity in humans. A 4-h 51Cr release assay was used to assess tumor cell lysis by hydrogen peroxide, superoxide, and hypochlorite. Nine of 11 GD2(+) human melanoma and neuroblastoma cell lines had equal or greater resistance to these oxidants as compared to a GD2(-) human carcinoma line (SKBr1-III) found by others (and confirmed by us) to be significantly more resistant to oxidative lysis than a murine cell line (P388D1) representative of those commonly used in cytotoxicity assays. To facilitate detection of oxidant-mediated lysis, subsequent studies of 3F8-mediated ADCC used GD2(+) targets that were relatively sensitive and others that were relatively resistant to oxygen radicals. Normal PMN and PMN obtained from children with chronic granulomatous disease, which do not generate reactive oxygen species, were equally effective in ADCC. Granulocyte-macrophage colony-stimulating factor, which primes oxidative responses of normal but not of chronic granulomatous disease PMN, enhanced ADCC by both kinds of PMN. During ADCC of 3F8-sensitized targets, with or without granulocyte-macrophage colony-stimulating factor, GD2(-) "innocent bystander" tumor cells (including P388D1) were not lysed, a finding consistent with unimportant extracellular release of cytotoxic mediators. Finally, antioxidant and antimyeloperoxidase moieties did not block ADCC. We conclude that oxidants are not key factors in 3F8-mediated lysis by PMN of human neuroectodermal tumor cells.

Antibodies, Monoclonal↗
PubMed1992

Desmoplastic primitive neuroectodermal tumor with divergent differentiation. Broadening the spectrum of desmoplastic infantile neuroepithelial tumors.

We report an unusual large, multicystic, posterior fossa neuroepithelial neoplasm involving the cerebellum, brain-stem, and quadrigeminal cistern of a 9-month-old girl. The neoplasm consisted of variably sized, sharply demarcated nests of small cells with a high nuclear-cytoplasmic ratio and moderately basophilic nuclei, embedded in a desmoplastic, immature-appearing, mesenchymal stroma. The nests contained mitoses but none were seen in the stroma. Glial fibrillary acidic protein (GFAP), neurofilament protein, synaptophysin, and cytokeratin (AE-1) were expressed in the nests. Mesenchymal cells were negative for neural markers but positive for vimentin and desmin. The neoplasm was interpreted as a mixed mesenchymal and primitive neuroectodermal tumor (PNET) with histologic features reminiscent of a recently described intraabdominal desmoplastic small cell tumor. The tumor responded poorly to chemotherapy and a second operation was performed 1 year later. The second specimen bore no resemblance to the original and consisted of epithelial-like nests and clusters of neoplastic cells frequently interrupted by sinusoidal vessels. Tumor cells had medium-sized vesicular nuclei with small nucleoli, and a granular cytoplasm. Occasional less cellular islands of neuropil-like tissue contained larger cells having eccentric, vesicular nuclei with prominent nucleoli and abundant pink cytoplasm. Mitoses were not conspicuous. Many cells expressed synaptophysin, neurofilament protein, and GFAP. Neurofilament protein was strongly positive in the larger, neuron-like cells and synaptophysin stained the neuropil-like areas strongly but was less prominent in the neuronal perikarya. Unexpectedly, the neuropil-like areas expressed epithelial membrane antigen, whereas the neuronal cells were negative for chromogranin A. The peculiar histologic picture, combination of phenotypic markers, and remarkable biologic behavior of this unusual tumor defies classification according to existing nomenclature and exemplifies the broad range of phenotypes expressed by primitive neuro-epithelial neoplasms.

Brain Neoplasms↗
PubMedDate not supplied

Primitive neuroectodermal tumor with choroid plexus differentiation.

A case of a cerebral juvenile undifferentiated round cell tumor with unusual formation of glandular structures is presented. The round cell component showed focal expression of glial fibrillary acidic protein, S-100 protein, neurofilament protein and neuron-specific enolase. The glandular part consisted of papillary epithelial structures with a central PAS-positive lumen. Ultrastructural findings supported the interpretation of these parts as choroid plexus. This route of differentiation was recognizable at the first operation at the age of 5 years and was much more prominent in the recurrence found 7 1/2 years later. The unusually benign course of this primitive neuroectodermal tumor (PNET) may be due to its intrinsic progressive tendency towards choroid plexus differentiation.

Biopsy↗
PubMed1984

Active nucleolus organizers are precisely positioned in adult central nervous system cells but not in neuroectodermal tumor cells.

Active nucleolus organizing regions (NOR) were stained with silver in isolated nuclei and chromosomes, monolayer cultures, and tissues for electron microscopy. In nuclei isolated in 0.8-1.3 M urea, only the NOR stained with silver, and round or fiber-like regions with a diameter of 200-250 nm with a substructure were delineated at appropriate nucleolar or acrocentric chromosomal sites. In whole cells, additional (non NOR) silver binding regions were noted. Electrophoresis studies on nuclear isolates indicated at least two different nuclear protein subsets were responsible for the observed silver binding; the NOR protein(s) was most tightly bound with respect to urea. Interphase cells displayed a more extensive NOR network than mitotic cells, suggesting an increase in rDNA transcription during interphase. Mature sperm cells showed no active NOR regions. Interphase neuroectodermal tumor cells generally contained large multiple NOR indicating extensive activity; the position of NOR in the nucleus was variable from cell to cell, even in cloned lines. In contrast to tumor lines, central nervous system (CNS) cells from various species all showed highly reproducible or non-random NOR locations within the nucleus of each cell type. The NOR of large neurons were always central and single, whereas small granule cell neurons displayed a few small NOR that were positioned more peripherally. These findings suggest that in highly differentiated cells, the NOR region is precisely positioned in the nucleus, regardless of non-identical chromosome locations of the NOR in different species. The variability of NOR in tumor cells indicates profound changes in nuclear structure that may be part of the neoplastic transformation.

Adult↗
PubMed1996

Chemotherapy for Childhood Medulloblastoma and Primitive Neuroectodermal Tumors.

Medulloblastoma is the most common form of childhood brain tumor, and management has evolved over the past two decades. Chemotherapy is now an integral part of the treatment of the majority, if not all, patients with this disease. Medulloblastoma is a chemosensitive tumor, and recurrent disease will often respond to a variety of different chemotherapeutic agents. The use of higher-dose chemotherapy supplemented with aggressive hematological support may improve outcome for patients with recurrent disease. The results of prospective randomized trials and large, single, institutional trials in children with newly diagnosed disease suggest that chemotherapy, when given during and after radiotherapy, improves outcome. This is especially true for children with more extensive disease at the time of diagnosis. Event-free survival rates as high as 85% have been reported in children with newly diagnosed medulloblastomas treated with radiation and adjuvant chemotherapy consisting of CCNU, vincristine, and cisplatinum. At the present time, there is no clear evidence that preradiation chemotherapy improves survival for children with medulloblastoma. In fact, two prospective trials suggest that treatment with pre-irradiation chemotherapy may result in poorer overall outcome than treatment with similar doses of radiation therapy or radiation therapy supplemented by postradiation chemotherapy. There is preliminary evidence that chemotherapy may allow for a reduction in the dose of craniospinal irradiation therapy required to control disease, especially for children with nondisseminated disease at the time of diagnosis. Treatment for infants with medulloblastoma and other primitive neuroectodermal tumors remains suboptimal. Some infants and young children will experience long-term disease control after treatment with chemotherapy alone or chemotherapy followed by radiation when the child is older. High-dose chemotherapy supplemented by autologous bone marrow rescue or peripheral stem cell rescue has been utilized in young infants with promising results. The need for postchemotherapy radiation therapy and the volume of radiotherapy required to control disease remain under study.

Journal Article↗