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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1998

Desmoplastic small round cell tumour of unknown primary origin with lymph node and lung metastases: histological, cytological, ultrastructural, cytogenetic and molecular findings.

Desmoplastic small round cell tumour (DSRCT) is an extremely aggressive neoplasm belonging to the family of "small round blue cell tumours", which includes primitive neuroectodermal tumour (PNET), Wilms' tumour and Ewing's sarcoma. DSRCT is considered to be a neoplasm derived from a primitive cell. It is immunohistochemically reactive with epithelial, neuronal and mesenchymal cell markers, demonstrating divergent differentiation along three cell lines. Originally thought to arise from serosal surfaces, the tumour has recently been reported in the central nervous system and ovary. The tumour in this case is a neoplasm that meets the histological, immunohistochemical, cytological and cytogenetic criteria of DSRCT; it is not associated with serosal membranes, and it has supraclavicular and axillary lymph node deposits and multiple pulmonary and brain metastases. Tumour cells from our case show cytogenetic similarities with Ewing's sarcoma and PNET. Electron microscopic findings suggest similarities between DSRCT and Wilms' tumour. Cloning and sequencing of PCR products showed in-frame fusion of EWS exon 7 to WT1 exon 8.

Adult↗
PubMed2002

Alterations of E-cadherin, alpha-catenin and beta-catenin expression in neuroendocrine tumors of the gastrointestinal tract.

Neuroendocrine tumors (NETs) of the gastrointestinal tract comprise a heterogeneous group of neoplasms arising from the diffuse neuroendocrine system. These tumors strongly differ from each other on the basis of different pathogenetic, clinical, functional, histological, and prognostic patterns. Previous studies have shown that abnormal and reduced expression of the E-cadherin/catenin complex in several human cancers is associated with tumor dedifferentiation, advanced clinical stages, and poor survival rate. We assessed correlations between the expression of E-cadherin and catenins, Ki-67, and the following clinicopathological factors: age, embryological site of origin, size, histological growth pattern, the depth of penetration into the intestinal wall, and the presence of metastasis. In this study, reduction of membranous E-cadherin expression to a variable degree was detected in more than two-thirds (42 of 51) of gastrointestinal NETs (19 foregut, 8 midgut, and 24 hindgut) belonging to the complete neuroendocrine neoplastic spectrum [18 well-differentiated NETs, 22 well-differentiated neuroendocrine carcinomas (NECs), and 11 poorly differentiated NECs]. The reduction of E-cadherin expression was concomitant with the reduction of alpha-catenin (44 of 51) and beta-catenin (35 of 51) expression. Our immunohistochemical analysis demonstrated significant differences of percentage of membranous positive cells of E-cadherin, alpha-catenin, or beta-catenin between normal tissues and well-differentiated NETs (P=0.0038, P=0.004, and P=0.0329, respectively), well-differentiated NECs (P<0.001, P<0.001, and P<0.001, respectively) and poorly differentiated NECs (P=0.0002, P<0.0002, and P=0.0002, respectively). Among the gastrointestinal NETs, there were significantly more positive cells of E-cadherin, alpha-catenin, or beta-catenin in well-differentiated NETs than well-differentiated NECs (P=0.0006, P=0.0065, and P=0.0001, respectively) or poorly differentiated NECs (P=0.0053, P=0.0041, and P<0.001, respectively). MIB-1 labeling index generally showed a low proliferative activity in well-differentiated NETs (0.49+/-0.37) and well-differentiated NECs (0.662+/-0.66). A high proliferation rate was observed in poorly differentiated NECs (41.518+/-16.59). MIB-1 labeling index was significantly higher in poorly differentiated NECs than well-differentiated NETs and well-differentiated NECs (P<0.0001 and P<0.0001, respectively). E-cadherin, alpha-catenin, and beta-catenin expression were correlated significantly with transmural tumor invasion (P<0.0001, P=0.0001, and P<0.0001, respectively) and with size (P=0.0013, P=0.0001, and P<0.0001, respectively). These results indicate that the alteration in the E-cadherin/catenin expression may be involved in the growth and progression of gastrointestinal NETs.

Adolescent↗
PubMed2000

Ewing's sarcoma/peripheral primitive neuroectodermal tumor (pPNET) arising in the omentum as a multilocular cyst with intracystic hemorrhage.

A rare case of Ewing's sarcoma/peripheral primitive neuroectodermal tumor arising in the greater omentum in a 41-year-old man is reported. The patient presented with a hemorrhagic mesenteric cyst that was disclosed by the results of an abdominal echogram, a computed tomography scan, and magnetic resonance imaging. A laparotomy showed a multilocular cyst with intra-cystic hemorrhage. Histologically, the tumor wall consisted of sheets of small round cells separated by thick desmoplastic stroma. Rosette formations or ribbon-like cell arrangements were absent. Further pathological examination revealed that the membrane of the tumor cells was positive for MIC-2, and negative for epithelial membrane antigen, cytokeratin, and desmin, which are usually positive in intra-abdominal desmoplastic small round-cell tumors. An EWS/FLI1 fused transcript was detected by reverse transcription-polymerase chain reaction. These findings confirmed the diagnosis of Ewing's sarcoma/peripheral primitive neuroectodermal tumor. The patient died of tumor recurrence 4 months after his first admission. The autopsied tumor tissue exhibited neural differentiation in certain regions. To our knowledge, this is the first case to be reported of Ewing's sarcoma/peripheral primitive neuroectodermal tumor arising in the omentum with unique pathological features and the occurrence of partial neural differentiation during the clinical course. This case pointed out to us, as gastroenterologists, that only thorough examination confirms a definitive diagnosis of small round-cell tumor of the abdomen, it also shows that Ewing's sarcoma/peripheral primitive neuroectodermal tumor should be included in the differential diagnosis of cystic lesions in the omentum.

Adult↗
PubMed2002

Primitive neuroectodermal tumor of the transverse colonic mesentery defined by the presence of EWS-FLI1 chimeric mRNA in a Japanese woman.

We report a case of primitive neuroectodermal tumor (PNET) arising in the transverse colonic mesentery. A 24-year-old Japanese woman was admitted to Kagoshima City Hospital with complaints of abdominal pain and sensations of abdominal fullness of 5 months' duration. On palpation, a mass the size of an infant's head was noted in the right flank. Abdominal computed tomography (CT) and ultrasonography showed a huge mass that consisted of multiple cystic components. On arteriography, a slight tumor stain appeared, with stretched and displaced tributaries of the right colic and middle colic arteries. Barium swallow examination demonstrated that the ascending colon was shifted to the right and small intestine to the left. We performed an en-bloc resection of the tumor in the transverse colonic mesentery, including the ascending colon, proximal jejunum (20 cm in length), and greater omentum. The resected tumor was 12 x 10 x 7 cm in size, 590g in weight, elastic soft in consistency, and multicystic. Histologically, the specimens showed a sheet-like proliferation of spindle-to-polygonal cells, and focally, the tumor formed rosette structures. Immunohistochemically, the tumor cells were positive for neuron-specific enolase (NSE) and mic-2. EWS-FLI1 chimeric mRNA was detected by reverse transcriptase-polymerase chain reaction (RT-PCR). Based on the above findings, we finally diagnosed the tumor as PNET of the colonic mesentery. There has been no recurrence for 20 months after operation. PNET arising in the mesentery is very rare, and we distinguished PNET from other tumors by immunohistochemical examination and by demonstration of the presence of EWS-FLI1 chimeric mRNA in the tumor.

Adult↗
PubMed2004

Primary primitive neuro-ectodermal tumor of cauda equina with intracranial seeding.

Primitive neuro-ectodermal tumor (PNET) of the cauda equina is a rare entity. 18 cases have been reported in the literature so far, including 4 cases with intracranial seeding. Moreover parenchymal involvement of brain has never been reported as a form of intracranial seeding from PNET of the cauda equina. A 31 year-old female patient, with PNET of cauda equina showing intracranial seeding 6 months after surgery, is presented in this report. To our knowledge, this is the first adult case of spinal cord PNET with parenchymal involvement of brain. The histopathological, clinical and radiological findings as well as treatment of the patient were evaluated.

Adult↗
PubMed1999

Crystal and molecular structure of 2-hydroxy-1-naphthaldehyde isonicotinoyl hydrazone (NIH) and its iron(III) complex: an iron chelator with anti-tumour activity.

Previous studies have demonstrated that 2-hydroxy-1-naphthaldehyde isonicotinoyl hydrazone (NIH) and several other aroylhydrazone chelators possess anti-neoplastic activity due to their ability to bind intracellular iron. In this study we have examined the structure and properties of NIH and its FeIII complex in order to obtain further insight into its anti-tumour activity. Two tridentate NIH ligands deprotonate upon coordination to FeIII in a meridional fashion to form a distorted octahedral, high-spin complex. Solution electrochemistry of [Fe(NIH-H)2]+ shows that the trivalent oxidation state is dominant over a wide potential range and that the FeII analogue is not a stable form of this complex. The fact that [Fe(NIH-H)2]+ cannot cycle between the FeII and FeIII states suggests that the production of toxic free-radical species, e.g. OH. or O2.-, is not part of this ligand's cytotoxic action. This suggestion is supported by cell culture experiments demonstrating that the addition of FeIII to NIH prevents its anti-proliferative effect. The chemistry of this chelator and its FeIII complex are discussed in the context of understanding its anti-tumour activity.

Antineoplastic Agents↗
PubMed2006

Askin tumor with metastasis to the scalp: a histochemical, immunohistochemical and ultrastructural study.

A 29-year-old woman presented with facial edema, and imaging disclosed a tumor extending from the anterior chest wall to the anterosuperior aspect of the mediastinum. Transbronchial cytology of the primary tumor and biopsy of the metastatic scalp lesion were performed. Histologically, the tumor consisted of closely packed small round cells. The neoplastic cells generally had round nuclei, finely dispersed chromatin, and small to prominent nucleoli. Histochemically, the cytoplasm of the neoplastic cells contained abundant glycogen and stained with Grimelius silver. Immunohistochemically, the neoplastic cell membranes reacted with CD99 (MIC2) and the neoplastic nuclei reacted with Fli-1, but various other markers, including lymphocyte and skeletal muscle markers, were not detected. No neoplastic cells were also reactive for chromogranin A, synaptophysin, and neurofilament. Ultrastructurally, some neoplastic cells had delicate cytoplasmic processes and contained membrane-bound dense core granules in the cytoplasm. Even if results are immunohistochemically negative for neuroendocrine markers, the combination of immunohistochemistry of CD99 (MIC2) and Fil-1 may be useful in diagnosing Askin tumor or its metastatic lesion.

12E7 Antigen↗
PubMed2004

Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report.

Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues. We present a case of a 16-year-old girl with primary cutaneous and subcutaneous PNET/ES of the abdominal wall. Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter. The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen. Immunohistochemical analysis showed positivity for CD99, CD56, S100, and glial fibrillary acid protein, and ultrastructural features included cytoplasmic glycogen and focal complex interdigitating synaptic junction-like cytoplasmic folds. Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13). Cytogenetic, interphase fluorescence in situ hybridization, and molecular genetic analyses failed to show t(11:22) (q24;q12) or abnormalities of chromosome region 22q12. The clinical behavior and atypical and complex cytogenetic abnormalities exhibited by the tumor in this patient are unusual and represent the most aggressive end of the clinical spectrum of cutaneous and subcutaneous PNET/ES.

Adolescent↗
PubMedDate not supplied

Primitive neuroectodermal tumors of the biliary and gastrointestinal tracts: clinicopathologic and molecular diagnostic study of two cases.

Primitive neuroectodermal tumor (PNET) is a prototypic malignant small round cell tumor of childhood that is characterized in most cases by t(11;22) resulting in an EWS-FLI1 gene fusion. Once thought to be uncommon, PNET now accounts for almost 20% of malignant soft tissue tumors in children. Increased recognition of PNET is partly due to advances in immunohistochemistry and molecular diagnostics, which have led to the identification of the tumor in non-classical sites. We report the clinical, histologic, immunohistochemical, and molecular findings of two visceral PNETs of the digestive system--one involving the small intestine and the other involving the hepatic duct. Histologically, each tumor was composed of malignant small cells growing in sheets, nests, and lobules; the tumor cells of both cases showed characteristic immunoreactivity for vimentin and O13 (CD99). Reverse transcription-polymerase chain reaction (RT-PCR) analysis for t(11;22) using nested primers was performed with RNA extracted from paraffin-embedded, formalin-fixed tissue and demonstrated an EWS exon 7 to FLI1 exon 5 fusion in both cases, confirmed by Southern blot hybridization and DNA sequence analysis. These results illustrate the expanded clinicopathologic profile of PNET, and demonstrate that visceral PNETs, despite their unusual sites of presentation, maintain the characteristic immunohistochemical and genetic features of PNETs at more conventional sites.

Adolescent↗
PubMedDate not supplied

Intraoral tumor of Chievitz in a child.

Juxtaoral organs known as organs of Chievitz are intramuscular embryonic structures found close to the angle of the mandible near the insertion of the pterygomandibular raphae. They are considered of neuroepithelial origin with no known function. We describe the first tumor of the organ of Chievitz which presented intraorally in a child. Immunohistochemically, the Chievitz nests showed positive reaction for vimentin, cytokeratins, and epithelial membrane antigen and ultrastructurally demonstrated cytoplasmic processes and intermediate filament bundles. These observations, together with light microscopic features, suggest that the epithelial nests of the organ of Chievitz are meningothelial rather than neuroepithelial.

Antigens, Neoplasm↗
PubMed1986

Neurosecretion of arginine vasopressin by an olfactory neuroblastoma causing reversible syndrome of antidiuresis.

A 26-year-old woman with the syndrome of inappropriate antidiuresis demonstrated complete recovery following the resection of an olfactory neuroblastoma. Tissue arginine vasopressin levels by radioimmunoassay, immunohistochemical staining of the tissue arginine vasopressin, postoperative normalization of plasma arginine vasopressin levels, and the clinical resolution are evidence in support of a neurally derived tumor being the direct source of neurosecretion of arginine vasopressin rather than neurohypophyseal secretion or secretion from non-neural tissues, as reported to date in the etiology of the syndrome of inappropriate antidiuresis.

Adult↗