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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1998

Molecular features in a biphenotypic small cell sarcoma with neuroectodermal and muscle differentiation.

We report a case of a 13-year-old girl with soft tissue sarcoma of the hand, which showed muscle and neuroectodermal immunophenotypes. Molecular studies were performed on RNA collected from fine-needle aspiration (FNA) cytology and peripheral blood samples by nested reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern blot analysis. This biphenotypic tumor showed simultaneous expression of EWS-FLI1 and PAX3-FKHR transcripts, specific of Ewing family tumors and alveolar rhabdomyosarcoma, respectively. Although childhood sarcomas with simultaneous muscle and neural differentiation have been described to have EWS-FLI1 transcripts, there are no reports of tumors with both transcripts. Cytological specimens are a good source of RNA for molecular studies.

Adolescent↗

PubMed1995

Small round cell tumors of bone and soft tissue. A morphometric and stereometric comparative analysis of 119 cases.

OBJECTIVE: To analyze the discriminative capability of morphometric assessment of nuclear morphology in the differential diagnosis of small round blue cell tumors (SRCTs) of bone and soft tissue. STUDY DESIGN: The study material consisted of glutaraldehyde-fixed, resin-embedded, semithin sections from 119 human tumors. Nuclear area, perimeter, maximum diameter, form factors and nuclear density were measured in at least 300 nuclei per case. RESULTS: Neuroblastoma (NB) (10 cases) showed the most regular pattern. Ewing's sarcoma (ES) (35 cases); atypical Ewing's sarcoma (AEs) (15 cases) and peripheral neuroectodermal tumors (PNET) (30 cases) showed no significant differences regarding area, perimeter or form factors, but AEs showed a lower mean nuclear density that was statistically significant. Rhabdomyosarcomas (6 cases) and osteosarcomas (OS) (11 cases) were used as controls and showed several morphometric and stereometric differences with other SRCTs, whereas microcellular OSs (6 cases) shared features of SRCTs and conventional osteosarcomas. CONCLUSION: Morphometric characterization of nuclear features reveals differences between the distinct groups of SRCTs. Although overlapping occurred between all these groups at the individual case level, this study provides new support for the existence of morphologic links within the family of ES and PNET.

Bone Neoplasms↗

PubMed2000

Subcutaneous primitive neuroectodermal tumour in the abdominal wall of a child: long-term survival after local excision.

Tumours of the peripheral primitive neuroectodermal tumour/Ewing's sarcoma (PNET/ES) family can rarely occur as primary lesions within the skin and subcutis. We present a case of subcutaneous PNET within the abdominal wall of a 33-month-old child. Histologically it was a small round cell tumour that marked strongly for CD99 and displayed ultrastructual evidence of neural differentiation. The tumour was completely excised, but due to an initial misdiagnosis no adjuvant therapy was given. Despite this, the patient is well without evidence of disease 10 years and 3 months after surgery. A review of similar cases reveals not all patients are as fortunate, suggesting combination therapy, including chemotherapy, is still the best treatment for these tumours, even when small and superficial. Our report therefore highlights the importance of recognising PNET/ES in the skin and subcutis so appropriate therapy can be administered.

12E7 Antigen↗

PubMedDate not supplied

Aspiration cytology, immunocytochemistry and electron microscopy of a malignant peripheral neuroectodermal tumor. A case report.

The findings of fine needle aspiration (FNA) cytology, immunocytochemical staining and electron microscopy (EM) in a case of malignant peripheral neuroectodermal tumor (PNET) presenting as a soft tissue mass in the lateral abdominal wall are reported. The immediate evaluation of the aspirate revealed cells of a small round cell malignant tumor. To provide a specific preoperative diagnosis, additional cytologic material was aspirated for immunocytochemical and ultrastructural investigations. While the results of EM were nonspecific, allowing only the exclusion of other small round cell malignancies, immunocytochemical staining of the aspirate was suggestive of a PNET. The diagnosis of PNET was corroborated by histopathologic and immunohistochemical findings. This case indicates that an exact preoperative categorization of small round cell malignant tumors can be made by FNA biopsy in otherwise equivocal cases when immunocytochemical and ultrastructural techniques are also utilized.

Abdominal Muscles↗

PubMed1977

Nerve tumors of the hand and forearm.

Peripheral nerve tumors comprise less than 5% of all tumors of the hand. The most common solitary nerve tumor is the neurilemmoma, which arises from the neural sheath, is well encapsulated, minimally symptomatic, and may be surgically enucleated without producing a neurological deficit. Neurofibromas may be solitary, multiple, or associated with von Recklinghausen's disease. They are usually centrally placed with nerve fibers traversing the tumor mass making it more difficult to remove the tumor without producing permanent neurological damage. Malignant tumors include neurofibrosarcomas which often are very aggressive, requiring wide excision or amputation, and the rare neuroepitheliomas. Reported nerve tumors, intraneural in location but nonneural in origin, include fibrofatty infiltration of the median and digital nerves, intraneural lipoma, hemangioma, and ganglion cysts. These lesions may be treated by decompression or excision, depending on the nature of the tumor. Four unusual cases are described.

Adult↗

PubMed2004

PNET of the hallux.

We present the case of a 21-year-old lady who presented with a peripheral primitive neuroectodermal tumour (pPNET) of the distal phalanx of the right great toe. She had an amputation through her metatarso-phalangeal joint, the diagnosis having been confirmed histologically. Although her resection margins were clear and radiological staging revealed no metastases, she was commenced on adjuvant chemotherapy due to the high rate of metastases associated with the disease. Her surgery came after two years of intermittent pain and swelling of her toe, during which time she was undergoing conservative management, with serial visits to the outpatients department, of a radiological (magnetic resonance imaging) diagnosis of a haemangioma. This case report details her background, diagnosis and treatment as well as presenting a literature review of what is an unusual tumour in a previously unreported site.

Adult↗

PubMed1993

Bone tumors other than osteosarcoma after retinoblastoma.

BACKGROUND: The most frequent second malignant neoplasm after retinoblastoma is osteosarcoma, which may be associated with radiation therapy or arise de novo in patients with bilateral retinoblastoma. METHODS: Clinical, pathologic, and diagnostic imaging findings in two patients with Ewing sarcoma (ES) and one with peripheral neuroepithelioma (PN) are presented. RESULTS: Second malignant neoplasms located in the extremities or pelvis were seen 12-18 years after initial diagnosis of unilateral (two patients) or bilateral (one patient) retinoblastoma. The patients with unilateral retinoblastoma were treated by enucleation only; the other received ocular radiation therapy and chemotherapy. To the knowledge of the authors, this is the first documentation of round blue cell tumor after unilateral retinoblastoma. CONCLUSIONS: ES and PN should be included in the differential diagnosis of malignant neoplasms occurring after treatment of unilateral or bilateral retinoblastoma.

Adult↗

PubMed1998

An autopsy case of peripheral neuroepithelioma in posterior mediastinum with p53 point mutation.

An 18-year old male was admitted to our hospital complaining of back pain. His chest computed tomography showed a tumor in the posterior mediastinum. Open biopsy was performed, and a diagnosis of peripheral neuroepithelioma was made. No genetic abnormalities were detected in the DNA obtained from the biopsy specimen. He received chemotherapy and radiation several times. These treatment regimens were effective, but he relapsed 14 months later and died of respiratory failure due to tumor growth. Autopsy examination revealed a large tumor which occupied almost the entire right thoracic cavity, but there was no evidence of metastasis to other organs. Chromosomal translocation t(14;17) (q24;p12.2) and point mutation of exon 5 of the p53 gene were detected.

Adolescent↗

PubMed2002

Peripheral primitive neuroectodermal tumour during pregnancy.

The case of a 25-year-old primipara in the second trimester of pregnancy, suffering from a peripheral primitive neuroectodermal tumour (pPNET) diagnosed by bone biopsy, is described. External irradiation was initially performed because of Jacksonian seizures due to a lesion in the right cerebral hemisphere. Appropriate shielding was used to reduce fetal exposure during brain radiotherapy. Caesarian delivery at the 27th week of gestation was performed because of tumour progression. The neonate had no evidence of disease and survived for 1 month. However, the placenta and ovaries showed metastases from the maternal pPNET. The patient died 14 months after initial diagnosis owing to the aggressiveness of the tumour, the rapid and extensive semination (bone marrow, lung, liver, craniospinal axis involvement) and the inability to adequately treat the patient with appropriate doses of chemotherapy.

Adult↗

PubMed1996

Malignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1.

A case of multifocal malignant peripheral neuroectodermal tumor (PNET) arising from a plexiform neurofibroma in a 4-month-old Chinese boy with neurofibromatosis type 1 (NF-1) is described. Cytogenetic culture demonstrated hypotriploid karyotype with an abnormal clone characterized by 59-60, XY, +2, +3, +6, +8, +8, +12, +i(13)(q10), +der(14)t(1;14)(q21;q32), +16, +19, +20, +mar[cp3] with no apparent abnormality of chromosome 17. The child was treated with combination chemotherapy comprising ifosphamide, vincristine and doxorubicin. Despite initial partial response the child finally died of tumor progression and pulmonary metastases 8 months after diagnosis. We believe this is the first reported case of PNET in a child with NF-1 and may support an association between these two disorders of neural crest origin.

Fatal Outcome↗

PubMed1994

MIC2 analysis of small cell carcinoma.

Small cell carcinomas (SCCs) and peripheral neuroectodermal tumors (PNETs) are two distinct neoplasms that show considerable histologic, immunohistochemical, and clinical overlap, but differ in their therapies and prognoses. In an attempt to further diagnostically distinguish the two, 33 SSCs were analyzed from both pulmonary and extrapulmonary sites for the MIC2 gene product, a cell surface antigen strongly and reliably expressed in PNETs and Ewing's sarcoma (ES). Two of the 33 SCCs stained positively, but the staining was less intense than that seen with PNET and ES. The remaining 31 tumors did not stain. These data indicate that, in combination with a panel of immunohistochemical stains, analysis of neuroendocrine tumors for MIC2 expression may be useful in distinguishing between small cell carcinomas of both pulmonary and extrapulmonary origins and soft tissue PNETs.

12E7 Antigen↗

PubMed1991

Expression of the natural killer cell-associated antigens CD56 and CD57 in human neural and striated muscle cells and in their tumors.

The expression of the natural killer cell-associated antigens CD56 and CD57 which are known to show sequence homologies to the neural cell adhesion molecule N-CAM was examined immunohistochemically in normal and regenerative human neural and straited muscle cells and in their tumors using monoclonal antibodies Leu-19 and Leu-7. The pattern of expression of CD56 and CD57 in neural tissue was assessed in comparison with that of Mr 68,000 neurofilament. Dendritic interstitial cells were discriminated from neural cells by application of the pan leukocyte antigen CD53. In normal tissue, CD56 was expressed in thin nerve fibers, fine varicose and sensory nerve endings, cell membranes of ganglion cells, and fetal striated muscle cells. Thick nerve fibers, perikarya of ganglion cells, and adult striated muscle fibers were CD56 negative. In the normal state, CD57 was restricted to thick nerve fibers. Enhanced expression or reexpression of CD56 was found in regenerative neural cells which, in part, were also CD57 positive and in regenerative, CD57-negative striated muscle cells. In neural tumors, CD56 was detectable in 3 of 3 benign and 8 of 13 malignant schwannomas, 1 of 4 peripheral neuroepitheliomas, 4 of 4 ganglioneuromas, and 8 of 8 (ganglio-)neuroblastomas, whereas CD57 was restricted to one benign and one malignant schwannoma. Furthermore, CD56, but not CD57, could be found in 8 of 8 rhabdomyosarcomas. All in all, the pattern of expression of CD56 is comparable to that of N-CAM. CD57 exhibits a very restricted binding pattern which, in most instances, is complementary to that of CD56. The absence of CD56 in some neural tumors might reflect alterations in cell-cell interactions. Its reexpression in regenerative striated muscle cells and in rhabdomyosarcomas suggests a role of CD56 as an oncodevelopmental antigen.

Antigens, Differentiation, T-Lymphocyte↗

PubMed1994

Interphase molecular cytogenetics of Ewing's sarcoma and peripheral neuroepithelioma t(11;22) with flanking and overlapping cosmid probes.

The translocation, t(11;22)(q24;q12), recurrently observed in Ewing's sarcoma and in peripheral neuroepithelioma has been recently cloned. The analysis of a series of ES/PNE has revealed that the chromosome 22 breakpoints are clustered in a small region of 7 kb, called EWSR1, and that those on chromosome 11 are spread over a larger region of 40-50 kb, called EWSR2. Cosmids from loci flanking or overlapping these two regions have been obtained. We demonstrate here that fluorescence in situ hybridization (FISH) with these cosmids allows the localization of the two breakpoints with a 10-kb resolution and leads to a rapid and reliable ES/PNE diagnosis.

Cell Line↗

PubMed1988

Indistinguishable patterns of protooncogene expression in two distinct but closely related tumors: Ewing's sarcoma and neuroepithelioma.

Genetic characterization of human tumors promises new insights of biological importance and clinical relevance. We have found that two solid tumors, peripheral neuroepithelioma and Ewing's sarcoma of bone, which share a common cytogenetic rearrangement, are characterized by an indistinguishable and highly reproducible pattern of protooncogene expression. c-myc, N-myc, c-myb, and c-mil/raf-1 are all expressed at similar levels in these tumors. c-fes and c-sis expression was not detected in any specimens of either tumor. In contrast, the protooncogene c-ets-1, located near the breakpoint of the chromosomal translocation in these tumors, is variable in its expression. We also detected high levels of choline acetyltransferase in these tumors, which suggests a common neural origin. Since it is likely that the clinical behavior and therapeutic responsiveness of tumors relate closely to their biological and genetic features, the pattern of protooncogene expression of individual tumors may provide a novel basis for their characterization.

Bone Neoplasms↗

PubMed1987

Translocation of c-sis protooncogene in peripheral neuroepithelioma.

Molecular genetic analysis of the c-sis protooncogene was performed on two neuroepithelioma cell lines carrying a t(11;22)(q24;q12). The c-sis protooncogene was found by in situ hybridization to be translocated from its germline position on chromosome #22 to the derivative chromosome #11 in each cell line. However, it was not rearranged or amplified in either cell line examined. In addition, we did not detect c-sis transcripts in Northern blots of poly (A)+ RNA. This is similar to results found in Ewing's sarcoma, which carries a cytogenetically indistinguishable translocation from neuroepithelioma.

Adolescent↗

PubMed1996

Malignant peripheral nerve sheath tumour with vascular differentiation: a report of four cases.

Four cases of malignant peripheral nerve sheath tumour showing vascular differentiation are described. One case was associated with neurofibromatosis 1 and contained angiosarcomatous, cartilaginous and rhabdomyoblastic elements. The other cases occurred in patients without neurofibromatosis and showed a spectrum of vascular lesions ranging from lobulated, haemangioma-like structures to angiosarcoma. These are the first recorded examples of this phenomenon not associated with neurofibromatosis. Immunohistochemical examination demonstrated the endothelial nature of the lesions in all cases and revealed cells positive for alpha-smooth muscle actin, probably pericytes, closely apposed to the endothelium.

Adult↗

PubMed1994

Peripheral neuroectodermal tumor of the chest (Askin tumor) as secondary neoplasm after Hodgkin's disease: a case report.

The authors present the case of a 20-year-old woman who developed a peripheral neuroectodermal neoplasm of the thoracopulmonary region (Askin tumor) 7 years after achieving complete remission of stage-IV Hodgkin's disease. The previous treatment had consisted of eight courses of alternating MOPP/ABVD combined with involved-field 20-Gy radiotherapy. The second neoplasm appeared in a nonirradiated area of the chest wall, with erosion of the ribs as shown by sonography and computed tomography. The histological pattern was in accordance with a generic diagnosis of a malignant small cell tumor; the immunostaining positivity of the neoplastic cells for the neuron-specific enolase allowed us to make the diagnosis of a tumor with a neuroectodermal origin. Partial resection of the neoplasm and four courses of chemotherapy including adriamycin, cisplatin, and ifosfamide induced a complete remission, confirmed by surgical restaging. She is alive and well 10 months after the completion of therapy. The clinical, radiological, and microscopic features of this tumor occurring as a secondary neoplasm after Hodgkin's disease are described.

Adult↗

PubMed1998

Neuroblastoma and peripheral neuroectodermal tumors.

Peripheral neuroblastic tumors in childhood present unique problems in terms of diagnosis, classification, and histologic determinants of prognosis. The proper specimen handling of these neoplasms requires integration of gross and microscopic pathologic examination along with cytogenetic, immunohistochemical, and electron microscopy studies. The appropriate gross examination and processing of these tumors are described with particular emphasis on ancillary studies. Important special studies such as immunohistochemistry, flow cytometry, and genetic and molecular oncologic investigation are stressed, and the appropriate methods of processing materials for these studies are discussed. The handling of small biopsy specimens, fine-needle aspirates, or both is also addressed. The staging of neuroblastoma varies according to Pediatric Oncology Group and Children's Cancer Study Group systems, and the importance of obtaining appropriate information to satisfy either system is noted. Ancillary information for classification of neuroblastoma and related neoplasms is also presented.

Adolescent↗