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Results for “Neuroectodermal Tumors”

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At least 811 records · Page 45Linked to original sources
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↗
PubMedDate not supplied

[Primitive neuroectodermal tumor of rare localization in two members of one family].

A 26-year-old man with primitive neuroectodermal tumour (PNET) is reported. The tumour originated from the cervical spinal cord and was resected partially. Few months later dissemination of the tumour to the meninges occurred. Familial history revealed that the first daughter of the patient had died in age of 14 months three years earlier of a tumour of the right cerebral hemisphere, also diagnosed as PNET.

Adult↗
PubMed1984

Peripheral neuroectodermal tumors of the chest wall in childhood.

A retrospective review of primary chest wall malignant tumors of childhood collected at the Children's Memorial Hospital of Chicago was undertaken. Among twelve instances of poorly differentiated neoplasms whose uniform, monotonous structure made accurate classification difficult or impossible by conventional histologic study, there were three tumors with features suggestive of neuroectodermal differentiation. Electron microscopic and immunohistologic findings further strengthened this interpretation, despite the fact that none of the patients had evidence of a primary neuroblastoma outside the chest wall. These results and a review of the pertinent literature support the conclusion that neuroectodermal neoplasms in childhood may present in peripheral somatic tissues with greater frequency than is commonly assumed. The importance of this distinction is discussed, particularly the need to distinguish these neoplasms from Ewing's sarcoma.

Adolescent↗
PubMed1981

[Pigmented neuroectodermal tumor (melanoameloblastoma)].

A pigmented tumor (melanoameloblastoma) in a 4-month-old infant localized in the upper jaw is described. The tumor consisted of cells with melanin granules in the cytoplasm. The detected neurosecretory granules and melanosomes in the parenchymal cells of the tumor suggest to be of neuroectodermal origin.

Child, Preschool↗
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↗
PubMed1996

Ewing's sarcoma and extracranial primitive neuroectodermal tumors.

The Ewing's family of tumors is comprised of bone and soft tissue, small round blue cell neoplasms of neuroectodermal origin defined by the chromosomal aberration t(11;22)(q24;q12), and closely related variants. Molecular methods now exist to facilitate diagnosis and to defect minimal residual disease. Multiagent chemotherapeutic regimens in concert with adequate local control yield improved survival rates in patients with localized disease at diagnosis. Patients with metastatic disease still suffer poor survival rates; programs attempting to cure metastatic patients with intensive therapy as facilitated by peripheral stem cell and autologous marrow rescue have shown some promise. Intensive regimens with and without rescue are being explored for high-risk patients.

Antineoplastic Agents↗
PubMed1984

The neuroectodermal tumor of bone.

Four small round cell malignant tumors of bone occurring in children are described. There was no catecholamine secretion and the clinical, radiologic, and biopsy diagnosis in each was Ewing's sarcoma. Glycogen was sparse both on imprints and in tissue sections. The tumors, when extensively sampled, had areas of a lobular growth pattern and Homer Wright rosettes. The rosettes were always focal and varied in complexity from case to case; they were rudimentary in one instance and markedly fibrillar in the most obvious instance. Neuron-specific enolase was demonstrated in tissue sections and in longterm cell cultures from three of the tumors. The cultured cells put out moderately long beaded processes in serum-free medium but had no catecholamine fluorescence. Electron microscopy of the tumor rosettes and the cultured cells showed processes containing aggregates of microtubules and only one case had rare neurosecretory granules. This study suggests that some small round cell tumors of bone and soft tissue in children, which present as Ewing's sarcoma, are neuroectodermal in nature.

Bone Neoplasms↗
PubMed1998

[Two children with unusual causes of torticollis: primitive neuroectodermal tumor and Grisel's syndrome].

Two children were presented with a torticollis. One, a 2-year-old girl, had a primitive neuroectodermal tumour of the cervical spinal cord which could not be radically removed surgically (the patient died three days later), the other, a 9-year-old boy, had the syndrome of Grisel. He was treated with antibiotics, head traction and atlantoaxial posterior spondylodesis. Acquired torticollis is a frequent innocent symptom in children with cervical lymphadenopathy following a respiratory tract infection. These cases demonstrate that additional investigation should not be delayed if relief of symptoms is not achieved very soon.

Atlanto-Axial Joint↗