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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1995

Very-high-dose short-term chemotherapy for poor-risk peripheral primitive neuroectodermal tumors, including Ewing's sarcoma, in children and young adults.

PURPOSE: To improve the prognosis of patients with poor-risk peripheral primitive neuroectodermal tumors (pPNETs; including peripheral neuroepithelioma and Ewing's sarcoma), while testing the feasibility of intensive use in adolescents and young adults of high-dose cyclophosphamide, doxorubicin, and vincristine (HD-CAV). PATIENTS AND METHODS: This report concerns previously untreated patients with newly diagnosed pPNET deemed poor-risk because of a tumor volume more than 100 cm3 or metastases to bone or bone marrow. The P6 protocol consists of seven courses of chemotherapy. Courses 1, 2, 3, and 6 include 6-hour infusions of cyclophosphamide on days 1 and 2 for a total of 4,200 mg/m2 per course (140 mg/kg per course for patients < 10 years old), plus 72-hour infusions of doxorubicin 75 mg/m2 and vincristine 2.0 mg/m2 beginning on day 1 (HD-CAV). Courses 4, 5, and 7 consist of 1-hour infusions of ifosfamide 1.8 g/m2/d and etoposide (VP-16) 100 mg/m2/d, for 5 days. Granulocyte colony-stimulating factor (G-CSF) and mesna are used. Courses start after neutrophil counts reach 500/microL and platelet counts reach 100,000/uL. Surgical resection follows course 3 and radiotherapy follows completion of all chemotherapy. RESULTS: Among the first 36 consecutive assessable patients (median age, 17 years), HD-CAV achieved excellent histopathologic or clinical responses in 34 patients and partial responses (PRs) in two patients. For 24 patients with locoregional disease, the 2-year event-free survival rate was 77%; adverse events were two locoregional relapses, one distant relapse, and one secondary leukemia. All six patients with metastatic disease limited to lungs achieved a complete response (CR) and did not relapse; one is in remission 36+ months from diagnosis, but the other patients are not assessable in terms of long-term efficacy of the P6 protocol because of short follow-up time (n = 3), additional systemic therapy (bone marrow transplantation), or septic death (autopsy showed no residual pPNET). All six patients with widespread metastases had major responses, including eradication of extensive bone marrow involvement, but distant relapses ensued. Myelosuppression was severe, but most patients received the first three courses of HD-CAV within 6 to 7 weeks. Major nonhematologic toxicities were mucositis and peripheral neuropathy. CONCLUSION: Excellent antitumor efficacy and manageable toxicity support the dose-intensive use of HD-CAV for pPNET in children, as well as in young adults. Consolidation of remissions of pPNET metastatic to bone and bone marrow remains a therapeutic challenge.

Adolescent↗
PubMed1994

Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Disease-free survival for patients with small sarcomas of bone has been impressively improved with the use of intensive combination chemotherapy and safe local control with surgery or radiation. The ability of monoclonal antibodies to recognize different antigens has allowed new insights into the histogenesis and has distinguished a neural variant now referred to as malignant peripheral neuroectodermal tumor. Both entities share the translocation t(11;22) (q24;q12) as a constant phenomenon. The breakpoint region has now been cloned, allowing for molecular identification and detection of tumor cells and opening a new era of diagnostic and staging possibilities. Patients with disseminated disease, either at diagnosis or in relapse, have benefitted from megatherapy regimens followed by bone marrow or peripheral stem cell rescue. However, this approach is still under investigation and remains to be standardized.

Adolescent↗
PubMed2006

Peripheral primitive neuroectodermal tumor of the cauda equina in an elderly patient. Case report.

Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification remains disputed, PNETs are recognized as primary tumors in both the central and peripheral nervous systems. These rare tumors usually occur in children or young adults and are typically metastatic to the spinal cord. The authors report the case of a 70-year-old man with no significant medical history, in whom a peripheral PNET was demonstrated that, based on clinical and imaging findings, manifested as a nonspecific intrathecal tumoral process arising from the cauda equina. Although this lesion is rare in the elderly, the authors' case illustrates the importance of making this diagnosis as early as possible so that a treatment plan may be devised. The use of chemotherapy in association with radiotherapy in relation to the resection of the tumor should be reconsidered.

Aged↗
PubMed2000

[Cardiac manifestations of peripheral primitive neuroectodermal tumor (pPNET): a rare case].

HISTORY: A 44-year-old man presented to his general practitioner with increasing exertional dyspnoea. After a syncope he was admitted to hospital. INVESTIGATIONS: Echocardiography on admission demonstrated a large pericardial effusion (45 mm over the right and 57 mm over the left ventricle) which, in view of its haemodynamic relevance, had to be removed by pericardiocentesis. Cytological examination of the fluid showed various blood constituents but no atypical cells. Computed tomography (CT) of the thorax revealed a homogeneous hyperdense structure in the area of the left atrium. After several more pericardiocenteses echocardiography showed a pericardial tumour between the left atrium and ventricle. Gastroscopy, coloscopy, abdominal CT, an octreotide scan and positron-emission tomography did not indicate an extracardiac tumour. Coronary angiography and video-assisted thoracoscopy were performed. They confirmed an epicardial tumour at the level of the left atrium. TREATMENT AND COURSE: The entire round and solid tumour of about 3 cm in diameter, at the tip of the left atrial appendage was removed under extracorporeal circulation. No adjuvant treatment was given. Histological and immunohistological tests of the resected specimen (CD-99 and NSE-positive) provided the diagnosis of malignant peripheral primitive neuroectodermal tumour (pPNET). CONCLUSION: There are at present no adequate data on optimal treatment of pPNET in the heart. This case demonstrates that even with a large such tumour long remission is possible after complete removal without any adjuvant treatment.

Adolescent↗
PubMed1990

Overexpression of the pseudoautosomal gene MIC2 in Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Ewing's Sarcoma (ES), the second most frequent bone tumor in childhood and adolescence, and the probably closely related peripheral primitive neuroectodermal tumor (pPNET) share a unique cytogenetic translocation between chromosomes 11 and 22. Both of them expose high amounts of a glycoprotein on their cell surface, which can be specifically detected by the mAb HBA-71. The cDNA coding for the HBA-71 antigen was isolated by screening a cDNA expression library constructed from a pPNET-derived cell line. Nucleotide sequencing revealed the HBA-71 antigen to be the product of the pseudoautosomal gene MIC2 previously identified by the mAb 12E7 in haematopoietic cells. This antigen is a glycoprotein with a molecular weight of about 29,000 and is expressed in low amounts in most human cell lines and probably normal tissues and tumors with only a few exceptions. In T-cells the antigen is involved in cell adhesion processes. In ES- and pPNET-derived cell lines MIC2 expression is significantly enhanced. No gross changes in posttranslational modification could be observed. The high expression results in easy and specific detection of the antigen in immunocytochemical analysis of paraffin embedded tissue sections making HBA-71 a useful tool in tumor diagnosis.

12E7 Antigen↗
PubMedDate not supplied

Bone marrow metastasis in Ewing's sarcoma and peripheral primitive neuroectodermal tumor: An immunohistochemical study.

Bone marrow metastases from small round cell tumors can present diagnostic difficulties. In this study, we assessed the value of immunohistochemistry, using two monoclonal antibodies to CD99, for the diagnosis of metastatic disease in bone marrow trephine specimens from patients with Ewing's sarcoma or primitive neuroectodermal tumor (PNET). The proportions of specimens showing metastases were 10.3% with routine staining and 20.7% with immunohistochemistry. The specimens that were negative on conventional light microscopy and positive with immunohistochemistry all showed other abnormalities. The results do not support the routine use of immunohistochemistry in specimens that are normal by conventional light microscopy, but indicate that useful information may be gained in cases where marrow histology is obscured by fibrosis, necrosis, or distortion artefact. Neither of the two antibodies tested was superior for this purpose.

12E7 Antigen↗
PubMed1998

Ewing's sarcoma and peripheral primitive neuroectodermal tumors after their genetic union.

The Ewing family of tumors has been defined by the presence of an EWS-ets gene rearrangement. Using molecular techniques for the visualization of this aberration, several tumors of unusual location and histology were recently identified and added to this group. Increasing experimental data suggest that EWS-ets fusions act as transforming transcription factors. Subtractive screening of transgenic fibroblasts has led to the identification of potential targets. The authentic cellular context for these aberrant gene products is still unknown and may be diverse, however. Clinical heterogeneity has stimulated a search for genetic factors that may relate to treatment response. The effect of the EWS-ets fusion transcript structure on localized disease has been recently confirmed and frequent p16 tumor suppressor alterations have been described. The presence of metastases at diagnosis as the major predictor of outcome has been assessed on a submicroscopic level by reverse transcriptase-polymerase chain reaction. Problems arising from these studies are discussed, although the biological basis for variable disease extension remains obscure.

Heterogeneous-Nuclear Ribonucleoproteins↗
PubMed2002

Metastatic peripheral primitive neuroectodermal tumor (PNET) masquerading as liver abscess: a case report of liver metastasis in orbital PNET.

Adult primitive neuroectodermal tumor (PNET) of the orbit is an extremely rare malignant tumor. We report the case of a 37-year-old woman with PNET metastasis of the liver 3 years after treatment of the primary right intraconal orbital PNET with resection and chemoradiation adjuvant therapy. Literature review revealed eight previous cases of orbital PNET, but this is the first case report of liver metastasis arising from orbital PNET.

Adult↗
PubMed2004

Peripheral primitive neuroectodermal tumor of the stomach in a 14-year-old boy: a case report.

CONCLUSION: Although PNETs are rare malignancies, they should be considered in the differential diagnosis of submucosal gastric tumours in adolescents with clinical alarm symptoms.NET was confirmed by detection of the characteristic EWS/FLI-1 fusion gene, resulting in a reciprocal translocation t(11;22)(q24;q12). Three distinct liver metastases were detected by CT, MRI, and PET. The tumour failed to respond to neoadjuvant polychemotherapy with vincristine, etoposide, doxorubicin, and ifosfamide. Subtotal gastrectomy was performed and, surprisingly, we found diffuse metastatic infiltration of the liver that had not been detected by preoperative staging. Due to the diffuse metastatic disease the young patient's prognosis has to be considered very poor. Because of the tumour's intense expression of CD117 (c-kit), the patient is now treated with the tyrosine kinase inhibitor imatinib (STI571).

Adolescent↗
PubMed2006

Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report.

OBJECTIVE: Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET family) are small, round, blue cell tumors that have a decided predilection for young patients and commonly arise in bone and soft tissue. We are reporting a rare case of cavernous sinus pPNET in a 48-year-old woman. CLINICAL PRESENTATION: A 48-year-old woman presented with headache, ipsilateral maxillary, and ophthalmic and oculomotor nerve palsies. Neuroimaging revealed a cavernous sinus lesion. INTERVENTION: The patient underwent debulking of the tumor, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings. Bone scans, bone marrow aspiration, and biopsy and chest computed tomographic scans showed no evidence of systemic involvement. The patient had adjuvant treatment with radiotherapy and chemotherapy. After 14 months, the patient had no neurological deficits, and neuroimaging showed stable disease, although some chemotherapy complications occurred. CONCLUSION: This is a case of cavernous sinus pPNET in a 48-year-old woman, in whom the diagnosis is supported by the presence of EWS-FLI1 fusion gene. This seems to be the first reported case of a cavernous sinus pPNET confirmed by molecular genetic analysis.

Cavernous Sinus↗
PubMed2006

[Recurrence of peripheral primitive neuroectodermal tumor of the orbit with systemic metastases].

CASE REPORT: A six-year-old boy presented with proptosis of the right eye. Imaging studies detected a mass in the medial wall of the right orbit. This mass was biopsied revealing a histopathologic diagnosis of primitive neuroectodermal tumor, so chemotherapy treatment was given. After seven years in remission he presented with a recurrence of the orbital tumor and was found to also have systemic metastases. Treatment with chemotherapy, radiotherapy and orbital exenteration was unsuccessful. DISCUSSION: The orbital occurrence of these tumors is extremely rare. Differentiation from other small round cell tumors requires immunohistochemical and ultrastructural techniques.

Child↗