Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult : a case report
OBJECTIVE: In adults, supratentorial primitive neuroectodermal tumor (sPNET) is a very rare undifferentiated embryoblastic neoplasm. Prognosis is worse in comparison to infratentorial medulloblastoma. Older age appears to be prognostically favorable. At present, 5-year survival rates remain below 50% in all age groups. Survival longer than 15 years in an adult has only been reported once so far.
CASE REPORT: In 1987, a 33-year-old-male patient presented with seizures following a six-month's history of dizziness. CT- and MRI-scans revealed a right occipital tumor with moderate contrast enhancement. The tumor was completely removed. The original histological diagnosis was that of an undifferentiated sarcoma, malignant hemangioendothelioma, grade III. The patient was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy). Admission for another reason in 2003 led to a re-evaluation of the original diagnosis. Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component. Up to 20% of tumor nuclei were labeled for Ki-67. Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent. According to these findings, the diagnosis was changed to a sPNET (WHO IVdegrees ). Other tumor entities were excluded by immunohistochemistry.
CONCLUSIONS: Although the prognosis of sPNET is reported to be poor, a small fraction with a rather benign biological and clinical behavior exists. Parameters determining long-term-survival in sPNET are not yet known. Whenever possible, complete surgical resection should be attempted followed by postoperative radiotherapy. The value of chemotherapy is an issue of continuous investigation.
| Medienart: |
Artikel |
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| Erscheinungsjahr: |
2006 |
|---|---|
| Erschienen: |
2006 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:77 |
|---|---|
| Enthalten in: |
Journal of neuro-oncology - 77(2006), 3 vom: 11. Mai, Seite 291-4 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Krampulz, Thomas [VerfasserIn] |
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| Themen: |
Biomarkers, Tumor |
|---|
| Anmerkungen: |
Date Completed 11.09.2006 Date Revised 19.04.2022 published: Print-Electronic Citation Status MEDLINE |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM161190723 |
|---|
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| 100 | 1 | |a Krampulz, Thomas |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult |b a case report |
| 264 | 1 | |c 2006 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ohne Hilfsmittel zu benutzen |b n |2 rdamedia | ||
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| 500 | |a Date Completed 11.09.2006 | ||
| 500 | |a Date Revised 19.04.2022 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a OBJECTIVE: In adults, supratentorial primitive neuroectodermal tumor (sPNET) is a very rare undifferentiated embryoblastic neoplasm. Prognosis is worse in comparison to infratentorial medulloblastoma. Older age appears to be prognostically favorable. At present, 5-year survival rates remain below 50% in all age groups. Survival longer than 15 years in an adult has only been reported once so far | ||
| 520 | |a CASE REPORT: In 1987, a 33-year-old-male patient presented with seizures following a six-month's history of dizziness. CT- and MRI-scans revealed a right occipital tumor with moderate contrast enhancement. The tumor was completely removed. The original histological diagnosis was that of an undifferentiated sarcoma, malignant hemangioendothelioma, grade III. The patient was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy). Admission for another reason in 2003 led to a re-evaluation of the original diagnosis. Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component. Up to 20% of tumor nuclei were labeled for Ki-67. Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent. According to these findings, the diagnosis was changed to a sPNET (WHO IVdegrees ). Other tumor entities were excluded by immunohistochemistry | ||
| 520 | |a CONCLUSIONS: Although the prognosis of sPNET is reported to be poor, a small fraction with a rather benign biological and clinical behavior exists. Parameters determining long-term-survival in sPNET are not yet known. Whenever possible, complete surgical resection should be attempted followed by postoperative radiotherapy. The value of chemotherapy is an issue of continuous investigation | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 7 | |a Biomarkers, Tumor |2 NLM | |
| 650 | 7 | |a Ki-67 Antigen |2 NLM | |
| 650 | 7 | |a Receptor, Nerve Growth Factor |2 NLM | |
| 700 | 1 | |a Hans, Volkmar H |e verfasserin |4 aut | |
| 700 | 1 | |a Oppel, Falk |e verfasserin |4 aut | |
| 700 | 1 | |a Dietrich, Uwe |e verfasserin |4 aut | |
| 700 | 1 | |a Puchner, Maximilian J A |e verfasserin |4 aut | |
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