Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature
Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associ...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Steinbichler, Teresa B [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2014 |
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| Schlagwörter: |
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| Anmerkung: |
© Steinbichler et al.; licensee BioMed Central. 2014 |
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| Übergeordnetes Werk: |
Enthalten in: World journal of surgical oncology - London : Biomed Central, 2003, 12(2014), 1 vom: 17. Dez. |
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| Übergeordnetes Werk: |
volume:12 ; year:2014 ; number:1 ; day:17 ; month:12 |
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| DOI / URN: |
10.1186/1477-7819-12-387 |
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| 520 | |a Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. | ||
| 650 | 4 | |a Chondrosarcoma |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Enchondroma |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Hemangioma |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Maffucci syndrome |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Kral, Florian |4 aut | |
| 700 | 1 | |a Reinold, Susanne |4 aut | |
| 700 | 1 | |a Riechelmann, Herbert |4 aut | |
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10.1186/1477-7819-12-387 doi (DE-627)SPR028828984 (SPR)1477-7819-12-387-e DE-627 ger DE-627 rakwb eng Steinbichler, Teresa B verfasserin aut Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Steinbichler et al.; licensee BioMed Central. 2014 Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Chondrosarcoma (dpeaa)DE-He213 Enchondroma (dpeaa)DE-He213 Hemangioma (dpeaa)DE-He213 Maffucci syndrome (dpeaa)DE-He213 Kral, Florian aut Reinold, Susanne aut Riechelmann, Herbert aut Enthalten in World journal of surgical oncology London : Biomed Central, 2003 12(2014), 1 vom: 17. Dez. (DE-627)369082907 (DE-600)2118383-1 1477-7819 nnns volume:12 year:2014 number:1 day:17 month:12 https://dx.doi.org/10.1186/1477-7819-12-387 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2014 1 17 12 |
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10.1186/1477-7819-12-387 doi (DE-627)SPR028828984 (SPR)1477-7819-12-387-e DE-627 ger DE-627 rakwb eng Steinbichler, Teresa B verfasserin aut Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Steinbichler et al.; licensee BioMed Central. 2014 Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Chondrosarcoma (dpeaa)DE-He213 Enchondroma (dpeaa)DE-He213 Hemangioma (dpeaa)DE-He213 Maffucci syndrome (dpeaa)DE-He213 Kral, Florian aut Reinold, Susanne aut Riechelmann, Herbert aut Enthalten in World journal of surgical oncology London : Biomed Central, 2003 12(2014), 1 vom: 17. Dez. (DE-627)369082907 (DE-600)2118383-1 1477-7819 nnns volume:12 year:2014 number:1 day:17 month:12 https://dx.doi.org/10.1186/1477-7819-12-387 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2014 1 17 12 |
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10.1186/1477-7819-12-387 doi (DE-627)SPR028828984 (SPR)1477-7819-12-387-e DE-627 ger DE-627 rakwb eng Steinbichler, Teresa B verfasserin aut Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Steinbichler et al.; licensee BioMed Central. 2014 Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Chondrosarcoma (dpeaa)DE-He213 Enchondroma (dpeaa)DE-He213 Hemangioma (dpeaa)DE-He213 Maffucci syndrome (dpeaa)DE-He213 Kral, Florian aut Reinold, Susanne aut Riechelmann, Herbert aut Enthalten in World journal of surgical oncology London : Biomed Central, 2003 12(2014), 1 vom: 17. Dez. (DE-627)369082907 (DE-600)2118383-1 1477-7819 nnns volume:12 year:2014 number:1 day:17 month:12 https://dx.doi.org/10.1186/1477-7819-12-387 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2014 1 17 12 |
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10.1186/1477-7819-12-387 doi (DE-627)SPR028828984 (SPR)1477-7819-12-387-e DE-627 ger DE-627 rakwb eng Steinbichler, Teresa B verfasserin aut Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Steinbichler et al.; licensee BioMed Central. 2014 Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Chondrosarcoma (dpeaa)DE-He213 Enchondroma (dpeaa)DE-He213 Hemangioma (dpeaa)DE-He213 Maffucci syndrome (dpeaa)DE-He213 Kral, Florian aut Reinold, Susanne aut Riechelmann, Herbert aut Enthalten in World journal of surgical oncology London : Biomed Central, 2003 12(2014), 1 vom: 17. Dez. (DE-627)369082907 (DE-600)2118383-1 1477-7819 nnns volume:12 year:2014 number:1 day:17 month:12 https://dx.doi.org/10.1186/1477-7819-12-387 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2014 1 17 12 |
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10.1186/1477-7819-12-387 doi (DE-627)SPR028828984 (SPR)1477-7819-12-387-e DE-627 ger DE-627 rakwb eng Steinbichler, Teresa B verfasserin aut Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Steinbichler et al.; licensee BioMed Central. 2014 Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Chondrosarcoma (dpeaa)DE-He213 Enchondroma (dpeaa)DE-He213 Hemangioma (dpeaa)DE-He213 Maffucci syndrome (dpeaa)DE-He213 Kral, Florian aut Reinold, Susanne aut Riechelmann, Herbert aut Enthalten in World journal of surgical oncology London : Biomed Central, 2003 12(2014), 1 vom: 17. Dez. (DE-627)369082907 (DE-600)2118383-1 1477-7819 nnns volume:12 year:2014 number:1 day:17 month:12 https://dx.doi.org/10.1186/1477-7819-12-387 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2014 1 17 12 |
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Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. © Steinbichler et al.; licensee BioMed Central. 2014 |
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Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. © Steinbichler et al.; licensee BioMed Central. 2014 |
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Introduction Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases. Case report We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary. Conclusion Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. © Steinbichler et al.; licensee BioMed Central. 2014 |
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| score |
7.3988304 |