Management of large dorsal diffuse plexiform neurofibroma

Salvatore D'Arpa, Adriana Cordova, Francesco Moschella, Adriana Tuttolomondo, Carlo Melloni

Risultato della ricerca: Article

1 Citazione (Scopus)

Abstract

Abstract Neurofibromas are considered one of the hallmarksigns of neurofibromatosis type 1(NF1). Diffuse plexiformneurofibroma is a congenital NF1-associated tumor, characterizedby overgrowth and interference with function of theaffected area. The rich vascular plexus associated with neurofibromas,together with their infiltrative pattern, makes themdifficult to eradicate. Complications of neurofibromas are rarebut include malignant transformation and potentially lifethreateninghemorrhages. The use of the term “giant” to definea neurofibroma is controversial because there is no clearconsensus and descriptions are limited to few case reports.We report a case of a large dorsal diffuse plexiform neurofibromaresponsible for significant worsening of a patient’squality of life. These features make us believe that the masscan be defined as “giant.” Comprehensive perioperative management,including arterial embolization of the feeding vesselsand stabilization of the patient’s conditions, is required toperform a safe surgical procedure in such a complicated case.
Lingua originaleEnglish
pagine (da-a)349-352
Numero di pagine4
RivistaDefault journal
Volume37
Stato di pubblicazionePublished - 2014

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Plexiform Neurofibroma
Neurofibroma
Neurofibromatosis 1
Blood Vessels
Neoplasms

All Science Journal Classification (ASJC) codes

  • Surgery

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Management of large dorsal diffuse plexiform neurofibroma. / D'Arpa, Salvatore; Cordova, Adriana; Moschella, Francesco; Tuttolomondo, Adriana; Melloni, Carlo.

In: Default journal, Vol. 37, 2014, pag. 349-352.

Risultato della ricerca: Article

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AU - Cordova, Adriana

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AU - Melloni, Carlo

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AB - Abstract Neurofibromas are considered one of the hallmarksigns of neurofibromatosis type 1(NF1). Diffuse plexiformneurofibroma is a congenital NF1-associated tumor, characterizedby overgrowth and interference with function of theaffected area. The rich vascular plexus associated with neurofibromas,together with their infiltrative pattern, makes themdifficult to eradicate. Complications of neurofibromas are rarebut include malignant transformation and potentially lifethreateninghemorrhages. The use of the term “giant” to definea neurofibroma is controversial because there is no clearconsensus and descriptions are limited to few case reports.We report a case of a large dorsal diffuse plexiform neurofibromaresponsible for significant worsening of a patient’squality of life. These features make us believe that the masscan be defined as “giant.” Comprehensive perioperative management,including arterial embolization of the feeding vesselsand stabilization of the patient’s conditions, is required toperform a safe surgical procedure in such a complicated case.

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