Multiple Gastrointestinal Stromal Tumors, Malignant Peripheral Nerve Sheath Tumor and Atypical Neurofibromatous Neoplasm With Uncertain Biologic Potential Developing in A Single Patient With Neurofibromatosis Type 1 Syndrome.
Int J Surg Pathol
; 32(7): 1415-1421, 2024 Oct.
Article
em En
| MEDLINE
| ID: mdl-38303154
ABSTRACT
Neurofibromatosis type 1 (NF1) is the most common human genetic disease. In these patients, the incidence of malignant peripheral nerve sheath tumors (MPNST) and gastrointestinal stromal tumors (GIST) is increased. A male patient in his forties with neurofibromatosis 1, presented with the coexistence of multiple GISTs located at intestinal and colonic mesentery, MPNST located at his leg and atypical neurofibromatous neoplasm with uncertain biologic potential located at colonic mesentery. By FISH, the MPNST harbored CDKN2A loss and recurred 1 year later. After reresection and radiotherapy, the patient is now disease-free without evidence of disease. Atypical neurofibromatous neoplasm with uncertain biologic potential is a newly defined entity, and it is important to discriminate it from low-grade MPNST, which requires more aggressive treatment methods. To the best of our knowledge, this is the first report describing synchronous GISTs, MPNST, and atypical neurofibromatous neoplasm with uncertain biologic potential developing in a single NF1 patient.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neurofibromatose 1
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Tumores do Estroma Gastrointestinal
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Neoplasias Primárias Múltiplas
Limite:
Adult
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Humans
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Male
Idioma:
En
Revista:
Int J Surg Pathol
Assunto da revista:
PATOLOGIA
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Turquia
País de publicação:
Estados Unidos