Malignant Peripheral Nerve Sheath Tumor in a Patient with Neurofibromatosis and Primary Hyperparathyroidism

Soft tissue pathology consultation changed the diagnosis: the tumor exhibited marked nuclear atypia, high mitotic rate, and areas of necrosis; the tumor extended to the surgical resection; and frozen section slide showed less cellular area with less atypia and was therefore diagnosed as neurofibroma...

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Bibliographic Details
Published in:The American surgeon 2019-06, Vol.85 (6), p.287-289
Main Authors: Herrera, Alexander, Kwayisi, Golda Meir, Reid, KMarie
Format: Article
Language:English
Subjects:
Computed tomography
Consultation
Genetic disorders
Genetic transformation
Hyperparathyroidism
Magnetic resonance imaging
Medical prognosis
Meir, Golda
Necrosis
Neurofibromatosis
Neurological disorders
Pain
Pathology
Patients
Peripheral nerves
Positron emission
Positron emission tomography
Radiation
Recklinghausen's disease
Sheaths
Soft tissues
Surgery
Tomography
Tumors
Citations: Items that this one cites
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Description
Summary:Soft tissue pathology consultation changed the diagnosis: the tumor exhibited marked nuclear atypia, high mitotic rate, and areas of necrosis; the tumor extended to the surgical resection; and frozen section slide showed less cellular area with less atypia and was therefore diagnosed as neurofibroma, Figure 2. The gold standard for imaging of MPNSTs is MRi with and without contrast.1 FDG PET/CT can also be used to detect malignant transformation in known neurofibromas with high sensitivity and specificity.4 MPNSTs presently have no standardized screening criteria for patients at increased risk, conferred by previous radiation and history of neurofibromatosis.2 In patients presenting with a first genetic "hit," in the form of neurofibromatosis, any identified visceral mass must be suspected to be malignant until proven otherwise. Brinkman M, Jentjens S, Boone K. Evaluation of the most commonly used (semi-) quantitative parameters of 18F-FDG PET/ CT to detect malignant transformation of neurofibromas in neurofibromatosis type 1.
ISSN:0003-1348
1555-9823
DOI:10.1177/000313481908500606