Malignant Peripheral Nerve Sheath Tumor With Osseous Heterologous Differentiation in Uncommon Locations (Heart and Retropharynx)
Por:
Machado, I, Arana, E, Cruz, J, Brotons, S, Vendrell, J, Escriba, I, Luisa Chust, M, Martínez N, Lavernia, J and Llombart-Bosch, A
Publicada:
1 ago 2016
Resumen:
We report two cases of malignant peripheral nerve sheath tumor (MPNST)
in an uncommon location (heart and retropharynx) both with divergent
osseous heterologous differentiation. We present the pathological and
immunohistochemical studies that confirmed the neurogenic origin. The
histopathology of the tumor arising in the retropharynx showed a
transition from a neurofibroma to MPNST, making this a new report of an
MPNST arising from a plexiform neurofibroma without neurofibromatosis.
Primary cardiac MPNST with osseous differentiation has never been
reported before. In conclusion, the histology of MPNSTs is very
heterogeneous, showing no specific diagnostic immunoprofile or genetic
alteration. Thus, it is important to rule out other histologically
similar tumors, particularly in cases arising in uncommon locations or
tumors with divergent heterologous differentiation.
Filiaciones:
Machado, I:
Inst Valenciano Oncol, Valencia, Spain
Arana, E:
Inst Valenciano Oncol, Valencia, Spain
Cruz, J:
Inst Valenciano Oncol, Valencia, Spain
Brotons, S:
Inst Valenciano Oncol, Valencia, Spain
Vendrell, J:
Inst Valenciano Oncol, Valencia, Spain
Escriba, I:
Inst Valenciano Oncol, Valencia, Spain
Luisa Chust, M:
Inst Valenciano Oncol, Valencia, Spain
Martínez N:
Hosp Gen Alicante, Alicante, Spain
Lavernia, J:
Inst Valenciano Oncol, Valencia, Spain
Llombart-Bosch, A:
Univ Valencia, Valencia, Spain
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