title: Malignant Peripheral Nerve Sheath Tumor With Osseous Heterologous Differentiation in Uncommon Locations (Heart and Retropharynx).
abstract: 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.
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain isidro.machado@uv.es.
authors.forename: Isidro
authors.initials: I
authors.lastname: Machado
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Estanislao
authors.initials: E
authors.lastname: Arana
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Julia
authors.initials: J
authors.lastname: Cruz
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Simon
authors.initials: S
authors.lastname: Brotons
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Juan
authors.initials: J
authors.lastname: Vendrell
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Ismael
authors.initials: I
authors.lastname: Escriba
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: María Luisa
authors.initials: ML
authors.lastname: Chust
authors.affiliation: Hospital General de Alicante, Alicante, Spain.
authors.forename: Natividad
authors.initials: N
authors.lastname: Martínez-Banaclocha
authors.affiliation: Instituto Valenciano de Oncología, Valencia, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Lavernia
authors.affiliation: University of Valencia, Valencia, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Llombart-Bosch
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor; D002454:Cell Differentiation; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009442:Neurilemmoma / Q000175:diagnosis / Q000473:pathology*; D018318:Neurofibroma, Plexiform / Q000473:pathology; D009999:Ossification, Heterotopic / Q000175:diagnosis / Q000473:pathology*; D010614:Pharynx; D012983:Soft Tissue Neoplasms / Q000175:diagnosis / Q000473:pathology*; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: divergent heterologous differentiation; heart and retropharynx; malignant peripheral nerve sheath tumor