6533b82dfe1ef96bd1291dcd
RESEARCH PRODUCT
Malignant Peripheral Nerve Sheath Tumor With Osseous Heterologous Differentiation in Uncommon Locations (Heart and Retropharynx)
Ismael EscribaAntonio Llombart-boschIsidro MachadoM. ChustJulia CruzEstanislao AranaSimon BrotonsNatividad Martínez-banaclochaJavier LaverniaJuan Bosco Vendrellsubject
AdultMalemedicine.medical_specialtyPathologySoft Tissue NeoplasmHeterologousSoft Tissue NeoplasmsMalignant peripheral nerve sheath tumor030204 cardiovascular system & hematologyBiologyPathology and Forensic MedicineHeart NeoplasmsYoung Adult03 medical and health sciences0302 clinical medicinePlexiform neurofibromaBiomarkers TumormedicineHumansNeurofibromamalignant peripheral nerve sheath tumorNeurofibromatosisNeurofibroma PlexiformOssification HeterotopicCell Differentiationmedicine.diseaseImmunohistochemistrydivergent heterologous differentiation030220 oncology & carcinogenesisPharynxImmunohistochemistryFemaleSurgeryHistopathologyheart and retropharynxAnatomyNeurilemmomadescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2016-01-01 |