Peripheral nerve sheath malignancy with multiple metastasis: a rare clinical case

Abstract

Background. Tumours of peripheral nervous system are represented by benign and malignant neoplasms with different clinical and biological traits. Malignant peripheral nerve sheath tumours of paraspinal localisation with the involvement of nerve structures are extremely rare and may occur isolated or comorbid with congenital neurofibromatosis. Current literature contains a few bioptic and selected autopsy clinical reports. Herewith, we present an own sectional observation of a rare malignant peripheral nerve sheath tumour with multiple metastasis supplemented with morphological and immunohistochemical descriptions.Clinical Case Description. An autopsy was performed on a 30-yo man’s cadaver. A tumour infiltrate was observed along Th5—Th9 of the spinal column intimately associated with thoracic vertebral bodies. Metastases were detected in the right lung, myocardium, peripancreatic and perirenal adipose tissue. Histological tumour examination revealed heterogeneous solid and rosette-like structures. Tumour immunophenotype: vimentin+, pancytokeratin-, CD45-, S-100+, NSE+, GFAP-, proliferative activity index (Ki-67 = 75-80%). This profile is descriptive of peripheral nerve sheath malignancy of high grade with multiple organic metastases.Conclusion. The sectional observation presented illustrates the difficulty to in vivo diagnose rare peripheral nerve sheath malignancies due to their infiltrative growth into spinal bone marrow and metastasis to organs (lungs, myocardium, peripancreatic and perirenal adipose tissue).