title: Ultrastructurally confirmed myofibrosarcoma: a series of 10 new cases, with a discussion on diagnostic criteria.
abstract: Some view ultrastructure as key to myofibrosarcoma diagnosis, whereas others argue that electron microscopy is too little used in contemporary practice to be considered an important diagnostic tool. These views are discussed in the context of 10 ultrastructurally confirmed cases of myofibrosarcoma, some occurring at rare sites such as skin and penis. Patient age ranged from 21 to 83 years, with a 6:4 male to female ratio. Size ranged from 2 to 7.5 cm and all had infiltrative margins. Histologically, all consisted of variably cellular fascicles of spindle cells with mild to moderately pleomorphic nuclei, small punctate nucleoli, and eosinophilic cytoplasm. All cases showed α-smooth muscle actin positivity and 2 showed very focal weak positivity for desmin. Ultrastructurally, the tumor cells contained rough endoplasmic reticulum, mainly peripheral smooth-muscle myofilaments, and fibronectin fibrils or fibronexus junctions at the cell surface. The most confident diagnosis of myofibrosarcoma is provided by ultrastructural examination. However, given the right histological appearance, use of a panel of antibodies that includes α-smooth muscle actin, desmin, and h-caldesmon, serves as an acceptable practical way of diagnosing myofibrosarcoma.
authors: The Christie NHS Foundation Trust, Manchester, UK. patrick.shenjere@christie.nhs.uk
authors: Patrick
authors: P
authors: Shenjere
authors: Brian
authors: B
authors: Eyden
authors: S Sankar
authors: SS
authors: Banerjee
authors: Bipasha
authors: B
authors: Chakrabarty
authors: Jonathan H
authors: JH
authors: Shanks
authors: Kanwal A
authors: KA
authors: Sikand
authors: Lia P
authors: LP
authors: Menasce
mesh_terms: D000199:Actins / Q000378:metabolism; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis; D003937:Diagnosis, Differential; D018870:Endoplasmic Reticulum, Rough / Q000648:ultrastructure; D017809:Fatal Outcome; D005260:Female; D005353:Fibronectins / Q000648:ultrastructure; D005354:Fibrosarcoma / Q000378:metabolism / Q000556:secondary*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008297:Male; D008545:Melanoma / Q000175:diagnosis; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D009130:Muscle, Smooth / Q000648:ultrastructure; D009210:Myofibrils / Q000648:ultrastructure; D009217:Myosarcoma / Q000378:metabolism / Q000556:secondary*; D009364:Neoplasm Recurrence, Local; D010413:Penis / Q000473:pathology; D012509:Sarcoma / Q000175:diagnosis; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology*; D014973:Xanthomatosis / Q000175:diagnosis; D055815:Young Adult
chemical_list: D000199:Actins; D014408:Biomarkers, Tumor; D005353:Fibronectins