Myofibroblastic tumours: neoplasias with divergent behaviour. Ultrastructural and flow cytometric analysis

Pathol Res Pract. 1999;195(9):619-32. doi: 10.1016/S0344-0338(99)80127-7.

Abstract

Myofibroblasts are spindle cells having ultrastructural features in common with smooth muscle cells and fibroblasts. In the last few years, tumours have been described in which myofibroblasts represent not only a reactive mechanism but also a true neoplastic component. They constitute new nosologic entities which might be termed "myofibroblastic tumours". Tumours with benign and, rarely, malignant behaviour are reported to belong to this group of lesions. Recently, a third tumour type with borderline biological course, named "inflammatory myofibroblastic tumour" (IMT), has been identified, a condition that has been regarded as a benign and reactive disorder for a long time. Only in recent reports has been demonstrated that, in spite of an apparently benign morphological pattern, some cases of IMT have a malignant course. In this connection, DNA analysis by flow cytometry is a valuable diagnostic tool, because it allows identification of the ploidy status, a procedure that is often useful for predicting the nature and the biological behaviour of the lesion. In this study, 11 cases of myofibroblastic tumours were examined retrospectively by evaluating clinicopathological features and DNA ploidy status by flow cytometry. The diagnosis of myofibroblastic tumour was confirmed by performing histology, immunohistochemistry, and electron microscopy in all patients. In detail, these 11 cases were composed of 1 benign myofibroblastoma, 1 myofibrosarcoma and 9 IMTs. Among these myofibroblastic tumours, all those with local recurrence or distant metastases (one myofibrosarcoma and three IMT) showed an aneuploid cell population demonstrable by flow cytometric analysis, whereas the other cases with benign course (one benign myofibroblastoma and six IMT) exhibited an euploid DNA content. These data suggest the following: a) Besides the rare myofibroblastomas and myofibrosarcomas, IMTs represent a larger group of lesions with potentially different biological and clinical course. b) DNA flow cytometric analysis is a reliable tool that support histopathological examination in characterizing those cases of IMT that, though being malignant, mimic benign lesions. Consequently, it establishes the basis for a different therapeutic approach according to the euploid or aneuploid DNA content.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Child
  • Child, Preschool
  • DNA, Neoplasm / analysis
  • Endoplasmic Reticulum, Rough / ultrastructure
  • Female
  • Fibroblasts / pathology
  • Fibrosarcoma / genetics
  • Fibrosarcoma / metabolism
  • Fibrosarcoma / ultrastructure*
  • Flow Cytometry
  • Granuloma, Plasma Cell / genetics
  • Granuloma, Plasma Cell / metabolism
  • Granuloma, Plasma Cell / pathology*
  • Humans
  • Immunoenzyme Techniques
  • Infant
  • Male
  • Middle Aged
  • Neoplasms, Muscle Tissue / genetics
  • Neoplasms, Muscle Tissue / metabolism
  • Neoplasms, Muscle Tissue / ultrastructure*
  • Ploidies
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / ultrastructure*

Substances

  • DNA, Neoplasm