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Soft Tissue Tumors

Goldblum, John R. MD; Montgomery, Elizabeth MD



Department of Anatomic Pathology

Cleveland Clinic Foundation

Cleveland, OH

Johns Hopkins Hospital

Baltimore, MD

The current issue of Pathology Case Reviews focuses on one of the more rapidly evolving topics in soft tissue pathology, that is, fibroblastic/myofibroblastic tumors. This expansive group of tumors is among the more common types of soft tissue tumors encountered by general surgical pathologists, and such lesions can be difficult to classify precisely and distinguish from other spindle cell lesions. This issue begins with a review of the histologic spectrum of one of the most common benign myxoid fibroblastic tumors of soft tissue: myxoma. Dr. John O’Connell from Surrey Memorial Hospital in British Columbia and Dr. Pieter Nielsen from Massachusetts General Hospital have emphasized some of the less well-characterized variants of myxoma, including superficial angiomyxoma and juxta-articular myxoma, and have elaborated on the histologic spectrum of the most common type of myxoma, intramuscular myxoma. Although these lesions all share common morphologic features, they arise in distinctive clinical settings and have unique morphologic characteristics that set them apart from other soft tissue myxomatous lesions.

Dr. John Goldblum from the Cleveland Clinic Foundation follows with a review that elaborates on the relationship between dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) as well as the concept of fibrosarcomatous change. This review emphasizes the clinical, morphologic, immunohistochemical, and molecular genetic overlap between DFSP and GCF and puts into perspective the clinical significance of fibrosarcomatous change.

One of the major goals of this edition of Pathology Case Reviews is to expand on some of the newer entities that have been described in this family of tumors in recent years. Dr. Elizabeth Montgomery from Johns Hopkins discusses one such entity, inflammatory myxohyaline tumor. Dr. Montgomery is one of the authors of the original description of this underrecognized entity, and she neatly outlines the differential diagnostic considerations of this morphologically distinctive lesion. Similarly, Dr. Andrew Folpe from Emory University provides an excellent review and update of low-grade fibromyxoid sarcoma (Evans’ tumor). This entity remains one of the most challenging lesions to recognize in the field of soft tissue pathology. In addition, Dr. Folpe describes the features of hyalinizing spindle cell tumor with giant rosettes and emphasizes the morphologic overlap between low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor. Similar to some of the concepts elaborated by Dr. Goldblum in his review of fibrosarcomatous change in DFSP, Dr. Folpe emphasizes the relationship between adequate initial surgical excision and clinical outcome. Finally, Dr. Cristina Antonescu from Memorial Sloan-Kettering summarizes the important clinical and morphologic features of the recently described sclerosing epithelioid fibrosarcoma. She also provides a working outline of the variants of fibrosarcoma and their relationship to one another.

In recent years, one of the more controversial topics that continue to be discussed in soft tissue pathology circles is the existence and recognition of sarcomas with myofibroblastic differentiation. Dr. Cyril Fisher from the Royal Marsden NHS Trust in London provides a superb review of this evolving concept and eloquently describes the morphologic and immunophenotypic features of myofibrosarcomas.

The issue closes with a concise summary of cytogenetic and molecular genetic aspects of fibroblastic/myofibroblastic neoplasms by Dr. Brian Rubin from the University of Washington. Entities including DFSP, GCF, inflammatory myofibroblastic tumor and congenital fibrosarcoma are discussed with an emphasis on the molecular genetic alterations that seem to be central to the pathogenesis of these tumors. Given the rapid evolution of molecular genetics in the field of soft tissue pathology, this review is particularly timely.

We think we have gathered an excellent group of authors to contribute to this issue of Pathology Case Reviews, and we hope the information herein is not only interesting but also useful in the everyday practice of surgical pathology.


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© 2002 Lippincott Williams & Wilkins, Inc.