Die Bedeutung von Referenzzentren in Diagnose und Therapie von Weichgewebssarkomen der Extremitäten

Abstract

Correct histopathologic diagnosis is essential for adequate treatment of soft tissue sarcomas. Due to the disorder's rarity, multitude of subgroups, sometimes varying histopathologic appearance, and occasionally inadequate biopsy specimens, diagnosis and grading are challenging. The records of 603 patients with soft tissue tumors of the extremities were reviewed concerning mismatches in primary and definite diagnoses relating to entity, evaluation of primary or recurrent tumor specimens, and the diagnosing pathology institution. For second opinions we referred to the Institute of Pathology of the Ruhr University at the Bergmannsheil Hospital in Bochum, Germany, and to the Pathology Institute of the University of Jena, also in Germany. Liposarcoma and malignant fibrous histiocytoma were the most often diagnosed subgroups at 24% and 22.6%, respectively. In the eight most frequent sarcoma types, malignant peripheral nerve sheath tumors and leiomyosarcoma had the highest rates of false primary diagnosis, 78.4% and 74.2% of cases, respectively. The diagnostic error ratio for nonspecialized pathologists in practice, community hospital pathologists, and academic medical centers was over 60%. For optimal treatment of soft tissue sarcomas, we suggest obtaining expert second opinion to ensure adequate surgical therapy and precise indications for radiation and chemotherapy.

Soft Tissue Sarcomas of the Arm – Oncosurgical and Reconstructive Principles within a Multimodal, Interdisciplinary Setting

“…It is generally recommended that STS should be treated in tumor centers ( 16 , 22 – 28 ). However, given the rarity and diversity of these tumors, it is not surprising that excisions are often performed without preoperative suspicion of malignancy and adequate preoperative diagnostic and staging work-up ( 22 , 26 , 29 ).…”

The cost-saving effect of centralized histological reviews with soft tissue and visceral sarcomas, GIST, and desmoid tumors: The experiences of the pathologists of the French Sarcoma Group

“…The tumor grade was not systematically incorporated into the shared database. As the difficulty in making the diagnosis can depend on the grade, this has an impact on discordant diagnoses with sarcoma [ 17 , 25 ] and other diseases [ 7 ]. However, the relevance of the grade has declined since the development of targeted therapies based on histological subtypes and molecular anomalies.…”

Malignant peripheral nerve sheath tumour (MPNST)

“…In the case of MPNST the percentage of incorrect initial histological diagnoses is the highest among all STSs, accounting for up to 78% when the diagnosis was made outside reference centres treating sarcomas. To be qualified as MPNST, it is necessary for STS to meet one of three specific criteria: 1) the tumour has developed in the peripheral nerve, 2) the tumour has developed from the nerve sheaths of a pre-existing benign tumour (neurofibroma or others), or 3) the set of histological features of differentiated Schwann cells can be identified in the tumour [2,37,38]. MPNST can be in the classical spindle cell form or in the form of pleomorphic and epithelioid (epithelial) [2].…”