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Undifferentiated high-grade pleomorphic sarcoma (UHPS): Diagnostic criteria, differential diagnosis, and treatment. An attempt to erasure the misnomer "MFH"

Undifferenzierte "high-grade" pleomorphe Sarkome: diagnostische Kriterien, Differentialdiagnosen und Behandlung

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Zusammenfassung

GRUNDLAGEN: Das "maligne fibröse Histiozytom" ("MFH") wurde über Jahrzehnte als das häufigste Weichteilsarkom des Erwachsenenalters propagiert, obwohl die Histiogenese dieser Tumorentität kontrovers diskutiert wurde. METHODIK: Überblick zu MFH. ERGEBNISSE: Innerhalb der letzten zwei Dekaden zeigten mehrere Studien, dass der Begriff "MFH" für eine Fülle unterschiedlicher niedrig differenzierter/pleomorpher Sarkome verwendet wurde, obwohl eine spezifischere Subklassifizierung und daraus resultierend eine bessere Prognoseabschätzung möglich wäre. SCHLUSSFOLGERUNGEN: Mitbedingt durch die derzeit verfügbaren Untersuchungsmethoden wie Immunhistochemie, Cytogenetik und molekulare Diagnostik, sollte der Begriff undifferenziertes pleomorphes Sarkom ("MFH") nur für eine kleine Gruppe von pleomorphen malignen Weichteiltumoren verwendet werden, in denen man keine spezifische Differenzierungslinie nachweisen kann. Die exakte Klassifizierung der unterschiedlichen niedrig differenzierten/pleomorphen Sarkome könnte zu besseren/spezifischeren Behandlungsmodalitäten und verlängertem Überleben von Betroffenen beitragen.

Summary

BACKGROUND: For many years malignant fibrous histiocytoma ("MFH") was accepted to be the most common soft tissue sarcoma in adults, its histiogenesis, however, remained controversial. METHODS: Review of the current status of "MFH". RESULTS: Within the last two decades several studies demonstrated that "MFH" has been used as a "waste paper basket" for several better classifiable, poorly differentiated soft tissue sarcomas. Therefore, reclassification of these tumors could led to better prognostification. CONCLUSIONS: In the 21st century with the insight of immunohistochemistry as well as improved cytogenetic and molecular diagnostic the term high-grade undifferentiated pleomorphic sarcoma (so-called "MFH") should be restricted to a group of soft tissue malignancies without a specific line of differentiation. Better classification of various high-grade sarcomas may contribute to more specific treatment options and hence lead to improved survival for patients.

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References

  • O'Brien JE, Stout AP. Malignant Fibrous Xanthomas. Cancer 1964;17:1445–55

    Article  PubMed  Google Scholar 

  • Ozzello L, Stout AP, Murray MR. Cultural characteristics of malignant histiocytomas and fibrous xanthomas. Cancer 1963;16:331–44

    Article  PubMed  CAS  Google Scholar 

  • Kempson RL, Kyriakos M. Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histiocytoma. Cancer 1972;29:961–76

    Article  PubMed  CAS  Google Scholar 

  • Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 1978;41:2250–66

    Article  PubMed  CAS  Google Scholar 

  • Enzinger FM. Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer 1979;44:2147–57

    Article  PubMed  CAS  Google Scholar 

  • Guccion JG, Enzinger FM. Malignant giant cell tumor of soft parts. An analysis of 32 cases. Cancer 1972;29:1518–29

    Article  PubMed  CAS  Google Scholar 

  • Kyriakos M, Kempson RL. Inflammatory fibrous histiocytoma. An aggressive and lethal lesion. Cancer 1976;37:1584–606

    Article  PubMed  CAS  Google Scholar 

  • Weiss SW, Enzinger FM. Myxoid variant of malignant fibrous histiocytoma. Cancer 1977;39:1672–85

    Article  PubMed  CAS  Google Scholar 

  • Bertoni F, Capanna R, Biagini R, Bacchini P, Guerra A, Ruggieri P, et al. Malignant fibrous histiocytoma of soft tissue. An analysis of 78 cases located and deeply seated in the extremities. Cancer 1985;56:356–67

    Article  PubMed  CAS  Google Scholar 

  • Enjoji M, Hashimoto H, Tsuneyoshi M, Iwasaki H. Malignant fibrous histiocytoma. A clinicopathologic study of 130 cases. Acta Pathol Jpn 1980;30:727–41

    PubMed  CAS  Google Scholar 

  • Fletcher CD, Unni KK, Mertens F. WHO Classification of tumors. Pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press; 2002

    Google Scholar 

  • Brooks JJ. The significance of double phenotypic patterns and markers in human sarcomas. A new model of mesenchymal differentiation. Am J Pathol 1986;125:113–23

    PubMed  CAS  Google Scholar 

  • Dehner LP. Malignant fibrous histiocytoma. Nonspecific morphologic pattern, specific pathologic entity, or both? Arch Pathol Lab Med 1988;112:236–7

    PubMed  CAS  Google Scholar 

  • Coindre JM, Mariani O, Chibon F, Mairal A, De Saint Aubain Somerhausen N, Favre-Guillevin E, et al. Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma. Mod Pathol 2003;16:256–62

    Article  PubMed  Google Scholar 

  • Fletcher CD. Pleomorphic malignant fibrous histiocytoma: fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol 1992;16:213–28

    Article  PubMed  CAS  Google Scholar 

  • Fletcher CD, Gustafson P, Rydholm A, Willen H, Akerman M. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol 2001;19:3045–50

    PubMed  CAS  Google Scholar 

  • Oda Y, Tamiya S, Oshiro Y, Hachitanda Y, Kinukawa N, Iwamoto Y, et al. Reassessment and clinicopathological prognostic factors of malignant fibrous histiocytoma of soft parts. Pathol Int 2002;52:595–606

    Article  PubMed  Google Scholar 

  • Angervall L, Kindblom LG, Merck C. Myxofibrosarcoma. A study of 30 cases. Acta Pathol Microbiol Scand [A] 1977;85A:127–40

    CAS  Google Scholar 

  • Hollowood K, Fletcher CD. Malignant fibrous histiocytoma: morphologic pattern or pathologic entity? Semin Diagn Pathol 1995;12:210–20

    PubMed  CAS  Google Scholar 

  • Antonescu CR, Dal Cin P, Nafa K, Teot LA, Surti U, Fletcher CD, et al. EWSR1-CREB1 is the predominant gene fusion in angiomatoid fibrous histiocytoma. Genes Chromosomes Cancer 2007;46:1051–60

    Article  PubMed  CAS  Google Scholar 

  • Rossi S, Szuhai K, Ijszenga M, Tanke HJ, Zanatta L, Sciot R, et al. EWSR1-CREB1 and EWSR1-ATF1 fusion genes in angiomatoid fibrous histiocytoma. Clin Cancer Res 2007;13:7322–8

    Article  PubMed  CAS  Google Scholar 

  • Deyrup AT, Haydon RC, Huo D, Ishikawa A, Peabody TD, He TC, et al. Myoid differentiation and prognosis in adult pleomorphic sarcomas of the extremity: an analysis of 92 cases. Cancer 2003;98:805–13

    Article  PubMed  Google Scholar 

  • Massi D, Beltrami G, Capanna R, Franchi A. Histopathological re-classification of extremity pleomorphic soft tissue sarcoma has clinical relevance. Eur J Surg Oncol 2004;30:1131–6

    Article  PubMed  CAS  Google Scholar 

  • Binh MB, Sastre-Garau X, Guillou L, de Pinieux G, Terrier P, Lagace R, et al. MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data. Am J Surg Pathol 2005;29:1340–7

    Article  PubMed  Google Scholar 

  • Pilotti S, Della Torre G, Mezzelani A, Tamborini E, Azzarelli A, Sozzi G, et al. The expression of MDM2/CDK4 gene product in the differential diagnosis of well differentiated liposarcoma and large deep-seated lipoma. Br J Cancer 2000;82:1271–5

    Article  PubMed  CAS  Google Scholar 

  • Pilotti S, Della Torre G, Lavarino C, Sozzi G, Minoletti F, Vergani B, et al. Molecular abnormalities in liposarcoma: role of MDM2 and CDK4-containing amplicons at 12q13–22. J Pathol 1998;185:188–90

    Article  PubMed  CAS  Google Scholar 

  • Hornick JL, Bosenberg MW, Mentzel T, McMenamin ME, Oliveira AM, Fletcher CD. Pleomorphic liposarcoma: clinicopathologic analysis of 57 cases. Am J Surg Pathol 2004;28:1257–67

    Article  PubMed  Google Scholar 

  • Nascimento AF, Bertoni F, Fletcher CD. Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. Am J Surg Pathol 2007;31:99–105

    Article  PubMed  Google Scholar 

  • Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, et al. Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol 1996;20:391–405

    Article  PubMed  CAS  Google Scholar 

  • O'Connell JX, Wehrli BM, Nielsen GP, Rosenberg AE. Giant cell tumors of soft tissue: a clinicopathologic study of 18 benign and malignant tumors. Am J Surg Pathol 2000;24:386–95

    Article  PubMed  Google Scholar 

  • Oliveira AM, Dei Tos AP, Fletcher CD, Nascimento AG. Primary giant cell tumor of soft tissues: a study of 22 cases. Am J Surg Pathol 2000;24:248–56

    Article  PubMed  CAS  Google Scholar 

  • Mertens F, Fletcher CD, Dal Cin P, De Wever I, Mandahl N, Mitelman F, et al. Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology. Genes Chromosomes Cancer 1998;22:16–25

    Article  PubMed  CAS  Google Scholar 

  • Forus A, Weghuis DO, Smeets D, Fodstad O, Myklebost O, van Kessel AG. Comparative genomic hybridization analysis of human sarcomas: I. Occurrence of genomic imbalances and identification of a novel major amplicon at 1q21–q22 in soft tissue sarcomas. Genes Chromosomes Cancer 1995;14:8–14

    Article  PubMed  CAS  Google Scholar 

  • Larramendy ML, Tarkkanen M, Blomqvist C, Virolainen M, Wiklund T, Asko-Seljavaara S, et al. Comparative genomic hybridization of malignant fibrous histiocytoma reveals a novel prognostic marker. Am J Pathol 1997;151:1153–61

    PubMed  CAS  Google Scholar 

  • Kattan MW, Leung DH, Brennan MF. Postoperative nomogram for 12-year sarcoma-specific death. J Clin Oncol 2002;20(1):791–6

    Article  PubMed  Google Scholar 

  • Tunn PU, Kettelhack C, Durr HR. Standardized approach to the treatment of adult soft tissue sarcoma of the extremities. Recent Results Cancer Res 2009;179:211–28

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Institute of Pathology, Medical University of Graz, Graz, Austria

    B. Liegl-Atzwanger & A. Beham

  2. Division of Oncology, Department of Internal Medicine, Medical University of Graz, Graz, Austria

    G. Hofmann

  3. Department of Orthopedic Surgery, Medical University of Graz, Graz, Austria

    A. Leithner

Authors
  1. B. Liegl-Atzwanger
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  2. G. Hofmann
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  3. A. Leithner
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  4. A. Beham
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Correspondence to B. Liegl-Atzwanger.

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Liegl-Atzwanger, B., Hofmann, G., Leithner, A. et al. Undifferentiated high-grade pleomorphic sarcoma (UHPS): Diagnostic criteria, differential diagnosis, and treatment. An attempt to erasure the misnomer "MFH". Eur Surg 41, 143–149 (2009). https://doi.org/10.1007/s10353-009-0474-9

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  • DOI: https://doi.org/10.1007/s10353-009-0474-9

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