Abstract
Background
Undifferentiated pleomorphic sarcoma/NOS (not otherwise specified; former pleomorphic and storiform MFH) of the extremities is a common malignant soft tissue tumor in adults. The objective of this study is to determine prognostic factors for the outcome after surgical treatment with respect to the recent developments in classification.
Methods
From 1996 to 2004, 140 undifferentiated pleomorphic sarcomas/NOS were identified out of 1,200 soft tissue sarcomas of the extremities that were treated at our institution and recorded in a prospective database. Overall survival (OS) and isolated local recurrence (ILR) were determined by Kaplan–Meier analysis. All tumors were retrospectively analyzed regarding prognostic factors of the disease, including patient’s background (primary or recurrent), histological grade (G2/G3), adjuvant chemotherapy and radiotherapy, size (T1–2) and depth of the tumor, and surgical margins (R0, R1, R2).
Results
In 123 patients, a wide resection was performed (limb-sparing surgery). In nine patients, an amputation was necessary. The overall 5-year survival rate was 72% (median follow up: 52 months). There was a significant difference between the group presenting with primary tumors (5-year survival: 84%, p < 0.05) and recurrent tumors (5-year survival: 62%, p < 0.05). Isolated local recurrence occurred in 36 patients.
Conclusions
In terms of OS and ILR, primary or recurrence, negative surgical margins, size and grading had a highly significant influence, whereas the site of surgery and adjuvant chemotherapy, adjuvant radiation and tumor depth did not. Prognosis for patients with undifferentiated pleomorphic sarcoma of the extremities depends predominantly on adequate wide resection of the primary tumor.
Similar content being viewed by others
References
Belal A, Kandil A, Allam A, Khafaga Y, El-Husseiny G, El-Enbaby A, Memon M, Younge D, Moreau P, Gray A, Schultz H (2002) Malignant fibrous histiocytoma: a retrospective study of 109 cases. Am J Clin Oncol 25:16–22
Bramwell V, Quirt I, Warr D, Verma S, Young V, Knowling M, Eisenhauer E (1989) Combination chemotherapy with doxorubicin, dacarbazine, and ifosfamide in advanced adult soft tissue sarcoma. Canadian Sarcoma Group—National Cancer Institute of Canada Clinical Trials Group. J Natl Cancer Inst 81:1496–1499
Bramwell VH (1988) Current perspectives in the management of soft-tissue sarcoma. The role of chemotherapy in multimodality therapy. Can J Surg 31:390–396
Coindre JM (1993) Pathology and grading of soft tissue sarcomas. Cancer Treat Res 67:1–22
Dei Tos A (2006) Classification of pleomorphic sarcoma: where are we now? Histopathology 48:51–62
Fletcher CD (1992) Pleomorphic malignant fibrous histiocytoma: fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol 16:213–228
Fletcher CD (2006) The evolving classification of soft tissue tumours: an update based on the new WHO classification. Histopathology 48:3–12
Fletcher CD, Gustafson P, Rydholm A, Willen H, Akerman M (2001) Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol 19:3045–3050
Hartmann JT, Oechsle K, Huober J, Jakob A, Azemar M, Horger M, Kanz L, Bokemeyer C (2006) An open label, non-comparative phase II study of gemcitabine as salvage treatment for patients with pretreated adult type soft tissue sarcoma. Invest New Drugs 24:249–253
Hartmann JT, Patel S (2005) New drug developments for patients with metastatic soft tissue sarcoma. Curr Oncol Rep 7:300–306
Issels RD (2006) High-risk soft tissue sarcoma: clinical trial and hyperthermia combined chemotherapy. Int J Hyperthermia 22:235–239
Lee SY, Obata Y, Yoshida M, Stockert E, Williamson B, Jungbluth AA, Chen YT, Old LJ, Scanlan MJ (2003) Immunomic analysis of human sarcoma. Proc Natl Acad Sci USA 100:2651–2656
Lee YF, John M, Edwards S, Clark J, Flohr P, Maillard K, Edema M, Baker L, Mangham DC, Grimer R, Wooster R, Thomas JM, Fisher C, Judson I, Cooper CS (2003) Molecular classification of synovial sarcomas, leiomyosarcomas and malignant fibrous histiocytomas by gene expression profiling. Br J Cancer 88:510–515
Lehnhardt M, Daigeler A, Hauser J, Puls A, Soimaru C, Kuhnen C, Steinau HU (2007) The value of expert second opinion in diagnosis of soft tissue sarcomas. J Surg Oncol 97:40–43
Lehnhardt M, Kuhnen C, Drucke D, Homann HH, Joneidi Jafari H, Steinau HU (2004) [Liposarcoma of the extremities: recent developments in surgical therapy. Analysis of 167 patients]. Chirurg 75:1182–1190
Matsumoto S, Ahmed AR, Kawaguchi N, Manabe J, Matsushita Y (2003) Results of surgery for malignant fibrous histiocytomas of soft tissue. Int J Clin Oncol 8:104–109
Mentzel T, Bainbridge TC, Katenkamp D (1997) Solitary fibrous tumour: clinicopathological, immunohistochemical, and ultrastructural analysis of 12 cases arising in soft tissues, nasal cavity and nasopharynx, urinary bladder and prostate. Virchows Arch 430:445–453
Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, Fletcher CD (1996) Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol 20:391–405
Mentzel T, Fletcher CD (1998) Recent advances in soft tissue tumor diagnosis. Am J Clin Pathol 110:660–670
Mentzel T, Katenkamp D, Fletcher CD (1996) [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. Pathologe 17:116–121
Nakayama R, Nemoto T, Takahashi H, Ohta T, Kawai A, Seki K, Yoshida T, Toyama Y, Ichikawa H, Hasegawa T (2007) Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma. Mod Pathol 20:749–759
Nielsen TO, West RB, Linn SC, Alter O, Knowling MA, O’Connell JX, Zhu S, Fero M, Sherlock G, Pollack JR, Brown PO, Botstein D, van de Rijn M (2002) Molecular characterisation of soft tissue tumours: a gene expression study. Lancet 359:1301–1307
O’Brien JE, Stout AP (1964) Malignant fibrous xanthomas. Cancer 17:1445–1455
Oda Y, Tamiya S, Oshiro Y, Hachitanda Y, Kinukawa N, Iwamoto Y, Tsuneyoshi M (2002) Reassessment and clinicopathological prognostic factors of malignant fibrous histiocytoma of soft parts. Pathol Int 52:595–606
Popov P, Tukiainen E, Asko-Seljavaara S, Huuhtanen R, Virolainen M, Virkkunen P, Blomqvist C (2004) Soft-tissue sarcomas of the upper extremity: surgical treatment and outcome. Plast Reconstr Surg 113:222–230, discussion 231–222
Poremba C (2006) [Soft tissue sarcomas: the role of histology and molecular pathology for differential diagnosis]. Verh Dtsch Ges Pathol 90:59–72
Randall RL, Albritton KH, Ferney BJ, Layfield L (2004) Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop 33:602–608
Singer S, Demetri GD, Baldini EH, Fletcher CD (2000) Management of soft-tissue sarcomas: an overview and update. Lancet Oncol 1:75–85
Skubitz KM, D’Adamo DR (2007) Sarcoma. Mayo Clin Proc 82:1409–1432
Skubitz KM, Skubitz AP (2003) Differential gene expression in leiomyosarcoma. Cancer 98:1029–1038
Steinau HU, Ehrl H, Biemer E (1988) [Limb-sparing surgery in recurrent soft tissue sarcomas?]. Chirurg 59:265–271
Steinau HU, Hebebrand D, Hussmann J (1993) [Reconstructive possibilities after extensive resection of malignant soft tissue tumors]. Chirurg 64:517–526
Steinau HU, Hebebrand D, Torres A, Vogt P (1998) Surgical management of soft tissue sarcomas: principles of resection and reconstructive plastic procedures. Schweiz Rundsch Med Prax 87:1061–1065
Steinau HU, Homann HH, Drucke D, Torres A, Soimaru D, Vogt P (2001) Resection method and functional restoration in soft tissue sarcomas of the extremities. Chirurg 72:501–513
Stoeckle E, Kantor G, Thomas L, Coindre JM, Bui BN (2004) Surgery of recurrent soft tissue sarcoma of the extremities and the trunk wall: a comparison to primary sarcoma. Bull Cancer 91:853–860
Tos AP (2006) Classification of pleomorphic sarcomas: where are we now? Histopathology 48:51–62
Weiss SW, Enzinger FM (1978) Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 41:2250–2266
Weiss SW, Enzinger FM (1977) Myxoid variant of malignant fibrous histiocytoma. Cancer 39:1672–1685
Weitz J, Antonescu CR, Brennan MF (2003) Localized extremity soft tissue sarcoma: improved knowledge with unchanged survival over time. J Clin Oncol 21:2719–2725
Yamaguchi U, Hasegawa T (2004) [Pathological evaluation of soft tissue sarcoma for diagnosis, prognosis and treatment]. Gan To Kagaku Ryoho 31:1340–1345
Acknowledgement
We thank Renate Klaassen-Mielke, Institute of Biometry and Statistical Analysis, Ruhr University Bochum for his help in statistical analysis.
We thank Amanda Daigeler for her formal English revision of the manuscript.
Author information
Authors and Affiliations
Corresponding author
Additional information
Marcus Lehnhardt and Adrien Daigeler contributed equally to the study.
Rights and permissions
About this article
Cite this article
Lehnhardt, M., Daigeler, A., Homann, H.H. et al. MFH revisited: outcome after surgical treatment of undifferentiated pleomorphic or not otherwise specified (NOS) sarcomas of the extremities—an analysis of 140 patients. Langenbecks Arch Surg 394, 313–320 (2009). https://doi.org/10.1007/s00423-008-0368-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00423-008-0368-5