Head and Neck Tumors: Management of Primary Undifferentiated Pleomorphic Sarcoma

Document Type : Original


1 Department of Head and Neck Surgery, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, C1181ACH.

2 Department of Oncology, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, C1181ACH.


Sarcomas account for 1% of all tumors originated in the head and neck regions in adults. They constitute a heterogeneous group of tumors of mesenchymal origin with multiple histological variants. The undifferentiated pleomorphic sarcoma (UPS) subtype is the most frequent variant with an incidence range of 2.7-38%.
Materials and Methods:
This retrospective case series was conducted on 11 patients who were diagnosed with UPS and treated in our institution in the last 11 years.
According to the results, 26.8% of the sarcomas corresponded to UPS variant. Out of 11 patients, 5 cases were female. The median age of the participants was 58 years (range: 18-74 years). Seven and four patients were T1b and T2b, respectively. Ten patients had no metastasis at the moment of diagnosis. Six patients were categorized as intermediate-grade, and the remaining (n=5) were classified as high-grade. The most frequent location was the scalp (n=3). In the patients undergoing surgery, the mean follow-up duration was 52 months. Disease-free survival (DFS) rates were obtained at 70%, 50%, and 50% in the 1st, 3rd, and 5th years, respectively, with the mean DFS of 36 months. In addition, the overall survival rates in the 1st, 3rd, and 5th years were 100%, 100%, and 83%, respectively.
TheUPS represented the most common histological subtype in our series. They tend to be intermediate or high-grade tumors. An acceptable global survival rate justifies surgical treatment as the main therapeutic tool.


Main Subjects

1. Gorsky M, Epstein JB. Craniofacial osseous and chondromatous sarcomas in British Columbia, a review of 34 cases.Oral Oncol 2000; 36:27–31.
2. Shellenberger T, Sturgis EM. Sarcomas of the head and neck region. Curr Oncol Rep 2009; 11: 135–42.
3. Aljabab AS, Nason RW, Kazi R, Pathak KA. Head and Neck Soft Tissue Sarcoma. Indian Journal of Surgical Oncology December 2011; Volume 2, Issue 4, pp 286–90.
4. Kraus DH, Dubner S, Harrison LB, Strong EW, Hajdu SI, Kher U, et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas.Cancer 1994; 74:697–702.
5. Patel SG, Shaha AR, Shah JP. Soft  tissue sarcoma of the head and neck: an update. 2001; Am J Otolaryngol 22(1):2–18.
6. Kauffman SL, Stout AP.Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 1961; 14: 469-82.
7. Fletcher CDM, Mertens F, Lyon. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and
Bone.  2002.
8. Fletcher CD. The evolving classification of soft tissue tumours - an update based on the new 2013 WHO classification. Histopathology2014; Jan;64 (1): 2-11.
9. Akerman M. Malignant fibrous histiocytoma--the commonest soft tissue sarcoma or a nonexistent entity?Acta Orthop Scand Suppl 1997; 273: 41-6.
10. Dehner LP. Malignant fibrous histiocytoma. Nonspecific morphologic pattern, specific pathologic entity, or both? Arch Pathol Lab Med 1988; 112(3): 236-7.
11. 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(3): 213-28.
12. Hollowood K, Fletcher CD. Malignant fibrous histiocytoma: morphologic pattern or pathologic entity?Semin Diagn Pathol 1995; 12(3): 210-20.
13. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014 Oct;151(4):
14. Tajudeen BA, Fuller J, Lai C, Grogan T, Elashoff D, Abemayor E et al.Head and neck sarcomas: the UCLA experience.Am J Otolaryngol. 2014 Jul-Aug; 35(4):476-81.
15. Potter BO, Sturgis EM. Sarcomas of the head and neck. Surg Oncol Clin Am. 2003 Apr; 12(2): 379-417.
16. Clavien PA,Barkun J,de Oliveira ML, Vauthey JN,Dindo D,Schulick RDet al. The Clavien-Dindo classification of surgical complications: five-year experience.Ann Surg 2009; 250:187–96.
17. American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition (2010) published by Springer New York, Inc.
18. Hardison SA, Davis PL, Browne JD.Malignant fibrous histiocytoma of the head and neck: a case series. Am J Otolaryngol.2013 Jan-Feb;34(1):10-5.
19. Barosa J, Ribeiro J, Afonso L, Fernandes J, Monteiro E. Head and neck sarcoma: analysis of 29 cases. Eur Ann Otorhinolaryngol Head Neck Dis, 2014 Apr;131(2):83-6.
20. Nieweg OE, Pruim J, Van Ginkel RJ, Hoekstra HJ, Paans AM, Molenaar WM. Fluorine-18-fluorodeoxyglucose PET imaging of soft-tissue sarcoma.J Nucl Med 1996; 37(2): 257-61.
21. Eary JF, O'Sullivan F, Powitan Y, Chandhury K, Vernon C, Bruckner JD.Sarcoma tumor FDG uptake measured by PET and patient outcome: a retrospective analysis.Eur J Nucl Med Mol Imaging 2002, 29(9): 1149-54.
22. Miraldi F, Adler LP, Faulhaber P. PET imaging in soft tissue sarcomas.Cancer Treat Res 1997, 91: 51-64.
23. Jager PL, Hoekstra HJ, Leeuw J, van Der Graaf WT, de Vries EG,Piers D. Routine bone scintigraphy in primary staging of soft tissue sarcoma; Is it worthwhile? Cancer 2000, 89(8): 1726-31.
24. Kern KA, Brunetti A, Norton JA, Chang AE, Malawer M, Lack E, et al. Metabolic imaging of human extremity musculoskeletal tumors by PET.J Nucl Med 1988, 29(2):181-6.
25. Mekhmandarov S, Azaria M, Engelberg S, Lieberman MN. Utility of 24 hour bone scan in evaluation of bone involvement by soft tissue sarcoma.Clin Nucl Med, 13(9): 649-51, 1988.
26.Chao AH, Sturgis EM, Yu P, Skoracki RJ, Guadagnolo BA, Hanasono MM. Reconstructive outcomes in patients with head and neck sarcoma. Head Neck. 2013 May;35(5):677-83.
27. Barkley HT, Martin RG, Romsdahl MM, Lindberg R, Zagars GK. Treatment of soft tissue sarcomas by preoperative irradiation and conservative surgical resection.Int J Radiat Oncol Biol Phys 1988, 14(4): 693-9.
28. Lindberg RD, Martin RG, Romsdahl MM, Barkley HT Jr. Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas.Cancer 1981, 47(10): 2391-7.
29. Link MP, Goorin AM, Miser AW, Green AA, Pratt CB, Belasco JB, et al. The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of extremity. N Engl J Med 1986 314:1600–606.
30. Cahan WG, Woodard HQ, Higinbotham NL, Stewart FW, Coley BL. Sarcoma arising in irradiated bone: report of eleven cases. Cancer 1948, 1(1) : 3-29.
31. Mark RJ, Poen J, Tran L, Fu YS, Selch MT, Parker RG.Post-irradiation sarcomas. A single-institution study and review of the literature. Cancer 1994, 73(10): 2653-62.
32. Mills EE. Adjuvant chemotherapy of adult high-grade soft tissue sarcoma.J Surg Oncol 1982, 21(3): 170-5.
33. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data.Sarcoma Meta-analysis Collaboration 1997. Lancet, 350(9092): 1647-54.
34. Leyvraz S, Bacchi M, Cerny T, Lissoni A, Sessa C, Bressoud A, et al. Phase I multicenter study of combined high-dose ifosfamide and doxorubicin in the treatment of advanced sarcomas. Swiss Group for Clinical Research (SAKK).Ann Oncol 1998, 9(8): 877-84.
35. Patel SR, Vadhan-Raj S, Burgess MA, Plager C, Papadopolous N, Jenkins J, et al. Results of two consecutive trials of dose-intensive chemotherapy with doxorubicin and ifosfamide in patients with sarcomas. Am J Clin Oncol 1998, 21(3): 317-21.
36.O'Neill JP, Bilsky MH, Kraus D. Head and neck sarcomas: epidemiology, pathology, and management. Neurosurg Clin N Am. 2013; Jan; 24 (1): 67-78.
37. Tran LM, Mark R, Meier R, Calcaterra TC, Parker RG. Sarcoma of the head and neck, prognostic factors and treatment strategies.Cancer 1992 70:169–77.
38. Le Doussal, Coindre JM, Leroux A, Hacene K, Terrier P, Bui NB, et al. Prognostic factors for patients with localized primary malignant fibrous histiocytoma: a multicenter study of 216 patients with multivariate analysis.Cancer 1996, 77(9):
39. Midis GP, Pollock RE, Chen NP, Feig BW, Murphy A, Pollack A, et al. Locally recurrent soft tissue sarcoma of the extremities.Surgery 1998, 123(6): 666-71.
40. Singer S, Antman K, Corson JM, Eberlein TJ. Long-term salvageability for patients with locally recurrent soft-tissue sarcomas.Arch Surg 1992, 127(5): 548-53; discussion 553-4.
41. Zagars GK, Mullen JR, Pollack A. Malignant fibrous histiocytoma: outcome and prognostic factors following conservation surgery and radiotherapy.Int J Radiat Oncol Biol Phys 1996, 34(5): 983-94.
42. Salo JC, Lewis JJ, Woodruff JM, Leung DH, Breannan MF. Malignant fibrous histiocytoma of the extremity. Cancer 1999, 85(8): 1765-72.