Head and Neck Soft Tissue Sarcoma

Back To Search

Head and Neck Soft Tissue Sarcoma

Head and Neck Surgery
Author Credentials 

Rizwan Aslam, DO, MSc
Assistant Professor of Otolaryngology
Department of Otolaryngology Head and Neck Surgery
Tulane University School of Medicine
New Orleans, LA

Module Summary

Sarcomas of the head and neck are rare tumors accounting for approximately 2 percent of all head and neck cancers with approximately 1000 cases diagnosed each year in the United States. Patients that develop sarcomas in this region generally present with a palpable mass, local skin changes, or symptoms arising from site specific complications such as hoarseness, nasal obstruction or cranial nerve deficits. Imaging studies are useful in the in the complete examination of the patient and allow for preoperative staging and planning of reconstruction. Prognostic factors that have been identified include gender, tumor stage, status of surgical margins, histiologic subtype and nodal status. Ideal treatment for these malignancies involves wide margin resections, however due to the proximity to vital structures and the small space in this anatomic region this may not always be possible. The role and benefit of radiotherapy and chemotherapy for treatment of this tumor type is controversial and varies by anatomic site.

Embryology

Learning Objectives 
  1. Review the cell origin of all sarcomas.
  2. Explain the development of the head and neck. 
  3. Differentiate between the various histologic types of head and neck sarcomas.
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627-33.
  3. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Anatomy

Learning Objectives 
  1. Describe the anatomy of the head and neck region.
  2. Know the most common anatomic regions for sarcomas to form in the head and neck. 
  3. Recognize that the head and neck region is full of important vasculature and anatomy, and these sarcomas can affect many surrounding structures.
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627-33.
  3. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Pathogenesis

Learning Objectives 

Identify the common risk factors for head and neck sarcomas.

References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627-33.
  3. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Basic Science

Learning Objectives 
  1. Understand the role of mesenchymal cells in the human body.
  2. Be able to identify cell markers to differentiate the various types of sarcomas.
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627-33.
  3. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  4. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Incidence

Learning Objectives 
  1. Understand the incidence of each type of head and neck sarcoma in the general population.
  2. Identify the patient populations most at risk for head and neck sarcomas.
  3. Know how age affects the likelihood of various types of sarcomas.  
References 
  1. Tajudeen BA, Fuller J, Lai C, et al. Head and neck sarcomas: the UCLA experience. Am J Otolaryngol. 2014;35(4):476-81.
  2. O'neill JP, Bilsky MH, Kraus D. Head and neck sarcomas: epidemiology, pathology, and management. Neurosurg Clin N Am. 2013;24(1):67-78.
  3. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Genetics

Learning Objectives 

Identify genetic mutations that may predispose patients to sarcomas. 

References 
  1. O'neill JP, Bilsky MH, Kraus D. Head and neck sarcomas: epidemiology, pathology, and management. Neurosurg Clin N Am. 2013;24(1):67-78.
  2. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  3. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.

 

Patient Evaluation

Learning Objectives 
  1. Know the pertinent symptoms and signs in the evaluation of head and neck sarcomas.
  2. Be able to list the positive and negative prognostic indicators of head and neck sarcomas.
  3. Interpret aspects of history and physical exam that help differentiate among the different types of head and neck sarcomas.
  4. Identify imaging techniques and findings supportive of head and neck sarcoma.
  5. State the adjunctive tests that may be helpful in the evaluation of neck mass suspicious for sarcoma.
  6. Know the signs and risk factors for sarcoma recurrence.
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  3. Tajudeen BA, Fuller J, Lai C, et al. Head and neck sarcomas: the UCLA experience. Am J Otolaryngol. 2014;35(4):476-81.
  4. Tran LM, Mark R, Meier R, Calcaterra TC, Parker RG. Sarcomas of the head and neck. Prognostic factors and treatment strategies. Cancer. 1992;70(1):169-77.
  5. Kraus DH, Dubner S, Harrison LB, et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer. 1994;74(2):697-702.
  6. Flint PW, Haughey BH, Robbins KT et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. Elsevier Health Sciences; 2014.

Measurement of Functional Status

Learning Objectives 
  1. Know the risk head and neck sarcomas pose to breathing, circulation, vasculature and digestion.
  2. Know the best tests to evaluate for each possible functional impairment by neck sarcoma. 
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Kraus DH, Dubner S, Harrison LB, et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer. 1994;74(2):697-702.

Imaging

Learning Objectives 
  1. Understand the role of diagnostic imaging in evaluating head and neck sarcomas.
  2. Compare the use of CT, MRI, and PET in head and neck sarcoma diagnosis.
  3. Be able to identify characteristic features on imaging for each type of sarcoma.
References 
  1. Wanebo HJ, Koness RJ, Macfarlane JK, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck. 1992;14(1):1-7.
  2. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.

Pathology

Learning Objectives 
  1. Describe the key histologic findings of each type of sarcoma. 
  2. Know the value of immunohistochemistry in differentiating the various sarcomas.
  3. Understand the histologic grading system for the various types of sarcomas.
References 
  1. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  2. O'neill JP, Bilsky MH, Kraus D. Head and neck sarcomas: epidemiology, pathology, and management. Neurosurg Clin N Am. 2013;24(1):67-78.
  3. Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627-33.

Treatment

Learning Objectives 

Understand the available treatment options for sarcomas of the head and neck.

References 
  1. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  2. Tudor-green B, Gomez R, Brennan PA. Current update on the diagnosis and management of head and neck soft tissue sarcomas. J Oral Pathol Med. 2017;46(9):674-679.
  3. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239-52.

Medical Therapies

Learning Objectives 
  1. Know the available medical therapies available for sarcoma of the head and neck.
  2. Understand the role of medical therapy for sarcoma of the head and neck.
References 
  1. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  2. Tudor-green B, Gomez R, Brennan PA. Current update on the diagnosis and management of head and neck soft tissue sarcomas. J Oral Pathol Med. 2017;46(9):674-679.
  3. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239-52.

Pharmacology

Learning Objectives 
  1. Understand the potential benefits and adverse effects of pharmacologic therapies associated with sarcoma of the head and neck.
  2. Understand how different pharmacologic therapies are affected by physiological processes.
  3. Understand the mechanism of action of available pharmacologic therapies for sarcoma of the head and neck.
References 
  1. Tudor-green B, Fonseca FP, Gomez RS, Brennan PA. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med. 2017;46(9):667-673.
  2. Tudor-green B, Gomez R, Brennan PA. Current update on the diagnosis and management of head and neck soft tissue sarcomas. J Oral Pathol Med. 2017;46(9):674-679.
  3. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239-52.
  4. Wildiers H, Highley MS, De bruijn EA, Van oosterom AT. Pharmacology of anticancer drugs in the elderly population. Clin Pharmacokinet. 2003;42(14):1213-42.

Surgical Therapies

Learning Objectives 
  1. Understand the different surgical approaches necessary for resection in the various regions of head and neck.
  2. Understand the prognostic factors that contribute to recurrence after surgery.
  3. Know the complications that can occur post-surgical reconstruction.
References 
  1. Kraus DH, Dubner S, Harrison LB, et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer. 1994;74(2):697-702.
  2. Pellitteri PK, Ferlito A, Bradley PJ, Shaha AR, Rinaldo A. Management of sarcomas of the head and neck in adults. Oral Oncol. 2003;39(1):2-12.
  3. Eeles RA, Fisher C, A'hern RP, et al. Head and neck sarcomas: prognostic factors and implications for treatment. Br J Cancer. 1993;68(1):201-7.
  4. Breakey RW, Crowley TP, Anderson IB, Milner RH, Ragbir M. The surgical management of head and neck sarcoma: The Newcastle experience. J Plast Reconstr Aesthet Surg. 2017;70(1):78-84.
  5. Chao AH, Sturgis EM, Yu P, Skoracki RJ, Guadagnolo BA, Hanasono MM. Reconstructive outcomes in patients with head and neck sarcoma. Head Neck. 2013;35(5):677-83.

Staging

Learning Objectives 

Be familiar with the commonly used systems used to stage sarcomas of the head and neck.

References 

Amin MB, Edge S, Greene FL et al. AJCC Cancer Staging Manual. Springer; 2016.
Shuman AG, Brennan MF, Palmer FL, et al. Soft tissue sarcoma of the head & neck: nomogram validation and analysis of staging systems. J Surg Oncol. 2015;111(6):690-5.

Case Studies

  1. A 12-year-old boy presents with chronic nasal obstruction of the right nasal cavity and a mass sensation. Imaging studies and biopsy are consistent with a diagnosis of stage II rhabdomyosarcoma. What considerations must be taken into preoperative planning and prognosis for this patient? What are the possible complications that can arise from treatment?  
  2. A 36-year-old man presents with swelling of the right side of the jaw and pain. Diagnostic studies are consistent with a diagnosis of primary osteosarcoma. What considerations must be taken into preoperative planning for resection and reconstruction of this patient? In what instances would chemotherapy be a viable treatment option for this patient? What are the possible adverse effects related to chemotherapy?
  3. A patient presents with a previously diagnosed chondrosarcoma arising from the laryngeal cartilage. What anatomic considerations must be taken into account for preoperative planning for this patient? What are the potential complications that could arise in resection of this tumor?

Complications

Learning Objectives 

Predict the complications that arise secondary to treatment.

References 
  1. Chao AH, Sturgis EM, Yu P, Skoracki RJ, Guadagnolo BA, Hanasono MM. Reconstructive outcomes in patients with head and neck sarcoma. Head Neck. 2013;35(5):677-83.
  2. Raney RB, Asmar L, Vassilopoulou-sellin R, et al. Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and - III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group. Med Pediatr Oncol. 1999;33(4):362-71.
  3. Eisele DW, Smith RV. Complications in Head and Neck Surgery. Mosby; 2009.

Review

Review Questions 
  1. What cell types are the most common origin for development of head and neck sarcomas?
  2. Which anatomic region within the head and neck is the most commonly involved site?
  3. What common risk factors contribute to the development of sarcomas?
  4. Which cell markers are most commonly used to differentiate sarcoma type?
  5. Which aspects of the history and physical exam can be used to differentiate the type of sarcoma?
  6. What risk factors could indicate possible recurrence?
  7. How can the sarcoma type be differentiated based on imaging features?
  8. When should the different treatment modalities be applied?
  9. How can aging affect the pharmacokinetic properties of chemotherapy used to treat various sarcomas of the head and neck?
  10. Which surgical approaches should be considered based on the anatomic location of the cancerous growth?
Resources 

Learner must Sign In to access AAO-HNSF education activities.

  1. Annual Meeting Webcast (AMW):