Adult Head and Neck Sarcoma

1
Adult Head and Neck Sarcoma

• Grand Rounds
• SIU Otolaryngology

2
Sarcoma

• Definition Malignant neoplasm of soft tissue or
  bone arising in fibrous, fatty, muscular,
  synovial, vascular, or neural tissue, usually
  first manifested as a painless swelling.

3
Overview

• Case presentation
• Soft tissue sarcoma
• Chondrosarcoma
• Osteosarcoma
• Rhabdomyosarcoma
• Most common soft tissue sarcoma in children
• Ewings Sarcoma
• 2nd most common malignant bone tumor in children
• Kaposi Sarcoma
• Soft tissue, skin mucosal
• Epidemic or non-classic
• Classic

4
Head and Neck Sarcoma

• Approximately 15-20 of all sarcomas occur within
  the head and neck
  
• Unlike SCCA, ETOH and smoking are not risk
  factors
  
• Genetic syndromes Li Fraumeni Syndrome
• Environmental exposures - radiation
• Medical conditions - lymphedema
• 80 of all sarcomas are of soft tissue origin
• 20 arise from bone
• 80 of head and neck sarcomas present in adults

5
Case Presentation

• 24 y/o F with 3 month history of palpable L
  submental mass
  
• Slow enlargement
• Tender
• Not associated with the thyroid, no cervical LAD,
  palpable on oral cavity exam
  
• No previous infection
• No previous radiation
• PHM not significant

6
Case Presentation

• FNA follicular epithelial cells with mild
  atypia, columnar cells present
  
• Well defined 2cm mass, echogenic central septum
• Increased vascularity of central solid portion

7
CT Scan
8
Case Presentation Continued

• Mass excised in OR 1cm margins
• Pathology
• Biphasic synovial sarcoma with glandular tissue
  surrounded by spindle cell component
  
• Immunohistochemistry for cytokeratin and
  epithelial components
  
• Post-op XRT

9
Soft Tissue Sarcoma

• Head and neck location rare
• 10 of all soft tissue sarcomas
• 1 of all head and neck tumors
• Variable male preponderance
• Median age 50-55 yrs (15-93)
• Most patients initially evaluated for a painless
  mass (68-95)
  
• Most common primary site
• Scalp face neck oral cavity sinuses,
  larynx, orbit

10
Soft Tissue Sarcoma
11
Soft Tissue Sarcoma

• Histologic subtypes
• Malignant fibrous histiocytoma
• Angiosarcoma
• Neurogenic sarcoma (malignant schwannoma, PNST)
• Dermatofibrosarcoma protuberans
• Fibrosarcoma
• Leiomyosarcoma
• Synovial sarcoma
• Liposarcoma
• Epithelioid sarcoma
• Sarcoma NOS
• Alveolar soft part sarcoma
• Desmoid (aggressive fibromatosis) - benign

12
Soft Tissue Sarcoma

• Diagnostic evaluation
• CT and/or MRI
• PET unclear significance
• Useful when pulmonary nodules present
• FNA and/or core biopsy
• Incisional biopsy
• Soft tissue sarcomas are prone to seeding

13
Soft Tissue Sarcoma

• Regional lymph node metastases
• Presentation - 1 of 44 patients (2) Barker et
  al. U Iowa
  
• Recurrence - 3 of 60 patients (5) Kraus et al.
  MSKCC
  
• Distant metastases
• Related to histologic grade and tumor size
• Lung most common
• Chest CT and or PET CT
• Metastases to other sites unlikely
• Brain MRI, bone scan

14
AJCC 2002 TNM Definition
15
AJCC Staging
16
Soft Tissue Sarcomas
W.M. Mendenhall et al., Univ of Florida
Head and Neck, Oct 2005

• Purpose To determine the optimal treatment for
  adults with HN soft tissue sarcoma
  
• Methods Literature review
• 461 patients, follow-up 3-5 years

17
Mendenhall et al.

• Histologic subtypes

18
Mendenhall et al.

• Histologic Grade
• Maximum Diameter ()

19
Treatment

• Surgery
• Radical resection
• Compartmental resection usually not feasible
• Surgery radiotherapy
• Low grade with close (
• High grade
• Radiotherapy
• Efficacy difficult to assess
• Adjuvant chemotherapy
• Efficacy in HN unclear
• Recent extremity data shows improvement in
  disease-free survival (p.04) and overall
  survival (p.03)

20
Outcomes

• 5-year overall local control 60-70
• Surgery RT surgery RT
• In contrast to 90 for the extremity
• Distant tumor recurrence
• 10-30 experienced distant metastases

21
Local Control

• Surgery /- RT , 5-year mean follow-up
• T1 92
• T2 40
• Grade 1 to 2 80
• Grade 3 to 4 48
• Negative margins 74
• Microscopically margins 70
• Gross residual disease 25

22
Survival Outcomes

• Distant metastasis free survival
• 75 90
• Disease free survival
• 45 60
• Cause-specific survival
• 60 70
• Overall survival
• 60 70

23
Prognostic Factors

• Histologic grade
• T stage
• Margin status
• Optimal treatment is complete resection
• Adjuvant RT likely improves the probability of
  cure
  
• High-grade, close or positive margins
• Efficacy of adjuvant chemo ill defined
• Considered in high-grade lesions

Conclusion
24
Cutaneous Angiosarcoma - Outcome
3 doses doxorubicin XRT 3000 cGy in 10 frac
tions over 2 weeks
Pre-treatment
46 months post treatment
25
Chondrosarcoma

• Recognized by ACS in 1939
• Malignant, tumor cells form chondroid and not
  osteoid
  
• Tissue origin
• Bone chondroid precursors
• Cartilaginous
• Most common laryngeal sarcoma
• Soft tissue
• Cartilaginous differentiation of primitive
  mesenchymal cells
  

26
Classification

• Tumor tissue origin
• Primary vs. secondary
• Histologic based microscopic appearance
• Conventional ( NOS)
• Myxoid
• Mesenchymal
• Anatomically based
• Central intramedullary
• Peripheral cortex
• Juxtacortical periosteal or soft tissue
• Grade

27
Staging

• TNM
• Skeletal
• T1 confined within bone cortex
• T2 invade beyond bone cortex
• Extraskeletal
• Soft tissue sarcoma T staging
• GSS (general summary stage)
• L locally confined
• R regional spread
• D distant spread

28
National Cancer Database Report on Chondrosarcoma
of the Head and Neck

• Brenton Koch et al., Univ of Iowa, Head and Neck
  July 2000
  
• NCDB data on all reported head and neck
  chondrosarcomas
  
• 1985 1995

29
Results - Demographics

• 355,019 patients
• 400 (0.1) chondrosarcoma
• Median age 51 years

30
Treatment Characteristics
31

• Overall survival
• 5 year 77.6, 10 year 64.3
• Disease-specific
• 5 year 87.2, 10 year 70.6

32
P0.027

• Grade 1 - 2
• 5 year 93.2
• Grade 3 - 4
• 5 year 67.3

33

• 5 year survival by site
• Head and neck bones 92.4
• Laryngotracheal 88.0
• Sinonasal 87.5
• Soft tissue 80.2

34
P0.006

• 5 year survival by histologic subtype
• Chondro (NOS) 91.4
• Myxoid 45
• Mesenchymal 53.2

35
Recommended Treatment

• Surgical excision
• 1-2 cm margins
• Radiation no survival data
• Adjunct (23.6)
• Primary (4.0)
• Chemotherapy
• Mesenchymal chondrosarcoma (57.5)
• High grade distant mets, potential errors
• Cervical metastases
• Overall incidence 5.6
• No prophylactic neck treatment recommended

36
NCDB Chondrosarcoma Negative Prognostic Factors

• Advanced stage (III-IV)
• Higher grade
• Myxoid or mesenchymal histology

37
Osteosarcoma

• Relatively small published series
• 10 of all osteosarcomas occur in the head and
  neck
  
• Mandible maxilla paranasal sinuses skull
• In US
• 900 new cases annually
• 1 per year in large cancer centers
• Present during 3rd 4th decade

38
Classification

• Origin
• Intramedullary
• Surface (cortex)
• Extraosseous (soft tissue)
• Type
• Osteoblastic/conventional High
• Dedifferentiated - High
• Telangectatic - High
• Chondroblastic -Low
• Fibroblastic - Low

39
Staging

• Enneking Staging System
• TNM

T extent of primary Intra/extra compartmental
M nodal or distant disease
40
Major Risk Factors

• Radiation exposure
• Pagets disease
• Skull
• Other - fibrous dysplasia, chronic osteomyelitis,
  myositis ossificans, trauma
  
• Survivors of childhood retinoblastoma
• XRT
• Genetic disposition
• p53 and Rb mutation

41
Metastatic Risks

• Low grade
• Low likelihood of metastases
• High grade
• Propensity to metastasize
• Lung
• Cervical LN
• Brain
• Other bones

42
Effectiveness of Chemotherapy

• Efficacy of long bone osteosarcoma neoadjuvant
  chemo established
  
• Completeness of histologic response ( less than
  5-10 tumor viability at surgical resection)
  correlated to improved survival
  
• Chemoresistance
• p-glycoprotein (p170) multi-drug resistance gene
• Head and neck survival benefits not yet
  established
  
• Neoadjuvant and adjuvant chemo commonly employed

43
Effectiveness of Chemotherapy
Smeele et al., Effect of Chemotherapy on Survival
of Craniofacial Osteosarcoma J Clin Oncol 1996

• 201 patients, 1974 1994
• Analysis stratified for use or non-use of
  chemotherapy
  
• Conclusion
• chemotherapy improves survival in craniofacial
  osteosarcoma
  
• advocated long bone protocols for use in the head
  and neck

44
University of Washington Experience
Oda, Weymuller et al., Head and Neck, 1997

• 13 patients from 1981 1996
• 8 men, 5 women
• Median age 40.9 years (2275 years)
• Mean follow-up 58 months

45
Histology, Grade and Location
46
Treatment

• High grade
• Neoadjuvant chemotherapy (3 cycles)
• Doxorubicin and cisplatin
• Surgical resection
• Adjuvant chemotherapy
• Low grade
• Surgical excision, /- chemo
• Radiation therapy - radioresistance
• Positive margins
• Unresectibility

47
Cumulative Overall Survival

• 5-year survival 72
• 3/13 (23) patients had local recurrence
• 2 of the 3 deaths

48
Chemotherapy Results

• One patient chemoresistant
• Two patients 90 tumor necrosis
• No development of metastatic disease

49
University of Washington Conclusion

• Head and Neck osteosarcoma is more similar than
  dissimilar to long bone osteosarcoma in terms of
  response to treatment modalities
  
• Surgical resection with clear margins provided
  the best chance for survival
  
• Neoadjuvant chemo is of particular value for high
  grade histology

50
Head and Neck SarcomaSummary

• Surgical resection is essential for local
  control
  
• Soft tissue or bone sarcoma
• Negative margins improve local control
• Postoperative radiation
• Low grade lesions with close or margins
• All intermediate and high grade lesions
• 40 70 Gy, external beam, brachy, or both
• Role of Chemotherapy unclear
• Improved tumor response in osteosarcoma
• Consider for high grade lesions or when
  metastases are present

51
Head and Neck SarcomaSummary

• Management of the Neck
• Elective neck dissection is NOT indicated if the
  neck is not clinically involved
  
• Overall, only 8-10 of sarcomas metastasize to
  nodes.
  
• Although certain sarcomas have a higher
  propensity for nodal metastases such as malignant
  fibrous histiocytoma, and synovial sarcomas, a
  neck dissection would not be warranted unless
  nodes are clinically or radiologically involved.
  
• Neck irradiation would have similar
  considerations.

American HN Society HN Sarcoma treatment
recommendations