Adult Head and Neck Sarcoma
Grand Rounds SIU Otolaryngology
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.
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
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
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
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
CT Scan
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
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
Soft Tissue Sarcoma
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
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
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…
AJCC 2002 TNM Definition
AJCC Staging
STAGE GROUPING
Stage IA Stage IB Stage IIA Stage IIB Stage III Stage IV or or Low T1a T1b T2a T2b T1a T1b T2a T2b Any T Any T NO N0 N0 N0 N0 N0 N0 N0 N1 Any N NX NX NX NX NX NX NX NX M0 M0 M0 M0 M0 M0 M0 M0 M0 M1 G1-2 G1-2 G1-2 G1-2 G3-4 G3-4 G3-4 G3-4 Any G Low Low Low Low High High High High High Low Any G Any G High G1 G1 G1 G1 G2-3 G2-3 G2-3 G2-3 Any G
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 H&N soft tissue sarcoma Methods: Literature review 461 patients, follow-up 3-5 years
MSKCC PMH
19821989
N=60
MGH U. Iowa TMH
19721993
N=57
UCSF U. Illinois
19611993
N=65
NCI
19702000
N=44
19811995
N=72
19801988
N=52
1969-1983 19631993
N=53 N=58
Mendenhall et al.
Histologic subtypes
Mendenhall et al.
Histologic Grade
Maximum Diameter (%)
Treatment
Surgery
– Radical resection
• Compartmental resection usually not feasible
Surgery + radiotherapy
– Low grade with close (<1cm) or + margins – High grade
Radiotherapy
– Efficacy difficult to assess
Adjuvant chemotherapy
– Efficacy in H&N unclear – Recent extremity data shows improvement in disease-free survival (p=.04) and overall survival (p=.03)
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
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%
Survival Outcomes
Distant metastasis free survival
– 75 – 90%
Disease free survival
– 45 – 60%
Cause-specific survival
– 60 – 70%
Overall survival
– 60 – 70%
Prognostic Factors
Histologic grade T stage Margin status
Conclusion
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
Cutaneous Angiosarcoma - Outcome
• 3 doses doxorubicin
• XRT – 3000 cGy in 10 fractions over 2 weeks
Pre-treatment
46 months post treatment
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
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
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
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
Results - Demographics
355,019 patients 400 (0.1%) chondrosarcoma Median age 51 years
Treatment Characteristics
Overall survival
– 5 year 77.6%, 10 year 64.3%
Disease-specific
– 5 year 87.2%, 10 year 70.6%
P=0.027
Grade 1 - 2
– 5 year 93.2%
Grade 3 - 4
– 5 year 67.3%
5 year survival by site
– – – – Head and neck bones – 92.4% Laryngotracheal – 88.0% Sinonasal – 87.5% Soft tissue – 80.2%
P=0.006
5 year survival by histologic subtype
– Chondro (NOS) – 91.4% – Myxoid – 45% – Mesenchymal – 53.2%
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
NCDB Chondrosarcoma Negative Prognostic Factors
Advanced stage (III-IV) Higher grade Myxoid or mesenchymal histology
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
Classification
Origin:
– Intramedullary – Surface (cortex) – Extraosseous (soft tissue)
Type:
– – – – – Osteoblastic/conventional – High Dedifferentiated - High Telangectatic - High Chondroblastic -Low Fibroblastic - Low
Staging
Enneking Staging System
Stage
IA
Grade
G1
Tumor
T1
Mets
M0
T: extent of primary
Intra/extra compartmental M: nodal or distant disease
IB IIA
IIB IIIA IIIB
G1 G2
G2 G1 or G2 G1 or G2
T2 T1
T2 T1 T2
M0 M0
M0 M1 M1
TNM
Major Risk Factors
Radiation exposure Paget’s disease
– Skull
Other - fibrous dysplasia, chronic osteomyelitis, myositis ossificans, trauma Survivors of childhood retinoblastoma
– XRT – Genetic disposition
• p53 and Rb mutation
Metastatic Risks
Low grade
– Low likelihood of metastases
High grade
– Propensity to metastasize
• • • • Lung Cervical LN Brain Other bones
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
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
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 (22–75 years) Mean follow-up 58 months
Histology, Grade and Location
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
Cumulative Overall Survival
5-year survival 72% 3/13 (23%) patients had local recurrence
– 2 of the 3 deaths
Chemotherapy Results
One patient chemoresistant Two patients > 90% tumor necrosis No development of metastatic disease
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
Head and Neck Sarcoma Summary
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
Head and Neck Sarcoma Summary
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 H&N Society; H&N Sarcoma treatment recommendations