Maxillary Lump
Presenters:
Dr James Badlani (H&N Fellow)
Dr Edward Roper (Pathology Registrar)
Expert Panel:
Prof Martin Tattersall (Medical Oncologist)
Dr Anthony Clifford (H&N Surgeon)
Dr Puma Sundaresan (Radiation Oncologist)
Dr Richard Boyle (Orthopaedic Oncological Surgeon)
Dr Annabelle Mahar (Sarcoma Pathologist)
Chair:
Dr Hubert Low
• 51 year old female presents to her dentist with a sudden onset of swelling and pain in top right jaw.
• Med Hx:
Anxiety (Sertraline)
Non-smoker
Occasional consumption of alcohol
• Family Hx: Mother had breast Ca.
19 October 2015
26 October 2015
What is the best way to obtain a tissue diagnosis?
Histopathology- curettage 4/11/15
• Diagnosis
Osteosarcoma
• Malignancy of mesenchymal cells that have the ability to produce osteoid or immature bone.
• Rare.
120 cases of 1° malignant bone tumour in Australia in 2012. Most of which were Osteosarcoma1.
• 6-8% of all osteosarcomas occur in the jaws2,3,4.
Osteosarcoma
Extragnathic Osteosarcoma
• Bimodal (1st-2nd decade & >6th decade)3.
• Young- metaphysis of femur and tibia. Older- axial skeleton, flat bones. Associations- Pagets and RTx.
• > 90% Metastasize3
• >80% High grade5
Osteosarcoma of the jaws
• 3rd-4th decade2,3,4.
• Mandible=Maxilla v Mandible>Maxilla
• 17-21% Metastasize3,4
• <20-50% high grade.
Presentation
• Swelling
• Pain
• Nasal obstruction
• Epistaxis
• CN V disturbance
• Trismus
Referred to Dr Clifford- 25/11/15.
Any further invetigations to stage this patient?
PET scan
AJCC TNM Staging System for Bone 2010
AJCC Staging
Staging
• TNM- T1N0M0 G2= Stage IB.
• SSS- IIB
NCCN Guidelines
NCCN Guidelines
Surgery
Margins
• Long bone: >1-4cm v 5cm(6).
• Jaws: 0.5cm(4,7)-3cm(8)
RECONSTRUCTION
OPTIONS TO CLOSE DEFECT:
1. Obturator
2. Locoregional flap
3. Free flap
-Osteomyofasciocutaneous
-Myofasciocuatenous
-Myofascial
-Fasciocutaneous
DENTAL REHABILITATION:
1. Obturator
2. Partial denture
3. Implants.
Histopathology
High grade
– Osteoblastic
– Chondroblastic
– Fibroblastic
– Small cell
Low grade
– Fibrous Dysplasia-like
Prognosis
Prognostic factors
Positive Prognostic factor
• CLEAR SURGICAL MARGINS
Negative Prognostic factors:
• Size >4-6cm(2,10)
• Age >60 years2
• High grade2,10
• Poor response to neoadjuvant chemotherapy10
Clear margins
Benefit with neo/adjuvant treatment
• Clinical, therapeutic and prognostic features of osteosarcoma of the jaws e Experience of 36 cases. Kämmerer et al 2012.
• Smeele L et al 199711- Improved survival of adjuvant chemotherapy irrespective of completeness of surgical margins.
Neo/adjuvant Chemotherapy
• >90% tumour response to neoadjuvant chemotherapy- good response/predictive for survival.
• Poor responders (>10% viable tumour)- switch adjuvant chemo agents (limited success) v adjuvant Radiotherapy.
Neo/adjuvant Chemotherapy
Adjuvant Radiotherapy
Surveillance
1. http://www.cancer.org.au/about-cancer/types-of-cancer/bone-cancer.html
2. Smith RB, Apostolakis LW, Karnell LH, Koch BB, Robinson RA, Zhen W, et al. National cancer data base report on osteosarcoma of the head and neck. Cancer 2003;98:1670–80.
3. Baumhoer D Et al. Osteosarcomas of the jaws differ from their peripheral counterparts and require a distinct treatment approach. Experiences from the DOESAK Registry. Oral Oncology 2014;50:147-153.
4. Kämmerer PW et al. Clinical, therapeutic and prognostic features of osteosarcoma of the jaws e Experience of 36 cases. Journal of Cranio-Maxillofacial Surgery 201;40:541-548.
5. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®). Bone cancer version 2.2016.. http://www.nccn.org.
6. Enneking WF, Spanier SS, Goodman MA: A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop 1980:153:106-120.
7. Kawaguchi N et al. The Concept of Curative Margin in Surgery for Bone and Soft Tissue Sarcoma .Clinic Orthop 2004;419:165-172.
8. Granowski-Lecornu M et al. Osteosarcoma of the Jaws: Factors Influencing Prognosis. J Oral Maxillofac Surg 69:2368-2375, 2011
9. Fernandes R et al. Osteogenic Sarcoma of the Jaw: A 10-Year Experience. J Oral Maxillofac Surg 65:1286-1291, 2007
10. Patel SG et al. Improved Outcomes in Patients with Osteogenic Sarcoma of the Head and Neck. Cancer 2002;95:1495-1503.
11. Smeele LE, Kostense PJ, van der Waal I, Snow GB. Effect of chemotherapy on survival of craniofacial osteosarcoma: a sys- tematic review of 201 patients. J Clin Oncol. 1997;15:363–367.
12. Guaddagnolo BA. Osteosarcoma of the Jaw/Craniofacial Region. Cancer 2009