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