Adenoid Cystic Carcinoma Minor Salivary Gland Origin

Transcription

1 Adenoid Cystic Carcinoma Minor Salivary Gland Origin Educational Session Presenter: Smith JA Supervisors: Palme CE, Gupta R

2 Content Case report Imaging Primary Therapy Surgery Adjuvant Therapy Radiotherapy Pathology

3 Case report 56y female patient Persistent left otalgia No otological explanation Airway NAD Swallowing NAD

4 Social history Non-smoker Professional voice user Past medical history Case Report O/E Nasal polyp Clinically no other lymphadenopathy Mass incidentally seen left arytenoid Micro and biopsy performed at another site

5 MRI

6 BIOPSY Biphasic neoplasm ->epithelial and myoepithelial cells Cribriform architecture Nests with lumens containing matrix

7 Biphasic salivary gland neoplasms -Adenoid cystic carcinoma -Epithelial-myoepithelial carcinoma

8 Cribriform architecture Favoured: ADENOID CYSTIC CARCINOMA

9 FLUORESCENT IN SITU HYBRIDISATION (FISH) Approximately 50% of adenoid cystic carcinomas will have a MYB-NFIB translocation

10 Fish no myb rearrangement detected

11 FDG-PET summary Primary tumour left arytenoid FDG avid positive node left neck No evidence of distant disease => What next?

12 FNA left neck level 4 & 5 Blood and occasional lymphocytes No malignant cells identified

13 FDG-PET and AdCC 18F-FDG PET-CT is not helpful to rule out distant metastasis if the primary SGC does not show enhanced FDG uptake AdCC with relatively low FDG uptake might be obscured by the normal physiologic FDG uptake of the salivary glands; Salivary glands are frequently affected by inflammatory processes wherein increased FDG uptake might result in a false-positive result. J.L. Roh, C.H. Ryu, S.H. Choi, et al.clinical utility of 18F-FDG PET for patients with salivary gland malignancies. J Nucl Med, 48 (2007), pp

14 Staging As per major salivary gland tumours T1 = <2cm, T2 = 2-4cm, T3 >4cm T4a skin, bone T4b SB, pterygoid plates, carotid N1, N2a, N2b, N2c, N3 M0/M1 T1, N0, M0

15 Treatment of the primary? Principles? Resection Reconstruction Options?

16 Treatment options: ablation Primary site Surgery Transoral laser/robotic resection Cordectomy via laryngofissure Partial laryngectomy Vertical Supra-cricoid Total laryngectomy RT/CRT

17 Non-surgical primary treatment Surgery with RT vs. RT alone Combined modality improved local control and survival 5 and 10 year local recurrence 56 and 43% RT alone 94 and 91% Surgery and RT 5 and 10 year survival 57 and 42% RT alone 77 and 65% Surgery and RT W.M. Mendenhall, C.G. Morris, R.J. Amdur, J.W. Werning, R.W. Hinerman, D.B. VillaretRadiotherapy alone or combined with surgery for adenoid cystic carcinoma of the head and neck. Head Neck, 26 (2004), pp

18 Treatment of the neck Nil Surgery Further staging information RT CRT Occult nodal disease in approx. 15% elective neck dissection?improves local control Patients do not die of nodal disease S.Y. Lee, B.H. Kim, E.C. Choi. Nineteen-year oncologic outcomes and the benefit of elective neck dissection in salivary gland adenoid cystic carcinoma. Head Neck, 36 (2014), pp

19 Reconstruction options Aims: restoration of function Options Mucosal free graft False cord advancement/pull-down Bi pedicled muscle/perichondrial flap Skin or muscular free flap Muscular ligamentous/cartilagenous free flap Laser reduction or injection thyroplasty

20 False cord advancement/pull-down

21 Bi-pedicled muscle flap

22 Free tissue transfer

23 Intra-operative: Ablation

24 Specimen

25 Intra-operative: Reconstruction

26 Post Operative Day 7

27

28 Pale circumscribed lesion 15x6x6MM

29 -WELL DEMARCATED, UNENCAPSULATDED -predominantly cribriform architecture

30 -PERINEURAL INVASION -EXTENSION INTO SUBMUCOSAL FAT

31 - Adenoid cystic carcinoma 15mm - Margins clear (closest-> deep margin= 3mm) - Perineural invasion present - No lymphovascular invasion - Lymph nodes 0/22 - Stage I (pt1n0)

32 Adenoid cystic carcinoma Biphasic tumour- epithelial & myoepithelial cells Lumens filled with hyaline or basophilic mucoid material Architecture can be cribriform, tubular of solid > 30% solid -> more aggressive course 10% of epithelial salivary neoplasms Most frequently parotid, submandibular and minor salivary glands

33 Adenoid cystic carcinoma 5 year survival approximately 35% 80-90% die of disease in years Local recurrence 16-85% Lymph node involvement 5-25% Distant metastasis 25-55% (bone, brain, liver) Perineural invasion is a common feature Other prognostic factors include tumour site, clinical stage, bone involvement and status of margins.

34 Post operative recovery Covering tracheostomy and laryngeal stent Both removed prior to discharge Discharged 3/52 Tolerating diet and fluids Initial degree of aspiration Breathy voice good cough

35 Summary so far T1 N0 M0 adenoid cystic Ca supra-glottis Completely excised Local peri-neural invasion

36 Patterns of recurrence Between 60% - 100% of patients recur at the primary site 30yrs FU 10 and 20 year survival: 65% and 28% Disease is incurable in the long term Death is from distant disease Lung mets common site 32 months from diagnosis to death Targeted molecular therapies not shown to be of benefit L. Ciccolallo, L. Licitra, G. Cantú, G. Gatta. EUROCARE Working Group. Survival from salivary glands adenoid cystic carcinoma in European populations. Oral Oncol, 45 (2009), pp J. Van der Wal, A.G. Becking, G.B. Snow, et al.distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during follow-up. Head Neck, 24 (2002), pp A.S. Jones, J.W. Hamilton, H. Rowley, D. Husband, T.R. Helliwell. Adenoid cystic carcinoma of the head and neck. Clin Otolaryngol Allied Sci, 22 (1997), pp