Downloaded 54 times
More Related Content
Ameloblastoma
- 1.
- 2. History 38 yearold male No addictions, no comordities No family h/o malignancy Presented with complaints of growth on the Right side of the hard palate and upper alveolus in 2013 He was evaluated under ENT, AIIMS Underwent infrastructural maxillectomy in September 2013
- 3. Post-op HPE-> Patient was put on follow up under ENT • 3x2x2 cm tumor in the maxilla, lies • 0.5 cm from anterior margin • 0.4 cm from posterior • 0.3 cm from medial • 0.4 cm from lateral resected margin • s/o Plexiform ameloblastoma, involving the bone • All margins free
- 4. Presenting complaints Patientnoticed a mass in the post-op cavity and right upper jaw since September 2015 (DFS of 2 years) Associated with a swelling over the right cheek Not associated with pain or reduced vision
- 5. Evaluation CT Face(15/10/15) -> Enhancing soft tissue in Right GBS with contiguous thickening of posterior buccal mucosa and soft tissue mass in the Rt infratemporal fossa ; Soft tissue thickening in the right sphenoid sinus with erosion and bony defects in floor and superolateral wall with minimal intracranial extraxial extension continuous with soft tissue in ITF inferiorly
- 6. Irregular enhancing massin the rt nasal cavity Soft tissue mass in the sphenoid sinus with expansion and destruction of adj bone
- 7. Mass in thert sphenoid sinus extending intracranially as an extraxial mass
- 8. MRI Face (24/11/15) Soft tissue lesions with lobulated outline in right spenoid sinus, Rt ITF, Rt nasal fossa
- 9. T2 bright mass seenin the Rt nasal cavity T2 bright mass in the Rt posterior etmoidal sinus extending inferiorly into Rt ITF with broad area of contact with ICA, anteriorly in close relation to optic canal
- 11. Surgery Right totalmaxillectomy + infratemporal fossa clearance + endoscopic sphenoid sinus clearance on 9/12/15 in ENT, AIIMS Intra-op Findings -> 4x2 cm firm tumor present close to condyle of mandible, upper alveolus and floor of sinus - 3x2 cm tumor seen in the roof of the maxillary sinus extending into the sphenoid sinus - 4 x 3 cm soft tissue in sphenoid sinus with attachment to superior, lateral and anterior wall - 1 x 1 cm soft tissue tumor mass extending into lateral pterygoid muscle
- 12. Post-op HPE 4x 3.6 x 1.8 cm tumor arising from the wall of the maxilla and extending into soft tissue of ITF Closest margin is posterior – 0.1 cm s/o ameloblastoma, follicular variant Extensive soft tissue infiltration into adipose tissue and skeletal muscle All margins free Sphenoid tumor specimen – similar morphology
- 13. Diagnosis Recurrent Ameloblastoma Referred for post-operative Radiotherapy ( i/v/o recurrent disease and close margins)
- 14. Plan Post-op Radiotherapy50 Gy/25#/5 weeks Planning CT with contrast done
- 15.
- 16.
- 17. Beam arrangement IMRTwith 7 equally spaced co-planar beams
- 18. Dose colour wash(47.5 Gy)
- 19.
- 20. Volume ofPTV receiving 95% dose : 92.12% VPTV 107% - 0.47% VPTV 115% - 0% CI – 1.02 HI – 1.33
- 21. OARs Constraints Achieved Spinalcord D max < 45 Gy 25.68 Gy Brainstem D max < 54 Gy 47.21 Gy Optic nerve D max < 54 Gy Lt – 49.11 Gy ; Rt – 50.01 Gy Optic Chiasma D max < 54 Gy 49.56 Gy Lt eye D max < 45 Gy 34.64 Gy Rt eye D max < 45 Gy 47.33 Gy Temporal Lobe D max < 65 Gy Lt – 50.92 Gy ; Rt – 50.99 Gy Parotid D mean < 26 Gy Lt – 11.77 Gy ; Rt – 6.37 Gy Cochlea D max < 45 Gy Lt – 30.02 Gy ; Rt – 34.7 Gy Lacrimal gland D mean < 30 Gy Lt – 13.13 Gy ; Rt – 17.58 Gy Lens D max < 10 Gy Lt – 9.02 Gy ; Rt – 8.95 Gy
- 22.
- 23. 20% 80% Amel (enamel) Blastos(germ) Ameloblastoma Different names over time -> cystosarcoma -> adamantine epithelioma -> adamantinoma -> finally ameloblatoma First reported by Cusack in 1827
- 24. Benign, slowgrowing, but locally invasive tumor of odontogenic origin Global incidence – 0.5 cases/ million-person years………. Quite a rare disease! Most cases – 30-60 years age group African Americans are at higher risk Usually originate within the bone (except for the peripheral subtype)
- 25. Presenting features Painlessswelling ( m.c.) Pain due to haemorrhage Paraesthesias due to PNI Tooth displacement Root resorption Unerupted 3rd molar
- 26. Diagnostic evaluation Pantomography CTMRI Honey comb
- 27. Imaging featuresare characteristic, but not pathognomonic Diagnosis is established by HISTOLOGY
- 28. Histopathology Resembles normalenamel epithelium May arise from -enamel cell rests -Cells of the sheet of Hertwig’s -Epithelial boundary of an odontogenic cyst -Basal cells of the buccal mucosa
- 29. Biological classification (WHO,2005) Solid/multicystic (91%) Unicystic (6%) Extra-osseous (2%) (peripheral) Desmoplastic (1%)
- 30. Microscopic patterns follicular plexiform acanthomatous spindle basal cell-like desmoplastic granular cell Clinical significance????
- 31. Malignant subtypes (notincluded in WHO) Metastatic amelobastoma Defined by a distant focus Metastases to lung, bone, liver, brain Ameloblastic carcinoma Defined by malignant histology
- 32.
- 33. 3 studies onmutational analysis published in 2014 – Kurppa et al, Sweeney et al and Brown et al Mc Clary et al; March 2015
- 34.
- 35. • Lack ofa proper staging system • Disease rarity • Absence of prospective studies • Development of recurrences after a long time Difficult to compare treatments
- 36.
- 37. Conservative Enucleation/curettage ofbony cavity OPD procedure High recurrence rate (65-95%) Radical En bloc resection with 1-2 cm margins Low recurrence rate (5-15%) Current standard of care
- 39. Radiotherapy Rarity ofthe tumor and Sx as primary modality of Rx -> data on outcome after RT is scarce Initial reports – So, these tumors were initially considered to be radioresistant due to poor outcomes in older series • MSKCC,1974 ( n=11) -> recurrence rate -100% • Robinson et al,1937 (n=18) -> recurrence rate – 72% But, most of these studies used orthovoltage radiation and radium needles instead of MV radiation
- 40. 10 ptswith ameloblastoma Used megavoltage radiation RT alone – 7 Sx and RT – 3 Recurrence rate – 20% Recommendation : 45 Gy over 4 weeks Concluded that ameloblastoma was not radioresistant Cancer 53:869-873, 1984
- 41. 41 ptswith ameloblastoma of jaw 8 pts underwent RT; 2 had progressive disease Conclusion : for multiple recurrences RT was effective and should be give post operatively in selected cases 1995;121(9):994-996
- 42.
- 43. RT can begiven…… Post-operatively in pts with gross/microscopic residual disease and recurrent disease, in pts who are poor candidates for surgery
- 44. Chemotherapy May besensitive to platinum agents May have a role in improvement of symptoms in non-surgical patients
- 45. Future directions Agentstargeting BRAF and SHH pathways BRAF inhibition by vemurafenib/dabrafenib/trametinib SHH inhibition by arsenic trioxide
- 46.