lesions of odontogenic epitheliuM PRESENTATION

ODONTOGENIC LESIONS

1. LESIONS OF ODONTOGENIC EPITHELIUM PRESENTER: DR. RAVI GAUTAM MODERATOR: DR. VIJAYALAXMI PATIL 1

2. Development and histology of tooth & gingiva Classification Odontogenic cysts- developmental and inflammatory origin Benign odontogenic tumors – Epithelial, mixed and mesenchymal origin Malignant odontogenic tumors Approach Recent advances Summary 2

3. Although all intraoral epithelium is of a stratified squamous type , there are regional histologic variations, such as nonkeratinized, para keratinized, and orthokeratinized DEVELOPMENT AND HISTOLOGY OF TOOTH & GINGIVA 3

4. The initial formation of the tooth begins with a down growth of the oral epithelium (dental lamina) into the ectomesenchyme of the jaws. The tooth bud is organised into three parts: the enamel organ, the dental papilla, and the dental follicle. 4

5. The cells of the enamel organ give rise to ameloblasts , which produce enamel. The cells of the dental papilla give rise to odontoblasts, dentin. 5

6. 6

7. 7

8. Once tooth formation is complete, remnants of the epithelium involved in tooth formation persist indefinitely in the tooth-bearing areas of the jaws and gingiva. They are the sources of odontogenic cysts and most odontogenic tumors 8

9. Odontogenic rests are typically round or strand like in form . Rests are distinguished by their bland cytology lack of ameloblastic differentiation of peripheral columnar cells with nuclear polarization and central stellate reticulum–like zones 9

10. WHO CLASSIFICATION (2017) 10

11. DENTIGEROUS CYST 2 nd most common odontogenic cyst of jaw AGE : 50 yrs GENDER : males SITE : associated with unerupted mandibular teeth Signs & symptoms Usually asymptomatic Bone expansion, pain & swelling RADIOLOGICAL FEATURES CENTRAL TYPE: LATERAL TYPE : CIRCUMFERENTIAL TYPE : 11

12. 12

13. ODONTOGENIC KERATOCYST Comprise 10 % of cysts of jaw Types: solitary /primordial (90%) multiple (10%) Multiple are a/w Nevoid BCC syndrome ( Gorlin syndrome) AGE: Second and third decades. GENDER: M=F SITE : Mandible Pain, swelling or discharge (more than that of dentigerous cyst) 13

14. GORLIN-GOLTZ Syndrome RADIOGRAPHIC FEATURES 14

15. 15

16. ERUPTION CYST (ERUPTION HEMATOMA) Subtype of dentigerous cyst In a/w erupting primary or deciduous teeth AGE : children SITE: first permanent molars (mandibular) and the maxillary incisors Typical C/F: bluish compressible dome shaped expansion of alveolar ridge over the erupting tooth. 16

17. Radiological features 17

18. GINGIVAL CYST OF NEW BORNS Bohn nodules Congenital odontogenic cyst Arise from dental lamina remnants in gingiva overlying edentulous ridge 1-3 mm whitish or yellow form nodules /papules (keratin accumulation) Usually multiple 18

19. 19

20. GINGIVAL CYST OF ADULTS AGE : 5 th – 6 th decade of life SITE : mandibular pre molar & incisor- canine region Signs and symptoms : Small, slowly enlarging, well circumscribed painless swelling. 20

21. 21

22. LATERAL PERIODONTAL CYST Arise from cystic remnants of dental lamina Age : 20 – 60 years Sex : Male Site : Lateral PDL regions of mandibular premolar- canine region SIGNS & SYMPTOMS Usually asymptomatic as it occurs on the lateral aspect of root of tooth. 22

23. 23

24. CALCIFYING ODONTOGENIC CYST Age : 4 th - 5 th decade Sex : Equal. Site : Mandibular pre molar- molar area in a/w embedded tooth or gingiva Painless enlargement of mandible/maxilla 24

25. 25

26. Q. A 30 year male on routine dental examination was found to have cystic swelling in the m axillary anterior region. On radiograph, it is radiolucent lesion associated with the non vital tooth. What is the diagnosis ? 26

27. RADICULAR CYST Results from severe pulpal inflammation, leading to pulp necrosis & periapical inflammation Quite often a radicular cyst remains behind in the jaws after removal of the offending tooth and this is referred to as a residual cyst . Age : 3 rd - 4 th decades Sex : Males. Site : Maxillary anterior region (incisors) Frequency: Commonest cystic lesion of jaws. 27

28. Primarily symptom less. Slowly enlarging hard bony swelling initially. Diagnostic criteria – associated teeth are non vital 28

29. 29

30. GLANDULAR ODONTOGENIC CYST Site: anterior mandible Radiography: multiple radiolucencies 30

31. Q. A 40 year old male presented with swelling of the mandible associated with pain and paresthesia . Radiograph showed soap bubble appearance. Macroscopy shows both solid and cystic areas. On IHC, it shows positivity for CK- 5, 14, 8, 18, 19 and calretinin. What’s the diagnosis ? 31

32. AMELOBLASTOMA Most common epithelial odontogenic tumor Comprise 1 % of tumors & cysts arising from jaw benign locally aggressive odontogenic tumor arises from odontogenic epithelium 32

33. Ameloblastoma Clinical feature : 30-50 years Male=female 80% in mandible Usually asymptomatic . Soap bubbles appearance 33

34. Types: conventional (solid & multicystic )- M.C. type Unicystic ameloblastoma Peripheral ameloblastoma 34

35. 35

36. . Histopathology: Usually there are many histological variants of ameloblastoma. ≥ 2 subtypes can coexist within a tumor Histological criteria for diagnosis : well differentiated epithelium minimal/ no atypia few mitoses nests, strands, plexiform networks Cyst linings with peripheral palisaded columnar cells dark stained basal nuclei polarized away from basement membrane vacuolated basilar cytoplasm edematous spindle – stellate reticulum like region budding of epithelium from nests Stroma is fibrous without any inductive mesenchymal effect 36

37. Variants of ameloblastoma Follicular ameloblastoma Plexiform ameloblastoma Acanthomatous ameloblastoma Granular cell ameloblastoma 37

38. Variants of ameloblastoma Basal cell ameloblastoma. trabecular pattern of growth with little evidence of palisading at the periphery. They have been mistakes with basal cell carcinoma Desmoplastic ameloblastoma: 38

39. Unicystic ameloblastoma: Age: 2 nd decade Mandible . the cyst enclose the crown of impacted tooth. Radio: present as unilocular cyst 39

40. 40 Three subtypes of unicystic ameloblastoma are recognized

41. Peripheral ameloblastoma Vascular/Hemangiomatous Ameloblastoma Keratoameloblastoma (KA) /Papilliferous KA Mucous cell Malignant variations of ameloblastoma 41

42. Adenomatoid odontogenic tumor 3-7% of all odontogenic tumors Arise from odontogenic epithelium of dental lamina or its remnants Age: 2 nd decade Female 65% in the maxilla 75% associated with impacted teeth 42

43. Gross: Round Predominantly cystic with focal solid areas Not invasive Don’t recur Expanding tumor 43

44. Q. A 40 year old male presented with the slowly growing painless mass in the mandible associated with the crown of the unerupted teeth. Radiograph showed radiolucent area with fine flecks of radio-opacities. 44

45. Calcifying epithelial odontogenic tumor Benign cystic neoplasm locally aggressive tumor originated from the rest of dental lamina " Pindborg" tumor. Age: 40 -50 years Sex: M=F Mandible (crown of the unerupted teeth) slowly growing painless mass 45

46. Squamous odontogenic tumor Benign Rare tumour arise from neoplastic transformation of rest of Malassez 2 nd -7 th decade Mandible and maxilla in equal frequency Painless swelling or as loosening of teeth 46

47. Squamous odontogenic tumors 47

48. Ameloblastic fibroma (young age, cuboidal epithelium, lacking stellate reticulum) Rare biphasic tumor (the epithelial and mesenchymal components are part of the neoplastic process) Resembles dental papillae Clinical feature: Age: <21y 70% in mandible (over an unerupted tooth) Commonly mistaken with ameloblastoma 48

49. Primordial odontogenic tumor POT is a mixed odontogenic tumor Young age Mandible, well-circumscribed pericoronal Radiolucent Composed of variable cellular loose fibrous tissue, entirely surrounded by cuboidal-to-columnar epithelium 49

50. ODONTOMAS Odontogenic tumor with production of calcified part of tooth Site: alveolar ridge (mandible/maxilla), middle ear Compound Complex 50

51. ODONTOMAS Complex Compound Ameloblastic Poorly differentiated lesion with Variety of calcified pattern but Not enough coordinated production of enamel, dentin or cementum to reach a point where an actual tooth can be identified Higher degree of differentiation than do complex odontoma Prominent epithelial component resembling ameloblastoma + dental hard & soft tissues, such as enamel & dentin Sex: F>M Masses of misshapen teeth known as denticles, identified Solid/cystic Benign tumor Extremely benign Though benign, but occasionally, aggressive Site: more in molar region of mandible Site: anterior region of jaw (maxilla > mandible) Currently, regarded as form of immature complex odontoma Incidental finding usually 51

52. ODONTOMAS Compound Complex 52

53. ODONTOMAS Compound Complex 53

54. DENTINOGENIC GHOST CELL TUMOR Benign tumor Both intra & extra- osseous (more common) Bony expansion presenting as slow growing firm swelling Intra-osseous Extra-osseous Age ( yrs ) 47 57 Sex M>F M=F Site Mandible>Maxilla Mandible > Maxilla Radiography Radiolucent, unilocular/ multilocular, well defined/ ill defined Conspicuous bowl shaped defect with well defined smooth border 54

55. 55

56. . Q. A 40 year old female presented with swelling in the in the maxilla anterior to the first molar. Radiograph showed multilocular radiolucency, associated with the crown of an unerupted toot. On gross, it is solid. What is the diagnosis? 56

57. Odontogenic fibroma Benign neoplasm Derived from connective tissue of odontogenic origin Age range - 9-80 years Female 60 % in the maxilla Localized bony expansion or with the loosening of adjacent teeth Widely scattered islands and strands of embryonic odontogenic epithelium and calcification. 57

58. Odontogenic myxoma Locally aggressive intraosseous lesion derived from dental papilla Often related to malformed or missing tooth 20-30 y M=F Mandible Slow growing painless, fusiform swelling that some time displace teeth 58

59. Odontogenic myxoma . 59

60. Cementoblastoma 2 nd and 3 rd decade. Pain is the diagnostic feature Mandible > maxilla . 60

61. Cemento- ossifying fibroma Arises from mesenchyma of periodontal ligament Sino nasal tract (ethmoidal sinus, supra-orbital frontal region Age: 1 st and 2 nd decade Sex: M=F Clinical features: Facial swelling, nasal obstruction, pain, sinusitis, headache & proptosis Radiography: lytic/ mixed lytic & radio-opaque osseous or soft tissue mass 61

62. Malignant Ameloblastoma and Ameloblastic Carcinoma Less than 1 % of the ameloblastomas show malignant behavior with the development of metastases. Malignant ameloblastoma is a tumor that shows histologic features of the typical ( benign ) ameloblastoma in both the primary and secondary deposits . Ameloblastic carcinoma : Microscopic features of ameloblastoma + malignant morphologic features such as lack of differentiation, hyper cellularity, nuclear atypia, increased mitotic figures, vascular & neural invasion 62

63. Odontogenic sarcoma (Ameloblastic fibrosarcoma / odontogenic fibrosarcoma) Presents with painful swelling Malignant counterpart of the ameloblastic fibroma in which the mesenchymal portion shows features of malignancy. Aggressive tumor Local recurrence > mets . 63

64. Sclerosing odontogenic Ca A recently described variant Small nests with thin cords of small polygonal epithelial cells (occasionally clear cells), embedded in abundant sclerohyaline stroma Often exhibits skeletal muscle & neural differentiation 64

65. Clear cell odontogenic ca Rare epithelial lesion of jaw of odontogenic origin. It simulates clear cell ca of salivary gland type of metastatic origin 65

66. Ghost cell odontogenic ca Rare malignancy Maxilla Can be solid & cystic Recurrent Metastatic 66

67. Primary intraosseous carcinoma, NOS Squamous cell carcinoma arising within bone from odontogenic epithelial nests a neoplasm arising from an odontogenic cyst or a metastasis M>F Mandibular molar area The diagnosis is made after exclusion of possible origin from a mucosal neoplasm invading bone 67

68. Odontogenic carcinosarcoma (ODCASA) A very rare neoplasm Fast growing large, firm swelling Extending from the lower left first molar to the coronoid process of the left mandible. Radiogram : radiolucent scalloped and multilocular, relatively well circumscribed Both the epithelial and the ectomesenchymal components show cytological features of malignancy. 68

69. Approach Imaging - non specific & overlapping. Patient’s age, clinical presentation, radiographs, CT scan and histopathologic study - narrows the differential diagnosis. Location: Majority - posterior mandible Few lesions - anterior mandible : giant cell reparative granuloma, cemento - osseous dysplasia (COD) 69

70. Relation to dentition: Few odontogenic lesions - periapical in location : radicular cyst , Cementoblastoma, and periapical cemental dysplasia Dentigerous cysts are typically pericoronal , (related to crown of teeth). Ameloblastoma , Pindborg tumor, odontoma , and keratocystic odontogenic tumor (KCOT) are associated with an impacted tooth . 70

71. Relation to inferior alveolar canal: The lesions with epicentre above the canal are likely odontogenic and the lesions below the canal are non odontogenic in origin Margins: Benign lesions are usually well circumscribed and inflammatory; malignant lesions are ill- defined. 71

72. Cortical integrity and soft tissue: Benign lesions are generally expansile with cortical thinning , whereas malignant or locally aggressive lesions cause cortical destruction with soft tissue component. Presence of periosteal reaction is suggestive of osteomyelitis (OM) or malignant etiology. 72

73. RECENT ADVANCES Reduced Vitamin E levels in KCOT - suggestive of the possible interrelation between Vitamin E and KCOT in vivo. Increase intake of Vitamin E - reduces the risk of recurrence in KCOT by reducing the dysregulation of Cyclin D1 and down-Regulation of mutant p53. 73

74. 74

75. Cyclooxygenase (COX)‑2 in dentigerous cyst and ameloblastoma - nature and behavior of odontogenic cysts and tumors; definitive target for a pharmacological approach in the management. COX‑2 expression- odontogenic epithelium of dentigerous cyst and ameloblastoma. It may contribute to local extension of these lesions by increasing the proliferation of their odontogenic epithelial cells 75

76. 76

77. Summary 77

78. 78

79. 79

80. REFERENCES 1. Mills E. S., Greenson K. J,Hornick L. J,Longacre A. T,Reuter E. V. Sternberg’s Diagnostic Surgical Pathology. In: Jaws, Oral Cavity, and Oropharynx, 6ed. Wolters Kluwer;2015.p877-897. 2. Fletcher D. M. C. Diagnostic Histopathology of Tumors. In: Tumors of the oral cavity, 4ed. Elsevier;2013.p254-267. 3. Rosai J. Rosai and Ackerman’s Surgical Pathology. In: Mandible and maxilla, 10ed. Elsevier;2011.p270-279. 4. Weidner,Cote, Suster,Weiss. Modern Surgical Pathology. In: Oral Cavity and Jaws, 2ed. Elsevier;2009.p343-356. 5. Barnes L. Surgical Pathology of the Head and Neck. In: Cysts and Cyst-like Lesions of the Oral Cavity, Jaws and Neck, 3ed. Informa healthcare;2009.p1163-1172. 6. Barnes L. Surgical Pathology of the Head and Neck. In: Odontogenic Tumors, 3ed. Informa healthcare;2009.p1201-1313. 80

81. 7. Tekkesin M S, Wright J M. The World Health Organization Classification of Odontogenic Lesions: A Summary of the Changes of the 2017 (4th) Edition.Turk J Path.2017. 8. Alsaegh M A, Miyashita H, Taniguchi T, Zhu S R. Odontogenic epithelial proliferation is correlated with COX-2 expression in dentigerous cyst and ameloblastoma.ExpTher Med J.2017;13:247-53. 9. Ayinampudi B K, Varikoti S B, Baghirath P V, Vinay B H, Gayathri C, Gannepalli A. Assessing Alpha‑Tocopherol Levels in Patients with Keratocystic Odontogenic Tumor: A Cross‑sectional Study. Indian J. Dent. 2017;28(2):122-25. 10. H. Kumamoto. Molecular pathology of odontogenic tumors. J Oral Pathol Med.2006; 35:65–74. 81

82. Post test Questions: Total 10 marks Total 9 Ques.; total duration= 9.5 min. Q1. Name any 4 lesions having these type of cells. (2 marks) (1min.) Q2. Name the odontogenic lesions having soap bubble appearance on radiograph (1 mark) (0.5 min.) Q3. what is the difference between complex and compound odontoma (1mark) (1.5 min.) Q4. Describe the findings and Identify (1mark) (1.5 min.) 82

83. Q5. Gorlin Goltz syndrome is associated with which odontogenic lesion? (0.5 mark) (0.5 min.) Q6. Name the pathways involved in regulators of tooth development. (0.5 mark) (1min.) Q7. The lesions below the inferior alveolar canal are odontogenic in origin (True/False). (0.5 mark) (0.5 min.) Q8. Name any 2 odontogenic lesions associated with impacted tooth. (1 mark) (1 min.) 83

84. Q9. What are these bodies? (a) Name any 2 lesions containing them (b) Name any 2 special stains to demonstrate them (2.5 marks) (2 min.) 84

85. Ans. 1: Dentinogenic ghost cell tumor, Ghost cell odontogenic carcinoma, Calcifying odontogenic cyst, Odontoma, Pilomatricoma , Craniopharyngioma Ans. 2: Ameloblastoma and odontogenic myxoma Ans. 3: 85

86. Ans. 4: Ans. 5: Gorlin Goltz syndrome is associated with odontogenic keratocyst Central core of the neoplastic epithelium shows squamous metaplasia . These central cells show more mature squamous differentiation. Acanthomatous Ameloblastoma 86

87. Ans. 6: Ans. 7: False. The lesions below the inferior alveolar canal are non odontogenic in origin Ans. 8: Ameloblastoma, Pindborg tumor, odontoma, and odontogenic keratocyst are associated with an impacted tooth . 87

88. Ans. 9: Rushton bodies- Intraepithelial hyaline bodies, found in dentigerous cyst, radicular cyst and odontogenic keratocyst . Special stains to demonstrate them- Orcein stain & Masson’s trichrome stain. 88