This document discusses pediatric neoplasms affecting the oral cavity. It begins by defining pediatrics and discussing risk factors for pediatric neoplasms such as radiation exposure and Down syndrome. It then classifies and describes several benign and malignant pediatric neoplasms that can affect the head and neck region, including congenital granular cell tumor, hemangioma, lymphangioma, juvenile ossifying fibroma, ameloblastoma, adenomatoid odontogenic tumor, odontoma, leukemia, lymphoma, and mucoepidermoid carcinoma. For each neoplasm, the document discusses etiology, clinical features, histopathology, differential diagnosis, immunohistochemistry, imaging features, and treatment approaches.

1. PEDIATRIC NEOPLASM AFFECTING THE ORAL
CAVITY WITH EMPHASIS ON ITS RECENT
ADVANCES
OBSERVER :DR.G.NANDHINI
PRESENTER :KRISHNA MOHAN

2. INTRODUCTION
• ‘pedia’ meaning child
• ‘iatrike’ meaning treatment
• “Pediatrics is the branch of
medicine that deals with the care
of children from conception to
adolescence in health and illness”.

3. PREDISPOSING RISK FACTORS FOR
PEDIATRIC NEOPLASMS
• Ionizing radiation exposure
• Down syndrome
• Early life exposure to infectious agents, parental, fetal or
childhood exposure to pesticides.
• Family history of childhood cancer
• Children with AIDS possess an increased risk of developing
certain cancers like Kaposi’s sarcoma.

4. CLASSIFICATION OF PEDIATRIC NEOPLASMS
AFFECTING HEAD AND NECK

5. BENIGN NEOPLASMS
CONGENITAL
GRANULAR CELL
TUMOR (CGCT):
• Ernst Christian Neumann in
1871
• Congenital epulis and
Neumann tumor
• Rare benign soft tissue
tumor present at birth with
female predominance

6. ETIOLOGY :
Odontogenic epithelial, pericytic and fibroblastic origin have been
proposed
CLINICAL FEATURES :
1. Lesions are found only in newborn
2. Lesions mostly occur in anterior maxilla and occasionally lesions are
found in the anterior mandible
3. Occurrence: Females > Males
4. Lesions are pedunculated,polypoid masses originating from crest of
alveolar ridge
5. Texture: Smooth surface
6. Size: several cm in diameter(1-9)
7. Color: Pink

7. HISTOPATHOLOGY
• Uniform pattern of granular cells
with outer surface of atrophic
stratified squamous epithelium.
• Lesion is composed of sheets of
large cells with a granular
cytoplasm and vascular,
noncollagenous stroma.
• Lesions contain autophagic
vacuoles

8. DIFFERENTIAL DIAGNOSIS :
• Granular cell tumor
• Verruciform xanthoma
IHC :
• Vimentin
• acid phosphatase
TREATMENT :
• Surgical Excision
• Recurrence is rare

9. HEMANGIOMA
Hemangiomas are benign congenital vascular neoplasms composed of vascular
endothelial cells that have the capacity for excessive proliferation and
represent one of the most common birthmarks in infants and children.
ETIOLOGY :
One hypothesis says that placental cells such as trophoblasts may be the cell of
origin for hemangiomas.
CLINICAL FEATURES:
• About 30% of haemangiomas are present at birth.
• Gender: female to male ratio is 2:1
• Age: 1st and 3rd decades.

10. • SITE: Face, neck ,scalp and back shortly after birth in oral cavity the lips,
tongue, buccal mucosa and palate are most common sites.
• Appear as a flat or raised lesion of the mucosa.
• Bright red (strawberry hemangioma) or blue reddish in color
• Pedunculated and globular and some are broad based and flat or slightly
raised.
• Compressibility test, continue pressure will push blood out of the lesion.
• If associated with tongue it may cause loss of mobility

11. HISTOPATHOLOGY
• Irregularly dilated blood
vessels lined by flat
endothelium and with walls
of varying thickness.
• Calcifications and formation
of phleboliths occur through
dystrophic calcification of
organizing thrombi
• Mild inflammation
frequently found.

12. DIFFERENTIAL DIAGNOSIS :
• Pyogenic granuloma
• Mucocele
• Ranula
• Hemangiomatosis
IHC :
Elastin ,trichrome
DIAGNOSTIC IMAGING :
• Ultrasonography (US)
• Computerized tomography (CT)
• Magnetic resonance imaging (MRI)
TREATMENT: disappear on there own
rare cases treated with drug or laser surgery

13. LYMPHANGIOMA(CYSTIC HYGROMA)
• Lymphangioma is a congenital
benign lesion, consisting of
localized centres of abnormal
development of lymphatic system.
• Benign tumours of lymphatic
vessels
• Also known as lymphatic
malformations are cystic
structures most commonly present
as a lump in the head, neck or
armpit areas.
• These cystic masses made up of
small cysts or larger cysts
contain thin fluid

14. CLASSIFICATION OF LYMPHANGIOMA
• Lymphangioma Simplex
• Cavernous lymphangioma
• Cystic lymphangioma
• Lymphangioma complex

15. CLINICAL FEATURES
• occurs on lips, tongue and floor of
the mouth
• soft, non-tender mass.
• occur at various sites but are more
frequent in the anterior two-thirds
of the tongue, where they often
result in macroglossia.
• Cause malocclusion, respiratory
obstruction and dysphagia
• Pebbly surface, resembling a
cluster of translucent vesicles

16. HISTOPATHOLOGY
• It consist of lymphatic
vessels with marked
dilatations.
• The lining endothelium is
thin and the spaces
contain proteinaceous
fluid and lymphocytes.
• Secondary hemorrhage
may be noticed in the
lymphatic vessels.

17. • The lymphatic spaces contain
lymphatic fluid, red blood cells,
lymphocytes, macrophages, and
neutrophils.
• Surrounding connective tissue
stroma consists of loose fibrotic
tissue with a number of
inflammatory cells.

18. DIFFERENTIAL DIAGNOSIS :
• Hemangioma
IHC :
• Factor VIII related antigen,CD 31
TREATMENT :
• Surgical excision is recommended.
• Lymphangioma, complete removal is difficult, and hence
recurrence is common.

19. JUVENILE OSSIFYING FIBROMA (JOF)
• rare benign but locally aggressive tumor with
high recurrent potentials.
• Distinct clinical entity, often confused with
malignant conditions because of its rapidly
progression
• Aggressive bone-forming neoplasm due to its
clinical behaviour and high recurrence rate.
Two patterns :
(1) Trabecular
(2) Psammomatoid,
Trabecular form:
• Seen in younger patients.
• Mean age is approximately 11 years,
Psammomatoid form:
• Appears outside of the jaws
• 70% arising in the orbital and frontal bones and
paranasal sinuses.
ETIOLOGY : jof are thought to originate from
periodontal ligament.
Trabecular
Psammomatoid

20. CLINICAL FEATURES
• Early to late childhood
• Maxilla > Mandible
• Singular, slow-growing, painless swelling
• May involve impacted or unerupted teeth
• Increased level of serum alkaline phosphatase
• Severe malocclusion
• Psammomatoid type more commonly affects Sino nasal and orbital bones
of the skull
• Trabecular type affects mostly gnathic bone, particularly maxilla

21. RADIOGRAPHIC FEATURES
• Radiolucent or mixed
radiolucent and radiopaque
appearance (ground glass),
• Lamina dura is usually
obscured and the cortical
plates thinned .

22. HISTOLOGIC FEATURES
•Trabecular variant
Abundant cellular fibrous connective
tissue in a whorled pattern
• Proliferating fibroblasts form
spicules of bone
• Areas of hemorrhage and small
clusters of multinucleated giant cells,

23. Psammomatoid form
• The psammomatoid pattern forms
concentric lamellate and spherical
ossicles that vary in shape and
typically have basophilic centers
with peripheral eosinophilic
osteoid rims

24. DIFFERENTIAL DIAGNOSIS :
Ossifying fibroma ,fibrous dysplasia ,cementoblastoma
IHC :
RUNX2
TREATMENT:
• Smaller lesions, complete local excision or thorough curettage
appears adequate
• Rapidly growing lesions, wider resection may be required

25. AMELOBLASTOMA
• Ameloblastoma, a benign
odontogenic tumor rarely seen in
the pediatric age group, account
for about 10 – 15% of all reported
cases.
• Ameloblastoma is a benign, but
Locally invasive tumor with a
high tendency to recur.

26. CLASSIFICATION :
• Solid/multicystic
• Peripheral
• Unicystic
Unicystic ameloblastoma, a less aggressive type is considered to more
common in the younger age group than adults, with about 50% of cases
occurring in the second decade.

27. CLINICAL FEATURES
• Unicystic ameloblastoma occur in mandible, frequently associated with
an impacted tooth.
• Painless swelling, producing facial asymmetry, displacement or
mobility of tooth and root resorption.
• Due to its slow growth, sometimes, it is diagnosed only in the adult age
• Unicystic ameloblastoma shows better prognosis than solid/multicystic
and peripheral types.

28. RADIOGRAPHIC FEATURE
• Well-defined unilocular
radiolucent lesion partially
surrounding the impacted
permanent first molar
• Root resorption,
• Displacement of the tooth

29. HISTOPATHOLOGICAL FEATURES
• Cystic lesion mainly lined by a
thin layer of nonkeratinizing
stratified squamous epithelium
were seen.
• Minimal inflammation in the thick
fibrous connective tissue wall.
• In the focal area, the lining
epithelium grew downward into
the underlying connective tissue
• This invaded epithelium
demonstrated a basal layer of
columnar cells with
hyperchromatic nuclei that
showed reverse polarity and
basilar cytoplasmic vacuolization
Luminal type

30. • The suprabasal epithelial cells
were loosely cohesive and
resembled a stellate reticulum.
Mural type Intraluminal
type
The fibrous connective tissue wall of
the cyst is infiltrated by ameloblastic
masses
The nodules of ameloblastoma proliferate
and project into cystic lining

31. DIFFERENTIAL DIAGNOSIS :
Calcifying cystic odontogenic tumor ,Keratocystic
odontogenic tumor ,Dentigerous cyst
IHC :
CK 5,CK6,CK 16,CK 14,CK 19,CD56
TREATMENT :
• Conservative management by enucleation followed by Carnoy’s
solution and peripheral osteotomy are the recommended treatment
options

32. ADENOMATOID ODONTOGENIC TUMOR
(AOT)
• Is a benign tumor of odontogenic origin
• It is a derived from odontogenic epithelium
that usually occurs around the crown of un
erupted anterior teeth of young patients
• ETIOLOGY : arise from remnants of the
dental lamina.

33. CLINICAL FEATURES

34. RADIOGRAPHIC FEATURES
• Well-circumscribed
• Unilocular radiolucency or
radiopaque-radiolucent mixed
lesion with well-defined
corticated or sclerotic border,
• usually surrounding an
unerupted tooth

35. HISTOPATHOLOGICAL FEATURES
• AOT is usually
surrounded by a well-
developed connective
tissue capsule.
• A single large cystic
space, or as numerous
small cystic spaces.
• The tumor is composed of
spindle-shaped or
polygonal cells forming
sheets and whorled
masses in a scant
connective tissue stroma.

36. • The amorphous eosinophilic
material is seen between the
epithelial cells, as well as in the
center of the rosette-like structure.
• The characteristic duct-like
structures are lined by a single
row of columnar epithelial cells,
the nuclei of which are polarized
away from the central lumen.
• The lumen may be empty or
contain amorphous eosinophilic
material.

37. DIFFERENTIAL DIAGNOSIS :
Dentigerous cyst ,odontogenic myxoma, odontogenic keratocyst
,calcifying epithelial odontogenic cyst
IHC :
Survivin
TREATMENT :
• Conservative surgical procedures like enucleation, curettage and simple
excision are adequate .

38. ODONTOMA
Odontomas are hamartomas consisting primarily of enamel and dentin and
variable amounts of cementum and pulp.
ETIOLOGY :
• local trauma
• Inflammatory and/or infectious processes
• Cell rests of serres (dental lamina remnants)
• due to hereditary anomalies (Gardner’s syndrome, Hermann’s syndrome)
• Alterations in the genetic component responsible for controlling dental
development.

39. TYPES :
Two types of odontomas are compound and complex.
Compound odontoma consists of dental tissues arranged in more orderly
pattern while complex odontoma is represented by well-formed tissues in a
disorderly pattern .

40. CLINICAL FEATURES
• Of the two types, compound odontoma is a pediatric lesion, with majority
of cases occurring before the age of 20.
• It frequently presents in the maxillary anterior region and is often
associated with an unerupted permanent tooth.
• It is usually asymptomatic

41. RADIOGRAPHIC FEATURES
• well-organized malformed
teeth or tooth-like structures,
usually is a radiolucent cyst
like lesion.
• A complex odontoma shows
an irregularly shaped oval
radiopacity usually surrounded
by a well-defined thin
radiolucent zone.
• In case of compound
odontoma in which extremely
small, malformed teeth or
tooth-like structures

42. HISTOPATHOLOGICAL FEATURES
• Decalcified (5% nitric acid) hard
tissue section shows thin, non-
keratinized cuboidal or flattened
epithelial cell lining and
underlying loosely arranged
fibrous connective tissue
containing small endothelial lined
vascular spaces and extravasated
RBCs.

43. • Presence of mature dental tissues
like enamel, dentin and cementum
arranged as unstructured sheets.
• Large mature tubular dentin was
apparently.
• Small eosinophilic-stained islands
of epithelial ghost cells
undergoing keratinization were
visible

44. DIFFERENTIAL DIAGNOSIS :
Osteoma ,Ameloblastic fibro odontoma ,calcifying epithelial odontogenic
tumor.
IHC :
CD 16,CD 17,CD 18
TREATMENT :
Surgical removal followed by histopathological analysis

45. MALIGNANT NEOPLASMS

46. LEUKEMIA
• Neoplastic proliferations of white
blood cells is called leukemia
• Most common neoplasm
occurring in the pediatric age
group.
• Most frequently occurring types
include acute lymphocytic
leukemia (ALL), followed by
acute myeloid leukemia (AML).
• Chronic form types : chronic
lymphocytic leukemia(CLL),
chronic myeloid leukemia (CML)
• 4 years of age.

47. RISK FACTORS

48. SYMPTOMS

49. HISTOPATHOLOGY

50. MANAGEMENT
• Complete all dental care procedures before the initiation of therapy to
reduce the risk of oral complications.
• Maintaining oral hygiene is recommended.
• Any emergency dental treatment during the course of therapy can be
provided if absolute neutrophil count exceeds 1000/mm3 and platelet
counts are appreciable

51. LYMPHOMA
After leukemia, lymphoma is the
second most common pediatric
malignancy, accounting for 20.3% of
cases in India.
TYPES :
Hodgkin’s lymphoma and non
Hodgkin’s lymphomas

52. CLINICAL FEATURES:
• Male predilection.
• Painless cervical and supraclavicular lymphadenopathy
• Non-Hodgkin’s lymphomas comprise 60% of the pediatric lymphomas
• Extra-nodal sites are more frequently involved in children than adults.
• Rapidly growing and aggressive tumor.
• Lymphomas may involve extra-nodal sites in oral cavity and oropharynx,
primarily located in Waldeyer’s ring, causing dysphagia and sore throat.
• Oral lymphomas are frequently seen in gingiva, palate and tongue and
grow rapidly resulting in bone destruction.

53. • Lesions in oral cavity are commonly seen in HIV-infected individuals.
• Lymphomas of salivary glands mostly involve parotid gland and are
commonly associated with Sjogren’s syndrome

54. HISTOPATHOLOGICAL FEATURES
Hodgkin’s lymphoma Non Hodgkin’s lymphomas

55. TREATMENT
• Chemotherapy given over 12 – 32 months.
• Multiagent chemotherapy alone or in combination with radiotherapy result
in better survival rates.

56. MUCOEPIDERMOID CARCINOMA (MEC)
Salivary gland tumors are rare in
children, accounting for about 10%
of all pediatric neoplasms of head
and neck .
ETIOLOGY :
MEC may develop secondary to
radiation or chemotherapy during
childhood.

57. CLINICAL FEATURES
• MEC is the most common
salivary gland tumor occurring in
children
• The age range 5 – 15 years.
• Parotid gland while few have
been reported in minor salivary
glands of palate, buccal mucosa
and lips.
• Those cases occurring in children
have good prognosis.

58. HISTOPATHOLOGICAL FEATURES
• Characterized by variable
components of squamoid,
mucinproducing, and intermedia
type cells. with a cystic and solid
growth pattern.
• Keratinzation is rare but usually
have Oncocytic, clear-cell and
sclerosing variants.
• Mucicarmine stainng and periodic
acid-Schiff (PAS) stain with
diastase demonstrate
intracytoplasmic staining in
mucinous cells.

59. DIFFERENTIAL DIAGNOSIS :
Cystadenoma ,adenosquamous carcinoma,polymorphous low grade adeno
carcinoma
IHC:
• Ck7 ,ck5,ck6,p63 ,p40
TREATMENT
• Complete removal of tumor
• The use of radiotherapy is considered in selected
cases keeping in mind the long-term adverse
effects in children .

60. ACINIC CELL CARCINOMA (ACC)
• Next to MEC, ACC is the
second most common salivary
gland malignancy in children.
• Slight female predilection
• Parotid gland.
• slowly growing painless mass
without any symptoms.
• local invasion with propensity
for recurrence and distant
metastasis.

61. HISTOPATHOLOGICAL FEATURES
• Well differentiated acinar
cell
• Cytoplasmic granules
• Slightly basophilic
cytoplasm
• Eccentricaly located nuclei

62. DIFFERENTIAL DIAGNOSIS :
Adenocarcinoma ,pleomorphic adenoma , MEC
IHC :
Keratin ,SOX 10 ,DOG 1
TREATMENT :
Surgery is the treatment while radiotherapy is given
only in selected cases

63. OSTEOSARCOMA
• Osteosarcoma is the most common
primary malignancy of bone in
children and young adults.
• It is also called as osteogenic
sarcoma
• Neoplastic cell ability to produce
osteoid or immature bone within
tumor

64. CLINICAL FEATURES
• Osteosarcoma is the most
common primary malignancy
of bone.
• First and second decades.
• Rapid bone growth during
adolescence is considered as a
cause for the development of
this lesion.
• Predilection for occurrence in
mandible.
• Pain in the involved region of
bone.
• Mucosal ulceration and
loosening of teeth can also
occur

65. RADIOGRAPHIC FEATURES
• Variable with combination of
bone destruction & bone
formation
• Sunburst appearence & Codman's
triangle (lifting of periosteum)

66. HISTOPATHOLOGICAL FEATURES
• Malignant spindle
mesenchymal cells with
pleomorphic nuclei,
scattered mitotic figures.
• Immature and
disorganized osteoid
production is a
characteristic hallmark
and must be present for
diagnosis

67. DIFFERENTIAL DIAGNOSIS :
Chondrosarcoma ,Fibrous dysplasia ,Ossifying fibroma ,Osteoblastoma ,
IHC :
Bone morphogenetic protein ,S 100 ,Keratins ,Epithelial membrane antigen
TREATMENT :
Wide resection.
Radiotherapy

68. RECENT ADVANCES

73. CONCLUSION
• Pediatric neoplasms vary from that of adults in various aspects like clinical
behavior, site predilection, rate of metastasis and survival rates.
• Hence, the diagnosis and treatment of these neoplasms should take the
differences into account.