This document summarizes two bone tumors - osteosarcoma and synovial sarcoma - and the epithelial tumor spindle cell carcinoma. It discusses the definition, demographics, clinical features, histopathology, differential diagnosis, treatment and prognosis of each tumor type. Key information includes that osteosarcoma commonly affects long bones and jaws in children and young adults. Synovial sarcoma has a poor prognosis with high recurrence and metastasis rates. Spindle cell carcinoma is an aggressive form of squamous cell carcinoma with spindle-shaped epithelial cells.

1. Seminar on
Spindle cell tumors of the oral cavity
Part - III
By: Dr. Madhusudhan reddy
III yearPG
VDC

2. • Part I seminar
I. Tumors of fibrous origin
II. Tumors of Fibro histiocytic origin
III.Tumors of adipose tissue origin
IV.Tumors of Smooth muscle origin
• Part II seminar
I. Tumors of Skeletal muscle origin
II. Tumors of Nerve tissue origin
III.Tumors of Vascular origin

3. • Part III seminar
I. Tumors of Bone
II. Tumors of epithelial origin

4. Good morning

5. Bone tumors

6. • Definition: Malignant, bone-forming tumor in which the
neoplastic cells form bone
• Account for approximately 20% of primary malignant
bone tumors
• Representing the most common primary
(nonhematopoietic) malignancy of the skeletal system
• Osteosarcomas of the jaws are uncommon and represent
6% to 8% of all osteosarcomas

7. • Demographics
• Age: Bimodal age distribution
– 60% occurring before age 25
– 13% to 30% occurring in individuals older than 40
years
• Sex: Slightly more common in male individuals

8. • Site: Long bones commonly affected, although any bone
may be involved
• maxilla and mandible are involved with about equal
frequency
• Mandible - posterior body and horizontal ramus rather
than the ascending ramus.
• Maxilla - inferior portion (alveolar ridge, sinus floor,
palate) than the superior aspects (zygoma, orbital rim).

9. • Common type – intramedullary
• Other types – juxtra cortical
– Paraosteal
– Periosteal

10. • Clinical features
• Localized pain with or without a mass
• Pathologic fracture
• Low-grade surface osteosarcoma may present as a
painless mass
• Swelling and pain are the most common symptoms
Loosening of teeth, paresthesia, and nasal obstruction

12. • Radiographic features:
• Radiographic appearance is extremely variable
– High-grade osteosarcomas are usually large, poorly
defined, destructive, mixed lytic, and blastic, and often
have a soft tissue Mass
– Low-grade osteosarcomas are sclerotic and frequently
arise on the cortical surface

16. Right maxilla - large osteoblastic destructive
mass, hair-on-end periosteal reaction
S Wang, H Shi; Osteosarcoma of the jaws: demographic and CT imaging features;
Dentomaxillofacial Radiology (2012) 41, 37–42
Body of the mandible showing periosteal
reaction

17. • Clinical differential diagnosis
• Odontogenic tumor
• Malignant bone tumor
• Osteoblastoma
• Metastatic tumor

18. • Histological variant of osteosarcoma
– Osteoblastic
– Chondroblastic
– Fibroblastic
– Malignant fibrous histiocytoma-like.
– Small cell
– Epithelioid
– Telangiectatic
– Giant cell rich

19. Histopathology
Osteoblastic osteosarcoma containing
pleomorphic malignant cells and
coarse neoplastic woven bone
Chondroblastic osteosarcoma with
neoplastic cartilage merging with
tumor bone

20. Fibroblastic osteosarcoma containing fascicles of
malignant spindle cells adjacent to deposits of
neoplastic bone

21. spindle cells can grow in sheets in
parosteal osteosarcoma
Intramedullary well-differentiated
osteosarcoma - trabeculae of neoplastic
woven bone are surrounded
by minimally atypical spindle cells

22. • Histological differential diagnosis
• Aggressive osteoblastoma
• Psuedomalignant osteoblastoma
• Myofibroblastic tumors
• Malignant neoplasms of bone
• Intraosseous squamous cell carcinoma
• Primary intraosseous neoplasm
• Malignant tumor of odontogenic origin
• IHC markers
• Variable and usually not helpful in diagnosing osteosarcoma

23. • Treatment
• Combination of surgery and chemotherapy
• Neoadjuvant (preoperative) chemotherapy followed by
radical surgical excision
• Adjuvant (postoperative) chemotherapy is used and
may be modified if poor histopathologic response to the
neo adjuvant regimen is noted
• Survival rate for localized conventional high-grade
osteosarcoma is 50% to 80%
• Low-grade surface osteosarcoma has a 90% to 100%
survival rate

24. Synovial tumor

25. • The term synovioma was coined by Smith in 1927
• Later in 1936 Knox suggested the name synovial sarcoma
(SS)
• Most common soft tissue malignancy after MFH,
liposarcoma, and rhabdomyosarcoma.
• H&N SS account for 6.8% of all SS occurring in the body
• Definition: Malignant mesenchymal tumor showing epithelial
differentiation
• Arises from primitive cells that have the potential to
differentiate into either mesenchymal or epithelial
components

26. • Demographics
• Age: Most common in young adults but may be seen at any
age
• Sex: No sex or race predilection
• Site: primary SSs of oral and maxillofacial sites
– Buccal mucosa
– Maxillary sinus
– Mandible
– Tongue
– Floor of the mouth

27. Clinical features
• Slow-growing, deep-seated,
palable mass associated with pain
in about 50% of cases

28. • Clinical differential diagnosis
• Fibrosarcoma
• Osteosarcoma
• Rabdomyosarcoma

29. • Histological classification of SS
– Biphasic type with distinct epithelial and spindle cell
components present in various proportions and
patterns
– Monophasic spindle cell type with little or no evidence
of epithelial differentiation
– Monophasic epithelial type
– Poorly differentiated type

30. Histopathology
Monophasic synovial sarcoma -
hypercellularity and hypocellularity,
moderately long fascicles, uniform
hyperchromatic spindled cells
Biphasic synovial sarcoma with
occult glandular differentiation

31. Poorly differentiated synovial sarcoma,
showing a malignant hemangiopericytoma
growth pattern
Biphasic synovial sarcoma, with overt
glandular differentiation

32. • Histological differential diagnosis
• Malignant peripheral nerve sheath tumor
• Fibrosarcoma
• Solitary fibrous tumor
• Benign fibrous histiocytoma
• IHC markers
• Limited expression of low- and high-molecular-weight
cytokeratins
• Limited expression of EMA
• S-100 protein expression in 20% of cases
• CD34 negative
• CD99 expression is common - in poorly differentiated
tumors
• Nuclear expression of TLE-1

33. • Treatment
• Adequate surgical excision with follow-up
• Recurrence rates is upto 70% (2 – 20 years)
• Metastasis - usually blood borne to lungs (94%)
• Five-year survival rate is about 36–51%
• Prognosis is affected
– Tumor size
– Location
– Patient age
– Histological subtype
– Extent
– Mitotic activity
– Margin of resection

34. Epithelial tumors

35. • Benign
• Nevus
• Malignant
• Melanoma
• Spindle cell carcinoma

36. Nevus

37. • Melanocytes are non keratinocytes
• Melanocytes in skin – protects against harmfull effects of
sun light.
• Present in the basal layers of the oral mucosa. along the
tips and peripheries of the rete ridges.
• 1 melanocyte to 15 keratinocytes
• Function – unknown
MS Hashemi Pour; Malignant melanoma of the oral cavity: A review of litrature; IJDR, 19
(1), 2008

38. • Melanocytes, nevus cells, and melanoma cells differ
– Cellular appearance
– Organization
– Biological characters
• Nevus cells
– Dendritic processes
– Round to spindle shaped cells
• Nevus cells lack
– Cytological atypia
– Pleomorphism
– Mitotic activity
MS Hashemi Pour; Malignant melanoma of the oral cavity: A review of litrature; IJDR, 19
(1), 2008

39. • Definition: Oral melanocytic nevi are hamartomas that
derive from nevomelanocytes cells that originate from the
neural crest
• Demographics
• Age: Third to fourth decade
• Sex: No Sex Predelection
• Site:
– Palatal mucosa (34% to 44%)
– mucobuccal fold (24%)
– buccal mucosa (11% to 22%)
– lip vermilion (18%)
– gingiva (12% to 23%).

40. Varient of nevus Clinical
appearance
% of nevus Histopathology
Intramucosal nevi Plaques or Nodules 64% to 80% Type A- epithelioid cells just
beneath the epithelium
Type B -lymphocyte-like or
neuroid spindle Cells
Type C- deeper in the lamina
propria
Blue nevi Macules or Plaques 8% to 17% Nevus cells with benign nuclei
Without junctional nests
Compound nevus Plaques or Nodules 6% to 17% Combination of intramucosal
and junctional nevus
Junctional nevi Macules or Plaques Rare Many nests of benign nevus cells
in the basal layer
Combined nevi Plaques or Nodules Rare Presence of both intramucosal
and Blue nevus

41. Clinical features

42. • Clinical differential diagnosis
• Amalgam tattoo
• Medication-induced pigmentation
• Oral melanotic macule
• Smoking associated pigmentation
• Post inflammatory pigmentation
• Peutz-Jeghers syndrome
• Kaposi sarcoma
• Malignant melanoma

44. Histopathology
Intramucosal nevus
Heavily pigmented nevus cells

45. Junctional nesting of
pigmented nevus cells
Epithelioid nevus cells appear
to hang from tips of rete
ridges
Compound nevus Junctional nevi

46. Subtly pigmented lesion in
lamina propria
Spindled pigmented dendritic
melanocytes
Blue nevus

47. Combined mucosal nevus
Superficial cells are spindled and
Epithelioid without nesting
Pigmented dendritic cells.

48. Pigmented spindle and epithelioid cells
Sheets of nevus cells and sclerosis. Sheets of pigmented epithelioid cells
Combined mucosal nevus

49. • IHC markers
• S-100 protein
• Melan-A
• HMB-45
• Treatment
• No treatment is required
• Surgical excision for cosmetic reasons

50. Melanoma

51. • Definition: malignant tumor of melanocytic origin
• Most common skin malignancy in
• Demographics
• Age: Fourth and the seventh decade of life, with an
average of 55-57 years old
• Sex: males are more commonly effected M:F – 2:1
• Site: hard palate (40%) > upper gingiva > lower
gingiva > buccal mucosa > tongue > floor of the mouth

52. Clinical features
• Macular lesions, nodular, sometimes
ulceration with regular or irregular edges
• Dark blue to black
• The symptoms
• Bleeding
• Pain
• Presence of melanotic pigmentation

53. • Criteria for diagnosis of melanoma is based on
“ABCD” system
– A – Asymmetry
– B – Border irregularity
– C – Color variegation
– D – Diameter greater than 6mm
• Growth patterns in melanoma
– Radial growth pattern – spreads horizontally
through basal layers
– Vertical growth pattern – invade the underlying
connective tissue

54. • Based on clinicopathological features
– Superficial spreading melanoma (70% of cutaneous)
– Nodular melanoma (15% of cutaneous)
– Lentigo maligna melanoma (5-10% of cutaneous)
– Acral lentigenous malanoma (common form of oral
melanoma)

55. • Clinical differential diagnosis
• Oral melanotic macule
• Medication induced melanosis
• Cushing syndrome
• Postinflammatory pigmentation
• Melanoacanthoma
• Nevi
• Addisons disease
• Peutz jeghers syndrome
• Amalgam tattoo
• Kaposis sarcoma

56. Histopathology
Superficial spreading melanoma –
spread of melanocytes along basal
portion of epidermis
Nodular melanoma – malignant
cells invading into dermis

57. Acral lentigenous melanoma – numerous stypical
melanocytes in basillar portion of epi spreading into
superficial lamina propria

58. • Histological differential diagnosis
• Poorlydifferentiated carcinoma
• Large cell anaplastic lymphoma
• Sarcomatoid carcinoma
• Epitheoid sarcoma
• Melanotic schwannoma
• Malignant fibrous histiocytoma
• Malignant peripheral nerve sheath tumor
• Lymphoma
• Rhabdomyosarcoma
• IHC markers
– S-100
– MART-1
– HMB-45

59. • Treatment and Prognosis:
• Treatment depends on the depth of invasion of the tumor
depending histopathologic evaluation

61. Spindle cell carcinoma

62. • Definition: Spindle cell carcinoma is an unusual form of
poorly differentiated squamous cell carcinoma (SCC)
consisting of elongated (spindle) epithelial cells that
resemble a sarcoma
• First applied by Shervin et al
• also called
– Carcinosarcoma
– Pseudosarcoma
– Sarcomatoid SCC
– “Collision” tumor
– Sarcomatoid carcinoma,
• Biphasic tumor composed of SCC cells and pleomorphic
spindle-shaped cells

63. • Demographics
• Age: Mean Age 57 years, with a range of 29 to 93 years
• Sex: Males have a slight predilection
• Site: Commonly - oral cavity , larynx,
• less frequently - Sinonasal area and pharynx
• In oral cavity – alveolar ridge, lateral border of tongue,
floor of the mouth

64. Clinical features
Exophytic, polypoid, frankly
infiltrative ulcer, swelling, pain and
the presence of a nonhealing ulcer

65. • Clinical differential diagnosis
• Fibroma
• Traumatic neuroma
• Pyogenic granuloma
• Solitary neurofibroma
• Verrucous carcinoma

66. Histopathology
Spindle cell proliferation in
short fascicles with storiform
pattern, ulcerated
Interface of invasive spindle cells
and mildy dysplastic surface
epithelium

67. Spindle, stellateshaped,
and epithelioid cells in myxoid
stroma
Epithelioid and spindle cells
with pleomorphic nuclei

68. • Histological differential diagnosis
• Nodular fascitis
• Desmoplastic melanoma
• Spindle cell mesenchymal neoplasm
• MFH
• Fibrosarcoma
• IHC markers
• Cytokeratins
• Vimentin
• EMA
• P53
• Ki67

69. CK positivity

70. Positive staining of p53 in the spindle cell
component

71. • Treatment
• SPCC is biologically aggressive than the conventional
SQCC
• Treatment is similar to that of SQCC
• 90% of cases have 3-year survival rate