this ppt is about malignant tumours of connective tissue origin. classifications, clinical features, radiological features and histological features of all tumors are discussed with pictures.

2. • A malignant tumor of fibroblasts.
• The tumor is most common in the extremities; only 10% occur
in the head and neck region .
Clinical features:
• Can occur at any age but are most common in young adults
and children.
• most often present as slow-growing masses that may reach
considerable size before they produce pain.
• can occur anywhere in the head and neck region .
• Nose and paranasal sinuses -- obstructive symptoms.

3. Histopathological features:
• Well-differentiated fibrosarcomas  fascicles of spindle-shaped cells
that classically form a "herringbone“ pattern.
• The cells often show little variation in size and shape, although
variable numbers of mitotic figures can usually be identified.
• Poorly differentiated tumors  the cells are less organized and may
• appear rounder or ovoid.
• Mild pleomorphism along with more frequent mitotic activity may be
seen.
• Poorly differentiated tumors tend to produce less collagen than do
well-differentiated tumors.

5. • Treatment of choice is usually surgical excision, including
a wide margin of adjacent normal tissue.
• Recurrence is noted in about half of cases, and 5-year
• survival rates range from 40% to 70%.

6. • Highly lethal round cell sarcoma
• James Ewing in 1921
• Composed of small undifferentiated round cells of uncertain
histogenesis
• Considered to be intraosseous counterpart of PNET ( Primitive
Neuro Ectodermal Tumor)
• 3rd most common osseous neoplasm after osteosarcoma &
chondrosarcoma
• Etiology
• Genetic mutation
• Reciprocal translocation between chromosomes 11 & 22
[t(11;22) (q24;q12)]

7. Clinical Features
• Age: Children & young adults, 5-25 yrs
• Sex: M>F
• Race: Whites>blacks (never)
• Site: long bones, pelvis & ribs
• I/o –Mandible>Maxilla
• Pain (intermittent),dull to severe in nature with swelling
• An episode of trauma often precedes
• Rapid growth of swelling
• Intraoral mass may ulcerate
• Facial neuralgia, lip paresthesia & loosening of teeth (jaw)
• Low grade fever, Leucocytosis, E.S.R.

8. Radiographic features
• Irregular diffuse radiolucency
with ill- defined borders
• Onion skin appearance due to
formation of layers of
subperiosteal bone -
resembling sclerosing
osteomyelitis
• Sun- ray appearance due to
osteophyte formation

9. Histopathological Features
• Extremely cellular
• Solid sheets of small round
cell with little stroma
• Variably sized nests -
separated by fibrovascular
septa- lobular pattern Small
round cell

10. • Small round cells - scanty
cytoplasm, ill defined borders,
relatively large round or ovoid
nuclei, dispersed chromatin
and hyperchromasia Small
round cells
• Treatment
• Surgery
• Chemotherapy
• Radiotherapy

11. • Malignant tumor of cartilaginous tissue, a counterpart of
chondroma
• Bones that arise from cartilaginous tissue are more liable
to develop chondrosarcoma, therefore a jaw lesion is
rather rare
• Jaw lesion has a poorer prognosis than those in other
bones
• Primary chondrosarcomas ( arising de novo)
• Secondary chondrosarcomas arising most commonly in
osteochondroma

12. Clinical Features
• Any age, 10-80 years, secondary at an earlier age
• M>F
• Metastasis relative rare & occurs late
• No pathognomic signs and symptoms
• Depending on grade-
• High grade-fast growth with excruciating pain
• Low grade- more indolent with pain & swelling

13. Oral manifestations
• Expanding painless swelling
• Mucosa intact
• Commonly alveolar ridge
/near antrum
• Resorption & exfoliation of
teeth
• Invasive & destructive
lesions & metastasize
readily

14. Histopathological Features
• More difficult to diagnose
• Sheets of chondrocytes
• Large, pleomorphic
chondroblasts with plump
nuclei & binucleated
chondrocyte

15. • Giant cartilage cells with single
or multiple nuclei
• Tumor lobules separated by
fibrous connective tissue septa

17. • Grading - based on cellularity and cytologic atypia
• Grade I-closely mimics chondroma
• Grade II-increased cellularity
• Grade III-highly cellular, more mitotic figures.
• Variants
• Clear cell chondrosarcoma
• Mesenchymal chondrosarcoma
• juxta-cortical chondrosarcoma
• Extra –skeletal chondrosarcoma
• Myxoid chondrosarcoma
• Dedifferentiated chondrosarcoma
• T/t: Radical Surgery.

18. • Third most common cancer in adolescent ( lymphoma & brain
tumor)
• Arises from primitive mesenchymal cells that have ability to
produce osteoid or immature bone
• Except hematopoietic naoplasms,most common malignancy
originate within bone
• Etiology –exact cause is unknown
• Predisposing factor -Rapid bone growth, exposure to radiation,
trauma
• Secondary to Paget’s disease
• Genetic predisposition –familial cases (RB gene), bone
dysplasias, Li-Fraumeni syndrome, Rothmund-Thomson
syndrome

19. Clinical features
• Bimodal Age distribution
• 10 to 25 years, young persons( corresponds with growth
spurts) & over age of 50
• Initial peak during period of greatest bone growth
• Long bones of extremities near metaphyseal growth
plates.
• Site – Femur, tibia, humerus, skull /jaw & pelvis
• Sex- M>F
• Pain & swelling, particularly with activity of involved bone

20. • Oral manifestations
• Maxilla= mandible
• Mandibular –posterior body & horizontal ramus
• Maxillary-inferior portion (alveolar ridge, sinus floor,
palate)
• Swelling with Facial deformity
• Pain, Tooth ache & Loosening of teeth, Paresthesia,
• Nasal obstruction

22. Radiographic features
• Variable
• Depends on amount of tumor bone synthesized by
malignant osteoblasts
• Radiolucent
• Mixed sclerotic & radiolucent lesion
• Dense sclerotic

23. • Three features
• Sunray ( sunbrust ) pattern
• Uniform widening of periodontal
ligament space
• Codman’s triangle

24. • Types
• Parosteal (juxtacortical)
osteosarcoma-slow
growth & good prognosis
• Periosteal osteosarcoma-
more aggressive
• Extraosseous
osteosarcoma-soft tissue

25. • Histopathological features
• Histologic Variants:
• Osteoblastic osteosarcoma
• Chondroblastic osteosarcoma
• Fibroblastic osteosarcoma
• Characterized by presence of osteoid formed by
malignant osteoblasts
• Stromal cells may be spindle shaped & atypical with
irregular shaped nuclei

27. • an unusual vascular neoplasm that was first described in 1872
by Moritz Kaposi.
• caused by human herpesvirus 8 (Kaposi 's sarcoma associ
• ated herpesvirus).
• The lesion most likely arises from endothelial cells, with some
evidence of lymphatic origin.
Four clinical presentations are recognized :
• I. Classic
• 2. Endemic (African)
• 3. Iatrogenic immunosuppression- associated
• 4. AIDS-related

28. Classic type. Classic (chronic) Kaposi 's sarcoma
• Is primarily a disease of late adult life, and about 90% of cases
occur in men.
• It mostly affects individ uals of italian . lewish, or Slavic
ancestry.
• Multiple bluish-purple macules and plaques are present on the
skin of the lower extremities.
• These lesions grow slowly over many years and develop into
painless tumor nodules.
• Oral lesions are rare and most frequently involve the palate.

29. Endemic Kaposi's sarcoma in Africa has been divided into
four sub types:
I. A benign nodular type, similar to classic Kaposi's sarcoma
2. An aggressive or infiltrative type, characterized by
progressive development of locally invasive lesions that
involve the underlying soft tissues and bone
3. A florid form, characterized by rapidly progressive and Widely
disseminated. aggressive lesions with frequent visceral
involvement
4. A unique Iymphadenopathic type, which occurs primarily in
young black children and exhibits generalized, rapidly growing
tumors of the lymph nodes, occasional visceral organ lesions,
and sparse skin

30. Iatrogenic immunosuppression associated
• most often occurs in recipients of organtransplants.
• It affects 0.4 % of renal transplant patients, usually
several months to a few years after the transplant.
• It is probably related to the loss of cellular immunity.
which occurs as a result of immunosuppressive drugs.
• disease may run a more aggressive course.

31. AIDS related:
• KS begins with single or, more frequently multiple lesions
of the skin or oral mucosa .
• The trunk, arms, head, and neck are the most commonly
involved anatomic sites
• Oral lesions are seen in approximately 50% of affected
patients and are the initial site of involvement in 20% to
25%.

32. • Although any mucosal site may be involved, the hard palate,
gingiva, and tongue are affected most frequently.
• When present on the palate or gingiva , the neoplasm can
invade bone and create tooth mobility.
• The lesions begin as flat, brown or reddish purple zones of
discoloration that do not blanch with pressure.
• With time, the involved areas may develop in to plaques or
nodules.
• Pain, bleeding , and necrosis may become a problem and
necessitate therapy

34. Histopathologic Features
Kaposi's sarcoma typica lly evolves through three stages:
I. Patch (macular)
2. Plaque
3. Nodular
PATCH STAGE 
• characterized by a proliferation of miniature vessels.
• This results in an irregular, jagged vascular network that
surrounds preexisting vessels.
• Sometimes normal structures, such as hair follicles or
preexisting blood vessels, may appear to protrude into
these new vessels (promontory sign ).
• The lesional endothelial cells have a bland appearance
and may be a ssociated with scattered lymphocytes and
plasma cells.

35. PLAQUE STAGE 
• demonstrates further proliferation of these vascular
channels along with the development of a significant
spindle cell componen
NODULAR STAGE 
• the spindle cells increase to form a nodular tumor like
mass that may resemble a fibrosarcoma or other spindle
cell sarcomas
• numerous extravasated erythrocytes are present, and
slitlike vascular spaces may be discerned.

37. Treatment and Prognosis
.
• For skin lesions in the classic form of the disease, radiation therapy
(especially electro n beam) often is used.
• Surgical excision can be performed for the control of individual lesions of
the skin or mucosa.
• The classic form of the disease is slowly progressive; only 10% to 20%
die of the disease after 8 to 10 years.
• The benign nodular, endemic African form of the disease is similar in
behavior to classic non-African Kaposi's sarcoma.
• However the other endemic African forms are more aggressive and the
prognosis is poorer.
•
• The Iymphadenopathic form runs a particularly fulminant course, usually
resulting in the death of the patient within 2 to 3 years.
• In transplant patients, the disease also may be somewhat more
aggressive, although the tumors may regress if immunosuppressive
therapy is discontinued or reduced.

38. • Malignant solid tumor involving cells of the
lymphoreticular or immune system such as B-
lymphocytes, T- lymphocytes & monocyte
• Almost always begins in the lymph node but may be 1st
diagnosed in the extra nodal lymphatic tissue, where
normal tissue is replaced by malignant lymphocytes.

39. • First described by Thomas Hodgkin in 1832
• Orderly involvement of lymph nodes group with the
development of systemic symptoms as disease progress
• Pathologically, the disease is characterized by the
presence of Reed Sternberg cells Owl eye appearance

40. Etiology
• Infectious agents - EBV
• Acquired Immunodeficiency status
• Genetic predisposition
• Chemical exposure
Clinical features
• Bimodal incidence
• First young adulthood (age 15–35)
• Second >55 years old
• M > F , Whites > Asians
• Painless enlargement of one or more lymph nodes
(Cervical, axillary, inguinal,Waldeyer ring)
• Feel rubbery and swollen & overlying skin is normal

41. • Lymph nodes are movable in initial stage
• Matted & fixed to surrounding tissues
• Spreads to other lymph nodes & involves spleen & other
extralymphatic tissues, such as bone, liver & lung

42. • Hepatomegaly, Splenomegaly, Nonspecific back pain.
Systemic symptoms:
• Night sweats, Unexplained weight loss (at least 10% of
the patient‘s total body mass in 6 months or less)
• Itchy skin (pruritis), Lassitude, Alcohol induced pain
• Pel –Ebstein fever - cyclical high & low grade fever
• ORAL MANIFESTATIONS: Very rare, as is primarily a
disease of lymph nodes

43. Histological classification by Rye system
• There are different subtypes of Hodgkin's disease:
1. Nodular sclerosis (30–60% of cases)
2. Mixed cellularity (20–40% of cases)
3. Lymphocyte depleted (less than 5% of cases) -
worse prognosis
4. Lymphocyte predominant (5–10% of cases) – Best
prognosis
5. Nodular lymphocyte predominant (5 %)- (Popcorn
cell ,a variant of RS cell whose nuclei resembles an
exploded kernel of corn)

46. Reed Sternberg Cell
• Characteristic malignant cell of HL -20-50 µm
• Amphophilic ,finely granular, homogenous cytoplasm
• Two mirror- image nuclei (Owl eyes) with eosinophilic
nucleolus & thick nuclear membrane

47. • Non-Hodgkins lymphoma (NHL) is a heterogeneous
disease with variable clinical presentation & course
• NHL arises from B & T lymphocytes but B cell lymphoma
are more prevalent (85 %).
• Multicentric & diffuse involvement of lymph nodes,
lymphoid organs & extralymphatic tissue
• Lymph nodes of Head & Neck region are commonly
involved

48. Hodgkin's Lymphoma
• Bimodal age
• Mediastinum nodes
• Extranodal in 4%
• Systemic symptoms
40%
• Orderly & slow
Progression
Non-Hodgkin's
Lymphoma
• > 67 yrs
• Mesenteric nodes
• Extranodal in 23%
• Systemic symptoms
27 %
• Less predictable in
their course

49. • The "New Working Formulation" divides lymphomas into
three categories –
• 1. Low grade - Indolent behavior
• 2. Intermediate - Unfavorable behavior
• 3. High grade - Aggressive behavior
• Low-Grade
A. Small lymphocytic (lymphocytic; plasmacytoid)
B. Follicular, predominantly small cleaved cell
C. Follicular, mixed, small cleaved and large cleaved cel

50. • Intermediate-Grade
D. Follicular, predominantly large cell, cleaved and/or non-
cleaved
E. Diffuse, small cleaved cell
F. Diffuse, mixed, large and small cell
G. Diffuse, large cell, cleaved or noncleaved
• High-Grade
H. Large cell, immunoblastic -(B- or T-cell type)
Lymphoblastic
J. Small noncleaved cell (Burkitt's and non-Burkitt's)

51. Etiology / Risk factors
• Genetic abnormality
• Acquired Immunodeficiency states
• Infectious agents - EBV, human T-cell leukemia virus),
and bacterial infections (e.g., helicobacter pylori)
• Physical and Chemical agents -pesticides, solvents,
arsenate, and lead, hair dyes, radiation exposure (high
dose), and paint thinners may increase the risk.

52. Clinical Features:
• Age – Older > 50 yrs
• Sex-M>F
• Lymphadenopathy: Painless, persistent enlargement of lymph
nodes
• Systemic symptoms: Fever, night sweat, weight loss fatigue,
pruritis
• Sign & symptom depend on site of involvement & result from
pressure of enlarged lymph node
• Organ specific symptoms: shortness of breath, ches pain,
cough, abdominal pain & distension
• Extranodal lesion are more common

53. Oral manifestations
• Oral lesions: most commonly –posterior hard palate,
buccal vestibule, gingiva
• Rapidly growing swelling ,
• may ulcerate
• Large, fungating, necrotic, foul-smelling masses
• Underlying bone involvement: tooth mobility & pain,
paresthesia of mental nerve

56. • Proliferation of lymphocytic
appearing cells, showing
varying degrees of
differentiation, depending on
type.

57. Clinical Ann Arbor staging classification
• Stage I - Involvement of a one lymph node region (I) or
single extralymphatic site (IE)
• Stage II - Involvement of multiple node regions on the
same side of the diaphragm (II) or of one lymph node
region and a contiguous extralymphatic site (IIE)
• Stage III - Involvement of lymph node regions on both
sides of the diaphragm, which may include the
spleen(IIIS) and/or limited to contiguous extralymphatic
organ or site (IIIE, IIIES)
• Stage IV - Disseminated involvement of one or more
extralymphatic organs.

58. • The absence of systemic symptoms is signified by adding
'A' to the stage
• The presence of systemic symptoms is signified by
adding 'B' to the stage.
• For localized extranodal extension from mass of nodes
which does not advance the stage, subscript 'E' is
added.

59. • Rapidly growing tumor (fastest growing malignancy in
humans) & type of NHL Peculiar to children of tropical
central Africa
• Denis Parsons Burkitt in 1950
• Primary tumor cell is poorly differentiated B lymphocytes
• Three main clinical variants:
1. Endemic variant
2. Sporadic variant
3. Immunocompromised

60. Endemic variant
• Most common malignancy of children in Africa.
• Often have chronic malaria
• Characteristically involves the jaw or other facial bone,
distal ileum, ovaries, kidney or the breast
• Age-children (peak prevalence-7 yrs)
• M>F

61. • Oral manifestations
• African form
• Maxilla>mandible
• Swelling of affected jaw or
other facial bones
• Loosening of the teeth
• Pain
• Swelling of lymph nodes,
rapidly growing

62. • Radiographic features
• Bone destruction Ill defined margins Patchy loss of lamina
dura-early sign of BL
Sporadic form
• Jaw is less commonly involved
• Abdominal tumors causing swelling & pain in affected area
Immunodeficiency associated
• Associated with HIV Post-transplant infection
• Patients taking immunosuppressant

63. • Major signs of BL
• Soft tissue mass associated with involvement of the jaw
or other facial bones
• Enlarged cervical lymph nodes
• Abdominal masses
• Ascitis

64. Histopathology –
• Proliferation of B lymphocytes
characterized by sheets of
uniform tumor cells
• Tumor cells exhibit round
nuclei with minimal cytoplasm
• “Starry sky” appearance:
Scattered macrophages with
abundant clear cytoplasm,
containing phagocytic cellular
debris

65. • Unifocal, monoclonal, neoplastic proliferation of plasma
cells, usually arises within bone
• Solitary bone plasmacytoma- Plasmacytoma of skeletal
system
• Extramedullary plasmacytoma-Soft tissue plasmacytoma
Clinical features
• Age-50-60 yrs, average 55 yrs
• Sex-M>F
• Site-red marrow containing axial skeleton(vertebral
column)
• Pain at site of skeletal lesion
• Discovered in routine radiographs

66. Oral manifestations
• Maxilla & mandible
• Extramedullary- gingiva, palate,
floor of mouth, tongue,
tonsils,etc.
Radiographic features
• Unilocular ,well defined
radiolucency with no evidence of
sclerotic borders

68. • Aggressive part of a spectrum of plasma cell neoplasms-
Multiple myeloma
• Multicentric in origin
Clinical features
• Age- typically disease of adults ,over 60 yrs, rarely before
40
• Sex-M>F
• Race-Blacks> Whites
• Bone pain-characteristic feature
• Pathologic #, Fatigue, Anemia.

69. Oral manifestations
• Mandible>maxilla
• Pain
• Swelling
• Expansion of jaws
• Mobility of teeth
Laboratory findings
• Serum-monoclonal Hypergammaglobulinemia
• Urine- Bence Jones Proteins