Malignant tumors of connective tissue origin

MALIGNANT TUMORS OF
CONNECTIVE TISSUE ORIGIN
Dr. Prashanth

Components of connective tissue
 Fibers- fibroblasts
 Cartilage – chondroblasts
 Bone – osteoblasts
 Blood vessels
 Nervous tissue
 Lipid tissue
 Lymphoid tissue
 Blood

Fibrosarcoma
 Malignant tumor of fibroblasts
 Earlier days it was considered a common
tumor. Because of separation of other
tumors from the group,, it is less
commonly diagnosed today
 10% occur in head and neck.

Clinical features
 Slow growing
 More in femur and tibia
 Oro-facial region nose and para nasal
sinus common, produces obstruction
 Common in young adults and children.
 D/D includes fibrous dysplasia, fibrous
histiocytoma, osteosarcoma, fibrous
histiocytoma,, etc

Histopathology
Herring bone pattern
3 grades: - Well differentiated
- Intermediate grade
- High grade

Contd..
 Well –differentiated
a) Show typical herring bone pattern
b) Malignant cells don’t show much
pleomorphism
c) But mitotic figures are seen
 Intermediate grade- little higher degree of
pleomorphism.
 High grade – high pleomorphism, more
mitotic figures, difficult to diagnose without
immunohistochemistry. Less collagen

Treatment
 Surgical excision
 50% recurrence rate.

Liposarcoma
 Malignant tumor of adipose tissue
 Second most common soft tissue tumor,
but head and neck region it is rare.

Contd..
 Primarily seen in adults
 Soft ,slow growing , ill-defined mass
 Yellowish colored
 Pain is not a common feature, seen during
late stage
 Neck is the common region
 In oral cavity cheek is the common region
 Tongue is second common region

Histopathology
 5 types
a) Myxoid
b) Round cell type
c) Well differentiated
d) Dedifferentiated
e) Pleomorphic
Common one is myxoid variety

 Myxoid – stroma is myxoid
 Round cell – most aggressive form
 Well differentiated- resembles lipoma
 De differentiated- combination of well
differentiated and poorly differentiated
 Pleomorphic – extreme cellular
pleomorphism with giant cells

Treatment
 Radical excision
 10 year survival rate is 50%.

Malignant fibrous histiocytoma
 Most commonly diagnosed of all
sarcomas of adults
 50 to 70 years of age
 Common in males
 Looks moderately firm, with or without
pain or ulceration, irregular nodular lesion.

Histopathology
 Many histological subtypes described but
STORIFORM-PLEOMORPHIC type is
common
 Storiform Pattern ; pattern seen by low-
power light microscopy, which is
characterized by loosely-arranged whorls
of elongated, spindled fibroblast-like cells.

Hemangiopericytoma
 Stout and Murray described this in 1942
 Benign but has a malignant counterpart
 Chromosomal translocations are detected

Clinical features
 Reddish to bluish mass
 Arises before to second decade or after
seventh decade
 No gender predilection
 Soft or rubbery in consistency
 Well demarcated from the surroundings
 Oral cavity is also one of the common
sites

Histopathology
 Derived from pericytes
 Consists of many vascular channels lined
by plump endothelial cells surrounded by
tightly packed oval and spindle cells
 STAGHORN pattern --- branching
vascular channels of varying sizes.

Kaposi’s Sarcoma
 First described by Moritz Kaposi in 1872.
 Earlier to HIV epidemic it was a rare tumor,
but now it is common because of its
propensity to develop in HIV infected
individuals.
 It is caused by HHV 8
 Lesion arises from endothelial cells
 4 types : Classic
Endemic (African)
Iatrogenic immunosuppression –
associated

Clinical features
 Classic type
Disease of late adult type
90% occurs in men
Multiple bluish purple macules and
plaques are
present on the skin of lower extremities
Lesions grow slowly and develop into
painless
nodules
Oral lesions are rare , if present they
involve the

Endemic type
 Seen in African people
 Varies from classical type presentation to
lymphadenopathic type which shows
tumors of lymphatic system.
IATROGENIC type shows features of classic
form but usually caused several months
after the transplant, as a result of
immunosuppressive drugs

Histopathology
 3 stages are seen
a) Patch
b) Plaque
c) Nodular
Patch stage – proliferation of immature
vessels which results in irregular jagged
vascular network that surrounds
preexisting vessels

 Plaque stage : further proliferation of
vascular channels with development of
spindle cell component
 Nodular stage: spindle cell population
increases to form a nodular tumor like
mass resembling fibrosarcoma or any
spindle cell tumor
The spindle cells infiltrate through
the collagen, forming slit like spaces,
especially towards the periphery of the

Treatment
 Depends on the subtype and stage
 For skin lesions radiation therapy
 For oral lesions it is a combination of
radiation therapy, chemo and surgery
 Only 10 to 20 % die
 Around 25% die due to secondary
malignancies that develop in
lymphoreticular system

Malignant peripheral nerve sheath
tumor
 Earlier was called as Neurofibrosarcoma
now designated as MPNST.
 Accounts for 10% of all soft tissue
malignancies
 Most common on extremities and trunk
 Oral tumors are rare , but most common
sites are mandible, lips and buccal
mucosa.

 Common in young adults
 The mean age in patients with
neurofibromatosis (29 to 36 yrs) is about
one decade younger than in those without
this condition ( 40 to 46 yrs)
 Tumor enlarges and sometimes exhibits
rapid growth

 Fascicles of atypical spindle cells
 Resemble fibrosarcoma
 Cells are more irregular in shape with
wavy or comma shaped nuclei
 In some tumors skeletal muscle, cartilage
or bone differentiation can be seen and
these tumors are designated as Malignant
triton tumors

Treatment
 Radical excision with radiotherapy and
chemotherapy
 Prognosis is poor especially in patients
with neurofibromatosis

Chondrosarcoma
 malignant tumor characterized by the
formation of cartilage
 Primarily a tumor of adulthood and
majority of patients are above 50yrs
 Long bones involved commonly, oral
involvement is rare
 Maxilla is commonly affected than
mandible in a ratio of 4:1

 Painless mass is a common presenting
sign
 Associated with loosening and separation
of teeth
 Nasal obstruction, congestion,, epistaxis,,
photophobia, vision loss

Radiological features
 Ill defined radiolucency with scattered and
variable amounts of radiopaque foci due
to calcification of cartilage matrix
 Some cases show densely calcified
masses

Histopathology
 Chondrosarcoma are composed of cartilage
showing varying degrees of maturation
 Typical lacunar formation within the
chondroid matrix is seen
 3 histological grades
- Grade 1
- Grade 2
- Grade 3
Grade 1 resembles chondroma while grade3 is
highly cellular with mitosis and plemorphism
Variants like clear cell type,, dedifferentiated
types can be found

Treatment
 Depends on the size and location of the
tumor
 Complete radical excision is the treatment
of choice
 Head and neck tumors show very poor
prognosis

Osteosarcoma
 Malignancy of mesenchymal cellsthat
have the ability to produce osteiod or
immature bone.
 Excluding hematopoietic malignancies
osteosarcomas are the common
malignancies that arise within bone.

Clinical features
 Demonstrate bimodal age distribution – 10
to 20 yrs and in people above 50 yrs.
 Osteosarcomas of jaw bone are
uncommon and represent only 6 to 8%.
 Common in third decade with a mean age
of 33 yrs.
 Male predominance is seen
 Maxilla and mandible are involved with
equal frequency

 Pain and swelling are the common
symptoms
 Loosening of teeth, parasthesia and nasal
obstruction are other associated
symptoms
 few people are symptomless for a long
period of time before diagnosis, indicating
that jaw osteosarcomas are slow in
growth.

Radiographic features
 Sunburst appearance
 Ill defined borders
 Symmetrical widening of the periodontal
ligament space around single or multiple
teeth.
 This may be an earlier sign

Histopathology
 Osteoid material
 Round cells to highly pleomorphic cells
 Sometimes chondroid material or highly
fibrous connective tissue
 Depending on the amount of osteoid,
cartilage or fibers tumor is categorized into
 Osteoblastic
 Chondroblastic
 Fibroblastic

Treatment
 Excision
 Radio therapy
 Chemotherapy
 If diagnosed early and excision is
achieved totally then 80% survival rate.

Ewing’s sarcoma
 It’s a distinctive primary malignancy of
bone composed of small round cells
 Neuroectodermal in origin
 A reciprocal translocation is seen between
chromosome 11 and 22.
 6% of all bone malignancies and third
after osteosarcoma and chondrosarcoma

Clinical features
 Peak prevalence in 2nd decade of life, with
80% younger than 20yrs
 Male predilection
 Whites more than blacks
 Jaw bones – 1% to 2%
 Indian data : Ewing sarcoma comprised
around 15 % of all bone malignancies.
 68% percent were 0–19 years, with 1.6
times risk of tumor in bones of limbs as
compared to other bones.
 The highest incidence rate (per million) was
in the 10–14 years age group (male −4.4,
female −2.9)

 Pain with swelling is the common feature
 Pain is intermittent in nature with dull to
severe
 Fever, leukocytosis and elevated ESR
leads to misdiagnosis of osteomyelitis
 Usually tumor penetrates the cortex and
lies over the bone as a soft tissue mass
 In oral cavity mandible is commonly
affected than maxilla

Radiological features
 Onion peel appearance seen in long
bones, it’s a periosteal reaction
 Jaw bones – ill defined radiolucency

Histopathology
 Sheets of small round cells with well
delineated nuclear outline and ill defined
cell borders
 Nests of tumor cells are separated by
fibrous septa
 If large round cells are seen – large cell or
atypical Ewing’s sarcoma
 75% contains glycogen granules
 Diagnosis is difficult and should be
differentiated from other round cell tumors
 IHC is a must – CD99

Treatment
 Excision, radiotherapy and multidrug
chemotherapy has increased the
prognosis from 5% to 80%.

Hodgkin’s lymphoma
 Malignant lympho-proliferative disorder
 Neoplastic cells are called as Reed-
Sternberg cells
 They make up for only 1% to 3% of the
mass, making it difficult for the diagnosis
 Linked to Epstein Barr virus
 100 % cure achievable

Clinical features
 Always begins in a lymphnode or a group of
lymph nodes
 The common sites are cervical and
supraclavicular nodes upto 75%
 Male predilection is observed
 Bimodal age presentation one peak – 15 to
35
another – above 50
yrs
 Presenting sign is persistently enlarging,

 Initially the nodes are movable later they
become fixed
 If untreated the lesion spreads to other
lymph node group
 Finally involves extra lymphatic tissues
like bone, liver, spleen and lungs
 Associated with other symptoms like
weight loss, fever, night sweats and
generalized itching
 Absence of these signs and symptoms are
considered better in terms of prognosis

Staging – Ann Arbor system
 Staging is important for the treatment
planning and estimating the prognosis

Histopathology
 Two variants - 1. nodular lymphocyte
predominant
2. classical type ( 5 sub-
types)
- lymphocyte rich
- nodular
sclerosis
- mixed cellularity
- lymphocyte
depletion
- unclassifiable

Reed – Sternberg cell
 Presence of this cell is very important for
diagnosis
 It is an atypical neoplastic lymphocytic cell
 Typically bi-nucleated – Owl’s eye
appearance
 In lymphocyte pre-dominant type it is
called as ‘POPCORN” cell

1. nodular lymphocyte predominant – popcorn cell
2. classical type ( 5 sub-types)
- lymphocyte rich – sheets of small lymphocytes with few
RS cells
- nodular sclerosis – makes upto 60 to 80% of cases
Gets its name because of the broad fibrotic bands
extending from the lymph node capsule into the
lesional tissue. RS cells appear to reside in lacunae,
so called as lacunar cells
- mixed cellularity – 15% of cases, mixture of small
lymphocytes, plasmacells, eosinophils and histiocytes,
RS cells are abundant.
- lymphocyte depletion – most aggressive form, contains
lot of bizarre RS cells and less lymphocytes

Treatment
 Radiotherapy
 Chemotherapy either MOPP(
mechlorethamine, oncovin, procarbazine
and prednisone)
Or ABVD ( adriamycin, bleomycin,
vinblastin, DTIC)
Survival rate is about 75% for ten years.

Non- Hodgkin’s lymphoma
 Diverse and complex group of malignancies
of lympho-reticular system
 Initially begins in lymph nodes and tend to
grow as solid masses
 Most commonly B-cell lineage, T-cell origin
are rare
 Three categories based on American system
– “working formulation for clinical use”
- Low grade ( 35to 40%)
- Intermediate grade ( 60%)
- High grade ( 5%)

Eitiology
 Congenital immunological deficiencies
 AIDS
 Organ transplants
 Auto immune diseases
 Epstein Barr virus - Burkit’s lymphoma
 HHV type 8
 HTLV-1

Clinical features
 Occurs primarily in adults
 Commonly develops in lymph nodes
 Non tender mass slowly enlarging for
months
 Initially two or three freely movable nodes
are noticed and as the malignancy
progresses nodes become numerous and
are fixed to adjacent structures
 Gradually involves two or three lymph
node groups are affected

 In oral cavity it appears as a extra nodal
disease
 It may develop in oral soft tissues or
centrally with in the jaws
 Patient complains of ill fitting dentures
 Lesions have boggy consistency ( muddy)
 Central lesions present with mild pain
which can be mistaken for tooth ache

Histopathology
 Proliferation of lymphocytic appearing
cells showing varying degrees of
differentiation
 Low grade – shows well differentiated
lymphocytes and high grade shows less
differentiated cells
 Nodular pattern – germinal center
formation
 Diffuse type – no resemblance to normal
architecture
 If arises within the node- destroys the

 Radiotherapy
 Multi-agent chemotherapy
 Watch and wait

Burkitt’s lymphoma
 Malignancy of B- lymphocyte origin
 Dennis Burkitt was the first person to
document the process.
 Types – 1. prevalent in Africa and have
predilection for jaws – African Burkitt’s
lymphoma
2. prevalent in other areas of world
Endemic Burkitt’s lymphoma
3. Sporadic or American Burkitt’s
Lymphoma

Clinical features
 50 to 70% of African type present in jaws
 Usually affects children ( peak prevalence
about 7years of age)
 Maxilla is more commonly affected than
mandible
 Posterior jaw is commonly
 American type – tend to affect wider age
group
- abdominal region is
typically affected, jaw lesions rare

 Facial swelling
 Proptosis
 Pain
 Tooth mobility
 Premature exfoliation of deciduous teeth and
enlargement of gingiva and alveolar bone
 Ill defined radiolucency
 Burkitt’s lymphoma is a highly
aggressive tumor with a doubling time of
twenty-four hours

Histopathology
 STARRY SKY appearance
 Sheets of small lymphocyte appearing
cells with round nuclei with minimal
cytoplasm
 Each nucleus has several prominent
nucleoli and numerous mitosis

Starry sky appearance
 Starry sky
appearance-
presence of
histiocytes with
abundant cytoplasm
( stars) in the
background of
malignant darkly
staining lymphoma
cells ( night sky)

Treatment
 Intensive chemotherapeutic regimen, use
of cyclophosphamide – 90% of people
respond to this
 Survival rate is increased to 85% due to
intensive multi-agent chemotherapy

Multiple myeloma
 Malignancy of plasma cell origin with
multi-centric origin within bone
 The abnormal plasma cells that compose
this tumor are monoclonal
 Since all malignant cells develop from a
single precursor cell , the genetic make up
of neoplastic cells are similar and all
produce same immunoglobulin

Clinical features
 Typically a disease of adults
 Diagnosis will be usually between 60 to
70% of cases
 Bone pain is the characteristic presenting
feature
 Anemia results in fatigue
 Petechial hemorahages of skin and oral
mucosa – due to lack of platelet
production
 Metastatic calcifications of the soft tissues

Bence-Jones proteins
 A Bence Jones protein is a monoclonal
globulin protein or immunoglobulin light
chain found in the urine, with a molecular
weight of 22-24 kDa
 Renal failure because of overburdening of
kidneys due to excess circulating Bence –
Jones proteins

Amyloid
 An abnormal fibrillar protein
 It is deposited in various soft tissues in
patients with multiple myeloma
 Oral mucosa, tongue- shows diffuse
enlargement
 Appear waxy firm and plaque like

Radiology
 Multiple well defined punched out
radiolucent lesion

Histopathology
 Diffuse monotonus sheets of neoplastic
plasmacytoid cells

Congo red stain
 To demonstrate amyloid
 Apple-green birefringence on viewing with
polarized light

Diagnosis
 Radiographic picture
 Histopathology
 Demonstration of ameloid
 Demonstration of BENCE-JONES protein
(M-PROTEIN) in urine by immuno
electrophoresis

TREATMENT
 Chemotherapy by using an alkylating
agent such as melphalan or
cyclophosphamide in conjugation with
prednisone

Plasmacytoma
 Unifocal, monoclonal, neoplastic
proliferation of plasma cells that arises
within the bone.
 Sometimes it is seen in soft tissues –
referred as EXTRA MEDULLARY
PLASMACYTOMA – 90% seen in head
and neck region.
 Mild variety of neoplasm but if not
detected and treated early it can give rise
to more serious tumor like multiple

 It’s a tumor of adult male diagnosed at
age of 55years
 In medullary variety spine is commonly
affected.
 Bone pain and swelling are the usual
associated symptoms
 Extramedullary 90% occurs in head and
neck region- tonsils, nasopharynx,
paranasal sinuses, nose and parotid gland
 Radiographically, the lesion is seen as
unilocular radiolucency without proper

Histopathology
 Identical to multiple myeloma
 Sheets of plasma cells with varying
degrees of differentiation

Treatment
 Radiation therapy – 4000 cGy

Leiomyosarcoma
 Malignant tumor of smooth muscles
 Common sites are uterine wall and
gastrointestinal tract
 Leiomyosarcomas of the oral cavity are
rare
 Common in middle aged and older adults
 Present as an enlarging mass with or
without pain

Histopathology
 Fascicles of spindle cells with abundant
eosinophilic cytoplasm and blunt – ended,
cigar – shaped nuclei
 Pleomorphism present
 Presence of five or more mitosis per ten
high power field should be considered
malignant
 Glycogen can be demonstrated within
cells by PAS staining
 IHC – desmin, smooth muscle actin,

Smooth
muscle
actin
Fascicles of
spindle
shaped cells
Cigar
shaped
nuclei

Treatment
 Radical surgical excision with adjunctive
chemotherapy and radiotherapy

Rhabdomyosarcoma
 Malignant tumor of skeletal muscle origin
 Most common soft tissue sarcoma of
children
 Most frequent site is head and neck
 Three basic microscopic patterns are seen
1. Embryonic
2. Alveolar
3. pleomorphic

Clinical features
 Primarily seen in first decade of life
 Rare in people older than 45 years of age
 60% occurs in males
 Embryonal rhabdomyosarcomas are most
common in first 10years of life and account for
60% of all cases
 Alveolar type occurs in persons between 10 to
25 years of age and account for 25 to 30% of
cases
 Pleomorphic type – over 40 years of age and

 Painless mass with infiltrative mass that
grow rapidly.
 In head and neck region , orbit is the
common location,
 Palate is the most frequent intra oral
location
 Certain rhabdomyosarcomas show
exophytic, polypoid growth pattern like
clusters of grapes these are called as
BOTRYOID (GRAPE LIKE)

Histopathology - Embryonal type
 Resembles various stages of
embryogenesis of skeletal muscle
 Vary from poorly differentiated to well
differentiated
 Poorly differentiated is difficult to diagnose
and consists of small round cells with
hyperchromatic nuclei and indistinct
cytoplasm
 Well differentiated shows round to oval
rhabdomyoblasts with distinct eosinophilic
cytoplasm

Alveolar type
 Aggregates of poorly differentiated round
to oval cells separated by fibrous septa

Pleomorphic type
 Loosely arranged and
haphazardly oriented
cells of variable
morphology. Both small
and large cells with
round or pleomorphic
shapes are present
 IHC stains for desmin
and myoglobin are
positive , so can be
distinguished from other
pleomorphic sarcomas

Treatment
 Surgery with multidrug chemotherapy
 Survival rate is increased to 60% now a
days

Malignant tumors of connective tissue origin

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