Sarcomas

of the Head and

Neck
Dr. Darwito
SUB BAG BEDAH ONKOLOGI
(HNB)

 Sarcomas are relatively uncommon tumors,

accounting for 1% of all malignancies
Soft tissue sarcomas arise from the
mesenchyme,:

including muscle,
endothelial cells,
cartilage,
and supporting elements.
This sub classification also includes tumors of
peripheral nerve origin.
2

 Approximately 80% of sarcomas originate

from soft tissues, while 20% arise from
bone.
Fewer than 5000 cases of sarcomas
occur per year in the United States

 Approximately 15-20% of sarcomas occur

within the head and neck;
the paranasal sinuses and neck are the
most frequent sites of origin
Approximately 80% of head and neck
sarcomas occur in adults
10-20% occur in children
4

 Certain sarcomas are related to:

1.genetic syndromes (eg, Li Fraumeni
syndrome, osteosarcoma),
2.environmental exposures (eg,
radiation, multiple sarcoma types),
3. medical conditions (eg, lymphedema,
angiosarcoma).

 In the head and neck, the most common

sarcoma in children is
rhabdomyosarcoma

in adults,:

osteosarcoma,
angiosarcoma,
malignant fibrous histiocytoma,
and fibrosarcoma are most common.
6

 Regional metastases occur in 10-15% of

head and neck sarcomas overall; most of
these arise from high-grade primary
lesions
Distant metastases are rare in the
absence of regional metastases,
presence of nodal metastases should
prompt a search for distant metastases
7

 The most common site of distant

metastases:
is the lung,
followed by the liver
and bone.

Local recurrence is common, and it is the

most common cause of treatment failure

 Imaging studies are used to demonstrate

the extent of tumor involvement :
1.CT scanning is useful in demonstrating bony
involvement and tumor calcification
2.MRI is superior to CT scanning in its ability to
depict the extent of soft tissue involvement by
sarcomas
Occasionally, imaging findings can suggest the
correct diagnosis, but definitive diagnosis requires
histologic evaluation of a representative biopsy
specimen.
9

 Sarcomas are commonly classified

according to their site of origin:
soft tissues
or bone.

Sarcomas involving cartilage

(chondrosarcoma) and peripheral nerve
tissue (malignant schwannoma)

10

 Soft tissue sarcomas include the following :

Angiosarcoma
Hemangiopericytoma
Malignant fibrous histiocytoma
Synovial sarcoma
Chondrosarcoma
Rhabdomyosarcoma
Malignant schwannoma
Liposarcoma
Leiomyosarcoma
Fibrosarcoma
Alveolar soft part sarcoma (ASPS)
Kaposi sarcoma (KS)
11

 Bone sarcomas include the following :

Osteosarcoma
Ewing sarcoma

12

STAGES OF SOFT TISSUE SARCOMAS

American Joint Committee on Cancer (AJCC) /International Union
Against Cancer (UICC)
Primary tumor
Tx - Primary tumor cannot be assessed
T0 - No evidence of primary tumor
T1 - Tumor less than 5 cm in greatest dimension (T1a, superficial; T1b, deep)
T2 - Tumor greater than 5 cm in greatest dimension (T2a, superficial; T2b, deep)

Regional lymph nodes

Nx - Lymph nodes cannot be assessed
N0 - No lymph nodes metastases
N1 - Lymph nodes metastases present

Distant metastases
Mx - Distant metastases cannot be assessed
M0 - No distant metastases
M1 - Distant metastases present

13

Histopathologic grade
Gx - Grade cannot be assessed
G1 - Well differentiated
G2 - Moderately differentiated
G3 - Poorly differentiated
G4 Undifferentiated

Combined
IA (G1-2, T1a-b, N0, M0) - Low-grade, small, and superficial or deep tumor
IB (G1-2, T2a, N0, M0) - Low-grade, large, and superficial tumor
IIA (G1-2, T2b, N0, M0) - Low-grade, large, and deep tumor
IIB (G3-4, T1a-b, N0, M0) - High-grade, small, and superficial or deep tumor
IIC (G3-4, T2a, N0, M0) - High-grade, large, and superficial tumor
III (G3-4, T2b, N0, M0) - High-grade, large, and deep tumor
IV (any G, any T, N1, M0) - Any metastasis

14

Angiosarcoma
Angiosarcomas account for fewer than 1%
of all sarcomas.
This rare malignancy arises from
endothelial cells of either :
lymphatic (the lesion is termed a
lymphangiosarcoma; )
or vascular origin (term is hemangiosarcoma)

15

 Approximately half of these lesions occur

in the head and neck.
The scalp and face are the most common
sites of involvement.
Angiosarcomas typically occur in those
aged 50-70 years, and males are more
commonly affected than females, with a
3:1 ratio
While most patients are asymptomatic at
the time of diagnosis, some have pain or
bleeding at the tumor site
16

 associated with angiosarcoma :

Long-standing lymphedema
Prior irradiation.
Exposure to vinyl chloride is associated with
angiosarcomas of the liver
Trauma, either in the form of direct tissue
injury or an adjacent foreign body or
prosthesis
17

 The AJCC staging system for soft tissue

sarcomas is used to grade angiosarcomas.
Low-grade lesions appear as raised, red or
purple, painless papules or nodules. Often, small
lesions surround a central lesion.
Low-grade lesions have minimal cellular atypia and
few mitotic figures, and 1 or more nodules may be
present in the dermis.

High-grade lesions are usually multiple and

ulcerated, with extensive local infiltration.
High-grade lesions have greater pleomorphism, more
hyperchromatic nuclei, and more mitotic figures
18

 Immunohistochemical staining for endothelial

cell markers, including :

factor VIII,
vimentin,
CD31,
CD34,
ulex europeus,
laminin,
BNH9,
and monoclonal antibody EN4 is used to diagnose
angiosarcoma
19

Wide local excision is the mainstay of treatment

Prognosis appears to be most affected by the ability

to perform complete surgical excision
TREATMENT

local recurrence occurs in more than 50% of cases

and is the most common pattern of treatment failure

Most recurrences are evident within 2 years of initial treatment.

Regional metastases are less common,
occurring in fewer than 20% of cases in most large series

regional lymph node dissection is recommended

in patients with scalp lesions or palpable lymphadenopathy
20

 Distant metastases occur in 30-50% of

cases
lungs and liver most frequently involved
Five-year survival rates of 12-41% have been
reported;
most investigators report survival rates lower than
30%.
lesions smaller than 7 cm are associated with a
better prognosis

21

Adjunctive radiation therapy is used

to improve locoregional control
Doses of 6000-7000 rads (6000-7000 cGy)

in high-grade tumors and

tumors in which resection margins are close.

22

 Work in molecular genetics holds promise

in improving survival rates in patients with
angiosarcomas :
mutations in the p53 tumor suppressor gene,
the c-ras proto-oncogene,
and the erbB proto-oncogene exist.

23

Hemangiopericytoma
Hemangiopericytomas are rare and account for
less than 1% of vascular neoplasms
These tumors arise from the vascular pericytes
of Zimmerman, which occur around capillaries
and postcapillary venules.
About 15-30% of hemangiopericytomas occur in
the head and neck; the sinonasal tract is the
most common site.

24

 No sex predilection exists, and 90% of

hemangiopericytomas arise in persons aged 5070 years
At gross examination, hemangiopericytomas
typically appear as bland polypoid growths in the
sinonasal tract.
In other regions, the typical presentation is that
of a slowly growing painless mass.
When they are large, a bruit may be auscultated.
Biopsy can be associated with significant
bleeding
25

 Lesions associated with a poorer

prognosis ;
are those larger than 6.5 cm with more than 3
mitotic figures per 10 high-power fields,
necrosis,
and hemorrhage.

Survival rates decrease dramatically when

more than 4 mitotic figures are present per
10 high-power fields.
26

 Complete surgical excision is the treatment of

choice for hemangiopericytomas
Local recurrence rates of 10-50% have been
reported; fewer than 20% of patients have local
recurrence after surgical excision
Regional and distant metastases occur in fewer
than 10% of cases and are usually associated
with recurrent disease at the primary site.

27

 An overall 10-year survival rate of 70%

has been reported
in patients with lesions with aggressive
histologic characteristics (eg, >4 mitoses
per 10 high-power fields, necrosis,
hemorrhage, large size) decreases to
29%.

28

 Hemangiopericytomas are considered relatively resistant

to radiation therapy
Chemotherapy is not standard in the treatment of
hemangiopericytomas, although adjuvant chemotherapy
may have a role in the treatment of distant metastatic
disease and when primary resection is not possible
without significant morbidity or disfigurement

REGIMENT:
Vincristine, doxorubicin, and cyclophosphamide
alfa-interferon may be of benefit in patients
with pulmonary metastases or unresectable primary tumors.
29

Malignant fibrous histiocytoma

Malignant fibrous histiocytoma is the most
common soft tissue sarcoma in adults.
Malignant fibrous histiocytomas are most
common in the soft tissues of the
abdomen and extremities, although 23%
occur in osseous sites.
Approximately 1-3% of malignant fibrous
histiocytomas occur in the head and neck
30

 The development of malignant fibrous

histiocytoma is associated with :
previous radiation treatment and,
less commonly, with the injection of silica as a
sclerosing agent.

Malignant fibrous histiocytoma is thought to

originate from fibroblasts or from a
mesenchymal precursor cell that can
differentiate into fibroblasts and histiocytes.
31

 Giant cell malignant fibrous histiocytoma

involves multinucleated giant cells,
histiocytes, and fibroblasts.
Often, osteoids accumulate at the
periphery of the lesion.
The angiomatous and myxoid subtypes
have the best prognosis because of a
lower propensity for systemic metastases.
32

 Immunohistochemistry is of little value in

the diagnosis of malignant fibrous
histiocytoma because no specific marker
for these lesions exists.
The diagnosis is made on the basis of the
histologic appearance.

33

The World Health Organization classification of

fibrous histiocytomas is as follows
Benign

Intermediate

Fibrous histiocytoma
Dermatofibroma
Deep histiocytoma
Juvenile xanthogranuloma
Reticulohistiocytoma
Xanthoma

Atypical fibroxanthoma
Dermatofibrosarcoma protuberans
Giant cell fibroblastoma
Plexiform fibrohistiocytic tumor
Angiomatoid fibrous histiocytoma

Malignant
Storiform-pleomorphic tumor
Myxoid tumor
Giant cell tumor
Xanthoma (inflammatory tumor)
34

 The overall mortality rate with malignant fibrous

histiocytoma is 40-45%.
Age appears to be correlated with mortality; one
group reported a mortality rate of 72% in
patients older than 60 years and a 33% mortality
rate in patients aged 20-39 years.
Local recurrence is present in 20-42% of cases;
Regional lymph node involvement occurs in 015% of cases;
Distant metastases are found in 25-35% of
cases.
Recurrence usually occurs within 2 years of
treatment
35

treatment of choice

Complete surgical resection

lymph node dissection if palpable cervical metastases.
Because regional lymph node metastases are present in fewer than 15% of patients
exception of tumors in the oral cavity
Adjuvant radiotherapy is reserved for
lesions with close or positive resection margins;
systemic chemotherapy is used to treat distant metastatic disease.

36

Synovial sarcoma
Synovial sarcomas represent 6-10% of all
soft tissue sarcomas.
Only 3-10% of synovial sarcomas arise in
the head and neck.
These tumors occur in younger people;
peak incidence is in those aged 20-40
years

37

 The most common presentation is that of:

a painless mass;
various associated symptoms may be
present,
depending on the location of the tumor.

Calcifications are present in more than

50% of tumors and may be noted on
radiography; this finding suggests the
diagnosis
38

 Synovial sarcomas do not arise from

synovial tissue, but rather, they originate
from pluripotential mesenchymal cells, and
they rarely occur within joint spaces.
Mesenchymal cells differentiate into 2
components:
an epithelial-like cell layer
and a connective tissue layer of spindleshaped cells.
39

 Three subtypes of synovial sarcoma are

described: biphasic, monophasic, and poorly
differentiated. Biphasic synovial sarcomas are
composed of epithelioid and spindle cells
Monophasic synovial sarcoma is composed of
one cellular type and may be derived from
epithelioid or spindle cells.
epithelioid stain positively for cytokeratin and
epithelial membrane antigen (EMA)
Spindle cells stain positively with vimentin, a
mesenchymal marker
40

 The AJCC classifies all synovial sarcomas as

high grade.
Regional lymph node metastases occur in fewer
than 5% of synovial sarcomas, whereas distant
metastases, most frequently to the lung, occur in
50% of cases
Local recurrence is present in approximately
40% of patients
Factors associated with a poor prognosis include
increasing age, tumor size greater than 5 cm,
and mitotic activity
41

Treatment

Surgical excision combined with postoperative radiation therapy

is the primary treatment
The addition of adjuvant radiotherapy
to surgery reduces the recurrence rates to 28-49%
Chemotherapy with ifosfamide compounds appears
to be of benefit in the treatment of distant metastases.

42

Rhabdomyosarcoma
Rhabdomyosarcoma accounts for 19% of all
sarcomas, and it is the most common soft tissue
sarcoma in children
The head and neck is the most common site of
occurrence; 45% of rhabdomyosarcomas occur
here
80% of rhabdomyosarcomas occur in children
younger than 12 years,
The most commonly involved sites are the orbit,
nasopharynx, temporal bone, and sinonasal
tract.
43

 Rhabdomyosarcoma is a malignant tumor of the

skeletal muscle cells, and it arises from the
rhabdomyoblast
In the head and neck, 4 subtypes of
rhabdomyosarcoma have been described:

embryonal,
alveolar,
pleomorphic,
and mixed

The embryonal subtype is the most common

(>70%),
44

 Pleomorphic rhabdomyosarcoma is the

least common subtype, and it is more
common in older patients
Rhabdomyosarcomas are
immunoreactive to desmin and
myoglobulin. Additionally, reactivity to
muscle-specific actin occurs in more
than 75% of cases
The outcome varies with the location of
the primary tumor, its size, local
recurrence, and metastasis
45

 The Intergroup Rhabdomyosarcoma

Study Group has classified lesions as :
orbital, parameningeal (includes temporal bone
and ear, paranasal sinuses, nasopharynx, and
infratemporal fossa),
and all other head and neck sites.

With this classification, 5-year survival

rates exceed 90% in orbital tumors,
69% in parameningeal sites, and 80% in
all other head and neck sites.
46

Therapy

Multimodality therapy with combination chemotherapy

(vincristine, actinomycin D, cyclophosphamide, adriamycin)
external-beam radiation therapy and
nonradical surgery is superior to any single-modality therapy

Surgery has a smaller role

in the treatment of this sarcoma;
it is performed for biopsy and tumor debulking.

47

The UICC tumor, node, metastasis

staging system for rhabdomyosarcoma
Primary tumor
T1 - Tumor confined to organ or tissue of origin
T2 - Tumor involving one or more contiguous organs or tissues

Regional lymph node

Nx - Lymph nodes cannot be assessed
N0 - No lymph nodes metastases
N1 - Lymph nodes metastases present
Distant metastases
Mx - Distant metastases cannot be assessed
M0 - No distant metastases
M1 - Distant metastases present
48

Histopathologic grade
Gx - Grade cannot be assessed
G1 - Well differentiated
G2 - Moderately differentiated
G3 - Poorly differentiated
G4 - Undifferentiated

Histopathologic grade
Gx - Grade cannot be assessed
G1 - Well differentiated
G2 - Moderately differentiated
G3 - Poorly differentiated
G4 - Undifferentiated

49

The Intergroup Rhabdomyosarcoma Study

Group clinical staging system for rhabdomyosarcoma is as follows:

Group I - Localized disease with tumor completely resected and

regional nodes not affected, with the following:
(1) confinement to the muscle or organ of origin or
(2) contiguous involvement (ie, infiltration outside the muscle or organ of origin),
(1) as through fascial planes
Group II - Localized disease with microscopic residual disease or
regional disease and no residual or microscopic residual disease
and the following
: (1) grossly resected tumor with microscopic residual disease (nodes negative),
(2) completely resected regional tumor (nodes positive or negative),
or (3) regional disease with grossly resected involved nodes and
evidence of microscopic residual disease
Group III - Incomplete resection or biopsy sample with gross residual disease
Group IV - Metastatic disease present at onset
50

PROGNOSIS

With this staging classification, the 5-year

survival rates:
83% for stage I disease,
70% for stage II disease,
52% for stage III disease,
20% for stage IV disease.

51

Malignant schwannoma
Malignant schwannomas, also known as
malignant peripheral nerve sheath tumors
or neurofibrosarcomas,
arise from peripheral or cranial nerves
and account for 5-10% of all sarcomas.
The lower extremity is the most commonly
involved site; however, as many as 20% of
malignant schwannomas occur in the
head and neck.
52

 Malignant schwannomas may arise sporadically

or in association with von Recklinghausen
disease or neurofibromatosis type I (NF-1).
The sporadic form most commonly arises in
persons aged 40-60 years, and females are
affected more often than males
The malignant triton tumor is a rare lesion
consisting of a peripheral nerve sheath tumor
that contains foci of rhabdomyosarcoma.
One third of triton tumors arise in the head and
neck, and one third are associated with NF-1.
An association with previous irradiation also
exists.
53

 As many as 50% of patients with malignant

schwannomas have evidence of NF-1 or a
positive family history.
About 5-15% of patients with NF-1 have
malignant schwannomas
Symptoms vary according to the site involved,
but the most common presenting symptom is a
painful enlarging mass.
Paresthesias,
weakness,
and muscle atrophy may also be present.
54

 The term malignant schwannoma is misleading

because tumors can arise from any nerve
sheath cell, including perineural fibroblasts and
fibroblasts, as well as Schwann cells
Grading of malignant schwannoma is based on
the degree of :
cellular pleomorphism,
mitotic activity,
cellularity.

Most malignant schwannomas (80%) are highgrade lesions.

55

Treatment

primarily surgical;
adjuvant radiation therapy is often used
because most of these tumors are high grade
Chemotherapy may have a role in the treatment
of inoperable disease,
recurrent disease, or disease that persists despite initial therapy.

56

PROGNOSE
Cervical lymph node metastases are rare
Distant metastases occur in 33% of cases and
most commonly involve the lung or bone.
Local recurrence is present in 50% of patients.
The overall survival rate is 50%.
Tumors smaller than 5.0 cm are associated with
a more favorable prognosis; multiple tumors in
association with NF-1 are associated with a
poorer prognosis
In sporadic malignant schwannoma, reported 5year survival rates are 50-75%
57

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