Sarcomas of The Head and Neck: Dr. Darwito
Sarcomas of The Head and Neck: Dr. Darwito
Sarcomas of The Head and Neck: Dr. Darwito
including muscle,
endothelial cells,
cartilage,
and supporting elements.
This sub classification also includes tumors of
peripheral nerve origin.
2
in adults,:
osteosarcoma,
angiosarcoma,
malignant fibrous histiocytoma,
and fibrosarcoma are most common.
6
10
Angiosarcoma
Hemangiopericytoma
Malignant fibrous histiocytoma
Synovial sarcoma
Chondrosarcoma
Rhabdomyosarcoma
Malignant schwannoma
Liposarcoma
Leiomyosarcoma
Fibrosarcoma
Alveolar soft part sarcoma (ASPS)
Kaposi sarcoma (KS)
11
12
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
factor VIII,
vimentin,
CD31,
CD34,
ulex europeus,
laminin,
BNH9,
and monoclonal antibody EN4 is used to diagnose
angiosarcoma
19
21
22
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
27
28
REGIMENT:
Vincristine, doxorubicin, and cyclophosphamide
alfa-interferon may be of benefit in patients
with pulmonary metastases or unresectable primary tumors.
29
33
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
treatment of choice
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
Treatment
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
embryonal,
alveolar,
pleomorphic,
and mixed
Therapy
47
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
PROGNOSIS
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
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
58
59
60