H&N Sarcomas of the head and neck Flashcards
What are the two most common head and neck
locations for osteosarcoma?
Mandible and maxilla
Hemangiopericytomas arise most commonly from what head and neck site?
The sinonasal cavity
What is the most common head and neck site of
origin of leiomyosarcoma?
The oral cavity
What is the most common head and neck site for
chondrosarcoma?
The sinonasal cavity
What hereditary syndrome is caused by a muta-
tion in the p53 tumor suppressor gene resulting in
a greatly increased risk of sarcomas as well as other cancers?
Li-Fraumeni syndrome
What condition is associated with half of all
neurogenic sarcomas (malignant peripheral nerve
sheath tumor)?
Neurofibromatosis type 1
What percentage of patients with head and neck
fibrosarcomas report a history of prior radiation
exposure?
10%
What are the four histologic subtypes of rhabdo-
myosarcoma?
● Embryonal
● Alveolar
● Anaplastic (previously pleomorphic)
● Mixed
What histologic subtypes of rhabdomyosarcoma
have the worst prognosis?
Alveolar and anaplastic
What is the most common head and neck sarcoma
in children?
Rhabdomyosarcoma
Head and neck rhabdomyosarcoma can be divided
into three sites that have staging and prognostic
value; what are they?
● Orbit
● Head and neck
● Nonparameningeal
● Parameningeal
What is the primary treatment for rhabdomyo-
sarcoma?
Chemotherapy is the mainstay of treatment. It is typically
used with radiation as an induction agent and then
concurrently. Typically, vincristine is the main agent used
with two other agents. These regimens have been
established by the Intergroup Rhabdomyosarcoma Studies
(IRS) now renamed the Children’s Oncology Group (COG)
What soft tissue sarcoma has the highest response
rate to adjuvant radiation therapy?
Liposarcoma
Synovial sarcoma is thought to be most likely
derived from what cell type?
Pluripotent mesenchymal cells
What are the most common head and neck sites
of origin for synovial sarcoma?
Hypopharynx and retropharynx
What recent chromosomal translocation has been identified in patients with epithelioid heman-
gioendothelioma?
t(1;3)(p36;q25)
What prognostic factor is included in the AJCC
staging system for sarcomas in addition to the
traditional TNM staging factors?
Histologic grade (G1–G3)
What is the T-staging system of soft tissue
sarcomas according to the AJCC?
● Tx: Primary tumor cannot be assessed
● T0: No evidence of primary tumor
● T1: Tumor < 5 cm in greatest dimension (T1a, superficial;
T1b, deep);
● T2: tumor > 5 cm in greatest dimension (T2a, superficial;
T2b, deep)
What prognostic factor at the time of surgical
excision of head and neck osteosarcoma plays the
most significant role in local control and survival
rates?
Surgical margin status
What is the primary treatment modality for osteosarcoma of the head or neck?
Surgery
What is the treatment modality of choice for
dermatofibrosarcoma protuberans?
Surgical resection
What is the classic initial symptom of angiosarcoma?
Unexplained bruising of the forehead or scalp, which may
progress in a rapid fashion in an elderly patient.
What features are associated with most strongly
associated with prognosis in angiosarcoma?
Size, grade, and depth. Tumors < 5 cm and superficial
tumors have significantly better survival than tumors > 5 cm
or deeply invasive tumors. High-grade tumors have also
been associated with worse prognosis.
What percentage of angiosarcomas will occur in
the head and neck region?
50%
What is the standard treatment for scalp angio-
sarcoma?
Wide local excision with postoperative radiation therapy
What areas of the head and neck are most likely to be involved with dermatofibrosarcoma protuber-
ans?
Scalp and supraclavicular fossa
What is the long-term prognosis for patients with dermatofibrosarcoma protuberans?
If it is adequately managed, dermatofibrosarcoma protuberans commonly recurs locally, but it seldom metastasizes.
Long-term survival is therefore excellent.
What modifications should be considered to Mohs surgery in the case of dermatofibrosarcoma, and
why?
Because of the infiltrating growth pattern exhibited by
dermatofibrosarcoma protuberans, some have advocated a
“modified Mohs” procedure with paraffin sections as
opposed to frozen sections, alternatively taking an extra
border of tissue from around the tumor.
Because of the infiltrating growth pattern exhibited by
dermatofibrosarcoma protuberans, some have advocated a
“modified Mohs” procedure with paraffin sections as
opposed to frozen sections, alternatively taking an extra
border of tissue from around the tumor.
Imatinib, a tyrosine kinase inhibitor, has been shown to induce partial or complete remission in dermatofibrosarcoma protuberans.
What are the relative recurrence rates for dermatofibrosarcoma protuberans treated with Mohs surgery and wide local excision respectively?
1.6% versus 20% (favoring Mohs) in a large meta-analysis
Describe the common history and findings in
patients with atypical fibroxanthoma.
A rapidly enlarging, red, ulcerated lesion within the field of
prior radiation treatment.
What is the treatment of choice for atypical
fibroxanthoma?
Simple excision with clear margins, often by Mohs surgery.
Nodal metastasis is rare.
How commonly does malignant fibrous histiocytoma recur?
Recurrence is common in malignant fibrous histiocytoma
and even more so in patients previously exposed to radiation. Local metastasis is uncommon, whereas distant
metastasis is more frequent.
Pleomorphic undifferentiated sarcoma is formally
known as what?
Malignant fibrous histiocytoma
To what site does pleomorphic undifferentiated
sarcoma most often metastasize?
Lungs
What are common histologic features of pleomorphic undifferentiated sarcoma (malignant fi-
brous histiocytoma)?
A storiform pattern of pleomorphic and bizarre cells with
foamy cytoplasm, marked atypia, and numerous mitotic
figures in a collagenous background
What are the risk factors for Kaposi sarcoma?
All Kaposi sarcomas are caused by HHV-8, but the groups
most at risk for the disease are patients with AIDS and those
who are on immunosuppression medications. Patients with
AIDS are 20,000 times more likely to have Kaposi sarcoma
than the general population and 300 times more likely than
renal transplantation patients.
