H&N Sarcomas of the head and neck

Question 1

What are the two most common head and neck

locations for osteosarcoma?

Answer 1

Mandible and maxilla

Question 2

Hemangiopericytomas arise most commonly from what head and neck site?

Answer 2

The sinonasal cavity

Question 3

What is the most common head and neck site of

origin of leiomyosarcoma?

Answer 3

The oral cavity

Question 4

What is the most common head and neck site for

chondrosarcoma?

Answer 4

The sinonasal cavity

Question 5

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?

Answer 5

Li-Fraumeni syndrome

Question 6

What condition is associated with half of all
neurogenic sarcomas (malignant peripheral nerve
sheath tumor)?

Answer 6

Neurofibromatosis type 1

Question 7

What percentage of patients with head and neck
fibrosarcomas report a history of prior radiation
exposure?

Answer 7

10%

Question 8

What are the four histologic subtypes of rhabdo-

myosarcoma?

Answer 8

● Embryonal
● Alveolar
● Anaplastic (previously pleomorphic)
● Mixed

Question 9

What histologic subtypes of rhabdomyosarcoma

have the worst prognosis?

Answer 9

Alveolar and anaplastic

Question 10

What is the most common head and neck sarcoma

in children?

Answer 10

Rhabdomyosarcoma

Question 11

Head and neck rhabdomyosarcoma can be divided
into three sites that have staging and prognostic
value; what are they?

Answer 11

● Orbit
● Head and neck
● Nonparameningeal
● Parameningeal

Question 12

What is the primary treatment for rhabdomyo-

sarcoma?

Answer 12

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)

Question 13

What soft tissue sarcoma has the highest response

rate to adjuvant radiation therapy?

Answer 13

Liposarcoma

Question 14

Synovial sarcoma is thought to be most likely

derived from what cell type?

Answer 14

Pluripotent mesenchymal cells

Question 15

What are the most common head and neck sites

of origin for synovial sarcoma?

Answer 15

Hypopharynx and retropharynx

Question 16

What recent chromosomal translocation has been identified in patients with epithelioid heman-
gioendothelioma?

Answer 16

t(1;3)(p36;q25)

Question 17

What prognostic factor is included in the AJCC
staging system for sarcomas in addition to the
traditional TNM staging factors?

Answer 17

Histologic grade (G1–G3)

Question 18

What is the T-staging system of soft tissue

sarcomas according to the AJCC?

Answer 18

● 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)

Question 19

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?

Answer 19

Surgical margin status

Question 20

What is the primary treatment modality for osteosarcoma of the head or neck?

Answer 20

Surgery

Question 21

What is the treatment modality of choice for

dermatofibrosarcoma protuberans?

Answer 21

Surgical resection

Question 22

What is the classic initial symptom of angiosarcoma?

Answer 22

Unexplained bruising of the forehead or scalp, which may

progress in a rapid fashion in an elderly patient.

Question 23

What features are associated with most strongly

associated with prognosis in angiosarcoma?

Answer 23

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.

Question 24

What percentage of angiosarcomas will occur in

the head and neck region?

Answer 24

50%

Question 25

What is the standard treatment for scalp angio-

sarcoma?

Answer 25

Wide local excision with postoperative radiation therapy

Question 26

What areas of the head and neck are most likely to be involved with dermatofibrosarcoma protuber-
ans?

Answer 26

Scalp and supraclavicular fossa

Question 27

What is the long-term prognosis for patients with dermatofibrosarcoma protuberans?

Answer 27

If it is adequately managed, dermatofibrosarcoma protuberans commonly recurs locally, but it seldom metastasizes.
Long-term survival is therefore excellent.

Question 28

What modifications should be considered to Mohs surgery in the case of dermatofibrosarcoma, and
why?

Answer 28

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.

Question 29

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.

Answer 29

Imatinib, a tyrosine kinase inhibitor, has been shown to induce partial or complete remission in dermatofibrosarcoma protuberans.

Question 30

What are the relative recurrence rates for dermatofibrosarcoma protuberans treated with Mohs surgery and wide local excision respectively?

Answer 30

1.6% versus 20% (favoring Mohs) in a large meta-analysis

Question 31

Describe the common history and findings in

patients with atypical fibroxanthoma.

Answer 31

A rapidly enlarging, red, ulcerated lesion within the field of
prior radiation treatment.

Question 32

What is the treatment of choice for atypical

fibroxanthoma?

Answer 32

Simple excision with clear margins, often by Mohs surgery.

Nodal metastasis is rare.

Question 33

How commonly does malignant fibrous histiocytoma recur?

Answer 33

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.

Question 34

Pleomorphic undifferentiated sarcoma is formally

known as what?

Answer 34

Malignant fibrous histiocytoma

Question 35

To what site does pleomorphic undifferentiated

sarcoma most often metastasize?

Answer 35

Lungs

Question 36

What are common histologic features of pleomorphic undifferentiated sarcoma (malignant fi-
brous histiocytoma)?

Answer 36

A storiform pattern of pleomorphic and bizarre cells with
foamy cytoplasm, marked atypia, and numerous mitotic
figures in a collagenous background

Question 37

What are the risk factors for Kaposi sarcoma?

Answer 37

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.