Malignant Soft Tissue Tumors

Question 1

What is a fibrosarcoma?

Answer 1

Malignant tumor of fibroblasts

Question 2

Histopathologic appearance of fibrosarcoma?

Answer 2

Cellular mass of spindle shaped cells demonstrating mild pleomorphism

Question 3

Tx for fibrosarcoma

Answer 3

Surgical excision including a wide margin of adjacent normal tissue

Question 4

What is a liposarcoma?

Answer 4

Malignant neoplasm of fatty origin

Most common soft tissue sarcoma

Question 5

What are the most common sites of lipsarcomas?

Answer 5

Thigh, retroperitoneum and iguinal regions

Question 6

How common are the liposarcomas of the head and neck?

Answer 6

rare

Question 7

What are the 3 major categories of liposarcoma?

Answer 7

3 major categories:

1. well-differentiated/atypical lipomatous tumor (most common)
2. myxoid/round cell liposarcoma
3. pleomorphic liposarcoma

Question 8

Tx for liposarcoma

Answer 8

Radical excision - tx of choice

Question 9

Survival rates for liposarcoma?

Answer 9

5 year survival rate = 59-70%

10 year survival = 50%

Question 10

Recurrence of liposarcoma?

Answer 10

~50%

Question 11

Oral liposarcoma prognosis

Answer 11

More favorable because of the predominance of well differentiated subtypes and because tumors are small and diagnosed

Question 12

What other disease is often seen with malignant peripheral nerve sheath tumor?

Answer 12

Half seen in NF-1 (neurofibromatosis)

Question 13

Tx for malignant peripheral nerve sheath tumor

Answer 13

Radical surgical excision

Question 14

Prognosis of malignant peripheral nerve sheath tumor

Answer 14

Poor especially in patients with neurofibromatosis

Question 15

What is Kaposi’s sarcoma?

Answer 15

Vascular neoplasm caused by HHV8 and KSHV

Question 16

Kaposi’s sarcoma has the propensity to develop in what patients?

Answer 16

HIV

Question 17

What cell lineage does Kaposi’s sarcoma lesions mosty likely arise from?

Answer 17

Lesions most likely arise in endothelial cells although there is some evidence of lymphatic origin

Question 18

What is leiomyosarcoma?

Answer 18

malignant neoplasm of smooth muscle differentiation

Question 19

What is the most common site of leiomyosarcoma?

Answer 19

uterine wall and GI tract

Question 20

How often do leiomyosarcomas occur in the oral cavity?

Answer 20

rare

Question 21

What is a rhabdomyosarcoma?

Answer 21

Malignant neoplasm characterized by skeletal muscle differentiation

Question 22

What is the most frequnt site of rhabdomyosarcoma?

Answer 22

head and neck

Question 23

Is rhabdomyosarcoma more frequent in young chilren or adults?

Answer 23

young children

Primarily occurs in the first decade of life but may occur in teens

Question 24

Clinical presentation of the rhabdomyosarcoma tumor

Answer 24

Tumor is most often a painless infiltrative mass that may grow rapidly

Question 25

Tx for rhabdomyosarcoma

Answer 25

local surgical excision followed by multiagent chemotherapy

Question 26

Prognosis of rhabdomyosarcoma

Answer 26

with the advent of multimodal therapy, prognosis has improved

Question 27

How common are metastatic tumors to the oral cavity?

Answer 27

Uncommon

Question 28

What tissues can metastasis to the oral cavity affect?

Answer 28

bone or soft tissues

Question 29

What is the mechanism of metastatic spread to the head and neck?

Answer 29

primary malignancies from immediately adjacent tissues might be able to spread by a lymphatic route **Batson's plexus** * explanation for blood-borne metastasis to the head and neck * valve-less verterbral venous plexus that might allow retrograde spread of tumor cells, bypassing filtration through the lungs

Question 30

What is the most common site for soft tissue metastases in the oral cavity?

Answer 30

gingiva, followed by the tongue

Question 31

What is the clinical appearance of metastatic lesions in the oral cavity?

Answer 31

Lesions usually appear as a nodular mass that oftne resembles a hyperplastic or reactive growth

Question 32

How can a metastatic lesion to the oral cavity affect adjacent teeth?

Answer 32

Adjacent teeth may be loosened by an underlying destruction of alveolar bone

Question 33

How does the presence of teeth direct location of the metastatic lesion in the oral cavity?

Answer 33

Presence of teeth may plan an important role in the preference of metastases for the gingiva

Question 34

How does the oral cavity affect malignant cell growth when there is metastases to the oral cavity?

Answer 34

Once malignant cells reach the oral cavity, the rich vascular network of inflamed gingival tissues may serve as a fertile site for further growth

Question 35

What are the most common sources for metastasis to the oral cavity?

Answer 35

Lung Kidney Melanoma Prostate Breast

Question 36

Prognosis of metastatic lesions to the oral cavity?

Answer 36

GEnerally poor because other metastatic sites are also present

Question 37

Management of metastatic oral lesion?

Answer 37

palliative