Martin: Soft Tissue

Question 1

Sarcomas with complex karyotypes which are pleomorphic and geneticaly heterogenous and carry a poor prognosis are most often seen in whom?

Answer 1

Adults

Question 2

A well-encapsulated mass of mature adipocytes that is soft, mobile, and painless arising most commonly in adults is known as what?

Answer 2

Lipoma

Question 3

Liposarcoma most often arises in whom and in which locations?

Answer 3

• Common malignant ST tumor, adults (50-60 y/o)
• Arise in deep ST of prox. extremities and retroperitoneum

Question 4

Which genetic abberations is associated with the myxoid type of liposarcomas?

Answer 4

t(12;16) –> 12q encodes MDM2 a potent inhibitor of p53

Question 5

What is the morphology of the well-differentiated type of liposarcoma?

Answer 5

Contains adipocytes with scattered atypical spindle cells

Question 6

A history of what is seen in some cases of nodular fasciitis?

Answer 6

Trauma

Question 7

Whom does nodular fasciitis arise in and in which locations; describe its typical presentation.

Answer 7

• Appear in UE’s (arms, forearm) of young adults (20-30 yo)
• Grow rapidly over several weeks or months; typically no larger than 5cm

Question 8

What is the prognosis and tx of nodular fasciitis?

Answer 8

Often spontaneously regress and if excised, rarely recurs

Question 9

What occurs in the palmar (dupuytren contracture) subtype of superficial fibromatosis?

Answer 9

• Irregular or nodular thickening of the palmar fascia; unilateral or bilaterally
• Slow progression to flexion contracture, mainly affecting 4th and 5th fingers of hand

Question 10

Patients with what germline mutation and syndrome are predisposed to developing deep fibromatosis (desmoid tumors)?

Answer 10

APC mutations –> familial adenomatous polyposis (FAP) - Gardner syndrome

Question 11

What are the most common soft tissue sarcomas of childhood and adolescence?

Answer 11

Embryonal and alveolar type of rhabdomyosarcoma

Question 12

Where do the pediatric forms of rhabdomyosarcomas most often arise?

Answer 12

Siuses, head and neck, and GU tract

Question 13

Which genetic aberrations and translocations are associated with alveolar rhabdomyosarcomas?

Answer 13

Fusion of FOXO1 with either PAX3 = (2;13) or PAX7 = (1;13)

Question 14

In sarcoma botryoides, where the tumor cells abut the mucosa of an organ, they form a submucosal zone of hypercellularity called what?

Answer 14

Cambium layer

Question 15

Where does embryonal rhabdomyosarcoma most often arise, characteristic morphology, and age group?

Answer 15

• Arise in genitourinary tract
• Patients 1-5 y/o
• Primitive spindle cells, “strap cells“

Question 16

Phenotype of multiple cutaneos leiomuomas may be transmitted as an autosomal dominant trait associated with what?

Answer 16

Uterine leiomyomas + renal cell carcinoma –> hereditary leiomyomatosis and renal cell cancer syndorme

Question 17

Leiomyosarcomas most often develop in which age group, sex, and structures; common presentation?

Answer 17

• Present as painless firm masses, most often in women who are 40-60 y/o
• Arise in deep ST’s of the extremities and retroperitoneum

Question 18

How does Synovial Sarcoma typically present and in which age group?

Answer 18

Pt’s in their 20s-40s presenting with deep-seated mass in thigh, leg, chest wall or head and neck that has been present for several years

Question 19

Which characteristic chromosomal translocation is associated with Synovial Sarcomas?

Answer 19

t(x;18) producing SS18-SSX1, -SSX2, or -SSX4

Question 20

Synovial sarcomas, especially the biphasic type can be differentiated from other sarcoma by (+) immunohistochemical staining for what?

Answer 20

Epithelial markers (i.e., keratin)

Question 21

What is the prognosis of Synovial Sarcomas and where do they metastasize?

Answer 21

• 5-year of 25% to 62% related to stage** and **patient age
• Commonly metz to lung and regional LN’s