Sarcoma

Question 1

Clinical behavior of desmoid tumors

Answer 1

Don’t metastasize but are locally aggressive

Question 2

Precipitants of desmoid tumors

Answer 2

1) High estrogen states - during or following pregnancy (extra-abdominal or abdominal)
2) Trauma
3) FAP

Question 3

Clinical course of desmoid tumors

Answer 3

highly variable clinical course (stable to spontaneously regressing to slow growing to rapidly progressive)

Question 4

Management of desmoid tumor

Answer 4

• IF asymptomatic or minimally symptomatic, initial period of observation (Let disease biology declare itself. Significant rate of spontaneous regression and periods of growth arrest. Placebo arm had a 20% response rate in sorafenib trial).
• IF causing significant symptoms, multimodality evaluation

Question 5

Management of gross residual disease after resection of a desmoid tumor

Answer 5

• Observation or RT rather than more surgery

Question 6

Management of large intra-abdominal desmoid tumors in the setting of Gardner syndrome

Answer 6

• Surgery still preferred

Question 7

Klinefelter presentation

Answer 7

• tall
• micropenis
• hypogonadism (small firm testes) and signs of androgen deficiency (gynecomastia, sexual dysfunction, osteoporosis)
• learning and developmental disabilities