Martin: Soft Tissue Flashcards
Sarcomas with complex karyotypes which are pleomorphic and geneticaly heterogenous and carry a poor prognosis are most often seen in whom?
Adults
A well-encapsulated mass of mature adipocytes that is soft, mobile, and painless arising most commonly in adults is known as what?
Lipoma
Liposarcoma most often arises in whom and in which locations?
- Common malignant ST tumor, adults (50-60 y/o)
- Arise in deep ST of prox. extremities and retroperitoneum
Which genetic abberations is associated with the myxoid type of liposarcomas?
t(12;16) –> 12q encodes MDM2 a potent inhibitor of p53
What is the morphology of the well-differentiated type of liposarcoma?
Contains adipocytes with scattered atypical spindle cells
A history of what is seen in some cases of nodular fasciitis?
Trauma
Whom does nodular fasciitis arise in and in which locations; describe its typical presentation.
- Appear in UE’s (arms, forearm) of young adults (20-30 yo)
- Grow rapidly over several weeks or months; typically no larger than 5cm
What is the prognosis and tx of nodular fasciitis?
Often spontaneously regress and if excised, rarely recurs
What occurs in the palmar (dupuytren contracture) subtype of superficial fibromatosis?
- Irregular or nodular thickening of the palmar fascia; unilateral or bilaterally
- Slow progression to flexion contracture, mainly affecting 4th and 5th fingers of hand
Patients with what germline mutation and syndrome are predisposed to developing deep fibromatosis (desmoid tumors)?
APC mutations –> familial adenomatous polyposis (FAP) - Gardner syndrome
What are the most common soft tissue sarcomas of childhood and adolescence?
Embryonal and alveolar type of rhabdomyosarcoma
Where do the pediatric forms of rhabdomyosarcomas most often arise?
Siuses, head and neck, and GU tract
Which genetic aberrations and translocations are associated with alveolar rhabdomyosarcomas?
Fusion of FOXO1 with either PAX3 = (2;13) or PAX7 = (1;13)
In sarcoma botryoides, where the tumor cells abut the mucosa of an organ, they form a submucosal zone of hypercellularity called what?
Cambium layer
Where does embryonal rhabdomyosarcoma most often arise, characteristic morphology, and age group?
- Arise in genitourinary tract
- Patients 1-5 y/o
- Primitive spindle cells, “strap cells“
Phenotype of multiple cutaneos leiomuomas may be transmitted as an autosomal dominant trait associated with what?
Uterine leiomyomas + renal cell carcinoma –> hereditary leiomyomatosis and renal cell cancer syndorme
Leiomyosarcomas most often develop in which age group, sex, and structures; common presentation?
- 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
How does Synovial Sarcoma typically present and in which age group?
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
Which characteristic chromosomal translocation is associated with Synovial Sarcomas?
t(x;18) producing SS18-SSX1, -SSX2, or -SSX4
Synovial sarcomas, especially the biphasic type can be differentiated from other sarcoma by (+) immunohistochemical staining for what?
Epithelial markers (i.e., keratin)
What is the prognosis of Synovial Sarcomas and where do they metastasize?
- 5-year of 25% to 62% related to stage** and **patient age
- Commonly metz to lung and regional LN’s
