1.

Sarcomas are a heterogeneous group of neoplasms that arise predominantly from cells of the
embryonic mesoderm.
2. Most primary soft tissue sarco mas originate in an extremity (50–60%); the next most common
sites are the trunk (19%), retroperitoneum (15%), and head and neck (9%).
3. Embryonal/ alveolar rhabdomyosarcomas are the most common soft tissue sarcomas of
childhood, whereas pleomorphic rhabdomyosar coma occurs predominantly in adults.
4. External radiation therapy is a rare but well-established risk factor for soft tissue sarcoma.
5. Oncogenes are genes that can induce malignant transformation and tend to drive cell
proliferation.
6. The two genes most relevant to soft tissue sarcoma are retinoblastoma (Rb) and p53.
7. Patients with germline mutations in p53 (Li-Fraumeni syndrome).
8. Neurofibromatosis type 1 (von Recklinghausen’s disease) is due to various mutations in the NF-
1 tumor suppressor gene.
9. Magnetic resonance imaging (MRI) is the preferred imaging technique for soft tissue sarcomas
of the extremities, whereas computed tomography (CT) is most use ful for evaluating
retroperitoneal, intra-abdominal, and truncal sarcomas.
10. However, fine needle aspiration is the procedure of choice to confirm or rule out the presence
of a metastatic focus or local recurrence.
11. Core Needle Biopsy has become the preferred technique for diagnosing soft tissue lesions.
12. Soft tissue sarcoma is most commonly staged using either the American Joint Committee on
Cancer (AJCC) system (generally used in the United States) or the World Health Organization
sys tem.
13. Histologic grade is the most important prognostic factor for patients with soft tissue sarcoma.
14. The preferred treatment for extrem ity sarcomas is wide local excision that includes resection
of the biopsy site.
15. Amputation is the treatment of choice for the 5% of patients with primary or recurrent
extremity tumors whose tumors cannot be grossly resected with limb-sparing proce dures and
preservation of function.
16. Isolated regional perfusion is a limb-sparing technique in which a soft tissue sarcoma is
perfused with high concentrations of tumor necrosis factor alpha (TNF-α) and melphalan.
17. Doxorubicin and ifosfamide are the two most active agents against soft tissue sarcoma.
18. Aldoxorubicin is a doxo rubicin derivative that serves as a prodrug of doxorubicin that
covalently binds to albumin in the blood until reaching the acidic tumor environment releasing
doxorubicin into the tis sue.
19. Trabectedin, a marine-derived alkaloid that binds DNA, affecting transcription and inducing the
formation of DNA double-strand breaks.
20. Palifosfamide is a stabilized formulation of the active metabolite of ifosfamide that has been
reported to be better tolerated than ifosfamide.
21. Pazopanib is an oral angiogenesis inhibitor that targets vascular endothelial growth factor
receptors, platelet derived growth factor receptor (PDGFR), and c-kit.
22. Olaratumab is a human antiplatelet-derived growth factor receptor α monoclonal antibody that
has antitumor activity in human sarcoma xenografts.
23. The most common initial site of distant metastasis of soft tissue sarcomas is the lung.
24. Myxoid liposarcomas frequently present as slow-growing, deep tumors in the lower extremity
and can metastasize to other soft tissue locations, including the retroperitoneum and
extremities.
25. Complete surgical resection is the most effective treatment for primary or recurrent
retroperitoneal sarcoma.
26. For sarcomas of the small or large intestine, segmental bowel resection is the standard
treatment.
27. The findings at presentation of a patient with cutaneous angiosarcoma often include an
expanding erythematous patch, red papular eruptions, bluish-black lesions, or bruise-like
discoloration overlying an area of induration.
28. Cystosarcoma phyllodes are generally not considered to be sarcomas because these tumors are
thought to originate from hormonally responsive stromal cells of the breast and are usu ally
benign.
29. Uterine leiomyosarcomas are smooth-muscle tumors and account for 35% to 40% of uterine
sarcomas. Standard treatment is Total abdominal hysterectomy (TAH).
30. Endometrial stromal sarcomas account for approximately 7% to 10% of uterine sarcomas.
31. Malignant mixed müllerian tumor accounts for 50% of uterine sarcomas and arises
predominantly in postmenopausal women.
32. Undifferentiated endometrial sarcoma is an aggressive malignancy that does not express
estrogen or progesterone receptors.
33. GASTROINTESTINAL STROMAL TUMORS. These tumors share phenotypic similarities with the
intestinal pacemaker cells known as the interstitial cells of Cajal.
34. The most common locations for GISTs are the stomach (60%) and small intestine (30%).
35. GISTs are most commonly diagnosed by upper endoscopy and/or CT of the abdomen.
36. Complete surgical resection with negative margins is the rec ommended treatment for
localized GISTs.
37. Treatment with imatinib mesylate (Gleevec, ST1571), a selec tive inhibitor of the KIT protein
tyrosine kinase, has resulted in impressive clinical responses in a large percentage of patients
with unresectable or metastatic GISTs.
38. Regorafenib is a structurally unique inhibitor of multiple cancer-associated kinases, including KIT
and PDGFR, with broad-spectrum anticancer activity in preclinical and early-phase trials.
39. Desmoid tumors are not low-grade sarcomas but can be locally aggressive, although they do not
metastasize.
40. Although usually sporadic, desmoids may occur in association with famil ial adenomatous
polyposis, a presentation that is referred to as Gardner’s syndrome and is linked to germline
mutations in the APC gene.
41. Dermatofibrosarcoma protuberans is a rare low-grade sarcoma arising in the dermis that rarely
metastasizes but is locally aggressive. Dermatofibrosarcoma protuberans presents as a nodular,
cutaneous mass that grows slowly and persistently.
42. Rhabdomyosarcoma is a small round cell tumor that dem onstrates muscle differentiation
upon light microscopy and immunohistochemical analysis.
43. Alveolar rhabdomyosarcoma is associated with cytoge netic translocation [t(2:13)(q35:q14)].
44.