Soft Tissue sarcoma Flashcards
Rare tumors
Most common site of soft tissue sarcoma
Extremeity 59%
Trunk 19%
Retroperitoneum 13%
Head and neck 9%
Most common histologic types of soft tissue sarcoma in adults (excluding Kaposis sarcoma)
Malignant fibrous histiocytoma 28% Leiomyosarcoma 12% Liposarcoma 15% Synovial sarcoma 10% Malignant peripheral nerve sheath tumors 6%
Most common soft tissue sarcoma of childhood
Rhabdomyosarcoma
Sarcomas do not seem to result from the progression or differentiation of benign soft tissue tumors except
Malignant peripheral nerve sheath tumors
Clinical behavior of most soft tissue sarcomas is similar and iis determined by
Anatomic location
Depth
Grade
Size
The most dominant pattern of metastasis is
Hematogenous in the lungs
Lymphnode metastases are
Rare
Is a well established risk factor for soft tissue sarcoma
External radiation therapy
Incidence of sarcomas has been reported among patients treated for cancer
Breast Cervix Ovary Testes Lymphatic system
Radiation exposure
Post irradiation sarcomas, the most common histologic types were
Osteogenic sarcoma
Malignant fibrous histiocytoma
Angiosarcoma
Lymphangiosarcoma
Radiation exposure
Usually diagnosed at advanced stages and generally have a poor prognosis
Post irradiation
Chemicals linked to soft tissue sarcoma
Phenoxyacetic acid
Chlorophenols
Several chemical carcinogen shave been associated with hepatic angiosarcomas
Thorium oxide (thoratrast)
Vinyl chloride
Arsenic
Patient with soft tissue sarcoma linked to chemicals died due to
Massive blood loss
No casual relationship has been establish
Trauma
Filarial infections
Lymphangiosarcoma
Oncogenes identified in association with soft tissue sarcoma
MDM2
N-MYC
C-erB2
ras
Oncogenes produce specific oncoproteins that either play a role
Nuclear function
Cellular signal transduction
Function as growth factors
Growth factor receptors
Two tumor suppressor genes that are most relevant to soft tissue tumors
Retinoblastoma tumor suppressor gene
P53 tumor suppressor gene (most common 30-60%)
Can lead to development of retinoblastoma or sarcoma of soft tissue and bone
Mutations or deletion of RB
Most common mutation in human solid tumors and have been reported in 30-60% of soft tissue
Mutations in the p53
Known as recklinghausen’s disease
Occurs in approximately 1 every 3000 persons
Neurofibromatosis type 1
Neurofibromatosis type 1
Due to mutations in the
NF-1 tumor suppressor gene
Neurofibromatosis type 1
How many percent have mutation in NF-1
50%
Initial assessment
Most commonly presents as
Asymptomatic mass
Initial assessment
The size at presentation is usually associated with the
Location of the tumor
Initial assessment
Smaller tumor located at the
Distal extremities
Initial assessment
Tumors can grow quite large before becoming apparent
Proximal extremities and
Retroperitoneum
Initial assessment
Soft tissue sarcoma often grow in a
Centrifugal fashion
Initial assessment
Impingement on bone or neurovascular bundles produces
Pain
Edema
Swelling
Initial assessment
Retroperitoneal soft tissue sarcoma almost always presents as a
Large asymptomatic mass
Initial assessment
Patient may present with
Obstructive gastrointestinal symptoms
Neurologic symptoms
Initial assessment
The differential diagnosis of a soft tissue mass includes benign lesions including
Lipomas
Lymphangiomas
Leiomyomas
Neuromas
Initial assessment
In addition to sarcomas, other malignant lesions such as
Primary or metastatic carcinomas
Melanomas
Lymphomas
Initial assessment
Small lesion not changed for years
Closely observed
Initial assessment
All other tumors considered
Biopsy to establish definitive diagnosis
Diagnostic imaging
Purposes
Local extent of tumor Stage Biopsy Benign or Monitor tumor changes Recurrences