Malignant Mesenchymal Tumors and Lymphoreticular Malignancies - part I Flashcards
malignant mesenchymal tumors
- fibrous - fibrosarcoma
- nerve - malignant peropheral nerve sheath tumor (MPNST)
- endothelial cell - Kaposi sarcoma
- muscle - rhabdomyosarcoma
lymphoreticular malignancies
- Langerhans cell histiocytosis
- leukemia
- lymphoma
- multiple myeloma/plasmacytoma
fibrosarcoma
malignancy of fibroblastic differentiation
who is affected by fibrosarcoma?
adults but any age
clinical features of fibrosarcoma
- soft tissue mass or centrally as an intrabony lesion
2. slow growing
T/F: pain is an early clinical feature of fibrosarcoma
false, late feature
histopathologic features of fibrosarcoma
- fascicles of spindle-shaped cells often forming a “herringbone” pattern
- variable number of mitoses
treatment of fibrosarcoma
wide to radical surgical excision
T/F: fibrosarcoma responds well to radiation or chemotherapy
false, little to no response to radiation or chemotherapy
how does fibrosarcoma metastasize?
blood
which organs does fibrosarcoma metastasize to?
- lung
- liver
- bone
what is malignant peripheral nerve sheath tumor (MPNST) also known as?
- neurogenic sarcoma
2. neurofibrosarcoma
what percent of MPNST is associated with NF1?
~50%
T/F: MPNST arise spontaneously
true
clinical features of MPNST
- may have pain
- may have nerve deficit
- may be in soft tissue or centrally as an intrabony lesion
histopathologic features of MPNST
- spindle-shaped cells with wavy nuclei
2. mitoses
treatment for MPNST
- surgical resection
- radical excision
- amputation
- ± radiation
how was Kaposi sarcoma (KS) first described by Moritz Kaposi in the late 19th century?
described as a disease affecting elderly white males of Mediterranean descent
Kaposi sarcoma was identified in what type of people?
- sub-Saharan Africans
- transplant recipients
- HIV+ males
what is KS caused by?
infection with HHV-8
what are the 4 clinical presentations of KS?
- classic
- endemic (African)
- iatrogenic (transplant-associated)
- epidemic (AIDS-related)
who is affected by classic KS?
elderly patients, most often male
where does classic KS typically appears?
on lower extremities
clinical features of classic KS
- slow growing
- painless
- purple-red
- macules –> plaques –> tumors