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
T/F: classic KS blanches on pressure
false, does NOT
who is affected by endemic (African) KS?
wide age range especially young adults and children
T/F: course of endemic (African) KS can vary widely from indolent skin lesions to aggressive tumors
true
who is most often affected by lymphadenopathic endemic (African) KS?
young children –> causes visceral tumors
cause of iatrogenic (transplant-associated) KS
solid organ transplants
T/F: iatrogenic KS can occur several months to years after transplant
true
where does iatrogenic KS affect?
skin and oral mucosa
what can cause regression of iatrogenic KS?
reducing degree of immune-suppression
who is affected by AIDS-related KS?
primarily HIV-infected adult male homosexuals (other types of KS seen in both genders; children in endemic)
AIDS-related KS skin lesions have a predilection for which locations?
face and lower extremities
where is the initial site of AIDS-related KS presentation?
oral cavity in ~22% of patients
where does AIDS-related KS affect intraorally?
- hard palate
- gingiva
- tongue
T/F: AIDS-related KS can invade bone
true
what happens when AIDS-related KS invades bone?
tooth mobility
histopathologic features of KS
- cellular spindle cell tumor within the CT, extravasated blood
- many poorly defined vascular slits
- HHV-8 positivity in endothelial cells and some spindle cells
treatment for KS
- excision for small cosmetically problematic lesions
- radiation
- injections of chemotherapeutic agents
prognosis of classic KS
fair
T/F: patients with classic KS usually die of something else and not the disease
true, MI, CVA, etc.
prognosis of endemic lymphadenopathic KS
poor
prognosis of iatrogenic KS
fair to poor
prognosis of AIDS-related KS
fair
what are the different types of rhabdomyosarcoma?
- embryonal
- alveolar
- pleomorphic
T/F: rhabdomyosarcoma is common
false, rare
who does rhabdomyosarcoma affect?
children or adolescents
what is the most common soft tissue sarcoma in children under 15 y.o.?
rhabdomyosarcoma
clinical features of rhabdomyosarcoma
- usually painless
- rapidly growing
- sarcoma botryoides
what is the most common location for rhabdomyosarcoma?
head and neck
what is the most common intraoral site for rhabdomyosarcoma?
palate
sarcoma botryoides
term used when rhabdomyosarcoma grows in a body cavity e.g. oral cavity, vagina
how does sarcoma botryoides appear clinically?
“bunch of grapes”