Malgnant Mesenchymal (3) Flashcards
Which malignant mesenchymal tumor is of fibrous tissue?
Fibrosarcoma
Which malignant mesenchymal tumor is of nervous tissue?
Malignant peripheral nerve sheath tumor (MPNST)
Which malignant mesenchymal tumor is of endothelial cell tissue?
Kaposi sarcoma
Which malignant mesenchymal tumor is of muscle tissue?
Rhabdomyosarcoma
What are the clinical features of a fibrosarcoma?
Slow-growing, pain in late stage
What are the histo features of a fibrosarcoma?
Spindle shaped cells in a “herringbone” pattern
T/F: Treatment for fibrosarcoma includes radiation and chemotherapy.
False
Wide surgical excision
What is the 5 year survival rate of fibrosarcoma and how does it metastasize?
50%
Via blood
What are two other names for a MPNST?
Neurogenic sarcoma or neurofibrosarcoma
What are some clinical features of MPNST?
50% associated with NF1
May arise spontaneously
May have pain
Those with NF1 will develop lesion earlier in life (late 20s)
What are the histo features of MPNST?
Spindle-shaped cells with wavy nuclei
+ mitosis
What is the treatment and prognosis for MPNST?
Surgical resection and radical excision
50% 5-year survival without NF-1
Worse prognosis with NF-1
What causes Kaposi sarcoma?
Infection with HHV-8
What are the clinical features of classic kaposi syndrome?
Lower extremities of older patients
Painless purple. Goes from macule -> plaque -> tumor.
Does not blanch with pressure
What are the clinical presentations of endemic kaposi syndrome?
Seen in Africa
Varies from indolent skin lesions to aggressive tumors
T/F: Latrogenic kaposi syndrome will be seen weeks after organ transplant.
False
Months-years
What tissues are affected with latrogenic kaposi syndrome?
Skin and oral mucosa
T/F: Oral cavity is often the initial site of presentation in patients with classic kaposi syndrome.
False
AIDS-related kaposi syndrome
Where are there likely to be oral lesions with AIDS related kaposi syndrome?
Hard palate, gingiva, tongue
Will invade bone leading to mobility
What is the treatment and prognosis for kaposi syndrome?
Excision of small lesions, radiation and chemo injections
Classic and AIDS related -fair
Endemic - poor
latrogenic -fair to poor
What are the three types of Rhabdomyosarcoma?
- Embryonal
- Alveolar
- Pleomorphic
What is the most common soft tissue sarcoma in children under 15 y.o.?
Rhabdomyosarcoma
What is the most common intraoral site for Rhabdomyosarcoma?
Palate
What is the proper term and appearance when a Rhabdomyosarcoma grows within a body cavity?
Sarcoma botryoides
Looks like a bunch of grapes
What are the histo features of rhabdomyosarcoma?
Small round cells with hyperchromatic nuclei
Strap-shaped rhabdomyoblasts
What is treatment/prognosis for Rhabdomyosarcoma?
Wide excision, chemo, radiation
Good; 90% 5 year survival
Which lymphoreticular malignancy involves a spectrum of disorders characterized by proliferation of histiocyte-like cells with varying numbers of eosinophils, plasma cells, lymphocytes, and giant cells?
Langerhans cell histiocytosis
What is a langerhans cell?
Tissue-resistant macrophages that serve as antigen-presenting cells