malignant mesenchymal neoplasm Flashcards
A _____________ is a malignancy of fibroblastic differentiation
Fibrosarcoma
why are Fibrosarcomas less frequently diagnosed in recent years?
Once considered among the most common soft tissue sarcomas, now a less frequent diagnosis with improved lesion subclassification through use of IHC
Clinical characteristics of Fibrosarcomas:
- Usually affects adults
- May present as a soft tissue mass or as an intrabony lesion
Treatment for Fibrosarcoma:
A) Wide to radical surgical excision
B) Little response to radiation or chemotherapy, similar to many soft tissue sarcomas
what % of Fibrosarcomas will reoccur after initial treatment? what is the 5-year-survival?
50% recurrence, 40-70% 5-year survival
what locations do Fibrosarcomas usually metastasize to?
Metastasizes to lung, bone, liver
what malignant lesion is also known as a “neurogenic sarcoma”
Malignant Peripheral Nerve Sheath Tumor
what causes Malignant Peripheral Nerve Sheath Tumors?
May arise spontaneously or in association with neurofibromatosis, type 1 (NF1)
how are Patients with NF1 different from a normal patient diagnosed with Malignant Peripheral Nerve Sheath Tumors?
NF1 patients affected earlier (mean age around 4th decade compared to 5th decade in spontaneous cases)
Histological characteristics of MPNST:
Malignant Peripheral Nerve Sheath Tumors
A) Invasive cellular proliferation of spindle-shaped cells with wavy nuclei
B) Similar appearance with routine hematoxylin and eosin-stained sections to several other tumors
C) Usually shows S100 positivity by IHC
what is the treatment protocol for MPNST?
Treatment consists of radical surgical excision +/- chemotherapy/radiation therapy
what are the 5-year AND 10-year survival rates for MPNST (neurogenic sarcomas)?
Overall, 50% five-year survival with 35% ten-year survival
__________________ was first described as a disease in elderly white males of Mediterranean descent
Kaposi Sarcoma
what are the 4 clinical presentations of Kaposi Sarcoma?
1) Classic
2) Endemic (African)
3) Iatrogenic (transplant-associated)
4) Epidemic (AIDS-related)
T/F: Kaposis sarcoma is caused by a previous infection with the HPV virus
FALSE
Result of infection by HHV-8
clinical characteristics of CLASSIC Kaposis Sarcoma:
A) The classic form of KS usually presents on lower extremities of older adult males
B) Initially, purple cutaneous macules that gradually develop into plaques and tumors
T/F: Oral involvement of Kaposis Sarcoma is relatively rare
True
what population groups are most likely to be diagnosed with ENDEMIC Kaposis Sarcoma?
Endemic (African) KS affects a wide age range of patients, including children
- Widely varied course, from indolent to aggressive
What parts of the body are involved in the lymphadenopathic form of Kaposis Sarcoma?
what is the prognosis for this form?
- Lymph nodes and viscera involved in lymphadenopathic form
- poor prognosis
characteristics of Iatrogenic (transplant-associated) Kaposis Sarcoma
A) usually affects the skin of a small percentage (less than 1%)
B) may effect oral mucosa
C) Reducing the degree of immune suppression may lead to regression of KS
Characteristics of AIDS-related Kaposis Sarcoma:
A) predominately affects gay or bisexual men
B) Rarely seen in women
C) Frequency has been declining, from 25% at onset of the epidemic to less than 10%
Of patients who develop AIDS-related KS, ____% will have oral lesions
70%
Treatments for Kaposis Sarcoma:
1) Management may include excision for small cosmetically unacceptable lesions
2) Radiation therapy for skin lesions
3) Systemic chemotherapy or intralesional injections with anticancer agents such as vinblastine
what is the prognosis for Kaposis Sarcoma?
give prognosis for all 4 forms
1) Classic – fair. Patients usually die of something else (MI, CVA, etc.)
2) Endemic – variable. Lymphadenopathic form – poor.
3) Iatrogenic (transplant-associated) – fair to poor. Lesions may regress with reduction in immunosuppressive therapy
4) AIDS-related – fair.
______________ is a relatively uncommon malignancy of skeletal muscle
Rhabdomyosarcoma
what population groups are at highest risk for developing Rhabdomyosarcoma?
Affects children/adolescents
Over ___% of Rhabdomyosarcoma cases are found in the head & neck
35%
clinical characteristics of Rhabdomyosarcoma:
Infiltrative, rapidly growing mass
When rhabdomyosarcoma grows into a body cavity, such as the oral cavity, the lesion may appear similar to a bunch of grapes, termed “_______________”
sarcoma botryoides
Treatments/reoccurance/survival for rhabdomyosarcoma:
A) Wide surgical excision combined with radiation therapy and multi-agent chemotherapy
B) Prognosis depends on the stage and subtype
C) 5-yr survival ranges from 44-72%
what malignancy is responsible for 50% of childhood soft tissue sarcomas?
Rhabdomyosarcoma
