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Flashcards in malignant mesenchymal neoplasm Deck (31)
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1
Q

A _____________ is a malignancy of fibroblastic differentiation

A

Fibrosarcoma

2
Q

why are Fibrosarcomas less frequently diagnosed in recent years?

A

Once considered among the most common soft tissue sarcomas, now a less frequent diagnosis with improved lesion subclassification through use of IHC

3
Q

Clinical characteristics of Fibrosarcomas:

A
  • Usually affects adults

- May present as a soft tissue mass or as an intrabony lesion

4
Q

Treatment for Fibrosarcoma:

A

A) Wide to radical surgical excision

B) Little response to radiation or chemotherapy, similar to many soft tissue sarcomas

5
Q

what % of Fibrosarcomas will reoccur after initial treatment? what is the 5-year-survival?

A

50% recurrence, 40-70% 5-year survival

6
Q

what locations do Fibrosarcomas usually metastasize to?

A

Metastasizes to lung, bone, liver

7
Q

what malignant lesion is also known as a “neurogenic sarcoma”

A

Malignant Peripheral Nerve Sheath Tumor

8
Q

what causes Malignant Peripheral Nerve Sheath Tumors?

A

May arise spontaneously or in association with neurofibromatosis, type 1 (NF1)

9
Q

how are Patients with NF1 different from a normal patient diagnosed with Malignant Peripheral Nerve Sheath Tumors?

A

NF1 patients affected earlier (mean age around 4th decade compared to 5th decade in spontaneous cases)

10
Q

Histological characteristics of MPNST:

Malignant Peripheral Nerve Sheath Tumors

A

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

11
Q

what is the treatment protocol for MPNST?

A

Treatment consists of radical surgical excision +/- chemotherapy/radiation therapy

12
Q

what are the 5-year AND 10-year survival rates for MPNST (neurogenic sarcomas)?

A

Overall, 50% five-year survival with 35% ten-year survival

13
Q

__________________ was first described as a disease in elderly white males of Mediterranean descent

A

Kaposi Sarcoma

14
Q

what are the 4 clinical presentations of Kaposi Sarcoma?

A

1) Classic
2) Endemic (African)
3) Iatrogenic (transplant-associated)
4) Epidemic (AIDS-related)

15
Q

T/F: Kaposis sarcoma is caused by a previous infection with the HPV virus

A

FALSE

Result of infection by HHV-8

16
Q

clinical characteristics of CLASSIC Kaposis Sarcoma:

A

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

17
Q

T/F: Oral involvement of Kaposis Sarcoma is relatively rare

A

True

18
Q

what population groups are most likely to be diagnosed with ENDEMIC Kaposis Sarcoma?

A

Endemic (African) KS affects a wide age range of patients, including children

  • Widely varied course, from indolent to aggressive
19
Q

What parts of the body are involved in the lymphadenopathic form of Kaposis Sarcoma?

what is the prognosis for this form?

A
  • Lymph nodes and viscera involved in lymphadenopathic form

- poor prognosis

20
Q

characteristics of Iatrogenic (transplant-associated) Kaposis Sarcoma

A

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

21
Q

Characteristics of AIDS-related Kaposis Sarcoma:

A

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%

22
Q

Of patients who develop AIDS-related KS, ____% will have oral lesions

A

70%

23
Q

Treatments for Kaposis Sarcoma:

A

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

24
Q

what is the prognosis for Kaposis Sarcoma?

give prognosis for all 4 forms

A

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.

25
Q

______________ is a relatively uncommon malignancy of skeletal muscle

A

Rhabdomyosarcoma

26
Q

what population groups are at highest risk for developing Rhabdomyosarcoma?

A

Affects children/adolescents

27
Q

Over ___% of Rhabdomyosarcoma cases are found in the head & neck

A

35%

28
Q

clinical characteristics of Rhabdomyosarcoma:

A

Infiltrative, rapidly growing mass

29
Q

When rhabdomyosarcoma grows into a body cavity, such as the oral cavity, the lesion may appear similar to a bunch of grapes, termed “_______________”

A

sarcoma botryoides

30
Q

Treatments/reoccurance/survival for rhabdomyosarcoma:

A

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%

31
Q

what malignancy is responsible for 50% of childhood soft tissue sarcomas?

A

Rhabdomyosarcoma