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Flashcards in malignant mesenchymal neoplasm Deck (31)
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1

A _____________ is a malignancy of fibroblastic differentiation

Fibrosarcoma

2

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

3

Clinical characteristics of Fibrosarcomas:

- Usually affects adults

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

4

Treatment for Fibrosarcoma:

A) Wide to radical surgical excision

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

5

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

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

6

what locations do Fibrosarcomas usually metastasize to?

Metastasizes to lung, bone, liver

7

what malignant lesion is also known as a "neurogenic sarcoma"

Malignant Peripheral Nerve Sheath Tumor

8

what causes Malignant Peripheral Nerve Sheath Tumors?

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

9

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)

10

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

11

what is the treatment protocol for MPNST?

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

12

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

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

13

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

Kaposi Sarcoma

14

what are the 4 clinical presentations of Kaposi Sarcoma?

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

15

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

FALSE

Result of infection by HHV-8

16

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

17

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

True

18

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

19

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

20

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

21

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%

22

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

70%

23

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

24

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.

25

______________ is a relatively uncommon malignancy of skeletal muscle

Rhabdomyosarcoma

26

what population groups are at highest risk for developing Rhabdomyosarcoma?

Affects children/adolescents

27

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

35%

28

clinical characteristics of Rhabdomyosarcoma:

Infiltrative, rapidly growing mass

29

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

30

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%