malignant mesenchymal neoplasm

Question 1

A _____________ is a malignancy of fibroblastic differentiation

Answer 1

Fibrosarcoma

Question 2

why are Fibrosarcomas less frequently diagnosed in recent years?

Answer 2

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

Question 3

Clinical characteristics of Fibrosarcomas:

Answer 3

• Usually affects adults

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

Question 4

Treatment for Fibrosarcoma:

Answer 4

A) Wide to radical surgical excision

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

Question 5

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

Answer 5

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

Question 6

what locations do Fibrosarcomas usually metastasize to?

Answer 6

Metastasizes to lung, bone, liver

Question 7

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

Answer 7

Malignant Peripheral Nerve Sheath Tumor

Question 8

what causes Malignant Peripheral Nerve Sheath Tumors?

Answer 8

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

Question 9

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

Answer 9

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

Question 10

Histological characteristics of MPNST:

Malignant Peripheral Nerve Sheath Tumors

Answer 10

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

Question 11

what is the treatment protocol for MPNST?

Answer 11

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

Question 12

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

Answer 12

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

Question 13

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

Answer 13

Kaposi Sarcoma

Question 14

what are the 4 clinical presentations of Kaposi Sarcoma?

Answer 14

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

Question 15

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

Answer 15

FALSE

Result of infection by HHV-8

Question 16

clinical characteristics of CLASSIC Kaposis Sarcoma:

Answer 16

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

Question 17

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

Answer 17

True

Question 18

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

Answer 18

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

• Widely varied course, from indolent to aggressive

Question 19

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

what is the prognosis for this form?

Answer 19

• Lymph nodes and viscera involved in lymphadenopathic form

- poor prognosis

Question 20

characteristics of Iatrogenic (transplant-associated) Kaposis Sarcoma

Answer 20

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

Question 21

Characteristics of AIDS-related Kaposis Sarcoma:

Answer 21

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%

Question 22

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

Answer 22

70%

Question 23

Treatments for Kaposis Sarcoma:

Answer 23

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

Question 24

what is the prognosis for Kaposis Sarcoma?

give prognosis for all 4 forms

Answer 24

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.

Question 25

______________ is a relatively uncommon malignancy of skeletal muscle

Answer 25

Rhabdomyosarcoma

Question 26

what population groups are at highest risk for developing Rhabdomyosarcoma?

Answer 26

Affects children/adolescents

Question 27

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

Answer 27

35%

Question 28

clinical characteristics of Rhabdomyosarcoma:

Answer 28

Infiltrative, rapidly growing mass

Question 29

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

Answer 29

sarcoma botryoides

Question 30

Treatments/reoccurance/survival for rhabdomyosarcoma:

Answer 30

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%

Question 31

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

Answer 31

Rhabdomyosarcoma