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Flashcards in Tumores Partes Moles Malignos Deck (85):
0

Como é a caracteristica de crescimento dos tumores de partes moles?

The mass typically is not invasive and grows in a cen- tripetal fashion, pushing normal anatomical structures away.

1

Como se apresenta os sarcomas de partes moles na ressonancia magnetica?

Soft tissue sarcomas generally demonstrate low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.

2

Metastases de qual origem primária as TC de abdome e pelve identificam?

Abdominal and pelvic CT scans are useful to detect retroperitoneal metastases in patients with myxoid liposarcomas and lymphatic spread in such lesions as synovial sarcoma, epithelioid sarcoma, and rhabdomyosarcoma.

3

Qual o tumor de partes moles benigno mais comum?

Lipomas are probably the most common benign tumors of connective tissue.

4

Em qual genero e idade está a maior incidencia de lipomas?

More common in middle-aged women.

5

Como se chama o lipoma que invade a sinovia?

Lipoma arborecente

6

Qual é a localização dos angiolipomas?

Angiomyolipoma is a type of lipoma usually found in the kidney. It is composed of smooth muscle, blood vessels, and fat.

7

Onde ocorrem os mielolipomas?

A fatty tumor known as myelolipoma, containing bone marrow elements, may occur within the adrenal medulla.

8

Como se trata os lipomas?

Ressecção marginal ou observação

9

Como se apresenta clinicamente o paciente portador de schwanoma?

Patients may report a painless mass but sometimes complain of referred pain in the distribution of the involved nerve. A Tinel sign can be present on physical examination.

10

Como avaliar a RNM de um paciente com tumor em nervo periferico?

The nerve may be visualized entering and exiting the mass on the coronal or sagittal MRI sequences. A “split fat sign” refers to a rim of fat that may be observed specifically on the T1-weighted MR image of schwannomas.

11

Quais são os 2 tipos de tecidos encontrados nos schwanomas?

the tumor consists of two types of tissue: Antoni A and Antoni B. Antoni A tissue is more typical of the tumor and consists of compact collections of spindle cells that show marked palisading. Antoni B tissue is myxomatous and degenerative, and within it are cystic spaces and oten thick-walled blood vessels.

12

O que são os corpúsculos de Verocay?

Verocay bodies are characteristically found in schwannomas and consist of an arrangement of two rows of palisading nuclei separated by fibrillary material.

13

Como tratar os schwanomas?

Often the lesion simply spreads the nerve fibers apart without anatomical or functional interruption, allowing the tumor to be removed by careful blunt dissection ater a longitudinal incision in the perineurium.

14

Qual tipo de tecido o neurofibroma produz mais que o schwanoma?

Neurofibroma is characterized by a much greater production of collagen than is neurilemmoma, and it, too, may occur as an isolated lesion.

15

O que é a Doença von Recklinghausen?

Neuroibromas also occur as a manifestation of von Recklinghausen disease (neurofibromatosis type 1), in which many such tumors may be found associated with café-au-lait spots and various other lesions.

16

Qual gene a maioria dos portadores de neurofibroma não expressa?

do not have the NF1 marker (gene located at chromosome 17q11.2)

17

Qual a caracteristica do neurofibroma plexiforme?

Sometimes a neuroibroma occurs in which multiple fascicles of a peripheral nerve are involved; this is referred to as a “plexiform neurofibroma,” and excising it completely may be impossible without sacrificing the nerve and its associated function.

18

Qual a caracteristica da condromatose sinovial?

Synovial chondromatosis is a monarticular synovial proliferative disease in which cartilaginous or osteocartilaginous metaplasia occurs within the synovial membrane of joints, bursae, or tendon sheaths.

19

Quais são as 3 fases da condromatose sinovial?

The disease has been classiied into three phases: (1) early, with synovial chondrometaplasia but no loose bodies; (2) transitional, with active synovial disease and loose bodies; and (3) late, with loose bodies but no synovial disease.

20

Quais são as articulações mais envolvidas no condromatose sinovial?

Most common in the knee and hip

21

Qual é o tratamento para a condromatose sinovial?

Treatment consists of arthroscopic or open synovectomy and removal of the loose bodies

22

Como é apresentação clinica do TGC de bainha de tendão?

It first appears as a slowly enlarging but painless mass. Occasionally, a radiograph shows bony erosion of adjacent cortices.

23

Como tratar o TGC de bainha de tendões?

Treatment is by marginal excision, which may prove technically dificult in larger lesions. Recurrences are frequent if the excision is incomplete.

24

Quais são as formas de apresentação da sinovite vilonodular pigmentada?

Pigmented villonodular synovitis may occur in a localized or difuse form. The localized form is identical histologically to giant cell tumor of tendon sheath. The difuse form also appears to be identical histologically to the localized form, but it involves the entire synovium.

25

Quais são os locais de acometimento mais comum da sinovite vilonodular pigmentar difusa?

The difuse form most commonly afects the knee, but the hip, ankle, shoulder, wrist, and other joints can be involved.

26

Qual a caracteristica do liquido articular puncionado em um paciente com sinovite vilonodular pigmentar?

Aspiration of the joint characteristically reveals serosanguineous or blood-tinged fluid.

27

Como se apresenta a sinovite vilonodular pigmentar na RNM?

MRI frequently is diagnostic, showing intraarticular masses that are dark on the T1-weighted and T2-weighted images.

28

Quais são as medidas terapeuticas para os pacientes portadores de sinovite vilonodular pigmentar?

Recommended treatment for the localized form is marginal excision and for the difuse form is total synovectomy.

29

Como é a apresentação clínica do hemangioendotelioma benigno?

the type of hemangioma composed of cellular masses of closely packed endothelial cells with many mitotic figures often is called a “strawberry hemangioma” or a “benign hemangioendothelioma.” It is found at birth or shortly there-after, is deep red, and may grow rapidly during the first few months of life. This type usually stops growing, however, and, in most instances, eventually disappears. Only occasionally does one require surgery.

30

Como é a apresentação clinica dos hemangiomas intramusculares?

Intramuscular hemangiomas can be painful. The pain often is associated with the increased blood low that occurs during increased activity or when the limb is in a dependent position.

31

Qual a caracteristica dos hemangiomas na RNM?

Hemangiomas characteristically have increased fat content as may be shown on MRI.

32

Quais são as opções terapeuticas para os hemangiomas intramusculares?

Some intramuscular hemangiomas are infiltrative and extremely difficult to resect except by radical surgery. In these cases, treatment options include embolization or injection with sclerosing agents.

33

Como se apresenta os tumores glomicos?

Glomus tumor, or glomangioma, is a rare but painful lesion. The skin and subcutaneous tissue of the hands and feet usually are afected, but a tumor may develop in any location in which a glomus body is found. A glomus body serves to control blood low and temperature through the autonomic control of the smooth muscle of the arterial wall. The subungual area of the fingers is a characteristic site of involvement, especially in women. Exquisite point tenderness and hyper- sensitivity is invariably present. Multiple lesions are more common in men.

34

Como tratar o tumor glomico?

Glomus tumors are cured by marginal excision.

35

Qual o local mais comumente afetado pela fasciite nodular? Qual a população mais comumente afetada?

Antebraço. Homem jovem.

36

Como é a apresentação e o tratamento para a fasciite nodular?

Patients usually complain of a rapidly expanding mass that has been present for several weeks. Treatment is by marginal resection.

37

Quais são as caracteristicas dos tumores desmoides?

Desmoid tumors (aggressive fibromatosis) are locally aggressive lesions of connective tissue origin that iniltrate surrounding tissues and have a marked propensity for persistence or recurrence. Although most authorities regard this lesion as benign, it can be locally very aggressive.

38

Qual a localização e quais pacientes são mais comumente afetados pelos tumores desmoides?

These lesions occur most frequently in the anterior abdominal wall of women who have borne children; lesions in other locations often are known as extraabdominal desmoids.

39

Quais são os locais mais comumente afetados por tumores desmoides extraabdominais?

Extraabdominal desmoids occur most frequently in the shoulder girdle, arm, thigh, neck, pelvis, forearm, and popliteal fossa.

40

O que é a doença de Lederhose?

Fibromatose superficial da fascia plantar.

41

Quais são as modalidade terapêuticas para os tumores desmoides?

Most institutions currently recommend wide resection alone or marginal resection followed by adjuvant radiation therapy. Other treatment options that have had reported success include systemic modalities such as tamoxifen, nonsteroidal antiinflammatory drugs, and low-dose cytotoxic agents.

42

Como são chamados atualmente os fibrohistiocitomas malignos?

MFH is now referred to as undifferentiated pleomorphic sarcoma.

43

Qual a faixa etária mais comumente afetada pelo sarcoma pleomórfico indiferenciado?

It most commonly occurs in patients 50 to 70 years old

44

Qual o local mais comumente afetado pelo sarcoma pleomorfico indiferenciado?

The thigh is the most common location.

45

Qual é o tratamento para o sarcoma pleomorfico indiferenciado?

Treatment is wide resection. Radiation can be used as adjuvant treatment for large tumors or for any case in which the margins are close.

46

Qual a taxa de recorrencia dos sarcomas pleomorficos indiferenciados?

With multimodal treatment, local recurrence should be less than 10%.

47

Qual a taxa de sobrevivencia dos pacientes tratados por sarcoma pleomorfico indiferenciado e quais são os fatores de pior prognostico?

Five-year survival is approximately 60%. Poor prognostic indicators include high grade, large tumor size, deep location, and presence of metastases.

48

Qual o segundo sarcoma de partes moles mais incidente?

Lipossarcoma

49

Quais são as caracteristicas clinicas dos lipossarcomas?

Most patients are adults older than 50 years old who complain of a large, painless, deep-seated mass located proximally in the extremities.

50

Quais são os subtipos histologicos de lipossarcoma?

Subtypes include well-diferentiated liposarcoma, myxoid liposarcoma, round cell liposarcoma, and pleomorphic liposarcoma.

51

Quais são as modalidades de tratamento para os lipossarcoma?

Treatment of a well-diferentiated liposarcoma is by surgery alone. Patients with well-diferentiated liposarcomas are at risk for local recurrence; however, metastases are rare and long-term survival is excellent. Treatment of other types of liposarcoma is similar to that already described for MFH.

52

Qual a região geralmente acometida pelo lipossarcoma mixóide?

Myxoid liposarcomas are unique in that they have a tendency to occur in the retroperitoneum.

53

Quais são os fatores que pioram o prognostico dos pacientes com lipossarcoma?

Patients with high-grade tumors have a 60% 5-year survival rate. Poor prognostic indicators include high grade, large size, proximal or deep location, and the presence of metastases.

54

Qual o sarcoma de partes moles mais comum no pé?

Sarcoma sinovial

55

Qual sarcoma de partes moles é comum nas extremidades (mãos e pés)?

Sarcoma sinovial

56

Qual é curiosidade em relação a terminologia do sarcoma sinovial?

The term synovial sarcoma is a misnomer. The term originates from the histological appearance of the cells, which can resemble synovial cells. The tumors do not arise from synovial tissue, however.

57

Qual a translocação genetica mais comumente encontrada no sarcoma sinovial?

A speciic gene translocation, t(X:18), is found in 90% of patients with synovial sarcomas.

58

Qual a porcentagem de metastases linfonodais dos sarcomas sinoviais?

Lymph node metastasis is relatively common (20%) and should be evaluated carefully on physical examination. Prognosis likewise is similar to that described for MFH.

59

Qual é o tratamento para o sarcoma sinovial?

O mesmo que o tratamento para o sarcoma pleomorfico indiferenciado

60

Quais são as caracteristicas dos fibrossarcomas?

he typical presentation is a 5- to 10-cm, slow-growing, painless mass in the deep sot tissues of the lower extremity in adults 30 to 50 years old. Imaging characteristics, treatment, and prognosis are similar to those described for MFH.

61

Quais são as caracteristicas dos sarcomas epitelioides?

It is a slow-growing malignant tumor usually occurring in adolescents and young adults and frequently involves the distal upper extremities, including the hands and fingers.

62

Qual o sarcoma de partes moles mais comum nas mãos?

Sarcoma epitelióide

63

Quais são os tratamentos disponíveis para os sarcomas epitelióides?

The recommended treatment is wide resection or amputation. Similar to synovial sarcoma, lymph node metastases are relatively common.

64

Qual a taxa de sobrevivencia e os fatores de pior prognostico nos pacientes com sarcoma epidelioide?

Despite its slow growth, the aggressive nature of the tumor is evidenced by a high local recurrence rate (>50% in some series). The 5-year survival rate is approximately 70%, but survival declines to 50% at 10 years, owing to the high incidence of late metastases. Prognosis is worse for patients with tumors larger than 3 cm or tumors in proximal locations.

65

Qual é a caracteristica dos dermatofibrosarcoma protuberante?

Rare neoplasm that arises just beneath the epidermis as one or several nodules and eventually may grow to form a large, bulky mass.

66

Qual é o local de acometimento mais comum dos dermatofibrosarcomas protuberantes?

It occurs more oten on the trunk than on the extremities and most frequently in the early to middle decades of life.

67

Qual é o tratamento de escolha para os dermatofibrosarcomas protuberantes?

The treatment of choice is wide resection

68

Quais são os subtipos histológicos dos rabdomiossarcomas?

embryonal, alveolar, and pleomorphic.

69

Quais são os subtipos de rabdomiossarcomas mais comuns na infância?

The embryonal and alveolar types occur in children and adolescents and are among the more common malignant tumors in these age groups.

70

Qual tipo de rabdomiossarcoma ocorre mais comumente em adultos?

Pleomórfico

71

Quais são as localizações mais comuns dos rabdomiossarcomas embrionais?

Embryonal rhabdomyosarcomas usually are located in the head or neck or in the genitourinary tract.

72

Qual o local de ocorrencia mais comum dos rabdomiossarcomas alveolares?

Alveolar rhabdomyosarcoma usually occurs in the head, neck, or extremities and typically is firmer and less myxoid than the embryonal type.

73

Quais são os locais mais comumente afetados pelos rabdomiossarcomas pleomorficos?

The pleomorphic type of rhabdomyosarcoma usually occurs on the extremities and is much less common than either of the other types.

74

Qual o tratamento e o prognostico dos rabdomiossarcomas?

Rhabdomyosarcoma frequently has a rapid and aggressive clinical course. Metastases occur in the lungs, lymph nodes, and bone marrow. Treatment is multimodal consisting of surgery, radiation, and chemotherapy. In contrast to adult sot tissue sarcomas, a dramatic improvement in patient survival has been observed with the use of multiple-agent chemotherapy. Overall 5-year survival is approximately 65%.

75

Qual a porcentagem aproximada de pacientes portadores de neurofibromatose apresentam transformação maligna de um neurofibroma?

Approximately 5% of patients who have neurofibromatosis develop malignant change in a neurofibroma, and pain should alert the clinician to the possibility of transformation.

76

Qual a idade média do paciente que apresenta transformação maligna de um neurofibroma?

These tumors typically arise along a major peripheral nerve in adults 30 to 50 years old.

77

Qual é o tratamento de escolha para os pacientes com tumor maligno de linhagem neuronal?

Treatment consists of wide resection and radiation. Chemotherapy should be considered for young patients with large, high-grade tumors. Local recurrence is high in some series because the tumor can extend along the perineurium of the involved nerve for some distance. Overall 5-year survival is approximately 50%.

78

Qual a idade dos pacientes afetados com osteossarcoma extraesquelético?

extraskeletal osteosarcoma generally afects adults older than age 40 years.

79

Qual a localização mais comum do osteossarcoma extraesquelético?

the deep sot tissue of the thigh is the most common location.

80

Qual é o tratamento de escolha dos osteossarcomas extraesqueleticos?

Treatment is multi-modal, including wide resection, radiation, and multiple-agent chemotherapy. Compared with osteosarcoma of bone and other sot tissue sarcomas, prognosis has generally been poor, with average 5-year survival being 25%.

81

Qual é a localização mais comum de sarcoma de Ewing extraesquelético?

The most common locations include the paravertebral musculature and the chest wall.

82

Qual agente imuno-histoquimico cora o sarcoma de Ewing extraesqueletico?

most stain positively for the MIC2 gene product.

83

Quais são as caracteristicas dos condrossarcomas mixoides extraesqueleticos?

Extraskeletal myxoid chondrosarcoma is a low-to intermediate-grade soft tissue sarcoma that generally afects adults older than 35 years old. It typically presents as a deep-seated, slowly enlarging mass. Imaging studies are nonspecific, although the tumors frequently are lobular. Treatment consists of wide resection. Radiation is used if margins are close.

84

Quais são as caracteristicas dos condrossarcomas mesenquimais extraesqueleticos?

Extraskeletal mesenchymal chondrosarcoma is a rare high-grade tumor that generally afects patients 15 to 35 years old. The most common locations include the head and neck. Treatment consists of wide resection, radiation, and chemotherapy. The prognosis generally has been poor.