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Flashcards in Tumores Ósseos Malignos Deck (98):
0

Qual o tumor ósseo não hematológico mais comum?

Osteossarcoma

1

Qual a faixa etária mais comumente afetada pelo osteossarcoma primário?

primary high-grade osteosarcoma occurs most commonly in the second decade of life.

2

Qual o pico de incidência do osteossarcoma parosteal?

Parosteal osteosarcoma has a peak incidence in the third and fourth decades

3

Em que idade geralmente ocorrem os osteossarcomas secundários?

Secondary osteosarcomas (e.g., those that occur in the setting of Paget disease or previous radiation therapy) are more common in older individuals.

4

Quais patologias estão relacionadas a maior chance de desenvolvimento de osteossarcoma?

Osteosarcoma may be more common in patients with the hereditary form of retinoblastoma, Rothmund-Thomson syndrome, and Li-Fraumeni syndrome.

5

Quais os locais mais frequentes de desenvolvimento de osteossarcoma?

Most primary osteosarcomas occur at the sites of the most rapid bone growth, including the distal femur, the proximal tibia, and the proximal humerus.

6

Qual a queixa deve deixar o médico atento para o risco de osteossarcoma?

Night pain may be an important clue to the true diagnosis; however, only about 25% of patients experience this phenomenon.

7

Quais são os achados radiográficos dos osteossarcomas?

he most common appearance is that of an aggressive lesion in the metaphysis of a long bone. Approximately 10% are primarily diaphyseal, and less than 1% are primarily epiphyseal. Although the lesion can be either predominantly blastic or predominantly lytic, more commonly areas of bone production and bone destruction are present. The lesion usually is quite permeative, and the borders are ill defined.

8

Quais são as reações periosteais encontradas nas radiografias dos osteossarcomas?

Periosteal reaction may take the form of a “Codman triangle,” or it may have a “sunburst” or “hair-on-end” appearance.

9

Qual o local mais comum de metástase por osteossarcoma?

Pulmões

10

Quais são as subcategorias dos osteossarcomas primarios?

Primary osteosarcomas are subcategorized as conventional osteosarcoma, low-grade intramedullary osteosarcoma, parosteal osteosarcoma, periosteal osteosarcoma, high-grade surface osteosarcoma, telangiectatic osteosarcoma, and small cell osteosarcoma.

11

Quais são as caracteristicas dos osteossarcomas periosteais?

Periosteal osteosarcoma is an intermediate-grade malignancy that arises on the surface of the bone. The most common locations are the diaphyses of the femur and tibia.

12

Qual a localização mais frequente dos osteossarcomas parosteais?

It has a peculiar tendency to occur as a lobulated ossified mass on the posterior aspect of the distal femur.

13

Como diferenciar miosite ossificante de osteossarcoma parosteal pela TC?

The ossification in myositis ossificans is more mature at the periphery of the lesion, whereas the center of a parosteal osteosarcoma is more heavily ossified.

14

Qual subtipo de osteossarcoma é o mais raro?

High-grade surface osteosarcoma is the least common type of osteosarcoma.

15

Qual é a aparencia radiográfica do osteossarcoma teleangectásico?

Telangiectatic osteosarcoma is a purely lytic lesion. On a radiograph, it can have an invasive appearance or it can have a ballooned appearance similar to that of an aneurysmal bone cyst.

16

Quais são os fatores mais associados ao desenvolvimento de osteossarcoma secundário?

The most common factors associated with secondary osteosarcomas include Paget disease and previous radiation therapy. The incidence of osteosarcoma in Paget disease is approximately 1% and may be higher (5% to 10%) for patients with advanced polyostotic disease. Pelvis is the most common location.
Other conditions that have been reported to be associated with secondary osteosarcomas include fibrous dysplasia, bone infarcts, osteochondromas, chronic osteomyelitis, melorheostosis, and osteogenesis imperfecta;

17

Como era e como é a taxa de sobrevida de longo termo dos pacientes com osteossarcoma?

Before the advent of multiple-agent chemotherapy, the prognosis for patients with osteosarcoma was dismal. Despite treatment consisting of wide or radical amputation, approximately 80% of patients died as a result of distant metastases, usually within 2 years. With today’s multiple-agent chemotherapy regimens and appropriate surgical treatment, most series report long-term survival of 60% to 75% for patients with high-grade osteosarcoma without metastases at initial presentation and 90% for those with low-grade lesions.

18

Qual a porcentagem aproximada de pacientes que se apresentam com metástase no momento do diagnóstico?

Approximately 15% of patients with osteosarcoma have detectable pulmonary metastases at the time of diagnosis.

19

Qual o fator prognóstico mais importante nos osteossarcomas?

Presença ou não de metástase (skip, pulmonar ou óssea)

20

Qual a relação da localização do osteossarcoma com seu prognóstico?

Skeletal location also is thought to be important because patients with more proximal tumors do worse than patients with more distal tumors.

21

Como é realizado o tratamento dos osteossarcomas?

Currently, at most musculoskeletal oncology centers, the treatment of high-grade osteosarcoma consists of neoadjuvant chemotherapy, wide or radical surgery (resection or amputation), and adjuvant chemotherapy.

22

Como avaliar se um tumor respondeu bem à quimioterapia neoadjuvante?

The histological response of the primary tumor to neoadjuvant chemotherapy has been shown to be a good predictor of long-term survival. Greater than 90% tumor necrosis indicates a very good prognosis.

23

Qual é a taxa de recorrência dos osteossarcomas?

About 10% of patients have local recurrence after wide resection or wide amputation.

24

Quais são os fatores de pior prognóstico para pacientes com recidiva dos osteossarcomas?

Poor prognostic factors include rapid relapse after completion of the initial treatment, many (more than eight) pulmonary nodules, large (>3 cm) pulmonary nodules, and unresectable pulmonary nodules. Patients with a few, small, resectable pulmonary nodules that occur late may have a 40% chance of cure with aggressive treatment.

25

Em quais faixas etárias ocorrem os picos de incidencia dos condrossarcomas?

It occurs over a broad age range, with peaks between 40 and 60 years for primary chondrosarcoma and between 25 and 45 years for secondary chondrosarcoma.

26

Quais são os locais mais comumentes afetados pelos condrossarcomas?

Most are located in a proximal location such as the pelvis, proximal femur, and proximal humerus. Although chondrosarcomas rarely occur in the hand, they are the most common primary malignancy of bone in this location.

27

Quais são as entidades benignas relacionadas aos condrossarcomas secundarios?

Secondary chondrosarcomas arise at the site of a preexisting benign cartilage lesion. They occur most frequently in the setting of multiple enchondromas and multiple hereditary exostoses. In Ollier disease (multiple enchondromas) the incidence of malignancy (most commonly chondrosarcoma) is approximately 25% by age 40 years, and in patients with Mafucci syndrome (multiple enchondromas with soft tissue hemangiomas) the incidence may be even higher.
Other conditions that have been reported to be associated with secondary chondrosarcoma include synovial chondromatosis, chondromyxoid ibroma, periosteal chondroma, chondroblastoma, previous radiation treatment, and ibrous dysplasia.

28

Quais são as características radiográficas dos condrossarcomas?

The radiographic appearance of chondrosarcoma frequently is diagnostic. Similar to enchondroma, it is a lesion arising in the medullary cavity with irregular matrix calcification. The pattern of calcification has been described as “punctate,” “popcorn,” or “comma shaped.” Compared with enchondroma, however, chondrosarcoma has a more aggressive appearance with bone destruction, cortical erosions, periosteal reaction, and, rarely, a soft tissue mass.

29

Qual a espessura do "cap" do osteocondroma deve levantar a suspeita de um condrossarcoma em um paciente esqueléticamente maduro?

Finally, the size of the cartilaginous cap of an osteochondroma, as evaluated with CT or MRI, is important in evaluating the possibility of a secondary chondrosarcoma. If the cartilaginous cap is larger than 2 cm in a skeletally mature patient, a secondary chondrosarcoma must be considered.

30

Quais são as caracteristicas dos condrossarcomas de células claras?

Clear cell chondrosarcoma has a strong tendency to arise in an epiphysis (especially the proximal femur). It may have benign radiographic features and can be confused with chondroblastoma or giant cell tumor.

31

Qual é o tratamento para os condrossarcomas de baixo e alto grau?

Extended curettage is considered adequate treatment only for low-grade lesions that are confined within the medullary canal. Those with soft tissue extension should be treated similar to high-grade lesions. The treatment of high-grade chondrosarcoma is wide or radical resection or amputation.

32

Qual a sobrevida dos pacientes com condrossarcoma?

Patients with low-grade lesions have been reported to have a greater than 90% 10-year survival rate, whereas patients with high-grade conventional chondrosarcoma are reported to have a 20% to 40% 10-year survival rate. he 5-year survival rate is less than 15% for patients with dediferentiated chondrosarcoma, with most deaths occurring in the irst 2 years.

33

Qual é o tumor osseo mais comum em crianças < 10 anos?

Ewing

34

Qual é a localização mais comum do sarcoma de Ewing?

The most common locations include the metaphyses of long bones (often with extension into the diaphysis) and the flat bones of the shoulder and pelvic girdles.

35

Em qual população o sarcoma de Ewing é raro?

Ewing sarcoma is exceedingly rare in individuals of African descent.

36

Além da dor, quais são os outros sintomas do sarcoma de Ewing?

In addition to pain, patients also may have fever, erythema, and swelling, suggesting osteomyelitis.

37

Como é a apresentação radiografica do sarcoma de Ewing?

Classically, Ewing sarcoma appears radiographically as a destructive lesion in the diaphysis of a long bone with an “onion skin” periosteal reaction. In reality, Ewing sarcoma more often originates in the metaphysis of a long bone but frequently extends for a considerable distance into the diaphysis.

38

Qual é o local mais comum de metástases no sarcoma de Ewing? E o segundo?

Pulmão e osso

39

Qual a translocação mais comum no sarcoma de Ewing? Quais outras translocações podem estar presentes?

The t(11;22) (q24;q12) is the most common translocation diagnostic of Ewing sarcoma and is present in greater than 90% of cases. Other diagnostic translocations, including t(21;22)(q22;q12) and t(7;22)(p22;q12), also have been identified.

40

Qual a coloração imuno-histoquimica específica para o sarcoma de Ewing?

CD99

41

Como diferenciar linfomas e sarcoma de Ewing histologicamente?

In addition, Ewing sarcomas usually are periodic acid–Schif positive (owing to intracellular glycogen) and reticulin negative. This is in contrast to lymphomas, which are periodic acid–Schif negative and reticulin positive.

42

Qual o pior fator para diminuição do prognóstico dos pacientes com sarcoma de Ewing?

The worst prognostic factor is the presence of distant metastases. Even with aggressive treatment, patients with metastases have only a 20% chance of long-term survival.

43

Qual outra expressão genética parece ter fator importante no prognóstico do sarcoma de Ewing?

Secondary genetic alterations, such as aberrant TP53 expression, may prove to be important.

44

Como tratar o sarcoma de Ewing?

At this time, the choice between surgery and radiation for treatment of the primary lesion must be made on an individual basis. Repeat staging studies should be obtained ater neoadjuvant chemotherapy. The repeat radiographs often show increased ossification, and repeat MRI often shows a marked decrease in the soft tissue mass. At this point, if it seems that the lesion can be resected with wide margins with an acceptable functional deficit, surgery should be the treatment of the primary lesion. If wide margins would be dificult to obtain or if the functional deficit resulting from surgery would be unacceptable, radiation of the primary lesion is an acceptable alternative. Radiation also can be used as an adjuvant ater a marginal resection or a contaminated wide resection.

45

Quais são as regiões mais acometidas pelos cordomas?

Greater than 50% of chordomas arise in the sacrococcygeal area, and more than 30% arise at the base of the skull.

46

Quais são os picos de incidência dos cordomas?

Peak incidence for sacrococcygeal chordomas occurs in the fifth to seventh decades, whereas the peak for sphenooccipital lesions is the fourth to sixth decades.

47

Quais são as apresentações clínicas dos pacientes com cordoma?

Patients with tumors in the sphenooccipital region may report headaches or symptoms related to cranial nerve compression. In the spine, symptoms can be caused by nerve root or cord compression. If an anterior mass exists with a cervical spine lesion, the symptoms may be similar to those caused by a retropharyngeal abscess. Patients with tumors in the sphenooc- cipital region may report headaches or symptoms related to cranial nerve compression. In the spine, symptoms can be caused by nerve root or cord compression. If an anterior mass exists with a cervical spine lesion, the symptoms may be similar to those caused by a retropharyngeal abscess. The most common presenting complaint for patients with sacrococcygeal tumors is low back pain. Bowel and bladder disturbance and sciatic pain also are common with sacral tumors. A palpable mass frequently is present on rectal examination.

48

Qual a porcentagem de cordomas se apresenta com calcificações na radiografia?

More than 50% of chordomas exhibit radiographically detectable calcification.

49

Quais são as opções terapêuticas para os cordomas?

The primary treatment is surgical resection with wide margins, even if this creates a neurological deficit, because progressive growth of the tumor would create a neurological deficit anyway and possibly metastatic disease. Resection that preserves the S3 nerve roots bilaterally results in relatively normal bowel and bladder function, whereas resection above this level results in incremental loss of bowel and bladder function. Resection of bilateral S2 nerve roots results in complete loss of control of bowel and bladder function. If wide margins cannot be obtained or if tumor contamination occurs intraoperatively, radiation may be beneficial. Radiation also may be beneficial for patients in whom resection is not feasible, although a cure is rarely, if ever, achieved in these patients.

50

Como se comportam as metastases nos cordomas?

Metastases are rare at initial presentation (<5%) but may occur later in 30% to 60%. In addition to the lungs, metastases are common in bone and in unusual locations such as skin, eyelid, brain, liver, and other internal organs.

51

Qual o local de acometimento mais comum dos adamantinomas?

It has a peculiar predilection for occurring in the tibia (approximately 85%) and may also involve the ipsilateral fibula.

52

Como é a apresentação radiografica dos adamantinomas?

The most common radiographic appearance is that of multiple, sharply demarcated radiolucent lesions in the tibial diaphysis. The radiolucent lesions are separated by areas of dense, sclerotic bone. Although the radiographic appearance is similar to that of osteofibrous dysplasia, adamantinoma usually has a more aggressive appearance.

53

Quais são as modalidades terapêuticas para os adamantinomas?

The optimal treatment of adamantinoma is wide resection or amputation. The tumor generally is radioresistant and chemoresistant. Local recurrence occurs in approximately 25% of patients, and amputation should be considered for these patients. Metastases are rare at presentation but may occur later in 30% of patients. Overall survival is approximately 85% at 10 years. Prognosis depends most on the adequacy of the surgical margin. Compared with patients who have marginal or intralesional surgical procedures, patients who have wide or radical procedures have significantly reduced rates of local recurrence and metastases (<10%).

54

Qual a terminologia utilizada pra os tumores malignos vasculares?

Although not strictly deined, most authors use the term hemangioendothelioma to describe low-grade malignant vascular tumors and the term angiosarcoma to describe high- grade malignant vascular tumors.

55

O que é a sindrome de Stewart-Treves?

Stewart-Treves syndrome refers to the occurrence of angiosarcoma in the setting of chronic lymphedema (e.g., in the upper extremity of a patient who has previously undergone a radical mastectomy).

56

Quais são as apresentações radiograficas dos tumores vasculares malignos?

The radiographic appearance of this lesion also is correlated with its grade. Low-grade tumors appear as well-demarcated lytic lesions that may or may not have surrounding reactive bone formation. High-grade tumors have a more permeative appearance. Periosteal reaction is unusual. Malignant vascular tumors have a peculiar tendency to be multicentric at presentation regardless of grade. Most commonly, multiple lesions are found within the same bone or within multiple bones of the same extremity.

57

Quais os antigenos são positivos para lesões vasculares (imuno-histoquimica)?

Although metastatic carcinoma and malignant vascular tumors may be keratin positive, factor VIII–related antigen, CD31, and CD34 should be positive only in vascular tumors.

58

Quais são as opções terapêuticas para os tumores vasculares malignos?

Treatment is individualized depending on the clinical situation. Solitary lesions are treated with wide resection if possible. Radiation can be used successfully in the treatment of surgically inaccessible lesions or in the treatment of multiple lesions. For high-grade lesions, adjuvant chemotherapy can be added to the treatment regimen. Prognosis depends most on grade. Patients with low-grade lesions may have better than an 80% chance for long-term survival, whereas patients with high-grade tumors have less than a 20% long-term survival rate.

59

Quais são as entidades relacionadas ao desenvolvimento secundário do fibrosarcoma ou histiocitoma fibroso maligno?

Approximately 25% of these tumors are considered to be secondary to a preexisting bone abnormality. The most commonly reported predisposing conditions include Paget disease, radiation, giant cell tumor, and bone infarction.

60

Como é a apresentação radiográfica do fibrossarcoma/histiocitoma fibroso maligno?

Radiographically, these tumors have an aggressive appearance and are typically purely lytic with indistinct borders. They may appear as an area of bone destruction adjacent to an otherwise typical area of Paget disease or bone infarction. Periosteal reaction is absent, unless a pathological fracture has occurred.

61

Como é o tratamento para o fibrossarcoma/histiocitoma fibroso maligno?

Igual ao osteossarcoma

62

Qual o tumor maligno primário mais comum dos ossos?

Multiple myeloma is the most common primary malignancy of bone, representing more than 40% of primary bone cancers. Its peak incidence is in the ith to seventh decades with a 2 : 1 male predominance

63

Qual é a localização mais comum do mieloma múltiplo?

The spine is the most common location, followed by the ribs and pelvis.

64

Como é a apresentação radiográfica do mieloma multiplo?

Radiographically, multiple myeloma appears as multiple, “punched-out,” sharply demarcated, purely lytic lesions without any surrounding reactive sclerosis.

65

Como é feito o diagnóstico do mieloma multiplo?

The diagnosis usually can be confirmed by serum immunoelectrophoresis, which shows a monoclonal gammopathy.

66

Qual o produto celular encontrado no osso que pode indicar um mieloma multiplo?

Amyloid production can be abundant. (With the exception of patients on long-term hemodialysis, the presence of amyloid in bone usually means a diagnosis of multiple myeloma.)

67

Como diferenciar um plasmocitoma de uma lesão osteomielitica cronica?

In patients with a solitary plasmacytoma, the pathological diferential diagnosis may include chronic osteomyelitis with abundant plasma cells. In this situation, immunohistochemistry can be helpful. Plasmacytoma exhibits monoclonal κ or λ light chains, whereas the plasma cells of chronic osteomyelitis are polyclonal.

68

Como diferenciar linfoma de mieloma usando imuno-histoquimica?

Lymphoma cells usually stain positive for CD45 (leukocyte common antigen) and CD20 (a B-cell marker), whereas myeloma cells usually are negative.

69

Qual é o tratamento de escolha para o mieloma múltiplo?

The primary treatment of multiple myeloma is chemotherapy.

70

Em qual idade o linfoma se torna mais comum?

Lymphoma can occur at any age but becomes more common in the sixth and seventh decades of life.

71

Quais são os locais (ossos) mais acometidos por linfoma?

The femur is the most common bone involved, followed by the pelvis, spine, and ribs.

72

Como é a aparência radiográfica do linfoma?

Radiographically, lymphoma usually appears as an ill-defined area of bone destruction—frequently diaphyseal— and oten has a permeative appearance. The cortex may be thickened, but a periosteal reaction rarely is seen. Frequently, a large portion of the bone or even the entire bone can be involved. The extent of the lesion may seem large compared with the patient’s symptoms. Radiographs can be entirely normal despite extensive involvement of the medullary canal as seen on bone scan or MRI. (Lymphoma usually should be included in the diferential diagnosis of a patient who has bone pain and an abnormal bone scan or MRI with normal radiographs.)

73

Qual é o tratamento de escolha para os linfomas?

The primary treatment of lymphoma is chemotherapy.

74

Quais são os sitios primários que mais lançam metástases para o osso?

Most carcinomas metastatic to bone are from the breast and prostate, followed by the lung, kidney, thyroid, and gastrointestinal tract in order of decreasing frequency.

75

Qual a aparência radiografica dos carcinomas metastáticos?

Breast cancer and prostate cancer typically produce blastic lesions. Kidney cancer and thyroid cancer usually are purely lytic. Lung cancer may produce a mixed appearance. If the lesion is distal to the elbow or knee, lung cancer is the most likely primary lesion. Additionally, metastatic lung cancer may have the distinct appearance of a “bite” taken out of the cortex.

76

Como é a escala de Mirels para osteossintese profilática e qual o valor acima do qual a osteossintese está indicada?

SCORE
VARIABLE 1 2 3
Site Upper limb Lower limb Peritrochanter
Pain Mild Moderate Funcional
Size 1/3 1/3-2/3 >2/3
Lesion Blastic Mixed Litic

Osteossíntese profilática indicada para pontuações > 7

77

O que significa a reação periosteal?

Tumor é extracompartimental

78

Qual indicação de radioterapia nos osteossarcomas?

Em altas doses quando inoperável com caráter paliativo

79

Quanto tempo após o inicio da quimioterapia está indicado a excisão cirurgica?

Por volta da 10a semana após o início da quimioterapia

80

Quais são as modalidades de hemipelvectomia?

- Interna: com preservação do membro
- Externa: retira o membro

81

No osteossarcoma, a metástase que denota pior prognóstico?

Óssea

82

Qual é a síndorme de "Li Fraumeni"?

Deficiencia do p53 (gene supressor de tumor). Comum neoplasias solidas como rim, mama e SNC. Osteossarcoma é o tumor osseo mais comum

83

Quando realizar radioterapia no tumor de Ewing?

Após a ressecção

84

Não é achado frequento no tumor de Ewing?

Skip metastase

85

Critérios diagnósticos de mieloma múltiplo?

Lesão de órgão alvo = CRAB (Hipercalcemia, insuficiencia renal, anemia, lesões ósseas)
10% de plasmócitos ativos no aspirado de medula
20% de plasmócitos ativos no esfregaço de sangue periférico

86

Qual o padrão caracteristico microscopico dos adamantinomas?

Ilhas de células epiteliais basalóides

87

Quais são as drogas utilizadas na quimioterapia para osteossarcoma?

Cisplatina, doxorrubicina, metotrexato, dexrazoxane, ciclofosfamida
Há diferença no ciclo de tratamento para metastáticos e não-metastáticos

88

A cintilografia é pouco sensivel para qual tipo de lesão?

Tumor de partes moles

89

Como é a classificação de Huvus para resposta à quimioterapia neoadjuvante?

Grau % de necrose Aparencia histológica

Grau I 0-49 Pouca/nenhuma necrose

Grau II 50-89 áreas com osteóide + tumor viável

Grau III 90-99 predominio de osteóide com focos de tumor viável

Grau IV 100 Sem evidência de tumor viável

90

Qual é o padrão histológico de o grau de malignidade do osteossarcoma parosteal?

Tecido ósseo mais fibroso e baixo grau de malignidade

91

Quais outros tumores apresentam a t(11,22) além do sarcoma de Ewin?

Tumores PNET (neuroectodérmicos)

92

Qual a região de maior prevalência do fibrossarcoma intraósseo?

1/3 distal do fêmur e 1/3 proximal da tíbia

93

A imagem radiográfica em "vela derretida"é tipica de qual tumor?

Melorreostose

94

Como diferenciar sarcoma de Ewing e linfoma pela imuno-histoquímica?

O anticorpo LCA (antigeno leucocitário comum) é expresso nos linfomas e o CD99 nos sarcomas de Ewing

95

Qual exame deve ser realizado no paciente com sarcoma de Ewing e que geralmente não é realizado em outros tipos de tumor?

Aspirado de medula óssea (procurar a t11,22 fora do local do tumor).

96

Qual é o tratamento padrão para o sarcoma de Ewing?

Quimioterapia neoadjuvante, controle local (cirurgia, radioterapia ou ambos) e quimioterapia adjuvante

97

O que é megaterapia?

Transplante de medula óssea