Tumores Benignos Flashcards

0
Q

Qual a predileção de envolvimento do osteoma osteoide?

A

Predilection for the lower extremity, with half the cases involving the femur or tibia.

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1
Q

Qual a população mais comumente afetada por osteoma osteoide?

A

Homem jovem (2a e 3a décadas de vida)

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2
Q

Qual é apresentação clássica do osteoma osteoide?

A

The typical patient with an osteoid osteoma has pain that is worse at night and is relieved by aspirin or other nonsteroidal antiinflammatory medications.

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3
Q

O que pode ocorrer com uma articulação ou com a coluna vertebral de um paciente com osteoma osteoide nessas localizações?

A

When the lesion is near a joint, swelling, stifness, and contracture may occur. When the lesion is in a vertebra, scoliosis may occur.

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4
Q

Qual é a aparência do osteoma osteoide no rx?

A

The lesion consists of a small (<1.5 cm) central radiolucent nidus with surrounding bony sclerosis.

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5
Q

Qual é o melhor metodo de imagem para diagnostico de osteoma osteoide?

A

Tomografia

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6
Q

Quando indicar biopsia para ostema osteoide?

A

Raramente

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7
Q

Como tratar osteoma osteoide?

A

Multiple treatment options are available, including medical treatment, percutaneous radiofrequency ablation, and open surgical procedures. If the patient’s symptoms are adequately controlled, and the patient is willing to undergo long-term medical management, antiinflammatory medication can be used as the definitive treatment. Patients treated in this manner usually experience spontaneous healing of the lesion within 3 to 4 years.
Most patients with lesions of the pelvis or long bones of the extremities can be treated with percutaneous radiofrequency ablation.

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8
Q

Qual a taxa de recorrencia do osteoma osteoide?

A

Menos que 10%

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9
Q

Como é realizada a tecnica de radiofrequencia?

A

This technique involves a CT-guided core needle biopsy after which a radiofrequency electrode is inserted through the cannula of the biopsy needle. The temperature at the tip is increased to 90°C for 6 minutes.

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10
Q

Em quais osteomas osteoides a tecnica de radiofrequencia é contra-indicada?

A

The procedure may not be indicated for vertebral lesions (due to risk of thermal injury to the spinal cord) or lesions of the small bones of the hands or feet (due to risk of thermal injury to the skin).

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11
Q

Quais são os locais mais comum de ilhas osseas/enostoses?

A

Almost any bone can be involved, but the pelvis and the femur are the most common sites.

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12
Q

Qual diagnostico diferencia deve ser feito com enostoses?

A

Osteopoikilosis is a rare condition consisting of multiple small bone islands throughout the skeleton. Autosomal dominant and sporadic forms of the syndrome have been identiied.

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13
Q

Qual é a aparencia radiografica das enostoses?

A

They typically are small, round or oval areas of homogeneous increased density within the cancellous bone. Radiating spicules on the periphery of the bone islands merge with the native bone creating a brushlike border.

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14
Q

Como tratar um paciente com enostoses?

A

Most patients with bone islands can be treated with observation with serial plain radiographs. As long as the lesions remain asymptomatic and do not grow, no further intervention is indicated. If a patient experiences pain, or if the lesion grows, biopsy is indicated to rule out more aggressive lesions, such as a sclerosing osteosarcoma, blastic metastasis, or sclerotic myeloma.

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15
Q

Qual a região mais comum de localização dos condromas?

A

Although any bone can be involved, the phalanges of the hand are the most common location. They are the most common tumor of the small bones of the hands and feet.

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16
Q

Como é o crescimento dos condromas?

A

They usually arise in the medullary canal, where they are referred to as “enchondromas.” Rarely, they arise on the surface of the bone, where they are referred to as “periosteal chondromas” or “juxtacortical chondromas.

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17
Q

Como se caracteriza a Doença de Ollier?

A

Multiple enchondromatosis, also known as Ollier disease, is a rare condition in which many cartilaginous tumors appear in the large and small tubular bones and in the flat bones. It is caused by failure of normal endochondral ossification.
The tumors are located in the epiphysis and the adjacent parts of the metaphysis and shat, and many bones may be afected.

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18
Q

Como se caracteriza a Síndrome de Mafucci?

A

Multiple lesions of the small bones of the hand may cause considerable disability. When associated with hemangiomas of the overlying soft tissues the disease is known as Mafucci syndrome.

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19
Q

Qual a porcentagem de malignização dos condromas na Doença de Ollier?

A

Approximately 25% of patients with Ollier disease are diagnosed with sarcomas by 40 years of age.

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20
Q

Qual o aspecto radiologico dos condromas?

A

Enchondromas are benign-appearing tumors with intralesional calcification. The calcification is irregular and has been described as “stippled,” “punctate,” or “popcorn”.

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21
Q

Como diferenciar radiograficamente um condroma de um condrossarcoma?

A

In more proximal locations (e.g., the pelvis, proximal humerus, or proximal femur), deep endosteal erosion (two thirds of the thickness of the cortex) frequently indicates a chondrosarcoma. An associated soft tissue mass is never present with an enchondroma and always indicates a chondrosarcoma.

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22
Q

Como tratar um paciente com condroma?

A

Treatment of patients with solitary enchondromas usually consists of observation with serial radiographs. If the lesion remains radiographically stable and asymptomatic, no further intervention is indicated. If a lesion grows, or if it becomes symptomatic, extended curettage usually is curative.

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23
Q

Como tratar um paciente com encondromatose multipla?

A

Treatment of patients with multiple enchondromatosis can be more diicult. Although the individual lesions usually are not treated, the more obvious deformities can be corrected by osteotomy. These patients also must be monitored indefinitely for malignant change.

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24
Q

Como se caracteriza o comportamento dos osteocondromas?

A

The lesions consist of a bony mass, often in the form of a stalk, produced by progressive endochondral ossification of a growing cartilaginous cap. In contrast to true neoplasms, their growth usually parallels that of the patient and usually ceases when skeletal maturity is reached. Most lesions are found during the period of rapid skeletal growth. About 90% of patients have only a single lesion. Osteochondromas may occur on any bone preformed in cartilage but usually are found on the metaphysis of a long bone near the physis.

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25
Q

Quais são os locais mais comuns de desenvolvimento de osteocondromas?

A

They are seen most often on the distal femur, the proximal tibia, and the proximal humerus.

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26
Q

O que é a doença de Trevor?

A

Trevor disease (dysplasia epiphysealis hemimelica) refers to an intraarticular epiphyseal osteochondroma. When multiple joints are involved, it is usually unilateral (hemimelica).

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27
Q

Quais são as caracteristicas geneticas da exostose multipla hereditaria?

A

Multiple hereditary exostoses is an autosomal dominant condition with variable penetrance. Most patients with this disorder have a mutation in one of two genes: EXT1, which is located on chromosome 8q24.11-q24.13, or EXT2, which is located on chromosome 11p11-12.

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28
Q

Quais são as caracteristicas clinicas da exostose multipla familiar?

A

Presence of many exostoses, but disturbances in growth also occur, such as abnormal tubulation of bones, producing broad and blunt metaphyses, and sometimes bowing of the radius and shortening of the ulna, producing ulnar deviation of the hand. The disease occurs only 5% to 10% as often as solitary osteochondroma and is more common in males.

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29
Q

Como são classificados os osteocondromas?

A

Osteochondromas are of two types: pedunculated and broad based or sessile.

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30
Q

Quais são as caracteristicas dos osteocondromas pedunculados?

A

Pedunculated tumors are more common, and any definite stalk is directed away from the physis adjacent to which it takes its origin. The projecting part of the lesion has cortical and cancellous components, both of which are continuous with corresponding components of the parent bone. The lesion is covered by a cartilaginous cap that often is irregular and usually cannot be seen on radiographs; occasionally, calciication within the cap may be seen. Typically, the cap is only a few millimeters thick in adults, although it may be 2 cm thick in a child.

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31
Q

Qual é a taxa de malignização dos osteocondromas?

A

Approximately 1% for patients with a solitary osteochondroma and 5% for patients with multiple hereditary exostoses.

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32
Q

Quando suspeitar de transformação maligna de um osteocondroma?

A

Malignant transformation should be suspected when a previously quiescent lesion in an adult grows rapidly; it usually takes the form of a low-grade chondrosarcoma. In these cases, the cartilage cap usually is more than 2 cm thick. Malignant transformation is best evaluated by CT or MRI.

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33
Q

Quais são as caracteristicas da exostose subungueal?

A

A similar lesion, subungual exostosis, may develop on a distal phalanx, especially of the great toe. Often there is a definite history of trauma. Excision is indicated when elevation of the nail produces pain. The history and location of the lesion distinguish it from a true osteochondroma.

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34
Q

Qual a porcentagem de crianças que se acredita possuirem fibromas não-ossificantes?

A

Nonossifying fibromas (also known as metaphyseal fibrous defects, fibrous cortical defects, and fibroxanthomas) are common developmental abnormalities and are believed to occur in 35% of children.

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35
Q

Comente a epidemiologia dos fibromas não-ossificantes.

A

Generally, these lesions occur in the metaphyseal region of long bones in individuals 2 to 20 years old. Although any bone may be involved, approximately 40% of these lesions are found in the distal femur, 40% in the tibia, and 10% in the fibula.

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36
Q

Quais são as caracteristicas radiologicas dos fibromas não-ossificantes?

A

On plain radiographs, a nonossifying ibroma appears as a well-defined lobulated lesion located eccentrically in the metaphysis. Multilocular appearance or ridges in the bony wall, sclerotic scalloped borders, and erosion of the cortex are frequent findings. There is no periosteal reaction in the absence of a pathological fracture.

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37
Q

Como tratar um fibroma não-ossificante?

A

Most nonossifying fibromas are asymptomatic and regress spontaneously in adulthood. Most pathological fractures can be treated nonoperatively.
Some authors have recommended treatment for lesions that are larger than 50% of the diameter of the bone because of a theoretical increased risk of pathological fracture, although this parameter is not universally accepted as an indication for surgery. Recurrence ater curettage is rare.

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38
Q

Quais são as caracteristicas de um “cortical desmoid”?

A

A cortical desmoid is an irregularity in the posteromedial aspect of the distal femoral metaphysis and usually is seen in boys 10 to 15 years old. It may be a reaction to muscle stress exerted by the adductor magnus. The lesion is best seen on an oblique radiograph made with the lower extremity externally rotated 20 to 45 degrees. Clinical symptoms, if any, include soft tissue swelling and pain. Radiographs and MRI reveal erosion of the cortex with a sclerotic base. A biopsy is not warranted. Treatment usually consists of observation.

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39
Q

Qual é a caracteristica da displasia fibrosa? Quais são as suas formas?

A

The hallmark is replacement of normal bone and marrow by fibrous tissue and small, woven spicules of bone. May exist in a monostotic or polyostotic form.

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40
Q

Quais são as localizações da displasia fibrosa? Quais outros achados clinicos podem estar associados?

A

Fibrous distrophy can occur in the epiphysis, metaphysis, or diaphysis. Associated abnormalities, such as sexual precocity, abnormal skin pigmentation, intramuscular myxoma, and thyroid disease, may be present.

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41
Q

Quais são os achados da síndrome de McCune-Albright?

A

McCune-Albright syndrome refers to polyostotic fibrous dysplasia, cutaneous pigmentation, and endocrine abdonormalites.

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42
Q

Como se caracteriza a sindrome de Mazabraud?

A

Mazabraud syndrome is polyostotic fibrous dysplasia with intramuscular myxomas.

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43
Q

Quais são as caracteristicas radiologicas da displasia fibrosa?

A

The radiographic appearance is characteristic, with the lucent area having a granular, ground-glass appearance with a well-defined sclerotic rim.

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44
Q

Como tratar a displasia fibrosa?

A

Surgical treatment is indicated when significant deformity or pathological fracture occurs or when significant pain exists. Actual and impending pathological fractures are best treated with intra-medullary fixation when possible. Deformities are corrected by osteotomy with internal ixation.

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45
Q

Qual tipo de medicação os estudos mostram alguma eficacia para pacientes com displasia fibrosa?

A

Studies suggest that treatment with bisphosphonates probably is beneficial for patients with extensive disease.

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46
Q

O que é a doença de Campanacci?

A

Osteoibrous dysplasia (ossifying fibroma of long bones, also known as Campanacci disease) is a rare lesion usually afecting the tibia and fibula.

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47
Q

Qual a região mais comumente afetada pela displasia osteofibrosa?

A

The middle third of the tibia is the most frequently afected location, and although the lesion usually is diaphyseal, it may encroach on the metaphysis. The tibia is enlarged and often bowed anterolaterally.

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48
Q

Qual é a caracteristica radiografica da displasia osteofibrosa?

A

The radiographs show eccentric intracortical osteolysis with expansion of the cortex.

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49
Q

Quais são os diagnosticos diferenciais da displasia osteofibrosa?

A

The lesion must be distinguished from adamantinoma and monostotic fibrous dysplasia.

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50
Q

Qual o curso natural da displasia osteofibrosa?

A

Unpredictble. Some lesions regress spontaneously during childhood; most progress during childhood, but not ater puberty. Recurrence rates are high ater curettage or marginal resection in children. Conversely, recurrence rates are low ater surgery in skeletally mature patients. Pathological fractures can be treated nonoperatively. Surgical management is aimed at preventing or correcting deformity.

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51
Q

Quais são as caracteristicas do fibroma desmoplastico?

A

Raro, localmente agressivo, incidencia maior em pessoas na 2-3a decadas de vida. Envolve mais comumente ossos longos tubulares, mas casos de envolvimento pelvico, coluna vertebral, cranio e mandibula já foram descritos.

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52
Q

Quais são os achados radiológicos dos fibromas desmoplasticos?

A

Radiographs typically reveal a well-circumscribed lytic lesion with a narrow zone of transition and frequently a thin rim of reactive bone. Cortical destruction may be present. The lesion sometimes appears septate. On MRI, desmoplastic ibroma, similar to other fibrous tumors, may show low signal intensity on T1- and T2-weighted images.

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53
Q

Qual o tratamento recomendado para os fibromas desmoplasticos?

A

This tumor does not metastasize, but local recurrence is common after simple curettage. Most authors have recommended wide resection.

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54
Q

Qual a faixa etaria de incidencia dos cistos osseos unicamerais?

A

Unicameral bone cysts are common lesions of childhood more consistent with a developmental or reactive lesion than a true tumor. Eighty-five percent occur in the first 2 decades with a 2 : 1 male predominance.

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55
Q

Quais são os locais de acometimento mais comum dos cistos osseos?

A

Most common in the proximal humerus and femur (child). In adults, the ilium and calcaneus are more common locations.

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56
Q

Qual o comportamento classico dos cistos osseos?

A

The lesions are most active during skeletal growth and usually heal spontaneously at maturity. Unicameral bone cysts often are asymptomatic, unless a pathological fracture has occurred. Two thirds of patients present with fractures, which can stimulate the cyst to heal.

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57
Q

Quais são os achados radiograficos dos cistos osseos unicamerais?

A

Plain radiographs reveal a centrally located, purely lytic lesion with a well-marginated outline and usually are diagnostic for a unicameral bone cyst. The cyst may expand concentrically but never penetrates the cortex. Prominent osseous ridges on the inner cortical wall may give it a multiloculated appearance. No periosteal reaction is present, unless there has been a fracture.

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58
Q

Qual é o sinal radiografico patognomonico de cisto osseo unicameral fraturado?

A

Occasionally (20%), a thinned cortical fragment fractures and falls into the base of the lesion, confirming its empty cystic nature. This “fallen fragment” sign is pathognomonic of a unicameral bone cyst with a fracture.

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59
Q

Como são classificados os cistos osseos unicamerais?

A

Unicameral bone cysts are classified as active when they are within 1 cm of the physis and latent when they are closer to the diaphysis.

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60
Q

Como tratar os cistos osseos unicamerais?

A

Small, asymptomatic lesions in the upper extremities can be treated with observation with serial plain radiographs. Larger lesions (lesions at risk for pathological fracture), symptomatic lesions, and lesions in the lower extremities usually are treated with curettage (with or without bone grafting or internal fixation) or aspiration and injection (often using corticosteroids, bone marrow aspirate, demineralized bone matrix, or other materials). Pathological fractures in the upper extremity can be treated conservatively because the fracture may initiate cyst “healing.” Fractures through unicameral bone cysts in the proximal femur should be treated with curettage, bone grafting, and internal fixation. Flexible intramedullary nailing has been used in the femur and humerus and provides early stability, often obviating the need for a cast, and decreases the risk of further pathological fracture.

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61
Q

Quais são os locais de acometimento mais comuns do cisto osseo aneurismatico?

A

Most common locations include the proximal humerus, distal femur, proximal tibia, and spine.

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62
Q

Quando acomete a vertebra, qual o local mais comumumente afetado?

A

Vertebral lesions, accounting for 15% to 20% of these entities, are located in the posterior elements with frequent extension into the vertebral body or to adjacent levels.

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63
Q

Qual é a faixa etária de incidencia do cisto osseo aneurismático e o genero mais acometido?

A

Most occur in patients younger than 20 years old, and there is a slight female predominance.

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64
Q

Quais são os achados radiográficos dos cistos osseos aneurismaticos?

A

Radiographs reveal an expansile lytic lesion that elevates the periosteum but remains contained by a thin shell of cortical bone. An aneurysmal bone cyst can have well-defined margins or a permeative appearance that mimics a malignancy. It is most often eccentrically located in the metaphysis.

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65
Q

Como diferenciar um cisto osseo simples de um cisto osseo aneurismarico na ressonancia magnetica?

A

When diferentiating between a unicameral and aneurysmal bone cyst using MRI, the presence of a double-density fluid level and intralesional septations usually indicates an aneurysmal bone cyst.

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66
Q

Como tratar os cistos osseos aneurismaticos?

A

We treat most aneurysmal bone cysts with extended curettage and grafting with a bone graft substitute. Because the lesion may produce heavy bleeding, tourniquet control is advised. Marginal resection sometimes is indicated for lesions in expendable bones. Lesions in the spine or pelvis can be treated with preoperative embolization to minimize surgical blood loss

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67
Q

Qual é a taxa de recorrencia dos cistos osseos aneurismaticos após o tratamento?

A

The recurrence rate after curettage of an aneurysmal bone cyst is 10% to 20%. Recurrence has been correlated with age younger than 15 years, centrally located cysts, and incomplete removal of the cystic cavity contents.

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68
Q

Quais são os locais tipicos de acometimento dos cistos intraosseos ganglionicos?

A

Occur typically in the ends of the long bones of middle-aged men, particularly the distal tibia, although the knee and shoulder are other common areas.

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69
Q

Quais são os achados radiograficos tipicos do cisto ganglionico intraosseo?

A

On radiographs and MRI they appear as uniloculated or multiloculated, well-demarcated, lytic defects with a thin rim of sclerotic bone.

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70
Q

Como tratar os cistos ganglionicos intraosseos?

A

Treatment of symptomatic lesions is by local excision of overlying sot tissues and curettage of the involved bone. Recurrence is uncommon.

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71
Q

Qual o local mais comum de acometimento dos cistos epidermoides?

A

Are found most often in the skull.

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72
Q

Qual a aparencia radiografica dos cistos epidermoides?

A

Radiographically, they appear as rarefied defects surrounded by sclerotic bone.

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73
Q

Qual a aparencia radiologica dos lipomas intra-osseos?

A

They usually appear as well-defined lucencies possibly with a thin rim of reactive bone. CT and MRI show well-defined lesions with the same signal characteristics as fat. Central necrosis or calcification sometimes is evident.

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74
Q

Quando indicar cirurgia para o lipoma intraosseo?

A

Surgery is indicated only for the rare symptomatic lesion. In these cases, simple curettage usually is curative.

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75
Q

Qual a porcentagem estimada de hemangioma na coluna vertebral assintomatica na população?

A

It is estimated that 10% of the population has asymptomatic lesions of the vertebral bodies.

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76
Q

Quais locais comuns de aparecimento de hemangiomas?

A

Vertebras e cranio

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77
Q

Qual o achado radiologico do hemangioma vertebral?

A

The radiographic appearance in the spine usually is characteristic, with thickened, vertically oriented trabeculae giving the classic “jailhouse” appearance. In cross section, these thickened trabeculae have a “polka dot” pattern on CT.

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78
Q

Qual o manejo dos hemangiomas?

A

Treatment usually is not necessary; however, multiple options exist for symptomatic lesions. Nerve root or cord decompression with spinal stabilization is needed for rare cases of vertebral collapse with neurological compromise. Most lesions of the long bones can be treated adequately with extended curettage.

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79
Q

Qual a população afetada pela doença de Paget?

A

Paget disease may afect 4% of people of Anglo-Saxon descent who are older than age 55 years, but it is rare in most other populations.

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80
Q

Quais são as fases da doença de Paget?

A

It is a disorder of unregulated bone turnover. Excessive osteoclastic resorption is followed by increased osteoblastic activity. An early lytic phase is followed by excessive bone production with cortical and trabecular thickening.

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81
Q

Quais são os achados radiograficos da doença de Paget?

A

Radiographic indings depend on the stage of the disease. In the lytic phase, bone resorption can take on a “blade of grass” or “flame” appearance beginning at the end of the bone and extending toward the diaphysis. Later the radiographs show bony sclerosis, thickened cortices, and thickened trabeculae. Bone scans usually are “hot”.

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82
Q

Qual o padrão caracteristico da biopsia na doença de Paget?

A

Biopsy usually reveals a characteristic “mosaic” pattern with widened lamellae, irregular cement lines, and fibrovascular connective tissue.

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83
Q

Qual o manejo da doença de Paget?

A

Medical management of Paget disease consists of non-steroidal antiinflammatory drugs, calcitonin, or bisphosphonates. Serum alkaline phosphatase levels and urine pyridinium crosslinks can be used to monitor the activity of the disease. Orthopaedic management consists of correcting deformity and treating pathological fractures. During periods of active disease, intraoperative bleeding from afected bones can be massive.

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84
Q

Qual o risco de um paciente com doença de Paget desenvolver um sarcoma secundário?

A

Approximately 1% of patients with Paget disease develop a secondary bone sarcoma, usually an osteosarcoma. This risk is probably higher for patients with polyostotic disease.

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85
Q

Quais são as causas de hiperparatireoidismo?

A

Primary hyperparathyroidism usually is caused by an adenoma of the parathyroid glands. Secondary hyperparathyroidism can occur in patients with chronic renal failure.

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86
Q

Qual é o diagnostico diferencial do tumor marrom do hiperparatireioidismo?

A

Tumor de celulas gigantes. In hyperparathyroidism (1) the giant cells are a little smaller, often occurring in a nodular arrangement, especially around areas of hemorrhage; (2) the stromal cells are more spindle shaped and delicate; and (3) evidence of osseous metaplasia within the stroma is prominent.

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87
Q

Como determinar o diagnostico de hiperparatireoidismo?

A

serum calcium, phosphorus, alkaline phosphatase, and parathyroid hormone levels, rather than by histological examination of a focal lesion.

88
Q

Em quais pacientes devemos suspeitar um infarto osseo?

A

Bone infarcts frequently are seen in patients with a history of corticosteroid use, alcoholism, sickle cell anemia, Gaucher disease, or dysbaric conditions.

89
Q

Quais são os achados radiograficos do infarto osseo?

A

Bone infarcts usually are well-deined metaphyseal lesions with irregular borders. The periphery of the lesion is calcified, in contrast to chondroid lesions, which are usually calcified throughout.

90
Q

Qual transformação maligna pode ocorrer em uma osteomielite com fistulização cronica?

A

Malignant change occasionally occurs in chronically draining osteomyelitic sinuses of long duration; squamous cell carcinoma and fibrosarcoma have been reported.

91
Q

Em quais tipos de pacientes a osteolise pós-traumatica foi descrita?

A

Pubis of elderly osteopenic women and in the distal clavicle of athletes, particularly weightliters. The radiographic appearance in both circumstances may simulate a malignant bone tumor.

92
Q

Geralmente, como é a evolução da dor nos tumores osseos?

A

The pain initially may be activity related, but a patient with a malignancy of bone often complains of progressive pain at rest and at night.

93
Q

Qual lesão benigna pode se apresentar com dor noturna inicialmente?

A

Notably osteoid osteoma may cause night pain initially.

94
Q

Qual população o tumor de Ewing é raro?

A

Ewing sarcoma is exceedingly rare in individuals of African descent.

95
Q

Qual o dado mais importante da anamnese de um paciente com queixa compativel com tumor osseo?

A

Age may be the most important information obtained in the history, however, because most benign and malignant musculoskeletal neoplasms occur within specific age ranges.

96
Q

Quais caracteristicas da lesão devem ser abordadas no exame físico?

A

A mass should be measured, and its location, shape, consistency, mobility, tenderness, local temperature, and change with position should be noted. Atrophy of the surrounding musculature should be recorded, as should neurological deficits and adequacy of circulation. Café-au-lait spots or cutaneous hemangiomas also may provide diagnostic clues. Potential sites of lymph node metastases should be palpated. Although lymph node metastases are rare with most sarcomas, they often are present with rhabdomyosarcomas, epithelioid sarcomas, and synovial sarcomas.

97
Q

Quais são os diagnosticos diferenciais para lesões epifisárias?

A

Chondroblastoma (ages 10-25)
Giant cell tumor (ages 20-40)
Clear chondrosarcoma (rare)

98
Q

Quais são os diagnosticos diferenciais para lesões diafisárias?

A
Ewing sarcoma (ages 5-25) 
Lymphoma (adult) 
Fibrous dysplasia (ages 5-30) 
Adamantinoma (consider in the tibia) 
Histiocytosis (ages 5-30)
99
Q

Quais são os diagnosticos diferenciais para lesões vertebrais?

A
Older than 40 Years
Metastases 
Multiple myeloma
Hemiangioma 
Chordoma (in sacrum) 

Younger than 30 Years
Vertebral body
Histiocytosis
Hemangioma

Posterior elements
Osteoid osteoma
Osteoblastoma
Aneurysmal bone cyst

100
Q

Quais são os diagnosticos diferenciais para lesões multiplas?

A
Histiocytosis
Enchondroma 
Osteochondroma 
Fibrous dysplasia 
Multiple myeloma 
Metastases 
Hemangioma 
Infection 
Hyperparathyroidism
101
Q

Quais são as caracteristicas radiograficas de lesões malignas e benignas?

A

Lesions of low biological activity are usually well marginated, often with a surrounding rim of reactive bone formation. Aggressive lesions usually have a less well-defined zone of transition between the lesion and the host bone because the host response is slower than the progression of the tumor. Cortical expansion can be seen with aggressive benign lesions, but frank cortical destruction usually is a sign of malignancy.

102
Q

Qual a aparencia tipica da displasia fibrosa no rx?

A

The irregular osteoid trabeculae in a collagenous stroma produce the classic radiographic “ground glass” appearance in ibrous dysplasia.

103
Q

Qual o achado tipico do osteossarcoma na radiografia?

A

Matrix ossification combined with destructive features of host bone is a radiographic finding in a typical osteosarcoma.

104
Q

Qual achado radiológico aproxima o diagnostico de um hemangioma?

A

Flebolitos

105
Q

Qual a utilidade da cintilografia nos casos de tumor osseo?

A

Technetium bone scans are used to determine the activity of a lesion and to determine the presence of multiple lesions or skeletal metastases.

106
Q

Em quais tipos de lesões a cintilografia pode ter um resultado falso negativo?

A

Bone scans frequently are falsely negative in multiple myeloma and some cases of renal cell carcinoma.

107
Q

Qual a substancia utilizada como marcador no PET-CT?

A

Fluorine-18 (F)-fluorodeoxyglucose

108
Q

Em termos gerais, quando considerar uma neoplasia de partes moles como um sarcoma?

A

In general, any soft tissue neoplasm deep to the fascia or larger than 5 cm in its greatest dimension should be considered highly likely to be a sarcoma.

109
Q

Deiscencia de ferida operatoria e infecção são mais comum acontecer com quais niveis de albumina e leucocitos?

A

Risks of wound healing problems and infection have been shown to be significantly greater in patients whose serum albumin value is less than 3.5 g/dL or whose total lymphocyte count is less than 1500/mL.

110
Q

VHS geralmente está aumentado em quais condições?

A

The erythrocyte sedimentation rate usually is elevated in infection; metastatic carcinoma; and small “blue cell” tumors, such as Ewing sarcoma, lymphoma, leukemia, and histiocytosis.

111
Q

Hipercalcemia está presente em quais eventos?

A

Hypercalcemia may be present with metastatic disease, multiple myeloma, and hyperparathyroidism.

112
Q

Fosfatase alcalina está elevada em quais eventos?

A

Alkaline phosphatase may be elevated in metabolic bone disease, metastatic disease, osteosarcoma, Ewing sarcoma, or lymphoma.

113
Q

Quando realizar a biopsia de um tumor musculoesqueletico?

A

Musculoskeletal neoplasms should be evaluated completely before biopsy is done. The diferential diagnosis, extent of the lesion, and potential resectability of the lesion can affect the type of biopsy, the placement of the biopsy incision, and the pathological management of the tissue obtained.

114
Q

Paciente >40a com lesão ossea dolorosa, quais são as hipoteses dignosticas?

A

Multiple myeloma and metastatic carcinoma are the most likely diagnoses even if the patient has no known history of carcinoma. If a patient has no known primary tumor, however, the most likely sources are lung cancer and renal cell carcinoma.

115
Q

Quais são os sitios primarios que mais lançam metastases osseas?

A

Prostate cancer and breast cancer are the two most common primary sources for bone metastases.

116
Q

Como abordar um paciente com lesão ossea sem sitio primario definido?

A

The evaluation begins with a history focusing on any previous malignancies, even in the remote past, followed by a physical examination that includes not only the involved extremity but also the thyroid, lungs, abdomen, prostate in men, and breasts in women. Laboratory analysis should include complete blood cell count, erythrocyte sedimentation rate, electrolytes, liver enzymes, alkaline phosphatase, serum protein electrophoresis, and possibly prostate-specific antigen. Plain radiographs of the involved bone and the chest should be obtained. A whole-body bone scan should be ordered to evaluate other possible areas of skeletal involvement, and a CT scan of the chest, abdomen, and pelvis should be obtained.

117
Q

Quais são as 6 razões pelas quais a biopsia só deve ser realizada após o estadiamento completo da lesão?

A

(1) the lesion may be a primary sarcoma of bone that may require a biopsy technique that allows for future limb salvage surgery; (2) another, more accessible lesion may be found; (3) if renal cell carcinoma is considered likely, the surgeon may wish to consider preoperative embolization to avoid excessive bleeding; (4) if the diag nosis of multiple myeloma is made by laboratory studies, an unnecessary biopsy can be avoided; (5) the pathological diagnosis is more accurate if aided by appropriate imaging studies; and (6) the pathologist and surgeon may be more assured of a diagnosis of metastasis made on frozen section analysis if supported by the preoperative evaluation. This is important if stabilization of an impending fracture is planned for the same procedure.

118
Q

Como é o estadiamento de Enneking para tumores benignos e malignos?

A

Benign

  1. Latent—low biological activity; well marginated; often incidental indings (i.e., nonossifying fibroma)
  2. Active—symptomatic; limited bone destruction; may present with pathological fracture (i.e., aneurysmal bone cyst)
  3. Aggressive—aggressive; bone destruction/soft tissue extension; do not respect natural barriers (i.e., giant cell tumor);

Malignant
STAGE GRADE SITE METASTASES
IA Low Intracompartimental None
IB Low Extracompartimental None
IIA High Intracompartimental None
IIB High Extracompartimental None
III Any Any Regional or distant

119
Q

Como é o estadiamento da American Joint Committee on Cancer (AJCC) para sarcomas de partes moles?

A

Tabela 24-2

120
Q

Como é o estadiamento da American Joint Committee on Cancer (AJCC) para osteossarcomas?

A

Tabela 24-3

121
Q

Em caso de uso de torniquete na realização da biopsia, como deve ser utilizado?

A

If a tourniquet is used, the limb may be elevated before inflation but should not be exsanguinated by compression to prevent “squeezing” the tumors cells into the systemic circulation.

122
Q

Quais são os tipos de biopsia realizada?

A

Tabela 24.4

123
Q

Qual é o objetivo do tratamento do paciente com doença metastatica para o osso?

A

The goal of treatment of a patient with metastatic carcinoma to bone is to minimize pain and to preserve function.

124
Q

Como age a radioterapia nas células?

A

Radiation causes cell death by inducing the formation of intracellular free radicals that subsequently cause DNA damage. The sensitivity of a cell to radiation depends on several factors, including (1) the cell’s position in the cell cycle (actively mitotic cells are most sensitive), (2) tissue oxygenation (local hypoxia provides a protective efect because oxygen free radicals cannot be formed in hypoxic tissue), and (3) the cell’s ability to repair DNA damage or its inability to undergo apoptosis (programmed cell death) in response to this damage.

125
Q

Qual a unidade de medida de radiação?

A

The dose of radiation is measured in gray (Gy): 1 Gy is equal to 1 joule of absorbed energy per kilogram; 1 rad is equal to 1 centigray (cGy).

126
Q

Quanto de radiação a maioria dos protocolos de tratamento de tumor osseo utiliza?

A

Most radiation treatment protocols deliver 150 to 200 cGy/d until the target dose is achieved. This dose ranges from 30 to 40 Gy for myeloma to 60 Gy for treatment of a sot tissue sarcoma.

127
Q

Qual é a sensibilidade dos tumores osseos para a radioterapia?

A

Most primary bone malignancies are relatively radioresistant. Exceptions are the small blue cell tumors, including multiple myeloma, lymphoma, and Ewing sarcoma, which are each exquisitely sensitive. Carcinomas metastatic to bone, with the exception of renal cell carcinoma, also frequently are sensitive to radiation treatment.

128
Q

Quais são os efeitos colaterais agudos e tardios da radioterapia?

A

Acutely, the most common complication is skin irritation. Initial erythema may progress later to desquamation, especially in patients who also are being treated with cytotoxic drugs. Other common acute side efects include gastrointestinal upset, urinary frequency, fatigue, anorexia, and extremity edema. Late efects include chronic edema, ibrosis, osteonecrosis, and pathological fracture. Malignant transformation of irradiated tissues (i.e., radiation sarcoma) is being reported with increasing frequency in survivors of childhood and adolescent cancers.

129
Q

Quais sequelas a radioterapia pode trazer para pacientes pediátricos?

A

Radiation therapy in children has several adverse sequelae, such as scoliosis, kyphosis, chest wall deformities, hypoplasia of the ilium, and limb-length discrepancy, as a result of radiation-induced growth arrest.

130
Q

Quais são as exceções benignas que podem ser tratadas com radioterapia?

A

Exceptions include an extensive pigmented villonodular synovitis that cannot be controlled by surgery or a large spinal giant cell tumor.

131
Q

Com o advento da quimioterapia, qual a taxa de sobrevida em 5 anos dos osteossarcomas?

A

With the use of modern chemotherapy protocols, the current 5-year survival rate for osteosarcoma is approximately 70%.

132
Q

Em quais tipos de lesões a quimioterapia não é utilizada?

A

In general, chemotherapy is not useful for cartilaginous lesions and most other low-grade malignancies.

133
Q

Quais complicações a quimioterapia neoadjuvante pode levar?

A

Neoadjuvant chemotherapy may increase significantly the risks of perioperative complications, especially delayed wound healing and infection.

134
Q

Por que se utiliza diversas drogas nos ciclos de quimioterapia?

A

Although it is presumed that most malignancies arise from a single cell, the actual tumor is composed of a heterogeneous population of cells. This is the result of rapid turn-over and genetic lability of these cells. As a result, various cells within the same tumor evolve diferent mechanisms of chemo-resistance. To combat this diversity in resistance, most chemotherapy regimens involve combinations of toxic drugs.

135
Q

Quais são os fatores que devem ser levados em consideração na escolha entre salvar o membro ou amputá-lo?

A

Simon described four issues that must be considered whenever contemplating limb salvage instead of an amputation, as follows:

  1. Would survival be afected by the treatment choice?
  2. How do the short-term and long-term morbidity compare?
  3. How would the function of a salvaged limb compare with that of a prosthesis?
  4. Are there any psychosocial consequences?
136
Q

Quais as complicações podem ocorrer após a amputação de um membro?

A

Complications include infection, wound dehiscence, a chronically painful limb, phantom limb pain, and appositional bone overgrowth requiring revision surgery.

137
Q

Em relação a morbidade cirurgica, como é esta na salvação do membro?

A

Limb salvage requires a much more extensive surgical procedure with greater risks for infection, wound dehiscence, flap necrosis, blood loss, and deep venous thrombosis. Long-term complications of limb salvage vary depending on the type of reconstruction. These include periprosthetic fractures, prosthetic loosening or dislocation, nonunion of the graft-host junction, allograft fracture, leg-length discrepancy, and late infection.

138
Q

Qual droga quimioterapica pode induzir cardiomiopatia e diminuir a função cardiaca dos pacientes?

A

Doxorubicin

139
Q

Em relação as recostruções dos membros, qual o fator principal que determina uma longevidade maior da mesma?

A

Regarding prosthetic or allograft-prosthetic composite reconstructions, location is the most important issue, with proximal reconstructions generally outlasting more distal reconstructions. (This is the inverse of the prognosis for overall patient survival, with distal sarcomas having a better prognosis than proximal sarcomas.)

140
Q

Quais são os tipos de margens cirurgicas existentes em oncologia ortopédica?

A

In orthopaedic oncology, the surgical margin is described by one of four terms: intralesional, marginal, wide, or radical.

141
Q

Como é definido as margens intralesionais?

A

An intralesional margin is one in which the plane of surgical dissection is within the tumor. This type of procedure is often described as “debulking” because it leaves behind gross residual tumor. This procedure may be appropriate for symptomatic benign lesions when the only surgical alternative would be to sacrifice important anatomical structures. This also may be appropriate as a palliative procedure in the setting of metastatic disease.

142
Q

Como é definido a ressecção marginal de um tumor?

A

A marginal margin is achieved when the closest plane of dissection passes through the pseudocapsule. This type of margin usually is adequate to treat most benign lesions and some low-grade malignancies.

143
Q

Como é definido a ressecção com margens amplas de um tumor?

A

Wide margins are achieved when the plane of dissection is in normal tissue. Although no specific distance is deined, the entire tumor remains completely surrounded by a cuf of normal tissue.

144
Q

Como é definido a ressecção radical de um tumor?

A

Radical margins are achieved when all the compartments that contain tumor are removed en bloc.

145
Q

Como é realizada a curetagem de um tumor?

A

Curettage is done by first making a large cortical window over the lesion. This window must be at least as large as the lesion itself. If the window is smaller than the lesion, the surgeon inevitably leaves residual tumor on the undersurface of the near cortex.

146
Q

O que é curetagem estendida?

A

“Extended” curettage includes the use of adjuvants, such as liquid nitrogen, phenol, polymethyl methacrylate, or thermal cautery to extend destruction of tumor cells.

147
Q

Quais nervos podem ser sacrificados numa ressecção de umero?

A

Resections of the proximal humerus frequently require sacrifice of the axillary nerve, and resections of the humeral shaft frequently require sacrifice of the radial nerve.

148
Q

Como Malaware classificou a ressecção da articulação do ombro?

A

Surgical classification of shoulder girdle resections.
Type I: intraarticular proximal humeral resection.
Type II: partial scapulectomy.
Type III: intraarticular total scapulectomy.
Type IV: extraarticular scapular and humeral head resection.
Type V: extraarticular humeral and glenoid resection.
Type VI: extraarticular humeral head and total scapular resection.

149
Q

Qual músculo escapular protege a parade toracica de ser invadido por tumores escapulares?

A

The subscapularis muscle often provides a good margin, protecting tumors of the scapula from direct chest wall invasion until late.

150
Q

Qual a ressecção proposta por Tikhoff-Linberg?

A

Total escapulectomia, parcial ou completa ressecção da clavícula e ressecção do umero proximal.

151
Q

Qual região deve ser realizada a biopsia da região proximal do umero?

A

Biopsy of a proximal humeral lesion should be done through the anterior third of the deltoid, taking care not to contaminate the deltopectoral interval. Contamination of this interval potentially could allow tumor cells to spread over a greater distance and would make a successful resection more difficult.

152
Q

Quando indicar amputação pelvica?

A

Amputation may be indicated if the internal or external iliac vessels are involved.

153
Q

Como é classificada a ressecção pelvica?

A

Resections of the pelvis are classified as type I, iliac; type II, periacetabular; type III, obturator; and type IV, sacral. The letter “H” is added as a sufix if the femoral head is resected with the specimen.

154
Q

Qual é a incisão padrão para ressecção pelvica?

A

The incision begins at the anterior superior iliac spine and courses parallel to the inguinal ligament to the pubic tubercle medially.

155
Q

Quais tipos de reconstruções pelvicas podem ser realizadas de acordo com a classificação das ressecções?

A

Type I resections do not require reconstruction. Type II resections can be reconstructed with an iliofemoral or an ischiofemoral arthrodesis, with an allograt-prosthesis composite, or with a saddle prosthesis. Type III resections do not require reconstruction because the pubis does not bear weight.

156
Q

Em qual local deve ser realizada a biópsia do femur proximal?

A

Biopsy of the proximal femur should be performed through a curved hole placed laterally at the junction between the insertion of the abductors and the origin of the vastus lateralis. This location minimizes the amount of contaminated tissue and the risk of pathological fracture.

157
Q

Qual o local mais comum de doenças malignas primarias do osso? E o segundo mais comum?

A

The distal femur is the most common location of primary malignancies of bone. The proximal tibia is the second most common location for primary malignancies of bone.

158
Q

Quais são as contra-indicações para uma cirurgia de salvação do membro em um tumor localizado na região proximal da tibia?

A

Contraindications to limb salvage include involvement of the popliteal vessels, displaced pathological fracture, recurrent tumor, and complications (e.g., infection, hematoma, poorly placed incision, or joint contamination) resulting from the biopsy.

159
Q

Em uma ressecção de femur distal e/ou tibia proximal, quanto de osso livre de lesão deve ser deixado para realizar a artrodese do joelho?

A

From a variety of imaging techniques, including CT, radiographs, and MRI, the length of bone to be resected can be determined, leaving a diaphyseal margin of at least 3 cm from the lesion.

160
Q

Como Winkelmann classificou a “rotationplasty”?

A

Winkelmann classified rotationplasty into ive groups, as follows:
Group AI—Lesion in distal femur. The distal femur, knee joint, and proximal tibia are resected; the lower leg is rotated 180 degrees, and the tibia is joined to the remaining femur.
Group AII—Lesion in the proximal tibia. The distalmost femur, knee joint, and proximal tibia are resected. Ater rotation of 180 degrees, the distal tibia is joined to the distal femur.
Group BI—Lesion in the proximal femur sparing the hip joint and gluteal muscles. The upper femur and hip joint are resected, and the leg is rotated 180 degrees. The distal femur is joined to the pelvis so that the knee functions as the hip and the ankle functions as the knee.
Group BII—Lesion in the proximal femur with involvement of hip joint and contiguous soft tissue. the upper femur, hip joint, and lower hemipelvis are resected, and the leg is rotated 180 degrees. The remaining femur is joined to the remnant of the ilium so that the knee functions as a hinged hip joint and the ankle functions as the knee.
Group BIII—Lesion in the midfemur. The entire femur is resected. The tibia is attached to the pelvis using an endoprosthesis.

161
Q

Pra que a “rotationplasty” foi desenvolvida?

A

Treatment of distal femoral osteosarcoma as an alternative to amputation.

162
Q

Qual a faixa de idade populacional mais comumente afetada pelo TGC e quais os locais mais comumentes afetados?

A

They typically occur in patients 20 to 40 years old, and there is a slight female predominance. The most common location for this tumor is the distal femur, followed closely by the proximal tibia. In the distal radius (the third most common location), these tumors frequently are more aggressive. Spinal involvement, other than the sacrum, is rare.

163
Q

Qual a porcentagem de incidencia de matastases pulmonares em pacientes com TGC?

A

Although these tumors typically are benign, pulmonary metastases occur in approximately 3% of patients.

164
Q

Qual a taxa de mortalidade em pacientes com metastases de TGC?

A

The overall mortality rate from disease for patients with pulmonary metastases is approximately 15%. Patients with recurrent lesions or primary lesions that appear aggressive radiographically (stage 3) are at higher risk for pulmonary metastases.

165
Q

Qual a porcentagem de TGC malignos e como eles são classificados?

A

Malignant giant cell tumors represent less than 5% of cases and are classified as primary or secondary. Primary malignant giant cell tumors are extremely rare and are defined as sarcomas that occur within lesions that otherwise are typical of benign giant cell tumors. Secondary malignant giant cell tumors are sarcomas that occur at the sites of giant cell tumors that have been treated, usually with radiation.

166
Q

Qual a porcentagem de pacientes se apresentam com fratura patologica por TGC no exame inicial?

A

In 10% to 30% of patients, pathological fractures are evident at initial examination.

167
Q

Como se apresentam os TGC nas radiografias?

A

The lesions are eccentrically located in the epiphyses of long bones and usually abut the subchondral bone. The lesions are purely lytic.

168
Q

Como se apresentam microscopicamente os TGC?

A

Microscopically, giant cell tumors are composed of many multinucleated giant cells (typically 40 to 60 nuclei per cell) in a sea of mononuclear stromal cells.

169
Q

Qual cuidado deve ser tomado se for utilizado enxerto autologo para preencher a cavidade deixada pela curetagem do TGC?

A

If autograt will be harvested from another site, separate gloves and instruments should be used because cross­contamination could lead to transplantation of tumor cells to the harvest site.

170
Q

Quais são as desvantagens do uso de enxerto osseo para preenchimento do defeito deixado pela curetagem?

A

There are two main disadvantages, however, to using bone graft: (1) the joint must be protected for an extended time to prevent a pathological fracture, and (2) tumor recurrence often is dificult or impossible to distinguish from graft resorption.

171
Q

O que pode ser utilizado para aumentar a rigidez do preenchimento do TGC?

A

Parafusos cruzados ou divergentes.

172
Q

Como manejar o paciente com metastase pulmonar de TGC?

A

In patients with pulmonary metastases, resection should be attempted. Chemotherapy has limited success, and irradiation should be reserved for symptomatic inoperable lesions.

173
Q

Em que faixa etária incide o condroblastoma?

A

Typically occurs in patients 10 to 25 years old, with a 2 : 1 male predominance.

174
Q

Quais os locais mais comumentes afetados pelos condroblastomas?

A

The distal femur, proximal humerus, and proximal tibia are the most common sites of occurrence. In older patients, chondroblastoma has a tendency to occur in flat bones.

175
Q

Quais são os achados radiograficos dos condroblastomas?

A

Radiographic indings usually are characteristic. This well-­circumscribed lesion usually is centered in an epiphysis of a long bone; however, it also may be located in an apophy­sis, such as the greater tuberosity or the greater trochanter. Often it has a surrounding rim of reac­tive bone, and 30% to 50% exhibit matrix calcifica­tion.

176
Q

Qual o diagnostico diferencial de condroblastoma em crianças?

A

In children, a well­ circumscribed epiphyseal lesion that crosses an open growth plate is highly suggestive of chondroblastoma but could also represent an infectious process.

177
Q

Qual é o diagnóstico diferencial de condroblastoma em adultos?

A

For adults, diferential diag­noses for an epiphyseal lesion include giant cell tumor and clear cell chondrosarcoma.

178
Q

Quais são as caracteristicas microscopicas do condroblastoma?

A

Microscopically, chondroblastoma consists of sheets of chondroblasts usually with a background of chondroid matrix. The cells are polygonal with distinct cytoplasmic out­ lines. Dystrophic calcification is frequently present and may surround individual cells, giving the classic “chicken wire” appearance.

179
Q

Como tratar os condroblastomas?

A

Although they typically are not as aggressive as giant cell tumors, surgical management is war­ranted for almost all chondroblastomas owing to the slowly progressive nature of the disease. Treatment consists of extended curettage and bone grafting or placement of bone cement. Adequate curettage always should take precedence over sparing the physis.

180
Q

Como deve ser feito o manejo pós-operatório dos condroblastomas?

A

Radiographs of the primary site and of the chest should be obtained every 6 months for at least 3 years and annually thereater. Recurrence occurs in 10% to 20% of patients and can be treated similar to a primary lesion. Benign pulmonary metastases occur in approximately 1% of patients and should be treated by resection. Malignant transformation of a chon­droblastoma is extremely rare.

181
Q

Qual a faixa etária de incidencia dos fibromas condromixóides?

A

Although chondromyxoid fibromas may occur at any age, most occur in patients 10 to 30 years old.

182
Q

Qual o local mais comumente afetado pelo fibroma condromixoide?

A

Proximal tibia is the most common location.

183
Q

Qual a aparencia do fibroma condromixoide na radiografia?

A

The radiographic appearance is that of a benign neo­plasm. It usually is a well­circumscribed lesion with a rim of sclerosis in the metaphysis of a long bone and may have a bubbly appearance mimicking a nonossifying fibroma. In contrast to other cartilaginous lesions, radiographic evidence of intralesional calcification usually is absent (except in the rare instance of a surface lesion in which calciication may be abundant).

184
Q

Qual o tratamento para o fibroma condromixoide?

A

Treatment consists of resection or extended curettage with bone grafting. Local recurrence occurs in about 20% of patients and is treated with repeat surgery. Sarcomatous change is rare.

185
Q

Qual a idade de incidencia mais comum dos osteoblastomas? Qual a predominancia por genero?

A

Most patients with osteoblastoma are 10 to 30 years old. There is a male predominance of 3 : 1.

186
Q

Qual o local mais incidente de osteoblastoma?

A

Although any bone may be involved, 40% to 50% of the lesions are located in the spine.

187
Q

Qual a caracteristica da dor no osteoblastoma?

A

Pain, which is the most common symptom, may be similar to that produced by an osteoid osteoma (i.e., worse at night and relieved by nonsteroidal antiinflammatory drugs). In the spine, painful scoliosis or neurological deficit may be present. In the lumbar spine, signs and symptoms of nerve root com­ pression may be evident, whereas in the thoracic spine, cord compression is more common.

188
Q

Qual a aparencia mais comum do osteoblastoma na radiografia?

A

The most common radiographic appearance is that of a bone­forming neoplasm in the posterior elements of the spine in a young patient.

189
Q

Quais são os diagnosticos diferencias de osteoblastoma?

A

The difer­ential diagnoses include aneurysmal bone cyst and osteoid osteoma.

190
Q

Como diferenciar um osteoblastoma de um osteoma osteoide na radiografia?

A

The nidus of an osteoid osteoma is less than 1.5 cm.

191
Q

Como tratar o osteoblastoma?

A

Treatment consists of extended curettage or resection. Bone grafting of the defect may be necessary. In the spine, instrumented fusion may be necessary if resection causes instability. Some authors recommend adjuvant radiation therapy for spinal lesions because revision surgery for recur­ rences in this area is dificult.

192
Q

Quais pacientes estão sujeitos a degeneração sarcomatosa do osteoblastoma?

A

Sarcomatous degeneration has been reported and may be more common in lesions previously treated with radiation.

193
Q

Qual é a tríade da doença de Hand-Schüller-Christian?

A

Hand-Schüller-Christian disease classically refers to the clinical triad of skull lesions, exophthalmos, and dia­betes insipidus.

194
Q

Quais são as características da doença de Letterer-Siwe?

A

Letterer-Siwe disease, another variation, usually has its onset before 3 years of age and is characterized by fever, lymphadenopathy, hepatosplenomegaly, and multiple bone lesions. Frequently is rapidly fatal.

195
Q

Qual a faixa etária caracteristica da histiocitose de células de Langerhans?

A

Patients usually are 5 to 20 years old and usually have progressive pain.

196
Q

Qual a apresentação clínica da histiocitose de células de Langerhans?

A

The clinical picture may be similar to that produced by osteomyelitis with pain at rest (and at night), fever, and local signs of inflamma­tion.

197
Q

Quais são os locais mais comumente afetados pela histiocitose de celulas de Langerhans?

A

Most common loca­tions are the vertebral bodies, the flat bones, and the diaphyses of long bones.

198
Q

Qual é o aspecto radiológico da histiocitose de células de Langerhans na coluna?

A

Radiographically, Langerhans cell histiocytosis can have various appearances. Marked flattening of the vertebral body, or vertebra plana, is a common manifestation.

199
Q

Quais outras doenças podem cursar com vertebra plana?

A

Other causes of vertebra plana include Ewing sarcoma, lymphoma, leukemia, Gaucher disease, aneurysmal bone cyst, and infection.

200
Q

Qual o aspecto radiologico da histiocitose de celulas de Langerhans nos ossos planos?

A

In flat bones, the lesions usually are well­circumscribed, “punched­out,” purely lytic lesions. The lesions may have a “hole within a hole” appearance because of diferent involvement of the two tables.

201
Q

Qual o aspecto radiologico da histiocitose de celulas de Langerhans na diafise dos ossos longos?

A

In the diaphyses of long bones, the lesions may have an aggressive permeative appearance with periosteal reactive bone formation.

202
Q

Qual proteína marca a célula de Langerhans microscopicamente?

A

The cells stain posi­tively for S­100 protein.

203
Q

Quais organelas são identificadas pela microscopia eletronica nas células de Langerhans?

A

Electron microscopy may identify characteristic organelles in the Langerhans cell cytoplasm called Birbeck granules.

204
Q

Qual o tratamento da histiocitose de células de Langerhans?

A

Recommended treatments have included corticosteroid injections, radiation therapy, and curettage with or without bone grating. If a lesion is asymptomatic, no treatment is necessary because lesions have been noted to regress spontaneously. For symptomatic lesions, if the diagnosis is established by needle biopsy, the lesion may be injected with methylprednisolone during the same proce­dure. Similarly, if the diagnosis is established by open biopsy, the lesion can be curetted during the same procedure. Care must be taken, however, to rule out infection before placing corticosteroids or bone graft into a lesion.

205
Q

Como tratar os casos de vertebra plana na histiocitose de células de Langerhans?

A

Vertebra plana likewise may be treated conservatively because most lesions spontaneously regress. Vertebral height typically is partially restored with growth in skeletally imma­ture patients. Temporary bracing may help to relieve symp­toms. Irradiation may be indicated for treatment of mild neurological signs. Surgical decompression and fusion with instrumentation is indicated for rapidly progressive neuro­ logical signs or cord compression that is not responsive to radiation therapy.

206
Q

O que é a Doença de Dahlin?

A

Osteoblastoma

207
Q

O que é a deformidade de Bessel-Hagen?

A

Deformidade do antebraço secundaria a osteocondromatose, com luxação da ARUD e neoformação articular pelos osteocondromas

208
Q

Diagnostico diferencial de cisto osseo simples?

A

Displasia fibrosa

209
Q

Diagnostico diferencial de cisto osseo aneurismatico?

A

Fibroma condomixóide

210
Q

Quais tumores apresentam celulas gigantes multinucleadas?

A

TGC
Cisto osseo aneurismatico
Tumor marrom
Paget

211
Q

Classificação de Campanacci para TGC?

A

Ver

212
Q

O condroma periosteal ocorre mais frequentemente em qual região?

A

Região proximal do úmero

213
Q

Qual é a lesão neoplásica que mais frequentemente acomete o centro de ossificação secundário de um osso longo?

A

Condroblastoma

214
Q

No condrossarcoma de baixo grau de diferenciação, qual é o aspecto dos padrões de calcificações?

A

Anelar

215
Q

A célula caracteristica do TGC tem aspecto semelhante a qual célula?

A

Osteoclasto

216
Q

Qual é o tumor descrito por Dahlin, chamando-o de osteoma osteóide gigante?

A

Osteoblastoma

217
Q

Qual é o tumor ósseo benigno mais comum?

A

Osteocondroma