2. Tumores do osso e cartilagem

Apresentação em tema: "2. Tumores do osso e cartilagem"— Transcrição da apresentação:

1 2. Tumores do osso e cartilagem
Benignos Condroma Osteocondroma Displasia Fibrosa Hemangioma Granuloma eosinofílico Osteoma osteóide Quisto ósseo aneurismático

2 2. Tumores do osso e cartilagem Benignos Condroma
Chondromas are one of the more common benign tumors of the chest wall. They are primarily seen in children and young adults. Chondromas usually occur at the costochondral junction anteriorly. Given their typical location and the young age of most patients, chondromas may be confused with costochondritis. Clinically, a mass (usually without pain) is present in the case of chondromas. Radiographically, the lesion is lobulated and radiodense; it may have diffuse or focal calcifications and may displace the bony cortex without penetration. Chondromas may grow to huge sizes if left untreated. Treatment is surgical resection with a 2-cm margin. One must be certain, however, that the lesion is not a well-differentiated chondrosarcoma. In this case, a wider 4-cm margin is required to prevent local recurrence. Therefore, large chondromas should be treated surgically as low-grade chondrosarcomas. ++ Crianças e jovens adultos Massa na junção costocondral anterior Podem atingir grandes dimensões se não tratados

3 2. Tumores do osso e cartilagem Benignos Osteocondroma
+ comum ++ Primeiras 2 décadas de vida Maioria solitários, arcos costais Potencial evolução para condrossarcoma Osteochondroma Osteochondromas are the most common benign bone tumor. Many are detected as incidental radiographic findings. Most are solitary. If a patient has multiple osteochondromas, the surgeon must have a high index of suspicion for malignancy, because the incidence of chondrosarcoma is significantly higher in this population. Osteochondromas occur in the first 2 decades of life, and they arise at or near the growth plate of bones. The lesions are benign during youth or adolescence. Osteochondromas that enlarge after completion of skeletal growth have the potential to develop into chondrosarcomas. Osteochondromas in the thorax arise from the rib cortex. They are one of several components of the autosomal dominant syndrome known as hereditary multiple exostoses. When part of this syndrome, osteochondromas have a high rate of degeneration into chondrosarcomas. Any patient with hereditary multiple exostoses syndrome who develops new pain at the site of an osteochondroma or who notes gradual growth in the mass over time should be carefully evaluated for osteosarcoma. Local excision of a benign osteochondroma is sufficient treatment. If malignancy is determined, wide excision is performed, with a 4-cm margin. Staging (referir) Osteochondromas are benign lesions and can be staged under theMusculoskeletal Tumor Society (MSTS) staging for benign lesions, as follows: Stage I - Inactive or static lesions Stage II - Actively growing lesions Stage III - Actively growing lesions that are locally destructive/aggressive Most osteochondromas are stage I or II.

4 2. Tumores do osso e cartilagem Benignos Displasia Fibrosa
Fibrous Dysplasia The ribs are a frequent site of origin of fibrous dysplasia. As with chondromas, fibrous dysplasia most frequently occurs in young adults. Pain is an infrequent complaint, and the lesion is typically located in the posterolateral aspect of the rib cage. The disease process may be localized to a single bone (monostotic fibrous dysplasia) or multiple bones (polyostotic fibrous dysplasia. Fibrous dysplasia may be associated with trauma. Radiographically, an expansile mass is present, with cortical thinning and no calcification. Local excision with a 2-cm margin is curative Patients with small, monostotic lesions may be asymptomatic, with the osseous abnormality identified incidentally on radiologic studies obtained for unrelated reasons. However, bone pain, swelling, and tenderness are common presentations in symptomatic patients Fracture is the most common complication in fibrous dysplasia. Comum nas costelas, posterolateral ++ Jovens adultos Possível associação com trauma

5 2. Tumores do osso e cartilagem Benignos Hemangioma
Bone hemangiomas are benign, malformed vascular lesions, overall constituting less than 1% of all primary bone neoplasms. Bone hemangiomas are usually asymptomatic lesions discovered incidentally on imaging or postmortem examination and mostly encountered in the middle-aged. The symptoms are largely nonspecific and depend on the site, size, and aggressiveness of the tumors. the radiographic hallmark of bone hemangiomas is a prominent trabecular pattern. Radiographically, bony hemangioma usually presents as lucent, well-demarcated defects. Computed tomography (CT) scanning is especially useful for assessing changes in bone trabeculae; the results support the plain radiographic findings and provide greater detail Figure 1. Rib radiography. A mass (arrow) is located in the left seventh rib and expands beyond the irregular cortex. Calcification inside the mass presents a so-called honeycomb appearance. Figure 2. Chest CT shows localized expansive growth of the tumor (arrow) projecting toward the thoracic cavity. Figure 3. Magnetic resonance imaging of this lesion shows a low signal on T1-weighted images (a) Adultos + Doentes assintomáticos/sintomas inespecíficos Padrão em “favo de mel” no raio-x

6 2. Tumores do osso e cartilagem Benignos
Granuloma eosinofílico Lesões osteolíticas Histiocitose de células de Langerhans/Solitários Diagnóstico 5-15 anos Dor associada (Atenção dx diferencial!) Eosinophilic Granuloma Eosinophilic granulomas are benign osteolytic lesions. They were originally thought to be destructive lesions with large numbers of eosinophilic cells. Yet eosinophilic granulomas of the ribs also can occur as solitary lesions or as part of a more generalized disease process of the lymphoreticular system termed Langerhans cell histiocytosis (LCH). In LCH, the involved tissue is infiltrated with large numbers of histiocytes (similar to Langerhans cells seen in skin and other epithelia), which are often organized as granulomas. The cause is unknown. Of all LCH bone lesions, 79% are solitary eosinophilic granulomas, 7% involve multiple eosinophilic granulomas, and 14% belong to other forms of more systemic LCH. Isolated single eosinophilic granulomas can occur in the ribs, skull, pelvis, mandible, humerus, and other sites. They are diagnosed primarily in children between the ages of 5 and 15 years. Because of the associated pain and tenderness, they may be confused with Ewing's sarcoma or with an inflammatory process such as osteomyelitis. Healing may occur spontaneously, but the typical treatment is limited surgical resection with a 2-cm margin

7 2. Tumores do osso e cartilagem Benignos Osteoma osteóide
+Jovens; M:F = 2-3:1 Dor contínua e intensa, pior à noite, melhor com salicilatos “Ninho” osteóide bem demarcado, rodeado por uma zona de esclerose Osteoid osteomas are usually smaller than cm and characterized by an osteoid-rich nidus in a highly loose, vascular connective tissue. The nidus is well-demarcated and may contain a variable amount of calcification. Surrounding the nidus is a zone of sclerotic but otherwise normal bone Osteoid osteoma is generally a condition of the young, but it can affect a wide range of individuals aged 8 months to 70 years. Men are affected more frequently than women. The male-to-female ratio is 2-3:1. Symptoms of osteoid osteoma can last from weeks to years before diagnosis and eventual surgery. Pain is the principal symptom of both initial and recurrent disease. It is described as a continuous, deep, aching, and intense pain with varying quality and severity. It is typically localized to the site of the lesion. Pain is usually worse at night (in 95% of patients) and diminishes by morning. The pain may awaken the patient (29% of cases). Alivio com salicilatos Radiography Osteoid osteoma elicits a profound osteoblastic response in surrounding medullary and cortical bone and shows the characteristic picture of sclerosis around a lucent nidus. It appears as cortical thickening and diffuse medullary sclerosis on radiographs. Radiography usually reveals a radiolucent area of about 1 cm in diameter, called the nidus, with a center that is sometimes calcified, resulting in a radiopaque point called the bell. The nidus is surrounded by a rim or halo of radiodense cortical hypertrophy or hyperostosis Figure: Osteoid osteoma of the posterior arc of the rib in a 25-year-old man. (a) Anteroposterior radiograph shows a teardrop-shaped nidus (arrow) with central mineralization. Mild reactive sclerosis and cortical expansion of the host bone also are seen in the surrounding bone. (b) Axial unenhanced CT image shows the calcified nidus (arrow) in the anterior cortex of the rib, with mild reactive sclerosis (arrowheads) surrounding the nidus.

8 2. Tumores do osso e cartilagem Benignos
Quisto ósseo aneurismático 2ª década de vida Lesão cística expansível Pode ser localmente agressivo Dor e/ou edema e/ou massa e/ou fratura patológica The aneurysmal bone cyst (ABC) is an expansile cystic lesion that most often affects individuals during their second decade of life and may occur in any bone in the body Although benign, the ABC can be locally aggressive and can cause extensive weakening of the bony structure and impinge on the surrounding tissues Recurrence is not uncommon Patients usually present with pain, a mass, swelling, a pathologic fracture, or a combination of these symptoms in the affected area. The symptoms are usually present for several weeks to months before the diagnosis is made, and the patient may also have a history of a rapidly enlarging mass. Other findings may include the following: Deformity Decreased range of motion, weakness, or stiffness Reactive torticollis Occasionally, bruit over the affected area Warmth over the affected area Radiographic findings usually consist of an eccentric or, less commonly, a central or subperiosteal lesion that appears cystic or lytic. Images may show expansion of the surrounding bone with a blown-out, ballooned, or soap-bubble appearance. Some views may show an eggshell-appearing bony rim surrounding the lesion. One may see the cystic spaces and, rarely, partially ossified septa. Fig1: Computed tomography of the lesion showed a multiloculated, expanding mass with fluid levels in the right 3rd rib. Fig2 Chest computed tomographic scan in patient 1 revealed the expansile mass destroying the anterior half of the first rib and bulging into the chest cavity. The cyst was surrounded by a thin and calcified cortex with periosteum. The arrows point to the aneurysmal bone cyst.

9 3. Tumores dos tecidos moles
Benignos Lipoma Leiomioma Neurofibroma Schwannoma Tumor desmóide Ganglioneuroma

10 3. Tumores dos tecidos moles Benignos Lipoma
+ comum anos, + obesos Massa bem circunscrita e capsulada, formada por adipócitos. Na parede torácica + profundos. Sem captação de contraste (exceto alguns septos) Mais comum dentro desta categoria. Lipomas are well-circumscribed encapsulated masses composed of adipocytes that differ very little from normal fatty tissue. They typically occur in patients who are 50–70 years of age, and they are most frequent in the obese. Most lipomas that originate in the chest wall are deep lipomas, which tend to be larger and less well circumscribed than superficial lesions. On CT and MR images, lipomas generally appear to be internally homogeneous and do not enhance after intravenous contrast material administration. However, multiple thin septa often are present that appear slightly enhanced on CT scans and have low signal intensity on fat-suppressed T1-weighted MR images. Fig direita:  Lipoma in a 42-year-old woman. Axial contrast-enhanced CT scan at the level of the pulmonary artery (P) shows a well-defined mass with the same attenuation as fat in the left part of the chest wall. Soft-tissue septa (arrows) are clearly visible at the periphery of the mass. Septa not only occur in benign lipomas but also are common in atypical lipomatous tumors and liposarcomas.

11 3. Tumores dos tecidos moles Benignos Leiomioma
Músculo liso Muito raro nesta localização Geralmente solitário Dor espontânea ou induzida por estimulo Leiomyomas are benign soft tissue neoplasms that arise from smooth muscle Malignant transformation probably does not occur Muito raro nesta localização Pain can be spontaneous or induced by cold or tactile (eg, pressure) stimuli. The pain or tenderness may be secondary to pressure on nerve fibers within the tumor; however, some authors believe it may be solely due to contraction of muscle fibers Fig: (A) Chest radiograph. A mass is seen in the right middle lung field on chest roentgenogram; (B) Chest contrast enhanced computed tomography scans. A solid, round pleural mass is seen in the right anterior chest wall, with heterogeneous density and calcification. It is well capsulated and compresses the lung parenchyma without any sign of infiltration of lung or chest wall.

12 3. Tumores dos tecidos moles Benignos Neurofibroma
Neurofibromas most commonly develop in patients between 20 and 30 years of age and may demonstrate a localized, diffuse, or plexiform pattern. The localized type is by far the most common, representing approximately 90% of lesions, with the vast majority being solitary and not associated with neurofibromatosis type I. The diffuse type is associated with a plaquelike elevation of the skin and thickening of the entire subcutis. Plexiform neurofibromas diffusely involve long segments of a nerve and its branches and have a “bag of worms” appearance. Neurofibromas are slow-growing neoplasms that originate from a nerve, may or may not be encapsulated, and may include components of cystic degeneration and calcification. In 60%–90% of affected patients, the diagnosis is either type 1 neurofibromatosis or multiple plexiform neurofibromas. Malignant degeneration occasionally occurs in these lesions, but the risk is low. Radiographs of the spine may show a widening of neural foramina because of tumor extension along spinal nerve roots. Most neurofibromas are hypoattenuated on nonenhanced CT scans and show heterogeneous enhancement after intravenous administration of contrast material. Many neurofibromas have a histologic pattern of zonal distinction, with a central zone composed of a highly cellular component and a peripheral zone composed of abundant stromal material, which results in a targetlike appearance on T2-weighted MR images. On these images, the mass is characterized by a rim of increased signal intensity that surrounds the central part of the tumor, which has a lower signal intensity. This sign is also evident on gadolinium-enhanced MR images, on which the central part of the tumor appears markedly enhanced. Fig cima: Neurofibroma in a 43-year-old man with known neurofibromatosis who presented with a large axillary mass and a 3-month history of right arm pain. (a) Contrast-enhanced CT scan with coronal two-dimensional reconstruction demonstrates a well-defined mass with central necrosis displacing the right subclavian artery. (b) Sagittal T2-weighted fast spin-echo MR image shows a regular mass with a high-signal-intensity area of necrosis. Fig baixo: Neurofibroma in a 26-year-old man with type 1 neurofibromatosis. Coronal T2-weighted (6,000/112) MR image of the thoracic inlet shows intraforaminal and perineural extension of a tumor (arrows) that has a targetlike appearance (ie, a center with signal intensity lower than that in the periphery). anos + localizado Neurofibromatose tipo 1

13 3. Tumores dos tecidos moles Benignos Schwannoma
Schwannomas, also known as neurilemomas or neurinomas, are encapsulated neoplasms that originate in nerve sheaths and are usually slow growing. Chest wall schwannomas arise from spinal nerve roots and intercostal nerves and typically occur in patients between 20 and 50 years of age. Small tumors tend to be spheroid, firm, and well circumscribed, whereas larger tumors are ovoid or irregularly lobulated. Schwannomas occasionally manifest as fibrous-walled cysts containing smaller solid nodules. Radiographs do not usually depict small schwannomas, but bone erosion or scalloping can occasionally be seen. Nonenhanced CT scans of schwannoma typically show a well-circumscribed homogeneous mass with attenuation slightly less than or equal to that of muscle. On CT scans acquired after contrast material administration, the attenuation of the mass is equal to or slightly greater than that of muscle, and any cystic or necrotic areas in the mass appear nonenhanced. The signal intensity of schwannoma on T1-weighted MR images is equal to or slightly greater than that of muscle and on T2-weighted images is markedly greater, with increased contrast between the high-signal-intensity nerve sheath tumor, intermediate-signal-intensity fat, and low-signal-intensity muscle. The nerve from which the tumor originated can often be seen along one side of the mass. Small tumors tend to enhance brightly and uniformly after intravenous administration of contrast material, whereas the enhancement pattern of larger lesions may be more heterogeneous because of central cystic change. The presence of bone erosion without destruction indicates the benign nature and slow growth rate of this lesion. The extreme pain that often accompanies percutaneous biopsy is further evidence of the neural origins of the tumor. Fig1: Schwannoma in a 43-year-old man. Axial nonenhanced CT scan of the right side of the chest wall depicts an extrapleural nodule that originated from intercostal soft tissue along the course of an intercostal nerve. The presence of heterogeneous attenuation (arrowhead) indicates myxoid degeneration, which is found occasionally in small lesions like this one. Fig2:40-year-old male with right intercostal schwannoma. Axial T1 image (non-contrast) shows a “bead-like” appearance of schwannoma. anos Crescimento lento Pequeno: esferóide, firme, bem circunscrito Grande: ovóide ou lobulado de forma irregular

14 3. Tumores dos tecidos moles Benignos Tumor desmóide
3ª-4ª década de vida Estruturas musculoaponevróticas/fasciais Associação com outras patologias Gravidez e trauma Dor e/ou massa na parede torácica Recorrência local Desmoid Tumors Desmoid tumors are unusual soft tissue neoplasms that arise from fascial or musculoaponeurotic structures. Histologically, they consist of proliferations of benign-appearing fibroblastic cells, abundant collagen, and few mitoses. Accordingly, some authorities consider desmoid tumors to be a form of fibrosarcoma. Desmoid tumors have recently been shown to possess alterations in the adenomatous polyposis coli/ -catenin pathway, and cyclin D1 dysregulation is thought to play a significant role in their pathogenesis. Associations with other diseases and conditions are well documented, especially those with similar alterations in the adenomatous polyposis coli pathway, such as familial adenomatous polyposis (Gardner's syndrome). Other conditions with increased risk of desmoid tumor formation include states of increased estrogen levels (pregnancy) and trauma. Surgical incisions (abdominal and thoracic) have been the site of desmoid development, either in or near the scar. Clinically, patients are usually in the third to fourth decade of life and have pain, a chest wall mass, or both. The tumor usually is fixed to the chest wall but not to the overlying skin. No radiographic findings are typical, but MRI may delineate muscle or soft tissue infiltration. Histologic diagnosis may not be possible by a needle biopsy because of low cellularity. An open incisional biopsy often is necessary for lesions over 3 to 4 cm, with attention to the caveats listed earlier. Desmoid tumors do not metastasize, but they have a strong propensity to recur locally, with local recurrence rates as high as 5 to 50%, sometimes despite complete initial resection with histologically negative margins. Such locally aggressive behavior is secondary to microscopic tumor infiltration of muscle and surrounding soft tissues. Surgery consists of wide local excision with a margin of 2 to 4 cm and with intraoperative assessment of resection margins by frozen-section analysis. Typically, a rib is removed above and below the tumor with a 4- to 5-cm margin of rib. A margin of <1 cm results in much higher local recurrence rates.If a major neurovascular structure would have to be sacrificed, which would lead to high morbidity, then a margin of <1 cm would have to suffice. Survival after wide local excision with negative margins is 90% at 10 years. Figure 1: (a): X-ray chest P-A view showing well defined opacity in the left hemithorax (upper and mid zones) abutting the chest wall. (b) CT scan thorax (section just below carina) showing a mass protruding into the left hemithorax and eroding the adjoining ribs

15 3. Tumores dos tecidos moles Benignos Ganglioneuroma
anos Origem nos gânglio simpáticos da parede torácica Normalmente assintomático Massa ovóide, capsulada com trabeculação Ganglioneuromas originate from the sympathetic ganglia in the chest wall. Although this tumor most often arises de novo in young adults, it also may occur as a maturation of neuroblastoma. The mass is composed of mature ganglion cells, Schwann cells, and nerve fibers, and it is often large and encapsulated, with delicate trabeculation. Ganglioneuromas usually manifest radiologically as ovoid, sharply marginated paravertebral masses. Calcification occurs in 25% of cases. The tumor has either homogeneous or heterogeneous attenuation on CT images and homogeneous intermediate signal intensity on both T1- and T2-weighted MR images. Curvilinear bands of low signal intensity are seen on both T1- and T2-weighted images, giving the lesion a whorled appearance Figura dta: Ganglioneuroma in an asymptomatic 57-year-old woman. Axial T2-weighted (6,000/112) MR image of the left ventricle (LV) shows curvilinear areas of low signal intensity (arrowheads) in the tumor, which give it a septated appearance. The tumor had a broad base and was attached to several vertebral bodies. Fig esq: Photograph of the cut surface of the resected specimen shows fibrous septa (arrowheads) that correspond to the curvilinear areas of low signal intensity on the T2-weighted MR image.