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NAZEER
INTRODUCTION
y A team comprising an orthopaedic surgeon,
radiologist, pathologist, radiation oncologist, and medical oncologist is necessary to treat the spectrum of musculoskeletal tumors. y Other surgical specialists frequently are required, such as a vascular surgeon, thoracic surgeon, or plastic surgeon. y The orthopaedic surgeon must be well versed in the principles of oncological surgery, and the radiologist and pathologist should have a special interest in bone and soft-tissue tumors.
are the first and most important steps in evaluating a patient with a musculoskeletal tumor. y Patients may present with pain, a mass, or an abnormal radiographic finding detected during the evaluation of an unrelated problem y Patients with bone tumors most frequently present with pain.
(e.g., female predominance with giant cell tumors), this is rarely of diagnostic significance.
y Family history occasionally can be helpful, as
1) evaluation of the patient's general health 2) a careful examination of the part in question. y A mass should be measured, and its location, shape, consistency, mobility, tenderness, local temperature, and change with position should be noted. y Atrophy of the surrounding musculature recorded, as should neurological deficits and adequacy of circulation. y Caf au lait spots or cutaneous hemangiomas also may provide diagnostic clues.
STAGING OF TUMOURS
y Benign and malignant tumors of bone and soft
GRADE
LOW
SITE
INTRACOMPARTMENTAL
METASTASES
NONE
IB
LOW
EXTRACOMPARTMENTAL
NONE
IIA
HIGH
INTRACOMPARTMENTAL
NONE
IIB
HIGH
EXTRACOMPARTMENTAL
NONE
III
ANY
ANY
Grade
Low
Size
Any
Depth
Any
Metastases
None
II
Low
5 cm
Any
None
High
>5 cm
Superficial
None
III
High
>5 cm
Deep
None
IV
Any
Any
Any
Regional or distant
Grade
Low Low High High Any Any Any
Size
8 cm >8 cm 8 cm >8 cm Any Any Any
Metastases
None None None None Skip metastasis Pulmonary metastases Nonpulmonary metastases
BENINGN TUMOURS
STAGE 1 ( LATENT )
y lesions are intracapsular. y usually asymptomatic, and frequently
incidental findings. y Radiographic features include a well-defined margin with a thick rim of reactive bone. There is no cortical destruction or expansion. y These lesions do not require treatment because they do not compromise the strength of the bone and usually resolve spontaneously.
STAGE 2( ACTIVE )
y lesions also are intracapsular. y are actively growing and can cause symptoms
or lead to pathological fracture. y They have well-defined margins on radiographs but may expand and thin the cortex. y Usually they have only a thin rim of reactive bone. y Treatment usually consists of extented curettage .
STAGE 3( AGGRESSIVE )
y lesions are extracapsular. y Their aggressive nature is apparent clinically
and radiographically. They usually have broken through the reactive bone and possibly the cortex. y MRI may show a soft-tissue mass, and metastases may be present in 5% of patients with these lesions. y Treatment consists of extended curettage and marginal or even wide resection, and local recurrences are common.
BIOPSY
definitive procedure. Biopsy should be done only after clinical, laboratory, and radiographic examinations are complete. y provide more information leading to a more accurate pathological diagnosis, and avoids artifacts on imaging studies. y Placement of the biopsy incision is a crucial decision because the biopsy track needs to be excised en bloc with the tumor. y If a tourniquet is used, the limb may be elevated before inflation but should not be exsanguinated by compression.
sampled, if possible. hole made in the bone, should be round or oval to minimize stress concentration and prevent a subsequent fracture. The periphery of a lesion usually contains the most viable tissue and is the best tissue on which to base a diagnosis. If a tourniquet has been used, it should be deflated and meticulous hemostasis ensured before closure, because a hematoma would be contaminated with tumor cells. A biopsy can be done by fine needle aspiration, core needle biopsy, or an open incisional procedure
ADJUVANT THERAPY
y In includes : y 1) RADIATION THERAPY y 2) CHEMOTHERAPY
RADIATION THERAPY Radiation causes cell death by inducing the formation of intracellular free radicals that subsequently cause DNA damage. The goal of radiation treatment is to deliver the highest possible dose of radiation to the tumor cells, while minimizing toxicity to normal tissues.
the most sensitive phase of the cell cycle are killed. y Most primary bone malignancies are relatively radioresistant. y Exceptions are the marrow cell tumors, including multiple myeloma, lymphoma, and Ewing sarcoma, which are each exquisitely sensitive. y Radiation therapy is associated with significant acute and long-term complications.
BRACHYTHERAPY
y Radiation can be delivered by brachytherapy y y
y y
(from the Greek, brachys, meaning close ). By this method, hollow catheters are implanted in the tumor bed at the time of resection. These catheters exit through the skin. Postoperative radiographic evaluation and computer calculations determine the optimal loading of the catheters with radioisotopes. This technique allows for high doses to be delivered to the target tissues. Currently using this technique more frequently with good results
Brachytherapy catheters
CHEMOTHERAPY
y 80% of patients with apparently localized
osteosarcoma actually have undetectable metastases, or micrometastases, on presentation. With the use of modern chemotherapy protocols, the current 5-year survival rate for osteosarcoma is approximately 70%. y Adjuvant chemotherapy refers to chemotherapy administered postoperatively to treat presumed micrometastases. y Neoadjuvant chemotherapy refers to chemotherapy administered before surgical resection of the primary tumor.
y Alkylating Agents
Mustards : Ifosfamide Cyclophoshamide Platinum Compounds : Cisplatin Carboplatin Antimetabolites: Methotrexate Topoisomerase
Interactive Agents
y Antitumor Antibiotics :Doxorubicin
Osteoma Osteoid osteoma Bone island 2) CARTILAGE LESIONS Chondroma Osteochondroma 3) FIBROUS LESIONS Nonossifying fibroma Cortical desmoid Fibrous histiocytoma Fibrous dysplasia Osteofibrous dysplasia Desmoplastic fibroma
3) CYSTIC LESIONS Unicameral bone cyst Aneurysmal bone cyst Intraosseous ganglion cyst Epidermoid cyst 4) FATTY TUMOURS Lipoma 5) VASCULAR TUMOURS Hemangioma 6) NON NEOPLASTIC CONDITIONS Paget disease Brown tumor of hyperparathyroidism
OSTEOID OSTEOMA
y Osteoid osteoma is a benign neoplasm most
often seen in young men. y Found in the second or third decades of life. y predilection for the lower extremity, with half the cases involving the femur or tibia. y The tumor may be found in cortical or cancellous bone. y Multicentric lesions have been reported. y No malignant change has ever been documented. y The typical patient with an osteoid osteoma has pain that is worse at night
y Imaging studies usually are diagnostic. y The lesion consists of a small (<1.5 cm) central
nidus with surrounding bony sclerosis. y Plain radiographs often are sufficient to make the diagnosis. y CT is the best study to identify the nidus and confirm the diagnosis. y The microscopic appearance consists of fibrovascular tissue with immature bony trabeculae that are rimmed by prominent osteoblasts. y The lesion usually is surrounded by a sclerotic rim.
y Treatment options
1) are medical treatment, percutaneous radiofrequency ablation, and open surgical procedures. (enbloc excision & burr down technique ) 2) lesions of the pelvis or long bones of the extremities can be treated with percutaneous radiofrequency ablation
y Differential diagnosis:
incidentally. y Almost any bone can be involved. It is unclear whether they represent a developmental abnormality or neoplastic process.
y Osteopoikilosis is a rare condition consisting of multiple small bone islands throughout the skeleton. y Autosomal dominant and sporadic forms of the syndrome have been identified.
y Diagnosed by plain radiographs. y They typically are small, round or oval areas of
homogeneous increased density within the cancellous bone . y Radiating spicules on the periphery of the bone islands merge with the native bone creating a brushlike border. y Most patients with bone islands can be treated with observation with serial plain radiographs. y 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.
location. They are the most common tumor of the small bones of the hands and feet. y Chondromas usually are asymptomatic. y arise in the medullary canal, where they are referred to as enchondromas. y Rarely, they arise on the surface of the bone, where they are referred to as periosteal chondromas or juxtacortical chondromas.
y y y
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. Tumors are located in the epiphysis and the adjacent parts of the metaphysis and shaft. Deformities include shortening caused by lack of epiphyseal growth, broadening of the metaphyses, and bowing of the long bones. Associated with hemangiomas of the overlying soft tissues, the disease is known as Maffucci
syndrome.
appearing tumors with intralesional calcification. y The calcification is irregular and has been described as stippled, punctate, or popcorn. y In more proximal locations, deep endosteal erosion (two thirds of the thickness of the cortex) frequently indicates a chondrosarcoma. y An associated soft-tissue mass is never present with an enchondroma and always indicates a chondrosarcoma. y Plain radiographs are usually sufficient to diagnose a chondroma.
is that of mature hyaline cartilage Treatment of patients with solitary enchondromas usually consists of observation with serial radiographs. y If the lesion remains radiographically stable and asymptomatic, no further intervention is indicated. y If a lesion grows, or if it becomes symptomatic, extended curettage usually is curative. y Recurrence rates are low.
OSTEOCHONDROMA
y Osteochondromas are common benign bone
tumors. y They probably are developmental malformations rather than true neoplasms . y Thought to originate within the periosteum as small cartilaginous nodules. y The lesions consist of a bony mass, often in the form of a stalk, produced by progressive endochondral ossification of a growing cartilaginous cap. y Their growth usually parallels that of the patient and usually ceases when skeletal maturity is reached. y Female : male ratio is 1.4 : 1
proximal tibia, and the proximal humerus. y Cause no symptoms and are discovered incidentally. Some cause pain by irritating the surrounding structures. y The physical finding usually is a palpable mass. y Multiple hereditary exostoses constitute an autosomal dominant condition. y Osteochondromas are of two types: 1)pedunculated 2) broad-based or sessile. Pedunculated tumors are more common
and cancellous components, both of which are continuous with corresponding components of the parent bone. y The lesion is covered by a cartilaginous cap that often is irregular and usually cannot be seen on radiographs. y Occasionally, calcification within the cap may be seen. y Incidence of malignant degeneration to be approximately 1% for patients with a solitary osteochondroma and 5% for patients with multiple hereditary exostoses.
Pathogenesis of osteochondroma
the lesion is large enough to be unsightly or produce symptoms from pressure on surrounding structures, or when imaging features suggest malignancy. y Patients with multiple hereditary exostoses may require osteotomies to correct deformity. y A similar lesion, subungual exostosis, may develop on a distal phalanx, especially of the great toe. y Often there is a definite history of trauma. y Excision is indicated when elevation of the nail produces pain.
believed to occur in 35% of children. y Lesions occur in the metaphyseal region of long bones in individuals 2 to 20 years old. y Approximately 40% are found in the distal femur, 40% in the tibia, and 10% in the fibula. y Also known as metaphyseal fibrous defects, fibrous cortical defects, and fibroxanthomas. y On X-RAY appears as a well-defined lobulated lesion located eccentrically in the metaphysis.
wall, sclerotic scalloped borders, and erosion of the cortex are frequent findings. y There is no periosteal reaction in the absence of a pathological fracture. y Histologically, the defect is filled with spindleshaped cells distributed in a whorled or storiform pattern. y Most nonossifying fibromas are asymptomatic and regress spontaneously in adulthood. y Treatment for lesions that are larger than50% of the diameter of the bone
FIBROUS DYSPLASIA
y Fibrous dysplasia is a developmental anomaly of
bone formation . y Exist in a monostotic or polyostotic form. y The hallmark is replacement of normal bone and marrow by fibrous tissue and small, woven spicules of bone. y occur in the epiphysis, metaphysis, or diaphysis. y McCune-Albright syndrome refers to polyostotic fibrous dysplasia, cutaneous pigmentation, and endocrine abnormalities. y Mazabraud syndrome is polyostotic fibrous dysplasia with intramuscular myxomas.
occasionally with and without prior radiotherapy. y The X-ray appearance is characteristic, with the lucent area having a granular, groundglass appearance. y Surgical treatment is indicated when significant deformity or pathological fracture occurs or when significant pain exists. y Recurrence rates are high after curettage and bone grafting, cortical bone preferred over cancellous grafts (or bone graft substitutes) because of their slower resorption.
FIBROUS DYSPLASIA
OSTEOFIBROUS DYSPLASIA
y Ossifying fibroma of long bones. y Also known as Campanacci disease. y Usually affecting the tibia and fibula. y Patients usually are in the first two decades of
life. y The middle third of the tibia is the most frequently affected location, and although the lesion usually is diaphyseal, it may encroach on the metaphysis. y The tibia is enlarged and often bowed anterolaterally.
fracture has occurred. y X-rays show eccentric intracortical osteolysis with expansion of the cortex. y Histological studies reveal zonal architecture with loose fibrous tissue in the center of the lesion and a band of bony trabeculae rimmed by active osteoblasts at the periphery. y The lesion must be distinguished from adamantinoma and monostotic fibrous dysplasia.
tumor. y 85 % occur in the first 2 decades with a 2 : 1 male predominance. y Unicameral bone cysts are most common in the proximal humerus and femur. y In adults, the ilium and calcaneus are more common locations. y The lesions are most active during skeletal growth and usually heal spontaneously at maturity.
y Unicameral bone cysts often are asymptomatic, unless a pathological fracture has occurred. y Two thirds of patients present with fractures, which can stimulate the cyst to heal. y Plain radiographs shows
1)centrally located, purely lytic lesion with a well. marginated outline (diagnostic for a unicameral bone cyst. 2)The cyst may expand concentrically, but never penetrates the cortex. 3) Prominent osseous ridges on the inner cortical wall may give it a multiloculated appearance. 4)No periosteal reaction is present, unless there has been a fracture.
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. y Unicameral bone cysts start as metaphyseal lesions that abut the physis in growing children. y Unicameral bone cysts are classified 1)active when they are within 1 cm of the physis 2)latent when they are closer to the diaphysis.
Pathogenesis : 1) focal defect in metaphyseal remodeling blocks interstitial fluid drainage. 2) This leads to increased pressure, which leads to focal bone necrosis and accumulation of fluid. Unicameral bone cysts are filled with a clear yellow, serous fluid, unless a pathological fracture has caused bleeding into the cavity. A fibrous membrane, usually less than 1 mm thick, lines the cyst wall. The lining of a unicameral bone cyst is composed of fibroblasts, rather than endothelial cells.
extremities can be treated with observation with serial plain radiographs. y Larger lesions (lesions at risk for pathological fracture), symptomatic lesions, and lesions in the lower extremities usually are treated with: 1)curratage (with or without bone grafting or internal fixation) or 2)aspiration and injection (often using steroids, bone marrow aspirate, demineralized bone matrix, or other materials).
Fractures through unicameral bone cysts in the proximal femur should be treated with curettage, bone grafting, and internal fixation( Flexible intramedullary nailing). y Steroid injection was described as an effective new treatment option that was inexpensive and involved less morbidity. y Reduces the recurrence rate. y Generally, 80 to 200 mg of methylprednisolone is used, depending on the size of the patient, the size of the lesion, and the age of the patient. y Works either by an antiprostaglandin effect or by decreasing the pressure of the cyst.
Other materials used for percutaneous treatment of unicameral bone cysts include :
y 1)autogenous bone marrow mixed with
allograft demineralized bone matrix, y 2)high-porosity hydroxyapatite, calcium sulfate, and cancellous allograft.
blood-filled reactive lesions of bone and are not considered to be true neoplasms. y Commonly involves proximal humerus, distal femur, proximal tibia, and spine. y Vertebral lesions, accounting for 15% to 20% of these entities, are located in the posterior elements y occur in patients younger than 20 years old. y there is a slight female predominance. y Most patients complain of mild to moderate pain present for weeks to several months.
mimic a malignancy. y Radiographs reveal an expansile lytic lesion that elevates the periosteum, but remains contained by a thin shell of cortical bone. y MRI shows the multiloculated cavities and fluid levels. y When differentiating 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.
circulatory disturbance leading to increased venous pressure and production of local hemorrhage. y Grossly, ananeurysmal bone cyst is a cavitary lesion with blood-filled septate spaces. y TREATMENT : * Most cysts are treated with extended curettage and grafting with a bone graft substitute. * 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.
treatment of aneurysmal bone cysts in locations where curettage would be extremely difficult. y Low-dose irradiation has been reported to be an effective method of treatment, often associated with rapid ossification. y The recurrence rate after curettage of an aneurysmal bone cyst is approximately 10% to 20%.
neoplasms. y Occur in patients 20 to 40 years old. y Slight female predominance. y The most common location for this tumor is the distal femur , followed by the proximal tibia. y In the distal radius (the third most common location), these tumors frequently are more aggressive. y Pain is the presenting complaint with a mass from few weeks to several months.
STAGING (RADIOLOGICAL)
GRADE 1 : well marginated border of thin rim of mature bone & cortex is intact. GRADE 2 : has relatively well defined margins but no radioopaque rim. The combined cortex and rim of reactive bone is rather thin and moderately expanded. GRADE 3 : tumour with fuzzy borders , suggesting a rapid and permeative growth , tumour bulged into the soft tissues
may be synchronously or metachronously multicentric. y typically are benign but pulmonary metastases occur in approximately 3% of patients. y Some patients with pulmonary metastases have spontaneous regression or remain asymptomatic for many years. y Some pateints have progressive pulmonary lesions, however, that lead to death even when tumors remained histologically benign. y mortality rate with pulmonary metastases is approximately 15%.
y Malignant giant cell tumors repersent < 5% y Are classified as primary or secondary.
1) 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. 2) Secondary malignant giant cell tumors are sarcomas that occur at the sites of giant cell tumors that have been treated, usually with radiation.
epiphyses of long bone. y In skeletally immature patients, giant cell tumors rarely arise in the metaphysis. y Radiographically, the lesions are purely lytic. y The zone of transition can be poorly defined on plain radiographs. In less aggressive tumors, a partial rim of reactive bone may be present y On MRI, the lesion usually is dark on T1weighted images and bright on T2-weighted images.
MRI also may reveal fluid-fluid levels typical of a secondary aneurysmal bone cyst, which occurs in 20% of patients 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. y The nuclei of the mononuclear cells are identical to the nuclei of the giant cells, a feature that helps to distinguish giant cell tumors from other tumors that may contain many giant cells. y Giant cell tumors frequently are locally aggressive. Most manifest as stage 2 or stage 3 lesions.
y TREATMENT :
1) Treatment consisted of simple curettage. 2) use of a power burr to enlarge the cavity 1 to 2 cm in all directions is now considered standard. 3) The use of adjuvants, such as liquid nitrogen, phenol, bone cement, electrocautery, or an argon beam coagulator, theoretically help to kill any remaining tumor cells. 4) To fill the defect after curettage, the surgeon has several options, including autograft bone, allograft bone, an artificial bone graft substitute, or methylmethacrylate bone cement.
aggressive, extended curettage followed by argon beam coagulation. y Bone cement is used to fill the cavity because of its ease of application. y Curettage may not be effective in some stage 3 tumors, and primary resection may be required after biopsy. y Around the knee, a hemicondylar osteoarticular allograft reconstruction or a rotating hinge endoprosthesis may be necessary.
primary resection and reconstruction with a proximal fibular autograft (either as an arthroplasty or as an arthrodesis) may be indicated . y For lesions in expendable bones (e.g., the distal ulna or proximal fibula), primary resection without reconstruction may be indicated. y For lesions in the spine or pelvis, radiation or embolization (or both) may be used . y In patients with pulmonary metastases, resection should be attempted.
metastases occur within 3 years but have been reported to occur 20 years later. y At minimum, patients should have radiographs of the primary tumor site and the chest at 3- to 4-month intervals for 2 years, at 6-month intervals for the following year, and annually thereafter. y Treatment of recurrent lesions is the same as for primary lesions. After biopsy shows that the tumor is still benign, repeat curettage or resection should be performed.
CHONDROBLASTOMA
y Represents 1% of all bone tumors. y Occurs in patients 10 to 25 years old. y 2 : 1 male predominance . y The distal femur, proximal humerus, and
proximal tibia are the most common sites of occurrence. y In older patients, chondroblastoma has a tendency to occur in flat bones. y Most patients complain of progressive pain that may mimic a chronic synovitis or other intraarticular pathological conditions. y Also referred as CODMAN S TUMOUR
y Radiographic findings
1) Well-circumscribed lesion usually centered in an epiphysis of a long bone. 2) Also located in an apophysis, such as the greater trochanter or the greater tuberosity. 3) Has a surrounding rim of reactive bone, and 30% to 50% exhibit calcification on plain radiographs. Soft-tissue extension is extremely rare. 4) In children, a well-circumscribed epiphyseal lesion that crosses an open growth plate is virtually diagnostic of chondroblastoma.
1) giant cell tumor and 2) clear cell chondrosarcoma. In contrast to chondroblastomas, however, giant cell tumors usually do not have a rim of sclerotic bone or intralesional calcification and may have a soft-tissue component. y MICROSCOPY 1) Sheets of chondroblasts usually with a background of chondroid matrix. 2) The cells are polygonal with distinct cytoplasmic outlines.
3) Calcification is present and may surround individual cells, giving the classic chicken wire appearance. 4) Multinucleated giant cells are abundant.
TREATMENT:
y Extended curettage and bone grafting or
placement of bone cement. y Recurrence occurs in 10% to 20% of patients and can be treated similar to a primary lesion. y Benign pulmonary metastases occur in approximately 1% of patients and should be treated by resection.
CHONDROMYXOID FIBROMA
y Represents less than 0.5% of all bone tumors. y Occur in patients 10 to 30 years old . y This has both myxoid and fibrous elements. y Proximal tibia is the most common location. y Patients typically complain of pain, y if the tumor is located in the hands or feet, a
painless mass or swelling may be the chief complaint. y Radiographic appearance is well-circumscribed lesion with a rim of sclerosis in the metaphysis. y bubbly appearance(lobulated) with bite like cortical destruction.
y Microscopically:
1) Chondromyxoid fibroma has zonal architectute appears lobulated. 2) The center of the lobules contains loose myxoid tissue, and the periphery contains a more cellular fibrous tissue. 3) The background often appears chondroid, although distinct areas of hyaline cartilage are rare. 3) Microscopic calcification may be present.
y TREATMENT :
Resection or extended curettage with bone grafting . y Local recurrence occurs in about 20% of patients and is treated with repeat surgery. y Sarcomatous change is rare.
OSTEOBLASTOMA
y bone-forming neoplasm, represents less than
1% of bone tumors. y Occurs in patients 10 to 30 years old. y Male predominance of 3 : 1. y 40% to 50% of the lesions are located in the spine. y Pain, which is the most common symptom, may be similar to that produced by an osteoid osteoma y i.e., worse at night and relieved by nonsteroidal antiinflammatory drugs.
deficit may be present. y In the lumbar spine, signs and symptoms of nerve root compression may be evident, y In the thoracic spine, cord compression is more common.
y RADIOGRAPHY : 1) The classic appearance of a calcified central nidus with a surrounding radiolucent halo in the posterior elements of spine . 2) The nidus of an osteoid osteoma is less than 1.5 cm.
3) The lesion may be radiolucent, sclerotic, or mixed. Lesions may be diaphyseal or metaphyseal . 4) may be primarily cortical or intramedullary.
y Microscopically:
1) The lesion resembles an osteoid osteoma. 2) It contains a fibrovascular stroma with production of osteoid and primitive woven bone. 3) Bony trabeculae are lined by a single layer of osteoblasts, which may be important in differentiating osteoblastoma from osteosarcoma
4) Other features favoring an osteoblastoma include sharp circumscription and a loose arrangement of the tissue.
y 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 for spinal lesions.
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