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Category Archives: Bibliographic References in English

Tumor de Células Gigantes

Giant Cell Tumor

Giant Cell Tumor

Characteristics, Diagnosis and Treatment

Giant cell tumor, also known as TGC, is a mesenchymal neoplasm that is notable for the proliferation of large multinucleated cells, called gigantocytes. These cells resemble osteoclasts and are found within a stroma of mononucleated cells. Due to its peculiar histological morphology, accurate diagnosis often requires a thorough analysis of the clinical and radiographic picture in order to avoid confusion with other pathological processes.

The main manifestation of a giant cell tumor is intermittent local pain, often accompanied by an increase in volume in the affected region and restriction of adjacent joint movements. The evolution period varies from 6 to 12 months, depending on the affected bone, with reports of trauma as the initial trigger of symptoms being common.

This type of tumor tends to affect a single bone, mainly long bones such as the femur, tibia, humerus and radius. However, in rarer cases, it can occur in bones of the axial skeleton, with a predilection for the sacrum. The incidence is more common between the third and fourth decades of life, affecting both sexes equally.

Radiographically, TGC appears as an epiphyseal lesion characterized by bone rarefaction, initially eccentric and later compromising the cortex. Diagnostic confirmation is obtained through histological analysis, which reveals the presence of multinucleated giant cells and spindle cell stroma.

The treatment of giant cell tumors is well established and aims at segmental resection of the lesion, whenever possible, ensuring safety margins for both bone and soft tissues. In cases where segmental resection is not feasible, such as in the cervical spine, endocavitary curettage followed by adjuvant therapy is indicated. Among the adjuvant therapeutic options are the CO2 laser, phenol diluted in 4% alcohol, liquid nitrogen and electrothermia.

The electrothermal technique has been shown to be effective in complementing curettage, providing a more complete cleaning of the tumor cavity. After electrothermia, milling of the cavity using appropriate instruments such as the slow drill is performed to ensure complete removal of the remaining tumor cells.

Filling the treated cavity can be done with different materials, such as autologous bone graft, bone substitutes or methylmethacrylate, each with its advantages and disadvantages. Post-treatment follow-up is essential to monitor disease recurrence and ensure the effectiveness of the treatment performed.

In summary, giant cell tumor is a complex condition that requires a multidisciplinary approach to ensure the best therapeutic outcome and the patient’s quality of life. Advances in diagnostic and treatment techniques have contributed significantly to improving the prognosis of these patients, offering increasingly effective and safe therapeutic options.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Sarcomas em Tecidos Moles

Sarcomas in Soft Tissues

Sarcomas in Soft Tissues

Introduction:

Orthopedic oncological surgery encompasses the treatment of musculoskeletal lesions, including benign and malignant bone neoplasms, pseudo-tumor lesions and benign and malignant soft tissue neoplasms. Soft tissue sarcoma is a malignant neoplasm derived from the mesoderm, occurring in soft tissues, such as muscles, fascia, tendons, etc., differentiating itself from carcinomas, which have embryonic origin in the ectoderm.

Etiology:

Most soft tissue sarcomas do not have a defined cause, but some risk factors are well described, such as previous radiotherapy, lymphedema, Li-Fraumeni syndrome, type I neurofibromatosis, individual genetic propensity and HIV infection.

Incidence:

It is a rare tumor, representing approximately 12% of pediatric neoplasms and only 1% of all malignant tumors in adults. In the USA, there are an estimated 12,000 new cases per year, resulting in around 4,700 deaths. Around 60% of soft tissue sarcomas arise in the limbs, predominantly in the thigh, followed by the chest wall and retroperitoneum.

Classification:

The classification of soft tissue sarcoma is based on histological subtype such as liposarcoma, synovial sarcoma, rhabdomyosarcoma etc. Histological grade is also used, being divided into Grade 1 (well differentiated), Grade 2 (moderately differentiated) and Grade 3 (poorly differentiated).

Clinical condition:

The initial clinical picture is characterized by a palpable tumor bulge, often painless, with progressive growth, mainly in the thigh. Some patients may experience pain and paresthesia due to the compressive effect of the tumor. Fever or weight loss are exceptional symptoms.

Staging:

At diagnosis, soft tissue sarcoma rarely metastasizes, occurring more frequently as large-volume, deep-to-muscular, high-grade tumors. The pattern of dissemination is mainly hematogenous, with predominant lung metastases.

Imaging exams:

Exams include radiography, magnetic resonance imaging, tomography, PET-CT and scintigraphy. Biopsy is indicated for histological diagnosis, and can be performed in several ways, including percutaneous needle biopsy, incisional (surgical), ultrasound or tomography-guided biopsy.

Pathology:

The pathologist plays a crucial role in the diagnosis, carrying out examinations such as freezing, to ensure the representativeness of the sample, and subsequently microscopic study in paraffin, for histopathological diagnosis as well as determining the histological grade of the tumor. Immunohistochemistry is an important resource to complement the study of the sample.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Sarcoma de Ewing Diagnóstico e Tratamento

Ewing’s sarcoma

Ewing's sarcoma

Diagnosis and Treatment

Ewing sarcoma diagnosis and treatment. Ewing sarcoma is a malignant tumor composed of small, round, undifferentiated cells that represents a significant clinical challenge due to its aggressive nature and rapid spread. This type of neoplasia has a maximum incidence in the first and second decades of life, being less common after the third decade, and exhibiting a 2:1 ratio between male and female sexes.

Although the exact origin of Ewing sarcoma cells has been the subject of controversy, recent studies suggest a neuroectodermal origin. These tumors frequently arise in the meta-diaphyseal region of the long tubular bones and in the pelvis, and their macroscopic characteristics include a bone lesion with a whitish-gray color and soft consistency. A distinctive feature is the formation of a thin lamellar reaction, resulting in an “onion skin” radiographic appearance and a large amount of extracortical lesion.

On histological examination, Ewing’s sarcoma presents uniformly distributed, small, round cells, similar to lymphocytes, but larger in size. The argent impregnation technique reveals a shortage of reticulin fibers, commonly found only around blood vessels and in lymphoma. Clinical manifestations include pain, swelling, local hypersensitivity and increased erythrocyte sedimentation rate, which may initially resemble osteomyelitis.

The differential diagnosis involves distinction with osteosarcoma, eosinophilic granuloma, rhabdomyosarcoma and osteomyelitis. The treatment currently adopted consists of preoperative multidrug therapy followed by surgery to resect the lesion, complemented by postoperative multidrug therapy. Reconstruction of the segment can be performed after resection, using endoprosthesis or biological solutions with autologous bone grafts. In children, growth plate autotransplantation techniques can be used, translating the fibula plate to the tibia or to other locations such as the shoulder and wrist, in these cases with the help of microsurgery.

Assessment of the response to preoperative chemotherapy is essential, having prognostic value and guiding subsequent treatment. This assessment is classified into grades, depending on the percentage of tumor necrosis observed. With advances in chemotherapy treatment, patients have achieved excellent responses and prospects for a “cure”, which has driven the use of biological solutions in surgical treatment, aiming to avoid complications associated with endoprostheses or bank grafts, whose durability may be limited.

Therefore, a comprehensive understanding of Ewing sarcoma, from diagnosis to therapeutic options, is essential to providing the best possible care to patients affected by this challenging malignancy.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Osteossarcoma de Superfície

Surface Osteosarcoma

Surface Osteosarcoma

Clinical Aspects, Diagnosis and Treatment

Surface osteosarcoma, also known as paraosteal or juxta-cortical, is a specific form of this type of bone tumor that originates on the external surface of the bone, without involvement of the bone marrow. In this type of lesion, the tumor growth zone is visible on the bone surface, indicating a more immature region of the bone. Surface osteosarcoma can present two distinct histological representations:

– Low Grade: Characterized by the almost complete absence of atypical mitoses and morphologically similar to mature bone. The diagnosis is usually confirmed through clinical evaluation and imaging tests.

– High Grade: It has similar clinical characteristics, but exhibits atypia and a polymorphic pattern of immature neoplasia.

Clinically, surface osteosarcoma generally presents a slow evolution and tends to occur in individuals in the third and fourth decade of life. The most common locations include the posterior and distal metaphyseal region of the femur, popliteal cavus, proximal humerus, and distal radius.

Radiologically, this form of osteosarcoma is characterized by a dense lesion of tumoral bone neoformation, with its base in continuity with the cortex of the affected bone. The surface of the lesion may have a cartilaginous layer, representing the most immature area of ​​the tumor.

One of the main differential diagnoses is myositis ossificans, which exhibits an immature central area in the lesion, while the periphery is more mature and calcified.

Treatment of low-grade surface osteosarcoma generally involves oncological resection of the lesion, which may be partial, parietal or segmental, followed by reconstruction with an autologous bone graft or endoprosthesis. High-grade osteosarcoma is treated in a similar way to central osteosarcoma, with protocols that include chemotherapy, surgery and then adjuvant chemotherapy.

In summary, understanding the clinical, diagnostic and therapeutic aspects of surface osteosarcoma is crucial to ensure an effective and personalized approach for each patient, always aiming to achieve the best clinical results and good quality of life.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Osteocondroma

Osteochondroma

Osteochondroma

Diagnosis and Treatment

Osteochondroma, also known as osteocartilaginous exostosis, represents the most common benign bone lesion, although its incidence may be even higher than that reported in the literature due to many patients presenting asymptomatic osteochondromas.

This condition generally develops in the first and second decades of life, being located in the metaphyseal region of long bones. Radiographically, it is characterized by presenting a tumor composed of cartilage and bone. A distinctive feature is that the central cancellous bone of the exostosis continues with the medullary of the affected bone, while the dense cortical layer of the tumor continues with the normal cortical bone. On the surface of the lesion, there is a band of cartilage, through which the growth of the lesion occurs, hence the name “osteochondroma” – tumor forming cartilage and bone.

Osteochondromas can present as sessile bases (with an enlarged base) or pedunculated bases. They can be single or multiple, characterizing hereditary multiple osteochondromatosis.

The standard treatment for osteochondromas is surgery, generally involving resection, especially when the lesion compromises aesthetics, compresses vascular or nervous structures or limits function. It is important to note that these tumors usually continue to grow while the patient is in the growth phase.

When an osteochondroma increases in size after skeletal maturity, it may indicate post-traumatic bursitis or, more worryingly, malignancy to chondrosarcoma, which requires resection with oncological margins.

The risk of malignancy is low, with solitary osteochondromas having a malignancy rate of about 1%, while multiple osteochondromatosis can be as high as 10%.

In summary, a comprehensive understanding of the characteristics, diagnosis and treatment of osteochondroma is essential to ensure an effective and appropriate therapeutic approach, always aiming for the patient’s well-being and quality of life.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Osteoblastoma

Osteoblastoma

Osteoblastoma

Treatment and Differentiation of Bone Lesions

Osteoblastoma is a rare bone neoplasm, radiographically characterized by areas of bone rarefaction, with denser foci of ossification. This tumor has two distinct clinical forms: genuine osteoblastoma, which is more common and is generally located in the pedicle of the spine or, less commonly, in the metaphysis of long bones; and aggressive osteoblastoma, also known as “malignant”.

Mainly occurring in the first and second decades of life, the clinical picture of osteoblastoma is marked by intense pain, which can lead to fractures and functional and neurological deficits, especially when it occurs in the spine, and can even cause antalgic scoliosis in some cases.

The differential diagnosis of osteoblastoma includes other bone lesions such as osteoid osteoma, aneurysmal bone cyst and osteosarcoma, and a careful evaluation is essential to determine the most appropriate therapeutic course.

Treatment of osteoblastoma generally involves en bloc resection of the lesion, with bone grafting when necessary. In regions such as the spine, where en bloc resection may be challenging, judicious curettage is performed. Furthermore, the use of local adjuvants, such as phenol and electrothermia, has been increasingly used in order to minimize the risk of recurrence.

In short, osteoblastoma is a pathology that demands specialized attention and a multidisciplinary therapeutic approach to guarantee the best clinical result and avoid future complications. Detailed knowledge about this condition is essential for an accurate diagnosis and an effective treatment plan, always aiming for the patient’s quality of life and well-being. Understanding the diagnostic nuances and available therapeutic options is critical to successful management of this condition. Therefore, it is crucial that healthcare professionals stay up to date on the latest advances in the field of osteoblastoma diagnosis and treatment.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Metástases Ósseas

Bone Metastases

Bone Metastases

Bone metastases appear as one of the most common complications in cancer patients, representing an important medical issue. This is in part due to bone’s ability to act as a filter for circulating tumor cells. Like the lung and liver, skeletal tissue has the potential to harbor metastatic cells, especially due to its slower sinusoidal circulation.

With the advancement of cancer diagnosis and treatment methods, we have seen an increase in patient survival. However, this also means that more individuals end up developing bone metastases, with tumors such as breast, prostate, lung, kidney and thyroid being the main responsible for these spreads to the skeleton.

The clinical manifestations of bone metastases often involve pain and, in some cases, can lead to pathological fractures, significantly compromising the patient’s quality of life. In these situations, orthopedic surgical intervention becomes essential to alleviate pain, restore function and avoid serious complications.

The most common sites for these injuries include the femur, humerus, vertebrae, pelvis, scapula, and tibia. The therapeutic approach generally involves resection and reconstruction surgeries, using techniques such as osteosynthesis with cement or endoprosthetic implants. In some cases, radiotherapy may be used as a palliative measure to relieve pain in patients who are not candidates for surgery.

In the context of multiple myeloma, one of the most common primary bone tumors, treatment usually involves chemotherapy and, in certain cases, radiotherapy. However, when bone lesions become significant or present a risk of fracture, the orthopedic surgical approach follows the same principles adopted for bone metastases from other types of cancer.

In summary, the management of bone metastases requires a multidisciplinary approach, involving oncologists, orthopedic surgeons and radiation oncologists, with the aim of providing pain relief, preserving function and improving the quality of life of patients affected by this complication of cancer.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Granuloma Eosinófilo

Eosinophilic Granuloma

Eosinophilic granuloma

History and Evolution of Understanding:

The history of Eosinophilic Granuloma dates back to 1938, when Schaerer diagnosed a lesion on the skull of a child, initially considered to be eosinophilic myeloma or eosinophilic osteomyelitis. It was later described as a new clinical entity by Otani and Ehrlich in 1940, called Solitary Granuloma of Bone. Farber and Green, in 1942, identified its possible relationship with Hand-Schuller-Christian disease and Letter-Siwe disease. In 1944, Jaffe and Lichtenstein introduced the term Eosinophilic Granuloma of Bone, consolidating its association with systemic forms of the disease, now called Langerhans Cell Histiocytosis.

Introduction and Epidemiology:

Eosinophilic Granuloma of Bone, which is the most common form of Langerhans Cell Histiocytosis, represents between 60% and 80% of cases. Despite being a rare benign bone lesion, it occurs mainly in children and adolescents, with a predominance of males. The majority of cases occur in individuals under 21 years of age, with the most affected age group being between five and 15 years. Axial involvement of the skeleton is predominant, with a variety of bones affected, such as the skull, pelvis, ribs and vertebrae, with the spine being responsible for around 10% of cases in children.

Clinical Manifestations and Diagnosis:

The most common symptom of Eosinophilic Granuloma is localized pain, often confused with other causes of headache when it affects the skull. Other forms of Langerhans Cell Histiocytosis can present systemic symptoms, such as fever and diabetes insipidus. The diagnosis is made based on imaging tests, which reveal a bone rarefaction lesion with cortical erosion, in addition to laboratory changes, such as increased ESR and CRP.

Treatment and Prognosis:

The treatment of Eosinophilic Granuloma varies according to the extent of the disease. In isolated cases, an expectant approach or percutaneous biopsy followed by intralesional corticosteroid infusion may be effective. Spontaneous resolution is possible, especially in children, due to the potential for bone remodeling. In more extensive cases or with multiple lesions, systemic treatment with corticosteroids and Vinblastine may be indicated. The prognosis is generally good, with a favorable resolution rate in 97% of cases of solitary lesions.

 

Conclusion :

Eosinophilic Granuloma of Bone, as the most common form of Langerhans Cell Histiocytosis, represents a diagnostic and therapeutic challenge. However, with a multidisciplinary approach and appropriate therapeutic options, most patients can have a favorable outcome. A thorough understanding of this clinical entity is essential for effective management and an optimistic prognosis.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Fraturas Patológicas em Crianças

Pathological Fractures in Children

Pathological Fractures in Children

The frequently used terminology “pathological fracture” can lead to an inappropriate interpretation, since the fracture itself is not pathological, but rather the bone, which can present various structural, metabolic, dysplastic changes, among others. Therefore, it is more appropriate to discuss fractures that occur in children with pre-existing bone changes.

The breadth of this topic requires a delimitation of the topics to be covered. Fractures in children resulting from infectious processes or metabolic disorders, such as rickets and osteopsatirosis, will not be discussed here. Our focus will be on stress fractures, differential diagnosis and fractures resulting from pre-existing tumor or pseudo-tumor bone lesions.

Among the most common benign tumor lesions in childhood that can lead to fractures, osteoblastoma and chondroblastoma stand out. Osteoblastoma, located in the metaphysis of long bones, initially eccentric cortical, is locally aggressive, causing microfractures due to erosion of the cortical bone. The progressive destruction of the cortex can lead to complete fractures, facilitating local dissemination and complicating oncological treatment. Chondroblastoma affects the epiphyseal region of growing long bones and can lead to arthralgia and deformity.

The treatment for these benign lesions is surgical, preferably carried out as soon as possible to prevent the progression of local bone destruction. Segmental resection is the best indication to avoid local recurrences. However, the articular location of chondroblastoma requires a specific surgical approach, followed by local adjuvants and, when necessary, bone grafting.

The most common malignant bone neoplasms in childhood, such as osteosarcoma and Ewing’s sarcoma, require early diagnosis and immediate treatment. In cases of fractures upon diagnosis, local oncological control may require ablative surgeries, such as Van-Ness gyroplasty, which alters joint function and requires psychological support for the patient.

In addition to malignant neoplasms, fractures in children may be associated with pseudo-tumor lesions, such as simple bone cyst, aneurysmal bone cyst, fibrous dysplasia and eosinophilic granuloma. Treatment varies according to the lesion and may involve resection, intralesional curettage and filling with autologous bone graft.

In summary, fractures in children associated with pre-existing tumor or pseudo-tumor bone lesions require a multidisciplinary approach and an individualized treatment plan, considering the location, extent and characteristics of the lesion, as well as the patient’s general condition. Early diagnosis and appropriate treatment are essential to prevent complications and ensure good functional recovery.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Fratura em Osso Patológico

Pathological Bone Fracture

Pathological Bone Fracture

Clinical Aspects, Diagnosis and Classification

In our experience dedicated to the study and treatment of patients with bone diseases, we have repeatedly come across cases of fractures that, in turn, concealed previously undiagnosed underlying pathological conditions. The simplified designation of “pathological fracture” may be inappropriate, since every fracture is, in fact, a pathological process. In this sense, it is more appropriate to use the terminology “pathological bone fracture”, which is often associated with neoplasms, whether primary or metastatic.

The pathological processes that can culminate in fractures are varied and range from bone dysplasias to circulatory disorders, including degenerative, inflammatory, infectious or neoplastic changes. For an accurate diagnosis, it is essential to consider the patient’s clinical aspects, the fracture mechanism, the results of imaging exams, laboratory tests and anatomopathological findings.

Bone fractures that mask underlying pathological conditions may result in inappropriate orthopedic interventions, highlighting the importance of a meticulous diagnostic approach.

We will classify in a didactic way, within the five chapters of General Pathology, the various underlying bone disorders:

  1. Bone Dysplasias: These changes encompass congenital or hereditary disorders that affect bone morphology, which may result in deformities and/or fractures. We highlight, among others, osteogenesis imperfecta, osteopetrosis and fibrous dysplasia, each with its distinct clinical and radiological characteristics.
  2. Metabolic Changes: The balance between the processes of bone formation and resorption is essential for maintaining bone structure. Disorders such as osteoporosis, osteomalacia and hyperparathyroidism can lead to a decrease in bone density, increasing the risk of fractures.
  3. Degenerative Diseases: These include conditions such as Langerhans cell histiocytosis and lipidosis, which can compromise the structure and integrity of bones, predisposing to the occurrence of fractures.
  4. Circulatory Disorders: Paget’s disease is a significant example, characterized by abnormal bone remodeling due to intraosseous circulatory disorders, which can result in spontaneous fractures or due to mild trauma.
  5. Blood Dyscrasias: In rare cases, blood disorders such as leukemia or hemolytic anemia can cause extensive bone infarcts, increasing the risk of fractures in pathological bones.

In summary, understanding the relationship between fractures and underlying pathological conditions is crucial to ensure an appropriate therapeutic approach and minimize complications. An accurate diagnosis and appropriate classification of underlying conditions are critical to guiding treatment and improving clinical outcomes for patients.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

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