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Bone Sarcoma

The word 'sarcoma' is derived from the Greek word for 'fleshy'. Sarcomas arise from Mesenchymal tissue which is the precursor to fibrous tissue, muscle, bone and fat. Bone sarcoma is malignant tumor or cancer of the bone. The difference between the terms sarcoma and carcinoma is that while sarcomas spread through blood, carcinomas through lymph fluid to the nearby lymph nodes.


Symptoms of bone sarcoma

  • Pain in the bones.
  • Pain may worsen in the nights.
  • Swelling, redness and tenderness at the site of bone pain.
  • Swelling of the leg or arm in longer bones such as above or below the knee or upper arm, shoulder etc.
  • Bone fracture after a routine movement – like throwing a ball.
  • As cancer weakens bones, broken bones tend to occur more easily.
  • Fatigue, fever and weight loss and anemia are other signs.

Causes of bone sarcoma

The cause is unknown in most cases although bone sarcoma can develop from benign lesions in the bone and areas that previously received radiation. Therefore persons who received radiation therapy or chemotherapy can be at increased risk. A genetic syndrome called Li Fraumeni and retinoblastoma and a disease called Paget's Disease are also associated with sarcoma.


Types of bone sarcoma

1. Osteosarcoma that occurs in the bones around the knee and upper arm.
2. Chondrosarcoma that begins at the cartilage and ends of bones and lines of joints and in pelvis, upper leg and shoulder.
3. The Ewing sarcoma family of tumors that occurs in bone but may also be present in the connective tissues located in the pelvis, legs and arms.
4. Multiple Myeloma which is a cancer of plasma cells that originates in bones.


Bone sarcoma diagnosis

A doctor examines the patient physically after ascertaining family medical history. Then the physician may recommend the following diagnostic tests:

  • X rays in various parts of the body to show location, size and shape of bone tumor.
  • Bone scans
  • CT scans which reveal cross sectional images to check if the tumor has spread.
  • MRI scans
  • PET scans that image the body's metabolic action to show if cancer has spread.
  • Biopsy

Treating Bone Sarcoma

Surgery plays a primary role in the treatment of bone sarcoma. The goal of surgery is twofold. On the one hand it can remove the cancerous tumors and restore the physical function and appearance. On the other hand, a cuff of normal tissue is taken from around the tumor cells to minimize the risk of tumor recurring at the local resection site.


After surgery, the reconstructive or plastic surgery team performs the delicate task of replacing bone. Musculoskeletal oncologists and reconstructive plastic surgeons use donor bone, the patient's own bone from other sites and metal implants to reconstruct limbs and restore function.

Radiation therapy: This can be performed before or after surgery or even during surgery though the use of brachytherapy. Radiation therapy is used to treat tumors or when there is disease recurrence at the site of the original tumor or other localized site. This is especially used in treating chondrosarcomas more often than bone sarcomas.

Chemotherapy: Chemotherapy is given before surgery in order to shrink the tumor and allow for better resection. Surgery and radiation can only act on a small area tumor site. Chemotherapy kills any cancer cells floating undetected elsewhere in the body.

Follow-up depends upon the grade and type of bone sarcoma. In case of Osteosarcoma and Chondrosarcoma, follow-up includes physical exam, chest x-ray which should be performed every three months for at least two years initially and then after four months for a year and then every six months for two years and subsequently annually.


Coccydynia

The coccyx also called the tailbone is located at the bottom of the vertebral column. The pain associated with this area - Coccydyniais is usually a localized form of inflammation. The pain occurring in the coccyx region can be very painful as it is connected to many muscle innervations in the gluteal region. The surrounding muscles that arise and enervate at this junction are gluteal, rectus femoris and the muscles governing the sphincters of the anus.


Coccydynia is caused by trauma to the lower spine. This condition is common in the emergency room as it is associated with falls and accidents. Many athletes complain of the tailbone, which may include other reasons such as nerve compression, ligament tear and fractures. Some studies indicate the involvement of the inter-vertebral discs, which in conditions such as cystic fibrosis can cause inflammatory response leading to coccydynia. This particular condition is characterized by degeneration of intercoccygeal and sacrococcygeal discs. Based on etiological conclusions, coccydyniais is defined as a condition which can be idiopathic or traumatic in origin.


In some cases coccydyina can be caused by the anatomical deformities in which bony spicules and coccygeal retroversion are found. These are termed as type II, III and IV forms of coccyx. Other causes include inflammation of the pelvic bone floor muscles and the soft tissues surrounding the coccyx and also compression of sacral nerve roots. Infections also play a major role in the onset of this condition. The most predominant forms of infections associated with coccydynia are tuberculosis, meningitis (bacterial and viral), arthritis and clostridium related infections. Sometimes, trauma to the coccyx results from an accident, poor posture or even during childbirth.


Symptoms

Although coccydynia is often mild in origin with respect to its occurrence, the progression of the condition can worsen and affect the lifestyle of a person - making the person slower as the pain radiates causing restricted movements and abstinence from prolonged physical activity, which may sometimes include daily routines. Coccydynia is more prominent in women than men due to the associated osteoporotic condition in which the calcium and phosphate imbalance is noticed to a larger extent in the deterioration of the bones. Elevated pain during sexual intercourse, premenstrual period and also in defecation are noticed. The pain is usually worse when sitting down or moving from sitting to standing position. Other symptoms include painful buttocks and hips and shooting pain down the legs.


Diagnosis

Radiological diagnosis is more significant in coccydynia as CT, MRI and PET scans reveal the gross anatomy of the coccyx. Sagittal rotation, hard stools, angle of pelvic rotation and angle of mobility can pave the way for definitive diagnosis of this condition.


Patients with coccydynia are advised to avoid long hours of sitting as this elevates the pressure on the tailbone. In most cases anti-inflammatory drugs are given and patients with persistent coccydynia are treated with cortisone injections. Physiotherapy can provide short-term relief from pain. Surgical interventions such as coccygectomy are done to remove any bony prominence in the coccyx region.



Paraneoplastic Syndrome

The first report of Paraneoplastic syndrome is attributed to a French physician, M Auche, who described the involvement in a peripheral nervous system in cancer patients in 1890. This syndrome is a group of signs and symptoms caused by a substance that is produced by a tumor, or in reaction to a tumor. Paraneoplastic syndrome is defined as a rare disorder triggered by a response to neoplasm of an immune system that is altered. These are clinical syndromes produced by tumor although they occur remotely from the tumor itself.


The symptoms may be of any nature – endocrine, neuromuscular or musculoskeletal, cardiovascular, cutaneous, hematologic, gastrointestinal, renal or miscellaneous. Fever is the most common sign. Other clinical symptoms may be benign but syndromes could be varied; from a malignant carcinoid syndrome to Cushing syndrome. In cancer patients, the central nervous system involvement can be detected and it is a prominent manifestation of cancer.

Because of their protean manifestations, Paraneoplastic syndromes are managed by a medical team of physicians, including medical oncologists, surgeons, radiation oncologists, endocrinologists, hematologists, neurologists and dermatologists.


Causes

This syndrome can be due to a number of causes, including hormones or biologically active products, made by tumor, which blocks normal hormone, autoimmunity, immune-complex production and immune suppression. But, this is not caused by the primary tumor itself, or by its metastases, or by compression, infection, nutritional deficiency, or treatment of tumor.

Patients with family history of malignancies are at an increased risk and should be screened for cancer. Paraneoplastic syndromes occur typically among middle-aged to older patients. They are mostly diagnosed with cancers of the lung, breast, ovaries, or lymphatic system or lymphoma.

These symptoms sometimes appear before the diagnosis of a malignancy. When the tumor breaks immune tolerance and begins to attack the normal tissue expressing that protein, this syndrome become prominent.


Types of Paraneoplastic Syndrome

Due to the complexity of this syndrome, its clinical presentations may vary greatly. Hence, Paraneoplastic syndromes is divided into four main categories - endocrine, neurological, mucocutaneous, and hematological syndromes as well as a host of others including rheumatologic, renal, gastrointestinal, Cutaneous and miscellaneous.


Fever, dysgeusia, anorexia, and cachexia are included under miscellaneous category. Fever is associated with lymphomas, acute leukemias, sarcoma and carcinomas.

Rheumatologic syndrome includes polyarthritis, particularly in patients with myelomas, lymphomas, acute leukemia, malignant tumors of the colon, pancreas, prostrate. Scleroderma may precede the evidence of tumor. Widespread form of malignancies of breast, uterus and lung can be observed.

Renal syndrome is characterized by hypokalemic nephropathy, and it occurs in 50% of individuals with ACTH secreting tumors of the lung. Nephrotic syndrome is observed in patients with Hodgkin lymphoma, non Hodgkin lymphoma, malignancies of lung, thyroid, colon, breast, ovary and pancreatic head.

Watery diarrhea and electrolyte imbalance, leads to asthenia, confusion and exhaustion, all characteristic of gastrointestinal syndromes. Severe prostaglandins lead to malabsorption and unavailability of nutrients.


Hematologic syndromes are related to anemia, thrombocytosis, disseminated intravascular coagulation, and leukemoid reactions from several types of cancers. Leukemoid reactions due to immature white blood cells in the blood stream are accompanied by hypereosinophilia and itching.

Cutaneous syndromes are manifested by itching and flushes, alopecia, or hypertrichosis. Blackish pigmentation of the skin usually occurs in patients with metastatic melanomas or pancreatic tumors.

Endocrine syndromes resemble common endocrine disorders. Increased serum and urine cortisol concentrations are common examples of this malignancy.


Neurologic or neuromuscular syndromes relate to cancers and such disorders affect 6% of all patients with cancer. Neuromuscular symptoms mimic common neurological conditions. Myasthenia gravis is a common Paraneoplastic syndrome in patients with thymoma, a malignancy arising from epithelial cells of the thymus.

Paraneoplastic limbic encephalitis is characterized by depression, seizures, irritability and short term memory loss and neurologic symptoms rapidly resemble dementia.

Paraneoplastic cerebellar degeneration causes gait difficulties, dizziness, nausea and diplopia. Sensory neuropathy affects lower and upper extremities and there is progressive memory loss, either symmetric or asymmetric.


Diagnosis

As Paraneoplastic syndromes may evolve over weeks to months, they usually stabilize regardless whether the patient's underlying condition improves or worsens. Since this is the first manifestation of cancer, patients should undergo investigation for cancer. Other diagnoses are membrane disease, dementia, encephalopathy, encephalitis, myelitis, anemia, bone marrow failure, chronic fatigue syndrome, mixed connective tissue disease, polycythemia vera and polymyalgia rheumatica among others.

Complete laboratory tests in blood, urine, cerebrospinal fluid, protein electrophoresis, assays for auto antibodies, Endoscopy and imaging studies including whole body scans and FDG-PET scans are prescribed to detect extremely small tumors especially useful for patients with neurologic diseases.


Treatment of Paraneoplastic Syndrome

Treatment for this syndrome varies depending upon the type and location of tumors. The first option of treatment of the underlying tumor is by therapeutic protocols, a combination of surgery, radiation, chemotherapy. Second therapeutic option is for patients with clearly identifiable antibodies by intravenous immunoglobulins, steroids, or plasma exchange.

Surgical treatment is typically directed toward the underlying neoplasm although some Paraneoplastic disorders may resolve rapidly without surgery on the primary tumor.


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Bibliography / Reference

Collection of Pages - Last revised Date: November 15, 2019