Tumor List

Case Symptoms and Presentation
Brown Tumor

Clinically, hyperparathyroidism presents as "stones, bones and groans". The stones refers to recurrent kidney stones. Bones refers to the bone lesions that occur in severe or prolonged cases. Groans is meant to describe the gastrointestinal symptoms of nausea, vomiting, peptic ulcers and pancreatitis as well as the obtundation that occurs with hypercalcemia.

Calcific Periarthritis

Patients present with local pain, tenderness, swelling, and warmth.

Metastatic Breast Cancer

Pain is the most common presenting symptom. Pathological fracture rarely occurs without a history of a few weeks or months of increasingly severe pain. In some cases the patient has tried to ignore or deny the symptoms. Sometimes a painful bone lesion is thought to be a "muscle pull" or a "sprain" and strong pain medicines are prescribed, allowing the patient to continue to tolerate very severe pain before the true nature of the problem is discovered. Systemic symptoms may also occur, such as hypercalcemia.

Metastatic Prostate Cancer

Diagnosing prostate cancer usually begins with elevated serum PSA and/or prostatic acid phosphatase, or the palpation of nodules on a digital rectal exam. Diagnosis is typically confirmed by a biopsy guided by transrectal ultrasonography. PSA is also currently the most useful marker for assessing the level of bone involvement in prostate cancer. Diagnosis of bone metastases is accomplished by a bone scan. The problem with bone scans is that they are considered sensitive in the initial diagnosis, but the specificity is relatively low. They are also not suitable for monitoring short-term response to treatment. Other markers are currently under investigation to measure the amount of bone involvement. One such marker, pyridinoline cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), is cleaved during type I collagen degradation which is indicative of bone turnover. The current thinking is that ICTP is more specific to bone than PSA, but further studies need to be done.

The actual mechanism by which metastatic cancer cells elicit osteoblastic reactions is not known. However, Koeneman et al have proposed that "in order to thrive in the bone environment, cancer cells must acquire 'bone cell-like' properties." It is believed that there are reciprocal cellular interactions between prostate cancer cells and bone stroma that result in proliferation of both prostate cancer and bone stromal cells. This interaction involves the participation of growth factors, such as TGF-ß, bFGF, and IGF on both bone and prostate, which is further modulated by the differentiating actions of bone morphogenetic proteins and PTH-related proteins. In the end, conditions are suited for osteoblastic growth, and the consequences are bone pain, inflammation, and increased risk for fractures.Also, vertebral tumors may compress the spinal cord and cause nerve damage. It is important to get a good feel for the level of bone involvement, because it has a significant impact on overall patient survival.

The current methods of treatment for prostate cancer are prostatectomy, radiation therapy, and hormone therapy. Hormone therapy can be accomplished by a few different methods. Orchiectomy and administration of estrogen are two options. Although 60-80% of patients with advanced prostate cancer improve following castration, there is an inevitable progression to a testicular androgen-independent state, in which adrenal androgens take over. There are also drugs that effect the pituitary gland directly, such as LHRH agonists, and some patients also receive antiandrogen drugs to block the effect of androgens produced from the adrenal glands producing a total androgen blockade.

Some patients with metastases often require additional treatment, such as second-line hormone therapy, which further suppress the production of androgens, or block their actions at the cancer cell itself. Other options are chemotherapy, radiation therapy, and bisphosphonates, which degrade osteoclasts, and prevent bone breakdown. Bisphosphonates are still under investigation for their effectiveness in metastatic prostate cancer. In cases in which the patient is in severe pain, administration of narcotics and a referral to a pain management specialist may be warranted.

It is recommended that patients with bone metastases should start androgen withdrawal therapy immediately upon diagnosis. However, hormone therapy does not provide a cure for the majority of cases of prostate cancer that have metastasized to bone. There are also many side effects of androgen ablation. Namely, decreased sexual functions, hot flushes, anemia, weight gain, and in the long run, bone loss and osteoporosis. A total androgen blockade has at best modest benefits.

The survival rate is projected at 8-10% at five years, and it has no advantage over orchiectomy at two years. Also, 60% of newly diagnosed patients with metastases die by 2 years. Routine periodic examinations and bone scans should be performed on a patient with metastatic prostate cancer. The goal is to prevent the progression of a metastasis to a pathological fracture.

Skeletal metastases are very common in prostate cancer. At autopsy, 84% of those with prostatic adenocarcinoma have skeletal metastases, while in 1982, of 20,000 new cases of prostate cancer, 21.5% of patients presented with clinical stage D (metastatic) prostate cancer. Skeletal metastases are also generally associated with poor prognosis. Only 23% of patients survived 5 years from initial diagnosis, and the 10-year survival rate is 10%.

As mentioned above, the most common sites for skeletal metastases are, in order of decreasing frequency, vertebrae, sternum, pelvic bones, ribs, and femurs.1 However, the most common sites for fracture are the medial cortex of the proximal femur and the vertebral bodies because these two sites are required to carry heavy loads.

Overall, the pathologic fracture rate from prostate cancer is relatively low compared to that of other metastatic cancers.9 The reason for this low rate is because of the bone forming osteoblastic reaction by the tumor. Even in those cases in which fractures do occur, the rate of healing approaches that of normal bone. Normal healing, in conjunction with effective radiotherapy and hormonal manipulation limits the need for surgical stabilization to only about one fourth of the patients who develop a pathologic fracture.

On the other hand, skeletal metastases that are predominantly osteolytic are rarely seen in prostate carcinoma.9 The authors feel that the osteolytic lesions that are seen in prostate cancer have a higher risk of fracture than osteoblastic metastases. That risk probably approaches that of other osteolytic metastatic cancers, such as breast cancer, lung cancer, and renal cancer. A patient with an impending pathological fracture can be treated with surgery, radiotherapy, chemotherapy, or hormonal manipulation.

The treatment of a pathologic fracture involves treating the neoplasm while also restoring structural stability; however, treatment of one may effect the treatment of the other. For example radiotherapy and chemotherapy may have adverse effects on the healing bone. For this reason, when postoperative radiotherapy is necessary, endoprosthetic replacements in the femur are the preferred method of surgical stabilization over osteosynthetic devices, because they do not require fracture healing. After surgical reconstruction, the wounds should be allowed to heal at least two weeks before starting radiotherapy or chemotherapy to reduce the risk of infection.

The oncologist and the orthopedic surgeon should jointly share postoperative follow-up, as about 25% of these patients will develop another bone metastasis requiring surgical stabilization. In a study performed by Wedin et al, 26 of 228 (11%) procedures for metastatic lesions of long bones led to failures necessitating reoperation. The overall failure rate of metastatic prostate cancer alone was 10%. Of the 26 failures, four were attributable to immediate failures, six to tumor progression, ten to nonunions, five to stress fractures of bone, and one to late dislocation of a humeral megaprosthesis and skin necrosis. Among 54 endoprostheses, only one (2%) patient had a reoperation, while among 162 operations involving osteosynthetic devices 22(14%) were failures. The reoperation rate in radiated fracture sites was 13% versus 10% in nonradiated sites. Five of the six patients who had reoperations for local tumor progression had not received radiotherapy to the fracture site, and eight of ten nonunions had been treated with radiotherapy. Also, all five patients who had a stress fracture had been treated with radiotherapy.9 All of this confirms that endoprostheses have a smaller failure rate that osteosynthetic devices, and that radiation may prevent tumor progression.

Metastatic Lung Cancer

Pain is the most common presenting symptom. Pathological fracture rarely occurs without a history of a few weeks or months of increasingly severe pain. In some cases the patient has tried to ignore or deny the symptoms. Sometimes a painful bone lesion is thought to be a "muscle pull" or a "sprain" and strong pain medicines are prescribed, allowing the patient to continue to tolerate very severe pain before the true nature of the problem is discovered. Systemic symptoms may also occur, such as hypercalcemia and hypertrophic pulmonary osteoarthropathy (painful thickening of the long and short tubular bones and clubbing of the fingers).

Metastatic Kidney Cancer

Pain is the most common presenting symptom. Pathological fracture rarely occurs without a history of a few weeks or months of increasingly severe pain. In some cases the patient has tried to ignore or deny the symptoms. Sometimes a painful bone lesion is thought to be a "muscle pull" or a "sprain" and strong pain medicines are prescribed, allowing the patient to continue to tolerate very severe pain before the true nature of the problem is discovered. Systemic symptoms may also occur, such as hypercalcemia. An occasional patient may have hypertesion from the tumor affecting the renin-angiotensin pathway. Hematuria is also a common sign, but small amounts of blood in the urine cannot be detected without a urinalysis When a patient has a metastasis and no site of origin can be found (a metastasis of unknown origin) the most likely site is the lung or kidney.

Chondroblastoma

Patients complain of aching pain in a joint. There is poor response to NSAID medication. Eventually a swelling or mass appears.

Chondroblastoma - Foot and Ankle

Patients complaint of pain and swelling or a mass near the joint. The pain can be severe. The nearby joint may be locally inflamed.

Chondroblastoma

Patients complain of aching pain in a joint. There is poor response to NSAID medication. Eventually a swelling or mass appears.

Chondromyxoid Fibroma

The clinical presentation is usually chronic pain, swelling and possibly a palpable soft tissue mass or restriction of movement. Only 5% of patients with CMF present with a pathological fracture.

Chondromyxoid fibroma - Foot and Ankle

Patients present with pain and a slow growing mass.

Chondrosarcoma

The presentation of chondrosarcoma depends on the grade of the tumor. A high-grade, fast growing tumor can present with excruciating pain. A low grade, more indolent tumor is more likely to present as an older patient complaining of hip pain and swelling. Pelvic tumors present with urinary frequency or obstruction or may masquerade as "groin muscle pulls".

Chondrosarcoma - Foot and Ankle

The lesion presents as a slow growing mass with mild pain.

Chordoma

The clinical presentation depends on the location of the tumor. Sacrococcygeal tumors often present as low back pain with no characteristic pattern or time course. Sacrococcygeal tumors can also present as bowel and bladder dysfunction. Presacral tumors can often be palpated on rectal exam, but this simple examination technique is often overlooked. Sacral tumors are often large at presentation as a large volume of tumor can be accommodated within the pelvis. Anterior cervical tumors can present as dysphagia and posterior cervical tumors can cause neurological deficits. Tumors at the base of the skull may present with headaches.

Chronic Recurrent Multifocal Osteomyelitis (CRMO)

Children present with pain, deep aching pain, limping, and may also present with fever. The metaphyseal area of long bones, the clavicle, and the shoulder girdle are common locations. Other sites such as the spine, ankle, and foot have been reported.

Dermatological manifestations may occur and include psoriasis, acne, and pustules on the palms of the hands and soles of the feet. Uveitis, and inflammatory bowel disease have also been described.

Majeed syndrome consists of CRMO and congenital dyserythropoietic aneama, has been reported in families. Te LPIN2 gene appears to play a role in these cases.

Clear Cell Chondrosarcoma

The tumor is a slow growing lesion that has a predeliction for the epiphysis of major long bones. Therefore, gradual onset of joint-related pain in the hip or shoulder is a typical presentation. Sometimes a pathological fracture will force the patient to see treatment for the lesion, even though there has been minor to moderate aching pain for years. The overall presentation of this lesion mimics that of a benign bone tumor.

Collagenous fibroma (desmoplastic fibroblastoma)

Patient presents with a slowly growing painless mass in the subcuticular tissues a peripheral site, most commonly the upper or lower extremities, which may involve skeletal muscle. The size may be from one to several centimeters, with masses as large as 20 cm possible.

Chondroblastoma - Foot and Ankle

Patients complaint of pain and swelling or a mass near the joint. The pain can be severe. The nearby joint may be locally inflamed.

Periosteal chondroma

Symptoms are present for 1 to 5 years. Patients complain of a tender swelling or mass.

Periosteal chondroma - Foot and Ankle

The lesion presents with pain and swelling.