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Orthopaedic tumors and masses

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Orthopaedic tumors and masses

Gregory Domson

Introduction
Orthopaedic oncology is a field of orthopaedic surgery that specializes in the diagnosis and
treatment of both benign and malignant tumors of the bones and soft tissues of the extremities,
pelvis, and spine. Although definitions vary, a tumor can be thought of simply as any mass in
the soft tissues or bone that otherwise should not be there. For example, a tumor may be a
neoplasm, which is an abnormal proliferation of abnormal cells; a hamartoma, which is an
abnormal proliferation of normal cells; or simply an infection causing a masslike effect. The
focus of this chapter will be the common neoplasms encountered by the musculoskeletal
oncologist.

Musculoskeletal neoplasms can first be divided into benign and malignant entities. Benign
neoplasms are proliferations of abnormal cells that have no potential to metastasize to other
areas of the body. Locally, some benign neoplasms can be aggressive and cause significant
problems. However, despite its local activity, if a neoplasm has the ability to travel to a distant
organ (such as the lungs or lymph nodes) it is considered malignant.

Malignant bone disease

There are many categories of malignant neoplasms. Some of these include carcinomas
(from epithelial origin), adenocarcinomas (from epithelial cells with secretory properties),
lymphomas (arising from lymphocytes), leukemia (from bone marrow cells), and melanomas
(from transformed melanocytes). A sarcoma is a malignant neoplasm that arises from cells of
mesenchymal origin. Mesenchymal tissues include those found in the limbs and pelvis: bone,
cartilage, muscle, fat, vessels, and nerves. Sarcomas are exceedingly rare. Every year in the
United States there are fewer than 10,000 new cases of bone sarcoma and fewer than 15,000
new soft tissue sarcomas.

Malignant bone disease often causes bone destruction or lysis. A permeative or moth-eaten
pattern of lysis (Fig. 10-1), in which the bone is aggressively destroyed with indistinct margins,
is usually displayed. A more geographic pattern, in which there is a clear margin between
normal and abnormal bone, is seen with benign tumors (Fig. 10-2).

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Figure 10-1. A permeative pattern of destruction from a metastatic lesion in the diaphysis of a
femur.

Figure 10-2. A geographic pattern of bone lysis seen in a simple bone cyst of the femur.

Metastatic disease
Metastatic carcinoma to bone is 25 times more likely to occur than primary bone sarcoma.
The five primary carcinomas that most commonly metastasize to bone are breast, prostate,
lung, kidney, and thyroid. In contrast to the small numbers of primary bone sarcoma, there are
more than a million new cases of these five carcinomas in the United States every year.
Metastatic carcinoma most commonly occurs in the thoracic and lumbar spine (theoretically
because of the valveless Batson”s venous system there) but can occur in virtually any bone. It
commonly presents as pain and can lead to weakened bone and pathologic fractures
(fractures that occur at normal physiologic loads).

Metastatic breast cancer is common in women with advanced disease. Radiographically, it is

classically a mixed lytic and blastic lesion; thatfor
Orthopaedics is, Physician
it causes lysis of bone and formation of bone
Assistants
(Fig. 10-3). It typically responds to radiation therapy but commonly requires surgical
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stabilization. Metastatic prostate cancer is also radiosensitive but typically is a purely blastic
process (Fig. 10-4). Lung, kidney, and thyroid disease usually cause purely lytic and
destructive lesions (Fig. 10-5). Renal cell carcinoma and thyroid disease are extremely
vascular lesions that often require embolization before open surgical treatment.

Figure 10-3. A mixed lytic and blastic pattern of metastasis seen with widespread breast
cancer.

Figure 10-4. Widespread blastic metastases throughout the pelvis, lumbar spine, and femurs.

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Figure 10-5. Purely lytic lung metastasis to the proximal humerus.

Because of the overwhelming preponderance of potentially metastatic carcinoma, any lytic

bone lesion in a patient older than 40 years of age should be considered metastatic disease
until proven otherwise. Workup for these patients should include radiographs of the entire
affected bone, magnetic resonance imaging (MRI) of the area to assess soft tissue extent, a
bone scan to assess other skeletal disease, and a computed tomography (CT) scan of the
chest, abdomen, and pelvis in an attempt to identify the primary site (Fig. 10-6). Often a biopsy
will still be necessary to secure a definitive tissue diagnosis.

Figure 10-6. A bone scan demonstrating widespread adenocarcinoma metastases.

Surgical treatment of metastatic disease can be challenging. Painful lesions in weight-

bearing bones should be aggressively stabilized, but many lesions may not have definite
surgical indications, especially in patients with limited life spans. A team approach with medical
oncology, radiation oncology, orthopaedic oncology, and the patient should be used to optimize
treatment and outcomes. Unfortunately, bone metastasis is an ominous finding with little
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Although much rarer, other malignancies such as melanoma, colon, bladder, and cervical
cancer can all metastasize to bone and should be suspected in patients with a positive medical
history. Metastatic bone disease in children is not common but can be seen with
neuroblastoma and Wilms tumor.

Multiple myeloma
Multiple myeloma, a malignant disease of monoclonal plasma cells, is the second most
common cause of lytic lesions in adults. It is more common in men in their 60s and in African
Americans. The bone lesions are well-defined, punched-out lytic areas that can be seen in any
bone (Fig. 10-7) and are often seen in the skull. Patients will often have anemia (from bone
marrow replacement by tumor), hypercalcemia (from the bone lysis), and a monoclonal protein
spike on urine and serum protein electrophoresis. Treatment is multimodal, requiring medical
oncology, radiation oncology, and orthopaedics.

Figure 10-7. Multiple, well-defined, “punched out” lesions seen in multiple myeloma.

Lymphoma
Metastatic disease and multiple myeloma account for the vast majority of malignant lesions
of bone in adults and should be the first and second entities on any differential diagnosis.
Lymphoma that arises primarily in bone, although rare, can also be seen. It is often in younger
and middle-age adults and classically has a large soft tissue mass with little bony change or
destruction. Surgery for lymphoma of bone is for biopsy and bone stabilization only; definitive
treatment with high cure rates is a combination of chemotherapy and radiation.

Primary sarcoma of bone

Except for chondrosarcoma (which is seen almost exclusively in adults) the primary
sarcomas of bone occur more commonly in the pediatric population. Therefore, entities like
osteosarcoma and chondrosarcoma should be considered at the bottom of the differential
diagnosis of malignant bone lesions in adults (behind metastatic disease, multiple myeloma,
and lymphoma). Primary sarcoma should be at the top of the differential diagnosis of
aggressive lesions in children. The primary sarcomas below account for the most common
entities.
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Secondary sarcomas of bone are rarely seen but should be considered when there is a lytic
lesion or a mass in a bone with preexisting Paget”s disease or previous radiation therapy
(osteosarcoma is the most common variant). Enchondromas and osteochondromas can
transform into chondrosarcomas less than 1% of the time. Rarely, secondary sarcomas can
arise from bone infarcts or fibrous dysplasia.

Osteosarcoma
Osteosarcoma is the most common primary sarcoma of bone. It is a high-grade disease that
has a bimodal age distribution; it arises mostly in children but also in the elderly (often
secondary to a preexisting condition like Paget”s disease). It can occur in any bone but is most
common around the knee. Radiographically, it is classically a mixed lytic and blastic lesion with
a soft tissue mass characterized by a “sunburst,” radial pattern of osteoid formation (Fig. 10-8).
Pathologically, the tumor is required to have malignant cells producing osteoid (Fig. 10-9).

Figure 10-8. Osteosarcoma of the distal femur. Note the blastic soft tissue mass.

Figure 10-9. High-power view of osteosarcoma. Malignant cells are evident among the pink
bands of osteoid.

The workup for osteosarcoma should include an MRI of the entire bone to assess tumor
extent (Fig. 10-10), assist with preoperative planning, and rule out any skip metastases
(anatomically separate areas of tumor in the same bone); a CT scan of the chest to evaluate
the lungs for metastatic disease (the most common site); and a bone scan to evaluate the
skeleton (the second most common site of metastatic disease). A biopsy performed by the
treating physician is the next step to secure thefordiagnosis.
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Figure 10-10. Coronal magnetic resonance imaging of the osteosarcoma seen in Figure 10-8.

Treatment for osteosarcoma is typically multidrug chemotherapy, followed by wide excision

of the lesion (80% to 90% of cases are limb salvage; see Fig. 10-11), followed by more
chemotherapy. Five-year survival rates are currently approaching 80%. Chemotherapy can
have ototoxic and cardiotoxic side effects.

Figure 10-11. The gross specimen from Figure 10-8 after chemotherapy and limb salvage
surgery.

Ewing’s sarcoma
Ewing’s sarcoma is the second most common primary sarcoma in children. Pathologically, it
is a high-grade tumor composed of monotonous, small round blue cells (Fig. 10-12). The
majority of Ewing”s sarcomas have a characteristic t(11,22) translocation that results in the
formation of the EWS-FLI1 oncogene.

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Figure 10-12. High-powered view of Ewing sarcoma showing the uniform, small, round, blue
cells.

Ewing’s sarcoma is often seen in flat bones and the metaphyseal-diaphyseal regions of long
bones. Classically, it has an “onion skin” appearance (multiple thin layers of periosteal reaction
at the site of the tumor; see Fig. 10-13). The workup for Ewing sarcoma is the same as for
osteosarcoma, but the prognosis is slightly worse. Treatment involves chemotherapy and local
treatment. Most tumors are surgically excised, but because Ewing sarcoma is sensitive to
radiation, it can be used to treat tumors that are otherwise unresectable.

Figure 10-13. Plain radiograph showing the layered, periosteal reaction known as “onion
skinning.”

Chondrosarcoma

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