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5 Orthopedic Infections
101
Chen AF, et al. What’s new in musculoskeletal infec-
tion: update across orthopaedic subspecialties. JBJS.
2017;99(14):1232–43. Kocher MS, Zurakowski D, Kasser JR. Differentiating
between septic arthritis and transient synovitis of the hip
in children: an evidence-based clinical prediction algo-
rithm. J Bone Joint Surg Am. 1999;81(12):1662–70.
https://doi.org/10.2106/00004623-199912000-00002.
PMID: 10608376. Olsen MA, et al. Risk factors for surgical site infec-
tion following orthopaedic spinal operations. JBJS.
2008;90(1):62–9.
Rasouli MR, et al. Risk factors for surgical site infec-
tion following total joint arthroplasty. JBJS. 2014;96(18):e158.
Connell MC. In: Wiesel SW, Delahay JN, editors.
Essentials of orthopedic surgery, vol. 615. NewYork: Springer; 2007.
Flow Investigators. A trial of wound irrigation in the ini-
tial management of open fracture wounds. N Engl J Med. 2015;373(27):2629–41.
Tumors oftheMusculoskeletal System
JonathanDay andBrockAdams
6

Background

Both benign and malignant tumors can arise from mesenchymal soft tissue or bone of the extremities and axial skeleton. All tumors of the musculoskeletal system arise from one of the following histological tissues: bone (oste­oid forming tumors), cartilage (chondroid forming tumors), muscle, or brous connective tissue. In addition, tumors can rarely arise from arteries or nerves.
The vast majority of tumors are benign. Malignant tumors of the musculoskeletal system are termed sarcomas, which comprise about 80 different types arising from mesenchymal or connective tissues [1]. It is estimated that roughly 16,000 new cases and 6000 deaths are attributed to sarcomas annually in the USA. Still, this
J. Day MedStar Georgetown Orthopedic Institute, Georgetown University School of Medicine, Washington, DC, USA
Department of Orthopedics, MedStar Georgetown University Hospital, Washington, DC, USA
B. Adams (*) MedStar Georgetown Orthopedic Institute, Georgetown University School of Medicine, Washington, DC, USA
Department of Orthopedics, MedStar Washington Hospital Center, Washington, DC, USA e-mail: Brock.W.Adams@gunet.georgetown.edu
accounts for less than 1% of all malignant cancer diagnoses [2].
Contrary to most malignant diagnoses that occur in the later decades of life, most bone sar­comas (osteosarcoma and Ewing’s sarcoma) occur during childhood or adolescence. Soft tis­sue sarcomas tend to be found starting in the third decade of life and become more common as age increases. Bone tumors typically present with pain, while soft tissue tumors tend to present as a painless mass.
It is fundamental for orthopedic surgeons to have a sound understanding of musculoskeletal tumors. This includes an understanding of the general workup and to the ability to determine which patients should be referred to a subspe­cialized orthopedic oncologist. Early detection, combined with proper techniques of diagnosis and treatment, can dramatically improve the chances of achieving functional limb salvage and minimizing morbidity. Advances in imag­ing, chemotherapy, and radiation therapy, cou­pled with a better understanding of the biological behavior of mesenchymal neo­plasms, have led to a rational basis for diagno­sis, staging, and treatment.
The aim of this chapter is to provide an up-to­date, comprehensive review of the most common benign and malignant tumors of the musculoskel­etal system, as well as the general workup, diag­nosis, treatment options, and prognosis.
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024 W. F. Postma et al. (eds.), Essentials of Orthopedic Surgery,
https://doi.org/10.1007/978-3-031-66215-7_6
103
104
J. Day and B. Adams
Natural History ofBone andSoft Tissue Tumors
Mesenchymal tumors have characteristic patterns of behavior and growth that help distinguish it from other tumors. These unique features form the basis for tumor staging and treatment.
Biology andGrowth
Sarcomas grow in a centrifugal fashion with the youngest and most viable cells toward the periphery. They tend to push the normal tissue away as they expand as opposed to many carci­nomas, which inltrate and invade the surround­ing tissue. In contrast to benign lesions that often are surrounded by a true capsule, malignant lesions are surrounded by a pseudocapsule formed by compressing the normal tissue sur­rounding the lesion as it grows. This is composed of a brovascular zone of reactive tissue that contains some tumor cells within it. The thick­ness of this layer can vary by degree of malig­nancy and histologic subtype.
High-grade sarcomas characteristically have poorly dened reactive zones. In addition, tumor nodules that are not in continuity with the main tumor may be present in tissue that appears nor­mal. Low-grade sarcomas rarely form tumor nod­ules beyond the reactive zone.
Local anatomy inuences the growth of sarco­mas by setting natural boundaries to extension. In general, sarcomas take the path of least resis­tance. Fascial boundaries such as the periosteum, compartmental fascia, or a vascular sheath form effective barriers. Bone tumors grow via three mechanisms: compression of normal tissue, resorption of bone by reactive osteoclasts, and direct destruction of normal tissue. Benign bone tumors grow and expand via the rst two mecha­nisms, whereas malignant ones expand via direct tissue destruction. In addition, benign tumors are usually unicompartmental, meaning they remain conned to and may expand the bone in which they reside. In contrast, malignant bone tumors are often bicompartmental, meaning they destroy the overlying cortex and push directly into the
adjacent soft tissue. With this concept in mind, soft tissue tumors may start in one compartment (intracompartmental) or between compartments (extracompartmental). This determination of anatomic compartments is especially important when considering limb-preservation surgery.
Five Patterns ofTumor Behavior
On the basis of biologic considerations and natu­ral history, all bone and soft tissue tumors, benign and malignant, may be classied into ve catego­ries, each of which shares certain clinical charac­teristics and radiographic patterns. Treatment of tumors is also similar for tumors in each group. The ve categories are as follows:
1. Benign/Latent These lesions grow slowly during the nor-
mal development of the individual. As the growth of the individual stops, the growth of the lesion stops and sometimes resolves spon­taneously. These lesions are typically not symptomatic and as a general rule do not progress to malignancy. Observation is the most common course of treatment. Examples include lipomas and brous cortical defects.
2. Benign/Active These lesions exhibit progressive growth
and bone lesions are often symptomatic. Treatment is with excision for soft tissue lesions or curettage for bone lesions. It is not necessary to remove the reactive zone. Chondroblastoma and osteoid osteoma are examples.
3. Benign/Aggressive These lesions are locally aggressive but do
not metastasize (with the notable exception below). Tumor can extend beyond the capsule and into the reactive zone. Treatment aims to remove the reactive zone by resecting a cuff of normal tissue for soft tissue tumors. For bone lesions this is approximated by remov­ing the reactive zone with a high-speed burr and using a physical or chemical adjuvant to kill remaining tumor cells. Giant cell tumor of bone (benign but can metastasize) and bro­matosis are in this group (Fig.6.1).
6 Tumors oftheMusculoskeletal System
Fig. 6.1 A giant cell tumor of bone with a secondary aneurysmal bone cyst. This represents an aggressive benign lesion. Note the easily recognizable sharply dened border. Plain radiographs are the most important study in the diagnosis of bone tumors
4. Malignant, Low Grade These lesions have the ability to metasta-
size but do so at a low rate. Tumor can expand beyond the compartment and tumors cells are present in the reactive zone. Treatment involves removal of the lesion with a cuff of normal tissue, but no adjuvant therapy is necessary. Examples are parosteal osteosar­coma and low-grade soft tissue sarcomas.
5. Malignant, High Grade These lesions grow rapidly and metasta-
size early and at a high rate. These tumors may have a poorly dened pseudocapsule and tumor nodules can extend beyond the reactive zone into the normal tissue. Exci­sion with a cuff of normal tissue is necessary for surgical treatment. Chemotherapy is required for bone tumors and some soft tis­sue tumors to lessen the rate of metastasis. Radiation is required for soft tissue tumors to reduce the chance of local recurrence. Clas­sic intramedullary osteosarcoma and undif­ferentiated pleomorphic sarcoma (UPS) are in this category.
105
Mechanisms ofTumor Spread
Unlike carcinomas, bone and soft tissue sarco­mas disseminate almost exclusively hematoge­nously. Because of this, the lungs are by far the most common site of metastatic spread with 80–90% of metastases found in the lungs. Bone is the next most common site of metastasis. Both bone and soft tissue sarcomas can spread via the lymphatic system to regional lymph nodes. The rate of lymphatic spread varies by disease entity, but in general is just under 5%.
The histologic hallmark of malignant sarco­mas is their potential to break through the pseu­docapsule to form satellite lesions. Discontinuous tumor, commonly referred to as a skip metastasis, is a tumor nodule located in the same bone or compartment as the primary tumor but is not con­tiguous with it.
The mechanism of spread and common sites of metastatic spread are important to keep in mind when evaluating a patient with a sarcoma and guide the choice of imaging studies neces­sary to determine the stage of disease.
Clinical andRadiographic Evaluation

Clinical Evaluation

It is often said that the importance of the clinical history and physical examination should not be overlooked, and this is certainly true in the evalu­ation of musculoskeletal tumors. The location of a tumor, the rate at which it is growing, pain, or any history of trauma are all key points to obtain from the history. On examination it is important to note the size of the mass, its relation to sur­rounding structures, whether it appears to be supercial or deep, tenderness, the appearance of the overlying skin, and whether it is rm or soft. While mesenchymal tumors spread hematoge­nously for the most part, an examination of the regional lymph nodes should be included.
Special attention should be paid to the char­acteristics of pain. In bone tumors, pain is an important feature distinguishing aggressive or
106
J. Day and B. Adams
malignant tumors from more latent lesions. Pain that arises from a bone lesion typically is described as a dull aching type pain. It is often present at rest as well as with activity and clas­sically wakes the patient up at night. This is in contrast to more common musculoskeletal sources of pain which are typically worse with certain types of activity and improve with rest. A lesion in the proximal humerus of a patient presenting with vague, dull pain waking them up at night and full range of motion of the shoul­der is worrisome. A lesion in the proximal humerus of a patient presenting with pain with overhead activity after an injury is not worri­some. Interestingly, the same does not hold true for soft tissue tumors. The most common pre­sentation of a soft tissue sarcoma is a painless, enlarging mass. While there are more painful benign soft tissue tumors than malignant ones, both benign and malignant soft tissue tumors may present as painless or painful masses.

Radiographic Evaluation

X-Rays
Conventional anteroposterior (AP) and lateral radiographs remain essential in the initial charac­terization and diagnosis of musculoskeletal tumors. Further advanced imaging is often dic­tated by initial plain radiographic ndings. Proper interpretation of a lesion on X-rays can be guided by answering four classic questions proposed by Dr. William F.Enneking:
1. What is the anatomic location and extent of the lesion?
2. What is the lesion doing to the bone?
3. What is the bone doing to the lesion?
4. What is the tissue type of the lesion?
Distinctions between benign, aggressive, and frankly malignant lesions can be made on the basis of this analysis. Benign lesions have well- dened borders often with a surrounding rim of sclerotic bone and do not penetrate the cortex. In contrast, malignant lesions have
poorly dened sometimes permeative borders and can destroy the cortex. Many bone lesions in particular have a characteristic appearance on X-ray and a diagnosis can often be made based off the X-ray along. In general, plain radiographs are the most important imaging study when determining the type of bone tumor (Fig.6.2).
Computed Tomography
Computed tomography (CT) allows high­resolution cross-sectional imaging of bone. While it can be used to evaluate soft tissue struc­tures, soft tissue tumors, and the extent of a tumor both in the intramedullary space and in the soft tissue, CT is most helpful looking at the calcied portions of the bone. Thinning or scalloping of the cortex and subtle periosteal reaction are best seen on CT. Similarly, faint matrix production may be difcult to discern on plain X-ray, but relatively easy to see on CT.CT can also be help­ful for preoperative planning purposes, especially in more complex structures such as the pelvis. A CT of the chest is often used for staging purposes.
Magnetic Resonance Imaging
Magnetic resonance imaging (MRI) provides detailed multiplanar information of both bone and soft tissue. It gives great visualization of ana­tomic compartments, vessels, other soft tissues, and bone. MRI shows the extent of the tumor in both the bone and soft tissue and its relation to the surrounding structures in great detail. It is valuable for preoperative planning. While MRI can be helpful from a diagnostic standpoint, diag­nosis typically cannot be made on the basis of the MRI characteristics alone. An MRI done to eval­uate a tumor should include a true T1 sequence, a T2 sequence with fat suppression, and a T1 post­contrast image with fat suppression (Fig.6.3).
Nuclear Medicine
Bone scintigraphy can be helpful in assessing the primary lesion and to screen for distant metastasis. The delayed phase of a techne­tium-99 scan is used most commonly. While
a
6 Tumors oftheMusculoskeletal System
Adamantoma
Osteoid osteoma
Diaphysis
Chondromyxoid fibroma
107
Round cell lesions:
Ewing sarcoma Histiocytic lymphoma Myeloma
Fibrous dysplasia
Fibrosarcoma
Osteosarcoma
Metaphysis
UPS
Physis
Epiphysis
Chondroblastoma
Fig. 6.2 Schematic of typical locations of common bone tumors. The anatomic location, whether diaphyseal, metaphyseal, or epiphyseal, is one of the most important
the level of uptake compared to the surround­ing bone can be used to distinguish between latent and more aggressive lesions, the level of discrimination is relatively low. Because of this, bone scan is used primarily to screen for metastatic disease.
Positron Emission Tomography (PET) is used to measure the metabolic activity of a lesion. F-18 ouro-2-deoxy-D-glucose (FDG) is most commonly used. Similar to bone scan, there is a correlation between uptake on PET and how aggressive the tumor is. It can be used to assess treatment response. PET is used less frequently for staging in sarcoma than some other malignan­cies, owing to the relatively predictable pattern of spread, but use of PET for sarcoma is becoming more widespread.
Enchondroma
Osteochondrom
ABC
GCT
determinants of tumor type. Cortical, medullary, or eccen­tric location is also important. One should always pay spe­cial attention to the presence of matrix formation
Ultrasound
Ultrasound can be used to evaluate soft tissue tumors. While it is operator dependent, it can often be obtained more quickly than axial imag­ing. Using Doppler, it can be used to assess the vascularity of the lesion. Ultrasound can also be used to guide needle placement for biopsy.
Angiography
While the need for true angiography has decreased as the image quality and availability of MRI has improved, it is sometimes still the best way to assess the vascularity or intravascular involvement of a tumor. Preoperative emboliza­tion of highly vascularized tumors prior to surgi­cal resection can signicantly reduce blood loss and intraoperative morbidity.
108
a b d
c
J. Day and B. Adams
Fig. 6.3 Radiographic (a), T1 weighted coronal MRI (b), axial CT (c), and whole body technetium bone scan (d) of a patient with a grade-2 chondrosarcoma. While the plain radiograph is most important diagnostically (black arrow), MRI shows the tumor extent in the bone and soft tissue

Staging

Staging of a bone or soft tissue sarcoma is the process of determining the local extent of the tumor and any potential sites of distant metasta­sis. If there is clinical or radiographic suspicion of an aggressive or malignant tumor, staging studies should be performed prior to biopsy. Staging can guide proper location and method of biopsy. X-rays should always be a part of the local imaging. Beyond this, initial staging typi­cally includes MRI of the entire bone or region affected, CT of the chest, and a technicium-99 whole body bone scan.
(white arrows), CT shows the faint chondroid matrix and abnormal cortex (red arrow), while bone scan shows increased uptake at the lesion and screens for additional lesions (blue arrow)

Staging Systems

The purpose of a surgical staging system is to organize patients into groups that correlate with overall outcome and survival. This has prognos­tic value and can guide treatment decisions for both physicians and patients. There are two widely used staging systems in orthopedic oncol­ogy. Historically, the most widely used system is the Musculoskeletal Tumor Society (MSTS) sys­tem based on the system proposed by William Enneking [3]. The system from the American Joint Committee on Cancer (AJCC) is the other widely used system and is the most common sys-
6 Tumors oftheMusculoskeletal System
109
tem used in specialties outside of orthopedics. Stage in both systems correlates with overall sur­vival with higher stage having a worse prognosis.
The MSTS system takes three factors into account. i.e., the tumor grade, local extent, and the presence or absence of metastatic disease. The same system is used for both bone and soft tissue sarcomas.
1. Surgical Grade
The histological grade of a lesion corre­lates with its growth rate or aggressiveness. Tumors are divided into two groups—low grade versus intermediate and high-grade. Low-grade lesions are malignancies with low potential to metastasize.
Table 6.1 AJCC (8th edition) staging system for soft tis- sue sarcomas
G, Histologic
grade
T, Primary tumor
N, Regional node
M, Distant metastasis
Stage groups IA G1, T1, N0, M0
G1 Well-differentiated, low grade G2 Moderately differentiated,
high grade
G3 Poorly differentiated, high
grade
T1
5cm in greatest dimension
T2
>5cm and 10cm in greatest dimension
T3
>10cm and 15cm in greatest dimension
T4 Tumor >15cm in greatest
dimension
N0 No regional lymph node
metastasis
N1 Regional lymph node
metastasis M0 No distant metastasis M1 Distant metastasis
IB G1, T2/3/4, N0, M0 II G2/3, T1, N0, M0 IIIA G2/3, T2, N0, M0 IIIB G2/3, T3/4, N0, M0 IV Any G, Any T, N1, M0
Any G, Any T, Any N, M1
2. Surgical Site Anatomic site is either intracompartmental
or extracompartmental. A compartment is dened as an anatomic space bound by natu­ral barriers to tumor extension such as a single bone. More aggressive tumors tend to expand outside of their original compartment.
3. Metastatic Disease The presence of metastatic disease is a
poor prognostic factor. The 8th edition of the AJCC staging system is slightly different for bone and soft tissue sarcomas [4]. It uses grade, tumor size, the presence of discontinu­ous tumor (for bone sarcomas), and the pres­ence of lymph node or distant metastasis (Tables 6.1 and 6.2). Recent literature has pro­posed alternative staging systems which con-
Table 6.2 AJCC (8th edition) staging system for bone sarcomas
G, Histologic
grade
T, Primary tumor
N, Regional node
M, Distant metastasis
Stage groups IA G1, T1, N0, M0
G1 Well-differentiated, low grade G2 Moderately differentiated,
high grade
G3 Poorly differentiated, high
grade
T1
8cm in greatest dimension T2 >8cm in greatest dimension T3 Discontinuous tumor in
primary bone site N0 No regional lymph node
metastasis N1 Regional lymph node
metastasis M0 No distant metastasis M1a Distant metastasis—lung M1b Distant metastasis—other
sites
IB G1, T2/3, N0, M0 IIA G2/3, T1, N0, M0 IIB G2/3, T2, N0, M0 III G2/3, T3, N0, M0 I VA Any G, Any T, N0, M1a IVB Any G, Any T, Any N, M1b
110
J. Day and B. Adams
sider other factors such as histologic grade, tumor size, and anatomic depth [5].

Biopsy

Biopsy Techniques

The planning and technique of a biopsy are very important. Biopsies should be performed after staging studies are obtained [6]. Biopsy should be done in the plane of eventual tumor resection and should be closely coordinated by the treating phy­sician. Biopsy should be taken from the periphery of the lesion where the most viable tissue and immature cells are found. In a bone lesion, the soft tissue component should be biopsied if pres­ent. Care should be taken not to contaminate potential tissue planes or aps that will compro­mise the management of the lesion. A poorly planned biopsy can result in delayed diagnosis, larger denitive procedures, increased need for ap coverage, and higher rate of amputation [7].
Core-Needle Biopsy
To minimize contamination and morbidity, core­needle biopsy of soft tissue and bone sarcomas is generally recommended over incisional biopsy (Fig.6.4). Core-needle biopsy provides adequate tissue for diagnosis with accuracy similar to open
biopsy. The risk of seeding the biopsy tract is lower [8]. In contrast to ne-needle aspiration, core-needle biopsy preserves the intercellular architecture in addition to the cellular appear­ance, which is important in the histologic diagno­sis of mesenchymal neoplasms.
Imaging should be performed prior to biopsy. Image guidance with either CT or ultrasound can be used as needed and can be useful in tumors in close proximity to neurovascular structures, areas of complex anatomy, or when signicant hetero­geneity or evidence of necrosis is present on imaging.
Incisional Biopsy
While a core-needle biopsy is sufcient in the majority of cases, an open, or incisional, biopsy may still be indicated when core-needle biopsy is not diagnostic or a high volume of tissue is neces­sary. This requires proper technique to minimize contamination. While the biopsy should always be done in the plane of eventual resection, this is most important in an open biopsy. A tourniquet is generally advised to help facilitate visualization of tumor and meticulous hemostasis should be maintained. Tissue dissection should be mini­mized, and dissection of neurovascular structures should be avoided.
Excisional Biopsy
Some small, supercial lesions may be removed as an excisional biopsy. This involves excising the lesion with a wide margin or a cuff of normal tissue. Criteria to consider excisional biopsy include small size, supercial location or rela­tively close to the fascia, minimal morbidity, and neoadjuvant therapy would not change surgical approach or outcomes.
Fig. 6.4 Clinical photograph of core-needle and speci­men. Multiple specimens from different portions of the tumor can be obtained through one biopsy site. The ortho­pedic oncologist should either perform or oversee place­ment of the needle
Overview ofSurgical Procedures
Surgical removal—including curettage, resec­tion, and amputation—is the mainstay of treating musculoskeletal tumors (Fig.6.5). The extent of the surgical procedure necessary is determined by both tumor type and the local anatomy. A clas­sication scheme of surgical procedures based on
6 Tumors oftheMusculoskeletal System
Radical
Wide
Marginal
Intralesional
Fig. 6.5 Schematic diagram illustrating different resec­tion types
the surgical plane as well as method of resection is summarized below. This provides a standard­ized terminology when discussing surgical treat­ment of both bone and soft tissue tumors and is widely used in clinical practice as well as the published literature.
1. Intralesional procedures involve opening the pseudocapsule and removing the tumor in piecemeal fashion. Biopsies are by denition intralesional. Most benign bone tumors are treated with an intralesional procedure or curettage.
2. Marginal procedures involve opening the pseudocapsule and removing the mass in a single piece. This leaves the pseudocapsule behind. Marginal resections are not used for sarcomas since tumor cells are known to reside in the pseudocapsule. Low-grade lipo­matous soft tissue masses are typically removed with a marginal resection.
3. Wide (intracompartmental) procedures, also referred to as en bloc resections, involve resecting the entire lesion including the surrounding pseudocapsule and a cuff of nor­mal tissue. There is no specic thickness of normal tissue necessary to perform a wide resection. As opposed to carcinomas, which often require a specic margin measured in
111
centimeters, a wide margin in sarcoma is dened as at least single layer of cells.
4. Radical (extracompartmental) procedures involve resection of the entire compartment (or compartments) involved by the tumor. Although this term still appears in the litera­ture, it most often is used because of differ­ences in terminology and billing codes and does not reect a true radical resection. A true radical resection at this point is primarily his­torical in nature and is not necessary unless done to achieve a wide margin as described above.
It is important to note that all the above can be performed via a limb-sparing approach or via amputation. Amputation does not automatically provide a wide margin. Both the local tumor extent and the local anatomy dictates how a par­ticular margin can be obtained.
In general, benign bone tumors can be ade­quately treated with either intralesional proce­dure (curettage) or a marginal excision. For benign bone lesions, this typically involves mak­ing a cortical window and using curets to remove the lesional material. A high-speed burr is then used to remove the reactive zone. An adjuvant is then often used to kill any remaining tumor cells. This may be a cryoablation to freeze the tumor cavity, use of an argon beam, or a chemical adju­vant such as phenol. Removing the reactive zone and use of an adjuvant are important in reducing the recurrence rate, which can be as high as 50% for some tumors after a simple curettage (Fig.6.6).
Malignant tumors as a rule should be excised with a wide margin. Most tumors, 90–95%, can be excised with a limb-sparing operation, but if a wide margin cannot be safely obtained, amputa­tion should be considered. As mentioned above, there is no specic thickness of normal tissue that must be maintained around the tumor to achieve a wide margin. That being said, the biology of the individual tumor type and its propensity for local recurrence and the effectiveness of adjuvant treatment in the form or radiation or chemother­apy should be taken into account when deciding how aggressively to pursue limb salvage.