Diagnosis and Management of Pediatric Primary Bone Tumors in the Emergency Department

Table of Contents

 

About This Issue

The nonspecific signs and symptoms and lack of systemic abnormalities in pediatric patients with primary bone tumors can lead to misdiagnosis and a delay in initiation of treatment. This issue reviews the clinical presentation of the malignant and benign pediatric bone tumors that are most commonly encountered in the emergency department (ED). Associated radiographic findings that can assist in differentiating bone tumors and guide further management are also reviewed. You will learn:

Common presentations of malignant and benign pediatric bone tumors

Key aspects of the history and physical examination that can help narrow the differential diagnosis

How the location within the bone can help determine the tumor type

Radiographic findings to help differentiate between malignant and primary bone tumors

When advanced imaging is warranted

Recommendations for management and disposition, including which patients need treatment in the ED, which patients should be admitted, and which patients require referral for outpatient follow-up

When outpatient follow-up is urgent and when it is emergent

1. Abstract
2. Case Presentations
3. Introduction
4. Critical Appraisal of the Literature
5. Etiology and Pathophysiology
6. Presentation, Radiographic Features, and Management by Tumor Type
   1. Malignant Tumors
      1. Osteosarcoma
      2. Ewing Sarcoma
   2. Benign Tumors
      1. Langerhans Cell Histiocytosis
      2. Osteochondroma
      3. Osteoid Osteoma
      4. Osteoblastoma
      5. Chondroblastoma
      6. Enchondroma
      7. Chondromyxoid Fibroma
      8. Unicameral Bone Cyst
      9. Aneurysmal Bone Cyst
      10. Nonossifying Fibroma
      11. Fibrous Dysplasia
      12. Osteofibrous Dysplasia
7. Differential Diagnosis
8. Prehospital Care
9. Emergency Department Evaluation
   1. History
   2. Physical Examination
10. Diagnostic Studies
    1. Other Studies
11. Treatment
12. Special Circumstances
13. Controversies and Cutting Edge
14. Disposition
15. Risk Management Pitfalls in the Emergency Department Diagnosis and Management of Pediatric Primary Bone Tumors
16. Summary
17. Time- and Cost-Effective Strategies
18. Case Conclusions
19. Clinical Pathway for Emergency Department Diagnosis and Management of Pediatric Primary Bone Tumors
20. Tables and Appendix
    1. Table 2. Summary of Radiographic Findings in Benign Versus Malignant Bone Tumors
    2. Table 1. Tumor Type by Location in Bone
    3. Appendix 1. Presentation and Radiographic Features by Tumor Type
21. References

Abstract

Musculoskeletal pain is a common chief complaint of children in the emergency department. Although nonspecific and typically benign, musculoskeletal pain should be investigated thoroughly with consideration for an underlying bone tumor, especially when it is a recurrent visit for pain. This issue reviews the specific signs, symptoms, and unique presentations the emergency clinician should know when evaluating a pediatric patient with musculoskeletal pain. Additionally, assessment of relevant radiographic findings to assist in differentiating bone tumors and guide further management are discussed.

Case Presentations

An 8-year-old previously healthy girl presents to the ED with pain in her left leg that is worse at night...

• She is a gymnast and has been practicing more intensely for an upcoming competition, but she denies any significant falls or other trauma to her leg. She had a cold last week and had fevers at that time, but otherwise denies new fevers, swelling, weight loss, night sweats, or pain in her other joints. The pain is not waking her from sleep, and she has only needed to take ibuprofen occasionally for the pain.
• On examination, she is afebrile and all of her vital signs are within normal limits. She localizes the pain to the proximal third of her tibia, and there is mild tenderness to palpation over this area. There is no erythema, swelling, or other skin changes overlying this area, and she has full range of motion of all of her joints in the lower extremity, with a normal gait.
• You wonder whether an x-ray and laboratory studies are truly indicated, as her history and physical examination appear relatively benign...

A 13-year-old girl with a history of asthma presents to the ED with a painful, swollen lesion on the anterior chest wall…

• She first pointed this out to her mother a few days ago, and they did not think much of it. Since that time, it appears to have enlarged significantly. The patient is now uncomfortable, and she is sometimes short of breath when she lays flat. The girl denies fevers, weight loss, or night sweats. Her mom says that the girl had complained of chest pain several times a few months ago, but those instances occurred during upper respiratory infections and asthma exacerbations.
• On examination, the girl is afebrile. Her vital signs are: heart rate, 133 beats/min; blood pressure, 119/75 mm Hg; respiratory rate, 38 breaths/min; and oxygen saturation, 98% on room air. She appears uncomfortable but nontoxic, prefers to be sitting up, and has a quarter-sized erythematous swollen lesion on her right anterior chest wall that is tender to very soft palpation, with no obvious crepitus or surrounding erythema.
• You wonder whether this presentation is infectious in etiology or potentially malignant. Regardless, you are concerned about her airway and breathing. Other than a chest x-ray and screening laboratory studies, you wonder what other workup would be immediately helpful and which consultants you should involve urgently.

Clinical Pathway for Emergency Department Diagnosis and Management of Pediatric Primary Bone Tumors

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Tables and Appendix

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Key References

Following are the most informative references cited in this paper, as determined by the authors.

1. * Gereige R, Kumar M. Bone lesions: benign and malignant. Pediatr Rev. 2010;31(9):355-362. (Review article) DOI: 10.1542/pir.31-9-355

2. * Vartevan A MC, Barnes CE. Pediatric bone imaging: differentiating benign lesions from malignant. Appl Radiol. 2018;47(7):8-15. (Review article)

3. SEER Cancer Statistics Review, 1975-2018. Accessed: June 15, 2021. Bethesda: National Cancer Institute. (Statistical report)

6. * McCarville MB. The child with bone pain: malignancies and mimickers. Cancer Imaging. 2009;9 Spec No A:S115-S121. (Review article) DOI: 10.1102/1470-7330.2009.9043

21. * Aboulafia AJ, Kennon RE, Jelinek JS. Benign bone tumors of childhood. J Am Acad Orthop Surg. 1999;7(6):377-388. (Review article) DOI: 10.5435/00124635-199911000-00004

Atesok KI, Alman BA, Schemitsch EH, et al. Osteoid osteoma and osteoblastoma. J Am Acad Orthop Surg. 2011;19(11):678-689. (Review article)

24. * Motamedi K, Seeger LL. Benign bone tumors. Radiol Clin North Am. 2011;49(6):1115-1134. (Review article) DOI: 10.1016/j.rcl.2011.07.002

26. * Copley L, Dormans JP. Benign pediatric bone tumors. Evaluation and treatment. Pediatr Clin North Am. 1996;43(4):949-966. (Review article) DOI: 10.1016/s0031-3955(05)70444-2

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Keywords: bone tumor, primary bone tumor, pediatric bone tumor, pediatric bone cancer, malignant, benign, osteosarcoma, Ewing sarcoma, Langerhans cell histiocytosis, osteochondroma, cartilaginous cap, osteoid osteoma, osteoblastoma, chondroblastoma, enchondroma, chondromyxoid fibroma, unicameral bone cyst, aneurysmal bone cyst, nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, musculoskeletal pain, Codman triangle, nocturnal pain, pathologic fracture