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ENCHONDROMA

Introduction

Enchondroma is the most common bone tumor of the hand and wrist. Enchondromas are benign but have histological characteristics that are similar to malignant lesions, making differential diagnosis challenging. Thus, an accurate diagnostic workup is essential. Enchondromas are thought to be displaced epiphyseal cartilage fragments from incomplete endochondronal ossification that end up in the metaphysis during growth. Enchondromas are the most common bone tumor in the hand with half of these tumors occurring in the hand.3-5

Incidence and Related Conditions

  • Data from the Bone Tumour Registry Hamburg, Germany (1976-2006)1:
    • Mean (SD) age was 36 (19) years; peak prevalence in 40s and 50s; age range 3-74
    • 11% of tumors were malignant
    • 70% of tumors were phalangeal; 25% were metacarpal; 6% were carpal
    • 69% of all tumors were cartilage tumors; encondromas accounted for 47% of the cartilage type
  • Ollier’s disease: hereditary form of multiple enchondromas, increased risk for malignancy
  • Maffucci’s syndrome: extremely rare, non-hereditary multiple encondromas associated with hemangiomata, increased risk for malignancy

Differential Diagnosis

  • Chondrosarcoma
  • Giant cell tumor of bone
  • Fibrous dysplasia
  • Unicameral bone cyst
ICD-10 Codes

  • ENCHONDROMA

    Diagnostic Guide Name

    ENCHONDROMA

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    ENCHONDROMA D16.12D16.11 

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Enchondroma
  • Enchondroma - ring finger with no obvious change in but tender over middle phalanx
    Enchondroma - ring finger with no obvious change in but tender over middle phalanx
  • Enchondroma - note slight dorsal swelling at arrow
    Enchondroma - note slight dorsal swelling at arrow
  • Enchondroma - student with writer's cramp at exams and tender at arrow
    Enchondroma - student with writer's cramp at exams and tender at arrow
  • Distal Phalanx Enchondroma with cortical thinning and distal phalanx expansion
    Distal Phalanx Enchondroma with cortical thinning and distal phalanx expansion
Symptoms
Pain
Fracture from trivial trauma, i.e. pathologic fracture
Deformity with or without pain
Typical History

Enchondromas usually present as incidental findings on routine X-rays, as pathologic fractures, or occasionally as painful areas in the finger or hand.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
Enchondroma
  • Enchondroma ring finger middle phalanx AP view. Note the cortical thinning and expansion.
    Enchondroma ring finger middle phalanx AP view. Note the cortical thinning and expansion.
  • Enchondroma ring finger middle phalanx lateral view. Note well circumscribed lytic lesion with a thin expanded intact cortex. Lesion contains punctuated scalped cartilage calcifications.
    Enchondroma ring finger middle phalanx lateral view. Note well circumscribed lytic lesion with a thin expanded intact cortex. Lesion contains punctuated scalped cartilage calcifications.
  • Enchondroma ring finger proximal phalanx AP view. Note well circumscribed lytic lesion with a thin expanded intact cortex. Lesion contains punctuated scalped cartilage calcifications.
    Enchondroma ring finger proximal phalanx AP view. Note well circumscribed lytic lesion with a thin expanded intact cortex. Lesion contains punctuated scalped cartilage calcifications.
  • Enchondroma with cortical thinning and index metacarpal head expansion
    Enchondroma with cortical thinning and index metacarpal head expansion
Treatment Options
Treatment Goals

Make a proper clinical and pathologic diagnosis

Provide proper treatment of any pathologic fracture

Provide proper treatment of the enchondroma

Remember the current treatment controversies:

  1. The small asymptomatic enchondroma treatment options
  2. Pathologic diagnostic problems with distinguishing enchondroma and low-grade chondrosarcoma
  3. The use or nonuse of surgical adjuncts after curettage
  4. Pathologic fracture fixation & immediate grafting vs fracture healing followed by delayed grafting
  5. Void management post curettage of the enchondroma – leave empty, autograft or bone substitute
Conservative
  • No symptoms: no treatment
  • Can heal during fracture healing occasionally
Operative
  • Symptoms: surgical curettage traditionally with autograft or bony substitute grafting. Recent series have shown no outcome differences between those who had curettage and bone graft and those who had simple curettage with no grafting.4,5-9
Treatment Photos and Diagrams
Enchondroma
  • Extensor tendon retracted. Small k-wire used as a drill to outline
    Extensor tendon retracted. Small k-wire used as a drill to outline
  • Note the thinned cortex with tiny dots of cartilage seen through the thinned cortex.
    Note the thinned cortex with tiny dots of cartilage seen through the thinned cortex.
  • Enchondroma with 90% of the cartilage removed with an ear curette. Very thorough cartilage removal indicated. Removed cartilage looks like tapioca pudding.
    Enchondroma with 90% of the cartilage removed with an ear curette. Very thorough cartilage removal indicated. Removed cartilage looks like tapioca pudding.
  • Enchondroma after curettage and grafting with allograft
    Enchondroma after curettage and grafting with allograft
CPT Codes for Treatment Options

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Common Procedure Name
Curettage and bone graft enchondroma metacarpal with graft
CPT Description
Excision or curettage of bone cyst or benign tumor of metacarpal with autograft (includes obtaining graft)
CPT Code Number
26205
Common Procedure Name
Curettage and bone graft enchondroma proximal, middle or distal phalanx with graft
CPT Description
Excision bone cyst benign phalanx with graft
CPT Code Number
26215
CPT Code References

The American Medical Association (AMA) and Hand Surgery Resource, LLC have entered into a royalty free agreement which allows Hand Surgery Resource to provide our users with 75 commonly used hand surgery related CPT Codes for educational promises. For procedures associated with this Diagnostic Guide the CPT Codes are provided above. Reference materials for these codes is provided below. If the CPT Codes for the for the procedures associated with this Diagnostic Guide are not listed, then Hand Surgery Resource recommends using the references below to identify the proper CPT Codes.

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CPT 2021 Professional Edition: Spiralbound

Complications
  • Recurrence, malignant transformation
  • Loss of motion
  • Pathologic fracture in patients treated nonoperatively
  • Surgical complications can include adhesions, neuroma and infection4
Outcomes
  • Excellent with complete surgical excision
YouTube Video
Enchondroma and the Pathologic Fracture
Key Educational Points
  • Most common primary bone tumor of the hand
  • When diagnosing enchondroma versus chondrosarcoma, the clinical presentation and X-ray appearance are more important than the histologic findings
  • Patients with Ollier's disease (multiple enchondromatosis) have higher incidence of malignant sarcoma, possibly as high as 30%
  • Patients with Maffucci's syndrome (multiple enchondromas with multiple hemangiomas) have an even higher chance of malignant transformation (reported as high as 100%)
  • Recurrence higher in patients with multiple enchondromas
  • Surgical results appear similar with allograft, autograft and curettage alone
References

New Articles

  1. Simon MJ, Pogoda P, Hövelborn F, et al. Incidence, histopathologic analysis and distribution of tumours of the hand. BMC Musculoskelet Disord 2014;15:182. PMID: 24885007
  2. Cha SM, Shin HD, Kim KC, Park IY. Extensive curettage using a high-speed burr versus dehydrated alcohol instillation for the treatment of enchondroma of the hand. J Hand Surg Eur 2013 ePub. PMID: 24369364
  3. Schaller P, Baer W. Operative treatment of enchondromas of the hand: is cancellous bone grafting necessary? Scand J Plast Reconstr Surg Hand Surg 2009;43(5):279-85. PMID: 19863432
  4. Sassoon AA, Fitz-Gibbon PD, Harmsen WS, Moran SL.  Enchondroma of the Hand: Factors Affecting Recurrence, Healing, Motion, and Malignant Transformation.  J Hand Surg 2012:37A:1229-1234. PMID: 22542061

  5. Demers C, Hamdy CR, Corsi K, Chellat F, Tabrizian Yahia L. Natural coral exoskeleton as a bone graft substitute: a review. Biomed Mater Eng 2002;12(1):15-35.  PMID: 11847406.

  6. Lin SY, Huang PJ, Huang HT, Chen CH, Cheng YM, Fu YC. An alternative technique for the management of phalangeal enchondromas with pathologic fractures. J Hand Surg January 2012;38A:104-109.  PMID: 23200218

  7. Zhang L, Yang M, Mayer T, Johnstone B, Les C, Frisch N, Parsons T, Mi QA, Gibson G. Use of microRNA biomarkers to distinguish enchondroma from low-grade chondrosarcoma. Connect Tissue Res 2016:1-7.  PMID: 27267924

  8. Zheng H, Liu J, Dai X, Schilling AF. Modified technique for one-stage treatment of proximal phalangeal enchondromas with pathologic fractures. J Hand Surg September 2014;39A:1757-1760.  PMID: 25106766

  9. Heaton D, Alexander H, Trumble T. Missed pathologic fracture from multiple myeloma. J Hand Surg July 2015;40A;1501-1503.  PMID: 26043801

Reviews

  1. Herget GW, Strohm P, Rottenburger C, et al. Insights into Enchondroma, Enchondromatosis and the risk of secondary Chondrosarcoma. Review of the literature with an emphasis on the clinical behaviour, radiology, malignant transformation and the follow up. Neoplasma 2014;61(4):365-78. PMID: 24645839
  2. Larbi A, Viala P, Omoumi P, et al. Cartilaginous tumours and calcified lesions of the hand: a pictorial review. Diagn Interv Imaging 2013;94(4):395-409. PMID: 23478067

  3. Goddu LAO. Enchondroma – A review of the literature of this subject with report of three cases operated upon by the author. Boston Med Surg J March 18, 1915; 172:402-406.  DOI: 10.1056/NEJM191503181721103.

  4. Lubahn JD, Bachoura A. Enchondroma of the hand: evaluation and management. J Am Acad Orthop Surg 2016;24:625-633.  PMID: 27454024

  5. Sassoon AA, Fitz-Gibbons PD, Harmsen WS, Moran SL: Enchondromas of the hand: factors affecting recurrence, healing, motion, and malignant transformation.  J Hand Surg June 2012;37A:1229-1234. PMID: 22542061

Classics

  1. Milgram JW. The origins of osteochondromas and enchondromas. A histopathologic study. Clin Orthop Relat Res 1983;(174):264-84. PMID: 6600991
  2. Eiken O, Carstam N. Enchondroma in the hand. Acta Chir Scand Suppl 1966;357:148-53. PMID: 5335617
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