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Chondroblastoma

Chondroblastoma also known as Codman tumor, calcifying giant cell tumor or epiphyseal chondromatous giant cell tumor is a rare tumor occurring in young patients. It commonly arises in the epiphyseal region of long bones and usually involves a single bone. Although a majority of them are benign, few can become malignant and even metastasize.


Presentation

Chondroblastomas occur predominantly in the second and third decade of life and their incidence is higher amongst males [1]. They account for <1% of all primary bone tumors [1] and arise from the secondary ossification centers [2] at the epiphyseal ends of long bones like the distal and proximal femur, proximal tibia, and proximal humerus [3] [4]. They can also be found in flat bones like the acetabulum and ilium, or other sites such as the talus, calcaneus, patella [5], and the temporal bone [6].

Characteristic clinical presentation of chondroblastomas is often vague joint pain or localized tenderness, without a history of trauma. A palpable mass, joint effusion, muscle atrophy and decreased range of joint motion may also be present. Constitutional symptoms are usually absent. Fractures are rare but may occur when the tumor is large. Recurrence rates of chondroblastoma have been reported to be higher when they have a component of aneurysmal bone cyst [7]. Chondroblastomas can be localized or can extend into the surrounding soft tissues. Some chondroblastomas behave aggressively and can recur following excision or metastasize to distant organs, especially the lungs. Deaths following widespread metastasis of chondroblastoma have been reported [8] [9] [10].

Constitutional Symptom
  • A clinical presentation of constitutional symptoms along with a metaphyseodiaphyseal lesion on radiographs in a young child usually points toward a diagnosis of Ewing's sarcoma or infection.[ncbi.nlm.nih.gov]
  • Constitutional symptoms are usually absent. Fractures are rare but may occur when the tumor is large. Recurrence rates of chondroblastoma have been reported to be higher when they have a component of aneurysmal bone cyst.[symptoma.com]
  • A rare case of metaphyseodiaphyseal chondroblastoma of proximal femur presenting with constitutional symptoms in a six-year-old child. Journal of Pediatric Orthopaedics B. 2012;21(2):174-8.[pubs.sciepub.com]
Soft Tissue Swelling
  • tissue swelling, and joint effusion in the affected area.[en.wikipedia.org]
  • Soft tissue swelling, mass, or joint effusion is present in about 20% of cases. Chondroblastoma is usually well-circumscribed, round or oval lesion ( 3 ) on the radiograph.[jtd.amegroups.com]
  • There is soft tissue swelling with osteolysis of the bone adjacent to the cement. This indicates recurrent or residual disease.[radiologyassistant.nl]
Trismus
  • The patient presented swelling in the left temporomandibular joint and trismus. Based oncomputed tomography and magnetic resonance imaging findings, a provisional diagnosis of synovial chondromatosis was made.[ncbi.nlm.nih.gov]
  • There was no evidence of trismus.[yumpu.com]
Malocclusion
  • These patients had temporary malocclusion and restricted motion postoperatively, which resolved within 3 to 12 months with conservative treatment. All patients have no recurrence to date with a mean follow-up period of 9 years.[ncbi.nlm.nih.gov]
Cutaneous Manifestation
  • Cardio-facio-cutaneous syndrome (CFCS) is a rare genetic disorder characterized by craniofacial deformities and heterogeneous cardiac and cutaneous manifestations.[ncbi.nlm.nih.gov]
Hearing Impairment
  • A case report of a 38-year-old man who presented with the left-sided hearing impairment and temporal swelling was reviewed. A CT scan revealed an osteolytic lobulated expansile mass.[ncbi.nlm.nih.gov]
  • The common clinical manifestations reported are hearing impairment, tinnitus and ear stuffiness along with a swelling in the temporal region.[neurologyindia.com]
Ear Fullness
  • We report here the case of a 67-year-old woman who presented with right-sided mixed hearing loss, a right external auditory canal mass, ear fullness, otalgia, blood-stained otorrhea, and pain around the TMJ, associated with difficulty in opening the mouth[ncbi.nlm.nih.gov]
Hip Pain
  • We present the case of a 15-year-old patient with right hip pain which was first attributed to adductor tendinitis.[ncbi.nlm.nih.gov]
  • Diwanji SR, Cho SG, Kong IK, Yoon TR: Hip pain in a 24-year-old woman. Clin Orthop 461:262-8,2007. Dwaik M, Devlin PB: Case report: metadiaphyseal chondroblastoma. Clin Radiol 45:131-3,1992.[orthopaedicsone.com]
Bony Swelling
Vertigo
  • The tumor occurred in a 12-year-old girl and was revealed by left hypoacusis, vertigo, and walking instability.[ncbi.nlm.nih.gov]
  • Furthermore, tumoral involvement of the temporal bone may result in hearing loss, tinnitus, or vertigo. On physical examination, patients usually report tenderness in the affected area along with limitation of motion and muscular atrophy.[musculoskeletalkey.com]
  • Other symptoms include otalgia, seizures, vertigo and facial palsy. [4], [8], [13], [14], [15] In the Mayo Clinic series of chondroblastomas of the skull and the facial bones, the mean age of presentation was 43.5 years. [7] Our patient was in his thirties[neurologyindia.com]
Dizziness
  • In cases involving the temporal bone, tinnitus, dizziness, and hearing loss have been reported.In a publication by Turcotte et al. it was found that the average duration of symptoms for patients with chondroblastoma was about 20 months, ranging from 5[en.wikipedia.org]

Workup

The workup of chondroblastoma relies mainly on radiology and histopathology. On plain X-rays, chondroblastomas appear as circumscribed radiolucent lesions, with sclerotic rims and smooth or lobulated margins located eccentrically within the epiphysis of long bones, typically the humerus, femur or the tibia. Approximately 50% of the tumors have internal calcifications [1] [10]. A variable amount of joint effusion is noticed in about 30% of patients [11]. Computed tomograms (CT) are preferred to plain X-rays as they can demonstrate more accurately the relationship of the tumor to the epiphyseal and articular region, the extension of the tumor to the cortex, as well as the periosteal reaction, and the internal calcification [1] [12]. CT scan can also reveal endosteal scalloping [11].

Magnetic resonance imaging (MRI) is performed to detect transphyseal or transcortical extension, the status of the bone marrow and the extent of the soft tissue edema [1] [13].

On histopathology, the characteristic finding in a chondroblastoma is the chicken wire pattern of linear deposition of calcification around individual chondroblasts [3] [14]. The reaction of neoplastic cells to S-100 protein and vimentin can be demonstrated with immunohistochemical staining.

Supportive workup for surgical treatment and to detect metastasis include a complete blood count, metabolic panel, serum chemistries and plain radiography of the chest.

Differential diagnosis of chondroblastoma include other bone tumors like giant cell tumor, clear cell chondrosarcoma, chondromyxoid fibroma, eosinophilic granuloma, and chondroblastoma-like chondroma and these have to excluded on the basis of their radiological and histological characteristics.

Karyotype Abnormal
  • abnormalities, there were recurrent breakpoints seen at 2q35, 3q21-23, and 18q21. [8] Epidemiology In the United States, chondroblastoma accounts for approximately 1% of all bone tumors.[emedicine.com]

Treatment

  • Treatment consisted of aggressive curettage, phenolization, and bone allograft. The patient was able to resume normal activities after treatment, and there was no recurrence of the chondroblastoma during a follow-up of 3 years.[ncbi.nlm.nih.gov]

Prognosis

  • Thus, except for the distinctive site of the long bone diaphysis, which made diagnosis difficult, the patients' ages, symptoms, X-ray and CT images, treatment, and prognosis were in accordance with typical lesions in the epiphysis and metaphysis.[ncbi.nlm.nih.gov]
  • Although most of them are benign, the prognosis is not predictable. A 14-year-old girl presented with recurrent slowly growing mass over the right side of the temporo-parietal region of skull vault.[najms.org]
  • […] this highly lauded three-volume reference provides an interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation, pathology, differential diagnosis, and prognosis[books.google.com]
  • […] recurrence (up to 1/3) Most common locations for recurrence to occur are proximal femur and pelvis Pathologic fractures Very rare Malignant transformation Extremely rare Pulmonary metastases or local invasion are possible, even with “benign” tumors Prognosis[learningradiology.com]

Etiology

  • We discuss the diagnostic considerations and possible etiology of chondroblastoma given this unusual location.[ncbi.nlm.nih.gov]
  • Updated, reorganized, and revised throughout, this highly lauded three-volume reference provides an interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation[books.google.com]
  • The distal femoral and proximal tibial epiphyses are most frequently involved, followed by the proximal humerus, where approximately 18% of chondroblastomas appear. [6] Etiology No risk factors are known for chondroblastoma.[emedicine.com]

Epidemiology

  • Updated Molecular and Epidemiologic diagrams added to all new chapters.[books.google.it]
  • This study was designed to review the epidemiologic characteristics and outcomes of surgical management in a large series of patients with extremity chondroblastoma.[ncbi.nlm.nih.gov]
  • Epidemiology: Chondroblastomas typically occur in adolescents and young adults. According to the largest studies, the average age is 22 years old (Ramappa, 2000).[posna.org]
  • […] patients with chondroblastoma. [7] Sjögren et al performed cytogenetic analysis of benign and malignant cartilage tumors, and while they observed no consistent karyotypic abnormalities, there were recurrent breakpoints seen at 2q35, 3q21-23, and 18q21. [8] Epidemiology[emedicine.com]
  • Figures 1, 2 and 3: Typical radiological findings of a lytic eccentric lesion affecting the epyphysis of the humerus (1 RX, 2 NMR and 3 bone scan) Epidemiology Chondroblastoma is a rare neoplasm accounting for less than 1% of all bone tumors.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Various theories have been proposed concerning the pathogenesis of chondroblastomas.[emedicine.com]

Prevention

  • We report the case of a patient with chondroblastoma of the femoral head who was treated with wide curettage of the lesion and implantation of an osteochondral allograft to prevent femoral head collapse.[ncbi.nlm.nih.gov]
  • Treatment & Care Options Neurosurgery, aimed at removing the tumor and preventing damage to the end of the affected bone, is standard treatment for chondroblastoma.[danafarberbostonchildrens.org]
  • The goal for treatment of chondroblastoma is to remove the tumor and prevent damage to the end of the affected bone. Treatment may include: Surgical removal of the tumor Bone grafting.[hopkinsmedicine.org]

References

Article

  1. Erickson JK, Rosenthal DI, Zaleske DJ, et al. Primary treatment of chondroblastoma with percutaneous radiofrequency heat ablation: report of three cases. Radiology. 2001;221 (2): 463-8.
  2. Yang J, Tian W, Zhu X, Wang J. Chondroblastoma in the long bone diaphysis: a report of two cases with literature review. Chin J Cancer. 2012 May; 31(5): 257 -264
  3. Turcotte RE, Kurt AM, Sim FH, et al. Chondroblastoma. Hum Pathol. 1993;24:944–949.
  4. Bloem JL, Mulder JD. Chondroblastoma: a clinical and radiological study of 104 cases. Skeletal Radiol. 1985;14:1–9.
  5. Ghekiere J, Geusens E, Lateur L, et al. Chondroblastoma of the patella with a secondary aneurysmal bone cyst. Eur Radiol. 1998;8:992–995.
  6. Bertoni F, Unni KK, Beabout JW, et al. Chondroblastoma of the skull and facial bones. Am J Clin Pathol. 1987;88:1–9.
  7. Huvos AG, Marcove RC, Erlandson RA, et al. Chondroblastoma of bone. A clinicopathologic and electron microscopic study. Cancer. 1972;29:760–771.
  8. Ramappa AJ, Lee FY, Tang P, et al. Chondroblastoma of bone. J Bone Joint Surg Am. 2000;82-A:1140–1145.
  9. Jambhekar NA, Desai PB, Chitale DA, et al. Benign metastasizing chondroblastoma: a case report. Cancer. 1998;82:675–678
  10. Kricun ME, Kricun R, Haskin ME. Chondroblastoma of the calcaneus: radiographic features with emphasis on location. AJR Am J Roentgenol. 1977;128 (4): 613-6.
  11. Greenspan A, Remagen W. Differential diagnosis of tumors and tumor-like lesions of bones and joints. Lippincott Williams & Wilkins; 1998
  12. Brower AC, Moser RP, Kransdorf MJ. The frequency and diagnostic significance of periostitis in chondroblastoma. AJR Am J Roentgenol. 1990;154 (2): 309-14
  13. Weatherall PT, Maale GE, Mendelsohn DB, et.al. Chondroblastoma: classic and confusing appearance at MR imaging. Radiology. 1994;190 (2): 467-74.
  14. Kunze E, Graewe T, Peitsch E. Histology and biology of metastatic chondroblastoma. Report of a case with a review of the literature. Pathol Res Pract. 1987;182:113–123.

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Last updated: 2019-06-28 12:15