Adamantinoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2] [3] [4]
Synonyms and keywords:
Overview
Historical Perspective
Adamantinoma was first discovered by Fischer in 1913.[1]
The first reported example is attributed to Maier in 1900 [2]. In 1913,[3] [4] named the lesion "primary adamantinoma of the tibia" because of its striking histologic resemblance to the jaw "adamantinoma" (ameloblastoma). In 1951, Schulenberg [5] suggested a unifying histogenetic concept for the adamantinomas of the appendicular skeleton.
The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
There have been several outbreaks of [disease name], including -----.
In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].
Classification
There is no established system for the classification of [disease name].
Adamantinoma is classified into 2 distinct types: classic and differentiated (Osteofibrous dysplasia (OFD) - like)
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Classic | Differentiated | ||
|---|---|---|---|---|
| Age | >20 years | <20 years, Children | ||
| Radiology | Soft tissue or intramedullary involvement | Intra cortical | ||
| Histopathology | Both epithelial and osteofibrous component, solid nests of basaloid cells | OFD (Osteofibrous dysplasia) like pattern, Scattered positivity of epithelial elements for cytokeratin | ||
| Behavior | Aggressive | Relatively benign |
Pathophysiology
Adamantinoma is a low grade, malignant bone tumor. This tumor is predominnatly located in Tibia ( most in mid-portion of tibia). [4] It is a biphasic tumor including epithelial and osteofibrous components.[5].
Most locations of the tumor include:[6]
- Tibia ( 80 to 85 percent of cases)
- Ipsilateral fibula (10 to15% of cases)
- Humerus
- Ulna
- Femur
- Fibula
- Radius
- Ribs
- Spine
- Small bones of the hand and foot
Gross pathology: [7]
- Yellow gray or grayish white tumor
- Fleshy or firm in consistency
- Some OFD like adamantinomas are solid
- Macroscopic cysts containing blood like fluid, occasionally
Microscopic examanination: [8]
- Admixture of both epithelial and osteofibrous component, most commonly solid nests of basaloid cells in classic adamantinoma
- Nuclear atypia in a few cases
- Several patterns of growth including
- Tubular
- Basaloid
- Squamous
- Spindle-cell
- Osteofibrous dysplasia-like variant
- A few cases from a large series have exhibited nuclear atypia
- Foci of calcification, giant cells [9]
- Foci of xanthoma and spindle cells[10]
Immunohistochemistry:
- Positives for keratins 14 and 19[11]
Causes
Adamantinoma may be caused by: [12]
- Displacement of basal epithelium of skin during embrogenesis
- Traumatic implantation
- Synovial origin
Differentiating Adamantinoma from Other Diseases
Adamantinoma must be differentiated from aneurrysmal bone cyst, unicamaral bone cyst, fibrous dysplasia, chondromyxoid fibroma, eosinophilic granuloma, giant cell tumor, chondromyxoid fibroma, osteomyelitis, chondrosarcoma, epithelial metastasis, hemangioendothelioma, nonossifying fibromas, angiosarcoma.
Epidemiology and Demographics
Adamantinoma is a rare bone cnacer ( 0.1–0.5% of all primary bone tumors ).
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
OR
In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
OR
In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
Patients of all age groups may develop [disease name].
OR
The incidence of [disease name] increases with age; the median age at diagnosis is 25 to 35 years.
[Chronic disease name] is usually first diagnosed among [age group].
OR
[Acute disease name] commonly affects [age group].
There is no racial predilection to [disease name].
OR
[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
Men are more commonly affected by adamantinoma than women. The men to women ratio is approximately 5 to 4.[13]
The majority of [disease name] cases are reported in [geographical region].
OR
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
Risk Factors
Risk factors in the development of adamantinoma may include Benign osteofibrous dysplasia. It maybe a precursor of adamantinoma.[14]
Screening
There is insufficient evidence to recommend routine screening for adamantinoma.
Natural History, Complications, and Prognosis
If left untreated, 15-30% of patients with adamantinoma may metastasize to other parts of the body such as lungs or nearby lemph nodes. [15]. Adamantinoma has mortality rate of 13%to 18%. [16]
OR
Complications of adamantinoma include metastases
OR
Prognosis is generally good. Male patint, short duration of symtoms, young age ( less than 20 years) and lack of squamous differentiantion of tumor are related with unfavorable clinical outcome. [17]
Prognosis
Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained.
Diagnosis
Diagnostic Study of Choice
The diagnosis of adamantinoma is based on the findings of radiologic studies such as xray, CT and MRI.
History and Symptoms
The initial symptoms of adamantinoma are often nonspecific. The most common symptoms of adamantinoma include swelling with or without pain. Less common symptoms of adamantinoma include pathological fracture( 23% of patients) [18] and neurological symptoms in spinal lesions.[19]
Physical Examination
Adamantinoma may present with bowing deformity of the tibia.[20]
Spinal lesions may be manifested by neurologic symptoms in addition to pain [35].
Laboratory Findings
Paraneoplastic hypercalcemia can be seen in tibial adamantinoma and pulmonary metastasis[21]
Electrocardiogram
There are no ECG findings associated with adamantinoma.
X-ray
An x-ray may be helpful in the diagnosis of adamantinoma. Findings on an x-ray suggestive of adamantinoma include central or eccentric, multilocular lesion in tibia. The tumor is found in the diaphyseal location. Metaphyseal extention or only involvement of metaphysis is seen occationally. The lesions are well circumscribed surrounded ring-shaped densities ( soap -bubble appearance). The lesion are more intra-cortical, but if they destroy cortex, extracortical soft tisuue invasion can be seen. The periosteal reaction can be minimal to prominnet.[22]
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with adamantinoma.
CT scan
CT scan may be helpful in the diagnosis of adamantinoma. Findings on CT scan suggestive of adamantinoma include cortical involvement and the soft tissue extension if it exist.
Chest CT scan can detect pulmonary metastasis. [23]
MRI
MRI is helpful in the diagnosis of adamantinoma. It is useful in detection distant cortical foci, soft tissue, and intramedullary extension. MRI is a very important diagnostic study for stating of tumor and tumor-free margins. Findings on MRI suggestive of adamantinoma include a solitary lobulated focus or multiple small nodules in one or more foci. Tumors demonstrate low signal intensity on T1-weighted images and high signal on T2-weighted images.[24]
Other Imaging Findings
Bone scan may be helpful in the diagnosis of adamantinoma. Findings on a nuclear medicine suggestive of adamantioma include:[25]
- Increased blood flow in the region of the tumor
- Increased accumulation of technetium-99m methylene diphosphate in the area of the tumor
Other Diagnostic Studies
There are no other diagnostic studies associated with adamantinoma.
Treatment
Medical Therapy
There is no treatment for adamantinoma. Radition therapy and chemotherapy are not effective. [26]
Surgery
Surgery is the mainstay of treatment for adamantinoma.
Primary Prevention
There are no established measures for the primary prevention of adamantinoma.
Secondary Prevention
There are no established measures for the secondary prevention of adamantinoma.
References
- ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
- ↑ Van Rijn, Rick; Bras, Johannes; Schaap, Gerard; van den Berg, Henk; Maas, Mario (2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatric Radiology. 36 (10): 1068–1074. doi:10.1007/s00247-006-0272-5. ISSN 0301-0449.
- ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
- ↑ Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266.
- ↑ Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. doi:10.1007/s00247-006-0272-5. PMID 16906392.
- ↑ Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266.
- ↑ Unni KK, Dahlin DC, Beabout JW, Ivins JC (November 1974). "Adamantinomas of long bones". Cancer. 34 (5): 1796–805. PMID 4426036.
- ↑ Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266.
- ↑ Donner R, Dikland R (February 1966). "Adamantinoma of the tibia. A long-standing case with unusual histological features". J Bone Joint Surg Br. 48 (1): 138–44. PMID 5909059.
- ↑ Weiss SW, Dorfman HD (March 1977). "Adamantinoma of long bone. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes". Hum. Pathol. 8 (2): 141–53. PMID 852865.
- ↑ Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T (October 2000). "Expression of cytokeratin 1, 5, 14, 19 and transforming growth factors-beta1, beta2, beta3 in osteofibrous dysplasia and adamantinoma: A possible association of transforming growth factor-beta with basal cell phenotype promotion". Pathol. Int. 50 (10): 801–7. PMID 11107052.
- ↑ Ryrie BJ (December 1932). "ADAMANTINOMA OF THE TIBIA: AETIOLOGY AND PATHOGENESIS". Br Med J. 2 (3752): 1000–1020.1. PMC 2522231. PMID 20777206.
- ↑ Moon NF, Mori H (March 1986). "Adamantinoma of the appendicular skeleton--updated". Clin. Orthop. Relat. Res. (204): 215–37. PMID 3514033.
- ↑ Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S (May 2006). "A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature". Tohoku J. Exp. Med. 209 (1): 53–9. PMID 16636523.
- ↑ Moon NF, Mori H (March 1986). "Adamantinoma of the appendicular skeleton--updated". Clin. Orthop. Relat. Res. (204): 215–37. PMID 3514033.
- ↑ Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266.
- ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
- ↑ Qureshi AA, Shott S, Mallin BA, Gitelis S (August 2000). "Current trends in the management of adamantinoma of long bones. An international study". J Bone Joint Surg Am. 82-A (8): 1122–31. PMID 10954102.
- ↑ Dini LI, Mendonça R, Adamy CA, Saraiva GA (August 2006). "Adamantinoma of the spine: case report". Neurosurgery. 59 (2): E426, discussion E426. doi:10.1227/01.NEU.0000223497.06588.4A. PMID 16883154.
- ↑ Qureshi AA, Shott S, Mallin BA, Gitelis S (August 2000). "Current trends in the management of adamantinoma of long bones. An international study". J Bone Joint Surg Am. 82-A (8): 1122–31. PMID 10954102.
- ↑ Altmannsberger M, Poppe H, Schauer A (1982). "An unusual case of adamantinoma of long bones". J. Cancer Res. Clin. Oncol. 104 (3): 315–20. PMID 7161313.
- ↑ Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. doi:10.1007/s00247-006-0272-5. PMID 16906392.
- ↑ Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. doi:10.1007/s00247-006-0272-5. PMID 16906392.
- ↑ Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC (December 2004). "MRI of adamantinoma of long bones in correlation with histopathology". AJR Am J Roentgenol. 183 (6): 1737–44. doi:10.2214/ajr.183.6.01831737. PMID 15547221.
- ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
- ↑ Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T (October 2000). "Expression of cytokeratin 1, 5, 14, 19 and transforming growth factors-beta1, beta2, beta3 in osteofibrous dysplasia and adamantinoma: A possible association of transforming growth factor-beta with basal cell phenotype promotion". Pathol. Int. 50 (10): 801–7. PMID 11107052.
- ↑ Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266.
- ↑ Khan MH, Darji R, Rao U, McGough R (July 2006). "Leg pain and swelling in a 22-year-old man". Clin. Orthop. Relat. Res. 448: 259–66. doi:10.1097/01.blo.0000195924.36103.11. PMID 16826129.
| Adamantinoma | |
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| Micrograph of an adamantinoma showing the biphasic histomorphology. H&E stain.. |
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WikiDoc Resources for Adamantinoma |
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Articles |
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Most recent articles on Adamantinoma Most cited articles on Adamantinoma |
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Media |
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Powerpoint slides on Adamantinoma |
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Evidence Based Medicine |
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Clinical Trials |
|
Ongoing Trials on Adamantinoma at Clinical Trials.gov Clinical Trials on Adamantinoma at Google
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|
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US National Guidelines Clearinghouse on Adamantinoma
|
|
Books |
|
News |
|
Commentary |
|
Definitions |
|
Patient Resources / Community |
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Patient resources on Adamantinoma Discussion groups on Adamantinoma Patient Handouts on Adamantinoma Directions to Hospitals Treating Adamantinoma Risk calculators and risk factors for Adamantinoma
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|
Healthcare Provider Resources |
|
Causes & Risk Factors for Adamantinoma |
|
Continuing Medical Education (CME) |
|
International |
|
|
|
Business |
|
Experimental / Informatics |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [5]
Overview
Adamantinoma is a rare bone cancer, making up less than 1% of all bone cancers. It predominantly arises in bone in a subcutaneous location (85% are in the tibia). Most commonly, patients are in their second or third decade, but it can occur over a wide age range.
Historical Perspective
- The condition was first described by Fischer in 1913.[1]
- Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, however they differ in histology and frequency of malignancy. The typically benign odontogenic tumor known as ameloblastoma was first recognized in 1827 by Cusack but did not yet have any designation.[2] In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by [3]and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.[4][5]
Epidemiology and Demographics
Adamantinoma is a rare bone cancer, making up less than 1% of all bone cancers. Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.
Risk Factors
Benign osteofibrous dysplasia may be a precursor of adamantinoma[6][7] or a regressive phase of adamantinoma.[8]
Pathophysiology
Gross Pathology
The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones.
Microscopic Pathology
Islands of epithelial cells are found in a fibrous stroma.
Natural History, Complications, and Prognosis
Complications
Metastases are rare at presentation but may occur in up to 30% of patients during the disease course.
Prognosis
Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained.
History and Symptoms
Patients typically present with swelling in long bones with or without pain.
Physical Examination
Extremities
The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula).[9]
Diagnosis
X Ray
The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.[10]
Treatment
Treatment consists of wide resection or amputation.This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastized to the lungs or other organs.[10]
See also
- ameloblastoma
- osteofibrous dysplasia
References
- ↑ Fischer B. Uber ein primares Adamantinom der Tibia. 12. Frankfurt: Zeitschr. f. Path.; 1913:422-441.
- ↑ J.W. Cusack (1827). "Report of the amputations of the lower jaw". Dublin Hosp Rec. 4: 1–38.
- ↑ L. Malassez (1885). "Sur Le role des debris epitheliaux papdentaires". Arch Physiol Norm Pathol. 5: 309–340 6:379–449.
- ↑ R.H. Ivey, H.R. Churchill, (1930). "The need of a standardized surgical and pathological classification of tumors and anomalies of dental origin,". Am Assoc Dent Sch Trans. 7: 240–245.
- ↑ Madhup, R; Kirti, S; Bhatt, M; Srivastava, M; Sudhir, S; Srivastava, A (Jan 2006). "Giant ameloblastoma of jaw successfully treated by radiotherapy". Oral Oncology Extra. 42 (1): 22–25. doi:10.1016/j.ooe.2005.08.004.
- ↑ Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S (May 2006). "A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature". Tohoku J. Exp. Med. 209 (1): 53–9. doi:10.1620/tjem.209.53. PMID 16636523.
- ↑ Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER. (1994). "Relationship between osteofibrous dysplasia and adamantinoma". Clin Orthop Relat Res. (309): 234–44. PMID 7994967.
- ↑ Gleason, Briana C., Liegl-Atzwanger, Bernadette. "Osteofibrous Dysplasia and Adamantinoma in Children and Adolescents: A Clinicopathologic Reappraisal". American Journal of Surgical Pathology. 32 (3): 363–376. doi:10.1097/PAS.0b013e318150d53e.
- ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (2008). "Adamantinoma: A clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
- ↑ 10.0 10.1 Ernest U. Conrad (2008). Orthopaedic Oncology: Diagnosis and Treatment. Thieme. pp. 143–145.