Adamantinoma

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Evidence Based Medicine
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Clinical Trials
Ongoing Trials on Adamantinoma at Clinical Trials.gov Trial results on Adamantinoma Clinical Trials on Adamantinoma at Google
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US National Guidelines Clearinghouse on Adamantinoma NICE Guidance on Adamantinoma NHS PRODIGY Guidance FDA on Adamantinoma CDC on Adamantinoma
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Definitions of Adamantinoma
Patient Resources / Community
Patient resources on Adamantinoma Discussion groups on Adamantinoma Patient Handouts on Adamantinoma Directions to Hospitals Treating Adamantinoma Risk calculators and risk factors for Adamantinoma
Healthcare Provider Resources
Symptoms of Adamantinoma Causes & Risk Factors for Adamantinoma Diagnostic studies for Adamantinoma Treatment of Adamantinoma
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International
Adamantinoma en Espanol Adamantinoma en Francais
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List of terms related to Adamantinoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: .

Synonyms and keywords:

Historical Perspective

Adamantinoma was first discovered by Fischer in 1913.

The first reported example is attributed to Maier in 1900 . In 1913, [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)

Features	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

A

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

OR

[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Pathophysiology

Adamantinoma is a low grade, malignant bone tumor. This tumor is predominnatly located in Tibia ( most in mid-portion of tibia). It is a biphasic tumor including epithelial and osteofibrous components..

Most locations of the tumor include:

• 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

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR

[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Causes

The etiology of the tumor is still a matter of debate.

Disease name] may be caused by [cause1], [cause2], or [cause3].

OR

Common causes of [disease] include [cause1], [cause2], and [cause3].

OR

The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

OR

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating ((Page name)) from Other Diseases

[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].

OR

[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].

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.

OR

[Disease name] commonly affects individuals younger than/older than [number of years] years of age.

OR

[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].

[Disease name] affects men and women equally.

OR

Men are more commonly affected by adamantinoma than women. The men to women ratio is approximately 5 to 4.

damantinoma mostly occurs in the second to fifth decade. The median patient age is 25 to 35 years, with a range from 2 years to 86 years. It is slightly more common in men than women, with a ratio of 5:4

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

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for [disease/malignancy].

OR

According to the [guideline name], screening for [disease name] is not recommended.

OR

According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

To assure the histological diagnosis, pathologists should employ immunohistochemistry for demonstrating the sometimes sparse epithelial cell nests when the radiological features are suggestive of adamantinoma.

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

he initial symptoms of adamantinoma are often indolent and nonspecific and depend on location and extent of the disease. The onset is insidious and its course shows a slow, progressive character. The patient often tolerates symptoms for many years before seeking medical attention. A history of significant trauma has been noted in about 60% of 200 cases reviewed by Moon and Mori [25]. Most patients present with swelling with or without pain. The patient may presents with bowing deformity of the tibia due to involvement of anterior tibial surface. Pathological fracture may be present in as many as 23% of the patients [39]. There have been two case reports of paraneoplastic hypercalcemia associated with tibial adamantinoma and pulmonary metastasis [40,41]. Spinal lesions may be manifested by neurologic symptoms in addition to pain [35].

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) and neurological symptoms in spinal lesions.

T

OR

The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Adamantinoma may present with bowing deformity of the tibia.

Spinal lesions may be manifested by neurologic symptoms in addition to pain [35].

Radiology

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

OR

Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

Template:WikiDoc Sources

Adamantinoma
Micrograph of an adamantinoma showing the biphasic histomorphology. H&E stain..

WikiDoc Resources for Adamantinoma
Articles
Most recent articles on Adamantinoma Most cited articles on Adamantinoma Review articles on Adamantinoma Articles on Adamantinoma in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Adamantinoma Images of Adamantinoma Photos of Adamantinoma Podcasts & MP3s on Adamantinoma Videos on Adamantinoma
Evidence Based Medicine
Cochrane Collaboration on Adamantinoma Bandolier on Adamantinoma TRIP on Adamantinoma
Clinical Trials
Ongoing Trials on Adamantinoma at Clinical Trials.gov Trial results on Adamantinoma Clinical Trials on Adamantinoma at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Adamantinoma NICE Guidance on Adamantinoma NHS PRODIGY Guidance FDA on Adamantinoma CDC on Adamantinoma
Books
Books on Adamantinoma
News
Adamantinoma in the news Be alerted to news on Adamantinoma News trends on Adamantinoma
Commentary
Blogs on Adamantinoma
Definitions
Definitions of Adamantinoma
Patient Resources / Community
Patient resources on Adamantinoma Discussion groups on Adamantinoma Patient Handouts on Adamantinoma Directions to Hospitals Treating Adamantinoma Risk calculators and risk factors for Adamantinoma
Healthcare Provider Resources
Symptoms of Adamantinoma Causes & Risk Factors for Adamantinoma Diagnostic studies for Adamantinoma Treatment of Adamantinoma
Continuing Medical Education (CME)
CME Programs on Adamantinoma
International
Adamantinoma en Espanol Adamantinoma en Francais
Business
Adamantinoma in the Marketplace Patents on Adamantinoma
Experimental / Informatics
List of terms related to Adamantinoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

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.
• 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. In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.

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 or a regressive phase of adamantinoma.

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).

Diagnosis

X Ray

The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.

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.

See also

• ameloblastoma
• osteofibrous dysplasia