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    Adamantinoma (Extragnathic Adamantinoma)

    Adamantinoma - intermed mag[1]

    Adamantinomas are rare, slow growing malignant bone tumors, which occurs almost exclusively in the long bones.

    Presentation

    One of the most common traits of this tumor is its predilection for the long bones. It occurs here in 97% of cases. On the long bones, it focuses on the tibia in 80% -85% of cases. It is therefore its most characteristic clinical feature [5]. In 10% to 15% of cases, the lesion is seen in the ipsilateral fibula. Statistically, this tumor remains prevalent in the tibia but other limb bones have been involved. The involvement of several short bones has been reported on rare occasions as well. In other of decreasing frequency, some other bones that have been involved at appoint or the other includes:

    • The humerus
    • The ulna
    • The femur
    • The fibula
    • The radius
    • The innominate bones
    • The ribs
    • The spine
    • The small bones of the hand and foot


    Only six case of spinal adamantinoma have been reported till date [6]. There have also been rare reports of adamantinomas arising exclusively from the pretibal soft tissues without any bony involvement. The initial symptoms of adamantinomas are usually indolent and non-specific.

    They are also dependent on the location and extent of the disease. The onset of the disease is insidious and the course shows a slow progressive character.

    In many cases the patients endure years of pain before seeking medical attention. In 60% of reported cases, trauma is common place. Apart from the pain reported, many patients also show swelling. The swelling may also be there without the pain.

    Due to the involvement of the anterior tibial surface, patients may present bowing deformity of the fibia [7]. In as many as 23% of patients, pathological fracture was present. Other rare cases show paraneoplastic hypercalcemia (linked with tibial adamantinoma) and pulmonary metastasis. In addition to the pain spinal lesions may be manifested by neurologic symptoms.

    Jaw & Teeth
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  • Entire body system
    Leg Swelling
    • He presented with slowly progressing leg swelling that evolved over 2 years without significant clinical manifestation.[pubmedcentralcanada.ca]
    Pain
    Surgical Procedure
    • Some of the tumors erode through the overlying cortex, but this is unusual in patients who have not undergone a prior surgical procedure.[musculoskeletalkey.com]
    Swelling
    • He presented with slowly progressing leg swelling that evolved over 2 years without significant clinical manifestation.[pubmedcentralcanada.ca]
    • Surrounding the lesion there was soft tissue swelling with displacement of the fat planes.[ijri.org]
    • Patients typically present with swelling with or without pain.[en.wikipedia.org]
    • On palpation there is localized hard swelling and tenderness of a varying degree.[boneandspine.com]
    • Symptoms of adamantinoma include pain, swelling, and redness over the tumor site, trouble moving the affected limb/bone and/or limping.[nicklauschildrens.org]
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  • Skin
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  • respiratoric
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  • gastrointestinal
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  • musculoskeletal
    Bone Disorder
    • The book details the pathologic and radiologic characteristics of all bone and joint diseases, including arthritis, metastatic bone disease, osteoporosis, trauma, osteomyelitis, developmental bone disorders, and tumor-like lesions.[books.google.de]
    Bone Pain
    • Symptoms of Adamantinoma Bone pain Localized swelling at tumor site Localized redness at tumor site Increased bone pain with activity Limping more symptoms...»[rightdiagnosis.com]
    Bony Swelling
    • Clinical examination revealed an irregular bony swelling with no ulceration or sinuses over the skin surface.[ijri.org]
    Fracture
    • […] primarily in young males between 10 - 30 years of age (most common after skeletal maturity); - pts present w/ firm, slowly enlarging mass that produces minimal disability; - on other occassions the presenting features will be pain, swelling or pathologic fracture[wheelessonline.com]
    • Bilateral stress fractures of the anterior part of the tibial cortex.[pubmedcentralcanada.ca]
    • This edition has new chapters on fracture callus; the growth plate and dwarfs; tissue/bone banking—bone grafts; and giant cell tumors and differential diagnosis of giant cell lesions.[books.google.de]
    • Answer 3: Bracing would be appropriate treatment for OFD in the setting of tibial bowing or pending pathologic fracture.[orthobullets.com]
    Leg Pain
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  • Workup

    Radiographs are used for confirmatory purposes and they show many lucent lesions that are sharply circumcised. There is often interspersed sclerotic bone in mid tibia. Some lesions also destroy cortex. Bowing of the tibia may be seen as well. The radiographic evolution of lesions often proves helpful in diagnosis as lesions continue to grow and erode through the cortex [8]. Unlike what is seen in most primary bine tumors, adamantinoma doesn’t show any periosteal reaction.

    Histological studies are carried out as well and they show fibrous and epithelial tissue in gland like pattern characteristically. Epithelial and mesenchymal cells will be visible too as well as nests of epithelial-like cells arranged in palisading or glandular pattern. A background of fibrous stroma is also common.

    Treatment

    Generally, treatment involves wide resection or amputation. During the course of the disease metastases may occur. This occurs in 30% of cases even though it is rare at presentation. Prognosis and response to treatment is great but the chances are lower when a wide surgical margin cannot be obtained.

    Chemotherapy does not work as the tumor is not sensitive to radiation [9]. The only time when chemotherapy is deployed is when the cancer has spread to the lungs or other sensitive organs.

    Prognosis

    In 25% of cases reported, adamantinomas metastasized into the lungs. This is why long-term followup is highly recommended [4]. With negative margin excision, recurrence is rarely recorded. Majority of children treated surgically for adamantinoma have very positive long term outlook. Their 10 year survival rate is placed around 85%.

    Complications

    Bone Tumor
    • Features comprehensive coverage of bone tumor pathology based on pathologic and clinical data on 11,500 benign and malignant bone tumors from patients treated at the MD Anderson Cancer Center.[books.google.com]
    • - I Adamantinoma Adamantinoma Tweets by @WebPathology Slide Index Brain Breast Head & Neck Mediastinum Peritoneum Genitourinary Lymph Node/Spleen Hematopathology Gynecologic Orthopedic Bone Tumors - I Bone Tumors - II Tumor-like Lesions of Bone Misc.[webpathology.com]
    • Adamantinoma is a rare bone tumor, most of which originates in the long bone.[jstage.jst.go.jp]
    • The cause of this bone tumor is unknown.[childrenshospital.org]
    • I would also like to thank the large international group of collaborating authors, who are also widely acknowledged for their specifc expertise in the area of bone tumors, for their outstanding contributions.[books.google.com]
    Edema
    Malignant Neoplasm
    • Diagnosis: Adamantinoma Salient Points: : Adamantinoma is a rare, low-grade malignant neoplasm, which occurs almost exclusively in two skeletal locations: the bones of the lower leg and the jaw.[njms2.umdnj.edu]
    • High Quality Pathology Images of Benign and Malignant Neoplasms and Related Entities 7958 Images : Last Website Update : September 23, 2017 Follow us: Home About Us Acknowledgements Feedback Contact Us Site Map Advertisement Home Orthopedic Bone Tumors[webpathology.com]
    • TERMINOLOGY Abbreviations Adamantinoma (ADA) Definitions Low-grade biphasic malignant neoplasm of bone, composed of epithelial and mesenchymal elements ETIOLOGY/PATHOGENESIS Unknown Controversial but likely epithelial cell origin May be related to displaced[basicmedicalkey.com]
    Neoplasm
    • Diagnosis: Adamantinoma Salient Points: : Adamantinoma is a rare, low-grade malignant neoplasm, which occurs almost exclusively in two skeletal locations: the bones of the lower leg and the jaw.[njms2.umdnj.edu]
    • In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by Malassez and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.[en.wikipedia.org]
    • High Quality Pathology Images of Benign and Malignant Neoplasms and Related Entities 7958 Images : Last Website Update : September 23, 2017 Follow us: Home About Us Acknowledgements Feedback Contact Us Site Map Advertisement Home Orthopedic Bone Tumors[webpathology.com]
    • Necmettin Erbakan University Meram Medicine Faculty Orthopaedics and Traumatology Department, Konya, Turkiye) Introduction: Adamantinoma is a primary low-grade, malignant bone tumor, of unknown histogenesis which is a rare neoplasm, comprise only 0.1–[ideas.repec.org]
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  • Etiology

    The etiology of the tumor is not known and this has added to the controversy surrounding its origin.

    Epidemiology

    The condition is very rare and so, less than 300 cases have been recorded in the past century [2]. Of the documented cases, it has been established that the condition occurs majorly in young adults who are 20-40 years of age.

    Sex distribution
    Age distribution

    Pathophysiology

    Adamantinomas are classified into two main types, the classic and differentiated. Classic adamantinomas is seen in patients already older than 20 years of age but differentiated adamantinomas happen in patients younger than 20. Additionally, these two classifications have distinctive radiographic and histologic differences.

    Adamantinoma patients often show varying signs and symptoms. Generally, they report swelling and pain. Since the tumor grows rather slowly, patients often describe discomforts that may have lasted for months or years. Even though it is difficult to figure out an accurate mortality statistics due to the rarity of the tumor, there is 10% 10-year survival rate [3]. When faced with this tumor, medical professionals carry out surgeries which may include either marginal or en bloc resection. Traditional therapy and chemotherapy have not proven effective regarding the effective treatment of the disorder.

    Prevention

    There are no proven ways to prevent this but regular exercise of the lower extremities and bone friendly nutrition is recommended.

    Summary

    Adamantinoma is a rare type of tumor and till date, the origin remains a controversial topic. The tumor was first described by Fischer in 1913 [1] and till date, less than 300 cases have been reported. The tumor occurs almost exclusively within the long bones and in 80% of cases, tumors occur in the tibia. The most commonly affected is the diaphyseal region.

    Patient Information

    Patients are advised to cooperate with medical personnel and accept any procedures recommended by them. Adamantinoma doesn’t respond to chemotherapy, radiation etc. So surgery is the treatment method in most cases [10]. The different types of surgery are:

    • Limb salvage surgery: This focused on helping preserve the limb by removing the tumor and some of the healthy tissue surrounding it.
    • Amputation: This will only be carried out if the tumor involves any major nerves or blood vessels.

    If the adamantinoma reoccurs, more surgery will be required.
    Healthy individuals are encouraged to immediately report any pain in the lower extremities that have lasted more than a week. This condition must not be confused with arthritis.

    Self-assessment

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    References

    1. Fischer B. Uber ein primares Adamantinom der Tibia. 12. Frankfurt: Zeitschr. f. Path.; 1913:422-441.
    2. Van Rijn R, Bras J, Schaap G, Van den Berg H, Maas M: Adamantinoma in childhood: report of six cases and review of the literature. Pediatr Radiol 2006, 36:1068-74.
    3. Mirra JM: Adamantinoma and fibrous dysplasia. In Bone tumors. 1st edition. Edited by Mirra JM. Lea & Febiger, Philadelphia; 1989:1203-1231.
    4. Fisher B: Primary adamantinoma of the tibia. Z Pathol 1913, 12:422-441.
    5. Schulenburg CA: Adamantinoma. Ann R Coll Surg Engl 1951, 8:329-353
    6. Ryrie BJ: Adamantinoma of the tibia: aetiology and pathogenesis. BMJ 1932, 2:1000-1003
    7. Baker PL, Dockerty MB, Coventry MB: Adamantinoma (so-called) of the long bones. J Bone Joint Surg Am 1954, 36-A:704-20.
    8. Yildiz C, Erler K, Atesalp AS, Basbozkurt M. Benign bone tumors in children. Curr Opin Pediatr 2003; 15:58.
    9. Wyers MR. Evaluation of pediatric bone lesions. Pediatr Radiol 2010; 40:468.
    10. Springfield DS, Gebhardt MC. Bone and soft tissue tumors. In: Lovell and Winter's Pediatric Orthopaedics, 6th, Morrissy RT, Weinstein SL (Eds), Lippincott Williams & Wilkins, Philadelphia 2006. p.493.
    11. Faivre L, Nivelon-Chevallier A, Kottler ML, et al. Mazabraud syndrome in two patients: clinical overlap with McCune-Albright syndrome. Am J Med Genet 2001; 99:132.



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    1. Adamantinoma - intermed mag, CC BY-SA 3.0

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