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.

musculoskeletal
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]
  • The condition affects a wide age range of individuals; however, it is typically seen in children and young adults The tumors may be present as painless or painful masses and may cause pathological fractures in the affected bones.[dovemed.com]
  • 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.com]
Leg Pain
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]
  • more...
  • respiratoric
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  • Entire body system
    Pain
    • The swelling may also be there without the pain.[symptoma.com]
    • Differentiated Classic – grow beyond cortex, older patients, sometimes metastasize Differentiated – confined to cortex, earlier age, do not metastasize Clinical Presentation Signs/Symptoms: pain & Swelling, painless swelling, or pain alone 50% have history[tumorsurgery.org]
    • Depending on the pain.[csn.cancer.org]
    • In our case the pain had been evolving for eight months.[pubmedcentralcanada.ca]
    • While symptoms of an adamantinoma may from child-to-child, the most common include: pain (sharp or dull) at the site of the tumor swelling and/or redness at the site of the tumor increased pain with activity or lifting limping decreased movement of the[childrenshospital.org]
    Swelling
    • Differentiated Classic – grow beyond cortex, older patients, sometimes metastasize Differentiated – confined to cortex, earlier age, do not metastasize Clinical Presentation Signs/Symptoms: pain & Swelling, painless swelling, or pain alone 50% have history[tumorsurgery.org]
    • The swelling may also be there without the pain.[symptoma.com]
    • 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.[omicsonline.org]
    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]
    • Data of Demographics, Surgical Procedures and Results Case Age, y Gender (M/F) Time to Diagnosis, mo Type of Surgery Location Time to Recurrence, mo Location of Metastasis Time to Metastasis Follow-Up Dead or Alive Survival 1 21 F 12 WR ICA Rt. distal[shafaorthoj.com]
    Leg Swelling
    • He presented with slowly progressing leg swelling that evolved over 2 years without significant clinical manifestation.[pubmedcentralcanada.ca]
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  • Jaw & Teeth
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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
    • […] of bone tumors.[books.google.com]
    • Dahlin's Bone Tumors presents a succinct, profusely illustrated summary of the largest single collection of well-diagnosed bone tumors anywhere in the world.[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]
    • 抄録 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]
    Congenital Abnormality
    • Guides practicing radiologists through the complexities of various disorders, such as arthritis, collagen vascular diseases, bone tumors, soft tissue tumors, infections, systemic diseases, developmental and congenital abnormalities, and metabolic diseases[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]
    • Benign and malignant neoplasms are discussed on the basis of differentiation.[books.google.com]
    • High Quality Pathology Images of Benign and Malignant Neoplasms and Related Entities 8173 Images : Last Website Update : January 9, 2018 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
    • The section on neoplasm simulators has been expanded to include some conditions, such as neuropathic joint, that may present as a neoplasm.[books.google.com]
    • 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 8173 Images : Last Website Update : January 9, 2018 Follow us: Home About Us Acknowledgements Feedback Contact Us Site Map Advertisement Home Orthopedic Bone Tumors -[webpathology.com]
    • General Information Primary intraosseous epithelial neoplasm Low-grade malignancy and predilection for tibia Associated with intraosseous fibro-osseous proliferation (osteofibrous dysplasia) Classic vs.[tumorsurgery.org]
    • Metastases are rare at presentation but may occur in up to 30% of patients during the disease course. am·e·lo·blas·to·ma ( am'el'ō-blas-tō'mă ) A benign odontogenic epithelial neoplasm; it behaves as a slowly growing expansile radiolucent tumor, occurs[medical-dictionary.thefreedictionary.com]

    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.

    Other symptoms

    Adamantinoma
    Aging
    • References [ edit ] Brazis PW, Miller NR, Lee AG, Holliday MJ (1995).[en.wikipedia.org]
    • Differentiated Classic – grow beyond cortex, older patients, sometimes metastasize Differentiated – confined to cortex, earlier age, do not metastasize Clinical Presentation Signs/Symptoms: pain & Swelling, painless swelling, or pain alone 50% have history[tumorsurgery.org]
    • Demographics: Age: 10 - 50 (50% are age 10 to 30) Sex: Males greater than or equal to females Site: 90% involve tibial diaphysis (some with anterior cortical involvement).[uwmsk.org]
    • (Age and Sex Distribution) Individuals between the ages of 3-72 years can develop Extragnathic Adamantinoma The condition is mainly seen in the 2nd and 3rd decades of life.[dovemed.com]
    Metastasis
    • Here, we present a case of adamantinoma of the rib with liver metastasis.[jstage.jst.go.jp]
    • In one case, inguinal lymph node metastasis occurred simultaneously with lung metastasis.[shafaorthoj.com]
    • After the diagnosis, there must be a complete and sufficient surgery to avoid a local recurrence or metastasis.[pubmedcentralcanada.ca]
    • The possible complications of Extragnathic Adamantinoma are as follows: Recurrence of the condition after treatment Tumor metastasis (spread of the tumor to other regions) can affect the regional lymph nodes and lungs; this tumor metastasis may ultimately[dovemed.com]
    • Adamantinoma of the tibia and fibula with pulmonary metastasis: an unusual presentation.[ijcasereportsandimages.com]
    Fibrous Dysplasia
    • Mesenchymal origin - composed of immature mesenchymal cells and spicules of dysplastic bone - closely resembles the pattern of fibrous dysplasia and its variant ossifying fibroma Adamantinoma is considered by some to be a malignant variant of fibrous[boneschool.com]
    • A differential diagnosis of aneurysmal bone cyst or Adamantinoma or fibrous dysplasia was made.[ijri.org]
    • Fibrous dysplasia of a rib Fibrous dysplasia (3) On the left images of a patient with fibrous dysplasia of the rib with remarkable expansion.[radiologyassistant.nl]
    • Answer 5: Fibrous dysplasia - again, neither the imaging nor the histology slide are consistent with fibrous dysplasia.[orthobullets.com]
    • G.: Fibrous dysplasia associated with adamantinoma of the long bones.[link.springer.com]
    Chemotherapy
    • As a low grade sarcoma, no chemotherapy is indicated.[orthobullets.com]
    • The patient has been well without adjuvant chemotherapy for 5 years after the operation.[jstage.jst.go.jp]
    • An adamantinoma does not typically respond to standard cancer therapies such as chemotherapy or radiation.[nicklauschildrens.org]
    • This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastasized to the lungs or other organs. [2] Prior name for ameloblastoma [ edit ] The typically benign odontogenic tumor known as ameloblastoma was first[en.wikipedia.org]
    • An adamantinoma doesn’t respond to some traditional cancer treatments, such as chemotherapy or radiation.[childrenshospital.org]
    Ameloblastoma
    • "Neuro-ophthalmologic Aspects of Ameloblastoma" .[en.wikipedia.org]
    • Synonyms Adamantinoma Mandibular Ameloblastoma Maxillary Ameloblastoma Odontogenic Tumor Disorder Subdivisions None General Discussion Ameloblastoma is a rare disorder of the jaw involving abnormal tissue growth.[webmd.com]
    • Compare: ameloblastoma . 2.[medical-dictionary.thefreedictionary.com]
    • […] adamantinoma in Medicine adamantinoma ad·a·man·ti·no·ma (ād'ə-mān'tə-nō'mə) n. pl. ad·a·man·ti·no·mas or ad·a·man·ti·no·ma·ta (-mə-tə) See ameloblastoma .[dictionary.com]
    • Note: It is described as resembling ameloblastoma , [2] but the resemblance isn't striking.[librepathology.org]
    Adolescence
    • "Osteofibrous Dysplasia and Adamantinoma in Children and Adolescents: A Clinicopathologic Reappraisal" .[en.wikipedia.org]
    • […] have symptoms for 5 or more years Epithelial component derives directly from mesenchymal tissue and gradually increases in amount, may undergo sarcomatoid transformation ( Am J Surg Pathol 2003;27:1530 ) Osteofibrous dysplasia-like form: children and adolescents[pathologyoutlines.com]
    • Zoom Discussion Adamantinoma is a rare primary low-grade malignant bone tumor, slightly prevailing in the male sex and affecting any age from adolescent to the elderly [ 2 Campanacci M.[em-consulte.com]
    • However, occurrence in adolescence and, rarely, in childhood has been reported.[boneandspine.com]
    • Osteofibrous dysplasia and adamantinoma in children and adolescents: a clinicopathologic reappraisal.[mdedge.com]
    Soap
    • […] females affected equally Age: range 3-72 years old 50% present in second or third decade Sites: Predilection for tibia (90%) favoring diaphyseal portion of bone Radiographic Presentation Sharply defined osteolytic defect (lobulated, multicystic, or “soap[tumorsurgery.org]
    • Definition Rare low-grade malignant tumour - cell of origin unknown Epidemiology Usually second or third decade of life Site 90% diaphysis of tibia Mandible X-ray Most common anterior cortex of tibia Soap-bubble appearance Eccentrically located - well[boneschool.com]
    • An adamantinoma has a "soap bubble" appearance in x-rays. ( Left ) This x-ray of the tibia taken from the side shows several smaller areas of tumor which is a sign of OFD. ( Right ) The typical "soap bubble" appearance of an adamantinoma is obvious in[orthoinfo.aaos.org]
    • The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles. [2] Diagnosis [ edit ] This section is empty.[en.wikipedia.org]
    • Factors associated with a more aggressive outcome include male sex, short duration of symptoms, pain, and inadequate initial treatment IMAGE FINDINGS Radiographic Findings Diaphyseal, well-circumscribed intracortical lytic foci showing characteristic “soap-bubble[basicmedicalkey.com]
    Radiation Therapy
    • See full list of 9 symptoms of Adamantinoma Treatments for Adamantinoma Surgical approach is the treatment of choice Neither radiation therapy nor chemotherapy has been proven effective more treatments...»[rightdiagnosis.com]
    • Unfortunately, neither radiation therapy nor chemotherapy has been proven effective in the treatment of this insidious tumor.[boneandspine.com]
    • Treatment: Treatment of adamantinoma is wide surgical excision - neither radiation therapy or chemotherapy is effective.[uwmsk.org]
    • Unfortunately, neither radiation therapy nor chemotherapy has been proven effective in the treatment of this insidious tumor. [5, 2 , 6, 3 ] Preferred examination Plain radiography, computed tomography (CT) scanning, and magnetic resonance imaging (MRI[emedicine.medscape.com]
    • The tumor also may respond to radiation therapy.[radiologyassistant.nl]
    Pulmonary Metastasis
    • Other rare cases show paraneoplastic hypercalcemia (linked with tibial adamantinoma) and pulmonary metastasis .[symptoma.com]
    • Six patients died because of this disease, in all of which pulmonary metastasis occurred.[shafaorthoj.com]
    • Adamantinoma of the tibia and fibula with pulmonary metastasis: an unusual presentation.[ijcasereportsandimages.com]
    • Pulmonary metastasis of a tibial adamantinoma.[link.springer.com]
    • Tibial adamantinoma with local recurrence and pulmonary metastasis: report of a case with histocytologic findings.[orthopaedicsone.com]
    Very Rare
    Bone Dysplasia
    • Bone dysplasia can affect young patients is marked by recurrence and metastasis, Cytokeratin research through imunohisto chemistry must be the rule.[pubmedcentralcanada.ca]
    Tumoral Calcinosis
    • CALCINOSIS 1257 100 PSEUDOTUMORAL SOFT TISSUE AND PERIOSTEAL OSSIFICATIONS 1265 NORAS LESION 1280 SUBUNGUAL EXOXTOSIS 1281 HYPERPLASTIC BONE CALLUS IN NEUROPATHY 1282 MYOPERIOSTITIS OSSIFICANS ASSOCIATED WITH CONGENITAL DISORDERS 1284 101 PIGMENTED VILLONODULAR[books.google.com]

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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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