| Adamantinoma | |
|---|---|
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| Micrograph of an adamantinoma showing the biphasic histomorphology. H&E stain.. | |
| Specialty | Oncology, rheumatology  |
Adamantinoma (from the Greek word adamantinos, meaning "very hard" [1] ) is a rare bone cancer, making up less than 1% of all bone cancers. [2] It almost always occurs in the bones of the lower leg [3] and involves both epithelial and osteofibrous tissue. [4]
The condition was first described by Fischer in 1913. [5] [6]
Patients typically present with swelling with or without pain. The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula). [3] Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.[ citation needed ]
Benign osteofibrous dysplasia may be a precursor of adamantinoma [4] [7] or a regressive phase of adamantinoma. [8]
Histologically, islands of epithelial cells are found in a fibrous stroma. The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles. [2]
Diagnosis is on plain radiography, or CT scan[ citation needed ]
Treatment consists of wide resection or amputation. Metastases are rare at presentation but may occur in up to 30% of patients during the disease course. Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained. This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastasized to the lungs or other organs. [2]
The typically benign odontogenic tumor known as ameloblastoma was first recognized in 1827 by Cusack. Still, it did not yet have any designation. In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by Malassez. [9] It was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill. [10] [11] Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas. However, they differ in histology and frequency of malignancy.[ citation needed ]
A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. There may be a lump, pain, or neurological signs from pressure. A bone tumor might present with a pathologic fracture. Other symptoms may include fatigue, fever, weight loss, anemia and nausea. Sometimes there are no symptoms and the tumour is found when investigating another problem.
An osteosarcoma (OS) or osteogenic sarcoma (OGS) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin and that exhibits osteoblastic differentiation and produces malignant osteoid.
A benign tumor is a mass of cells (tumor) that does not invade neighboring tissue or metastasize. Compared to malignant (cancerous) tumors, benign tumors generally have a slower growth rate. Benign tumors have relatively well differentiated cells. They are often surrounded by an outer surface or stay contained within the epithelium. Common examples of benign tumors include moles and uterine fibroids.
Fibroadenomas are benign breast tumours characterized by an admixture of stromal and epithelial tissue. Breasts are made of lobules and ducts. These are surrounded by glandular, fibrous and fatty tissues. Fibroadenomas develop from the lobules. The glandular tissue and ducts grow over the lobule to form a solid lump.
Ameloblastoma is a rare, benign or cancerous tumor of odontogenic epithelium much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusack. This type of odontogenic neoplasm was designated as an adamantinoma in 1885 by the French physician Louis-Charles Malassez. It was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.
Phyllodes tumors, are a rare type of biphasic fibroepithelial mass that form from the periductal stromal and epithelial cells of the breast. They account for less than 1% of all breast neoplasms. They were previously termed cystosarcoma phyllodes, coined by Johannes Muller in 1838, before being renamed to phyllodes tumor by the World Health Organization in 2003. Phullon, which means "leaf" in Greek, describes the unique papillary projections characteristic of phyllodes tumors on histology. Diagnosis is made via a core-needle biopsy and treatment is typically surgical resection with wide margins (>1 cm), due to their propensity to recur.
A craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue that occurs most commonly in children, but also affects adults. It may present at any age, even in the prenatal and neonatal periods, but peak incidence rates are childhood-onset at 5–14 years and adult-onset at 50–74 years. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm. It has a point prevalence around two per 1,000,000. Craniopharyngiomas are distinct from Rathke's cleft tumours and intrasellar arachnoid cysts.
Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.
Canalicular adenoma is a benign, epithelial salivary gland neoplasm arranged in interconnecting cords of columnar cells. This is a very rare benign neoplasm, that makes up about 1% of all salivary gland tumors, or about 4% of all benign salivary gland tumors.
A schwannoma is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves.
A dentigerous cyst, also known as a follicular cyst, is an epithelial-lined developmental cyst formed by accumulation of fluid between the reduced enamel epithelium and the crown of an unerupted tooth. It is formed when there is an alteration in the reduced enamel epithelium and encloses the crown of an unerupted tooth at the cemento-enamel junction. Fluid is accumulated between reduced enamel epithelium and the crown of an unerupted tooth.
An odontogenic keratocyst is a rare and benign but locally aggressive developmental cyst. It most often affects the posterior mandible and most commonly presents in the third decade of life. Odontogenic keratocysts make up around 19% of jaw cysts.
Calcifying odotogenic cyst (COC) is a rare developmental lesion that comes from odontogenic epithelium. It is also known as a calcifying cystic odontogenic tumor, which is a proliferation of odontogenic epithelium and scattered nest of ghost cells and calcifications that may form the lining of a cyst, or present as a solid mass.
An ameloblastic fibroma is a fibroma of the ameloblastic tissue, that is, an odontogenic tumor arising from the enamel organ or dental lamina. It may be either truly neoplastic or merely hamartomatous. In neoplastic cases, it may be labeled an ameloblastic fibrosarcoma in accord with the terminological distinction that reserves the word fibroma for benign tumors and assigns the word fibrosarcoma to malignant ones. It is more common in the first and second decades of life, when odontogenesis is ongoing, than in later decades. In 50% of cases an unerupted tooth is involved.
The calcifying epithelial odontogenic tumor (CEOT), also known as a Pindborg tumor, is an odontogenic tumor first recognized by the Danish pathologist Jens Jørgen Pindborg in 1955. It was previously described as an adenoid adamantoblastoma, unusual ameloblastoma and a cystic odontoma. Like other odontogenic neoplasms, it is thought to arise from the epithelial element of the enamel origin. It is a typically benign and slow growing, but invasive neoplasm.
Thymic carcinoma, or type C thymoma, is a malignancy of the thymus. It is a rare cancer that is often diagnosed at advanced stages. Recurrence following treatment is common, and thymic carcinoma is associated with a poor prognosis.
Cementoma is an odontogenic tumor of cementum. It is usually observed as a benign spherical mass of hard tissue fused to the root of a tooth. It is found most commonly in the mandible in the region of the lower molar teeth, occurring between the ages of 8 to 30 in both sexes with equal frequency. It causes distortion of surrounding areas but is usually a painless growth, at least initially. Considerable thickening of the cementum can often be observed. A periapical form is also recognized. Cementoma is not exclusive to the mandible as it can infrequently occur in the maxilla and other parts of the body such as the long bones.
A ceruminous adenoma is a benign glandular neoplasm which arises from the ceruminous glands located within the external auditory canal. These glands are found within the outer one third to one half of the external auditory canal, more common along the posterior surface; therefore, the tumor develops within a very specific location.
Ameloblastic carcinoma is a rare form of malignant odontogenic tumor, that develops in the jawbones from the epithelial cells that generate the tooth enamel. It is usually treated with surgery; chemotherapy has not been proven to be effective.
Pancreatic mucinous cystic neoplasm (MCN) is a type of cystic lesion that occurs in the pancreas. Amongst individuals undergoing surgical resection of a pancreatic cyst, about 23 percent were mucinous cystic neoplasms. These lesions are benign, though there is a high rate of progression to cancer. As such, surgery should be pursued when feasible. The rate of malignancy present in MCN is about 10 percent. If resection is performed before invasive malignancy develops, prognosis is excellent. The extent of invasion is the single most important prognostic factor in predicting survival.