| Adamantinoma | |
|---|---|
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| Micrograph (using H&E stain) of an adamantinoma showing the biphasic histomorphology. | |
| 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]
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]
X-rays of the affected area show a well defined tumour in bone, with multiple lobules giving a "soap bubble" appearance. [9] MRI can provide a more useful guide to its severity. [9]
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. [10] It was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill. [11] [12]
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.
A serous tumour is a neoplasm that typically has papillary to solid formations of tumor cells with crowded nuclei, and which typically arises on the modified Müllerian-derived serous membranes that surround the ovaries in females. Such ovarian tumors are part of the surface epithelial-stromal tumour group of ovarian tumors. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.
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.
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 Müller 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.
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. Despite its more common appearance in the bone region, it can affect soft tissue.
Calcifying odontogenic 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.
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 and 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.
Salivary gland tumours, also known as mucous gland adenomas or neoplasms, are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800 to 1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.
A renal oncocytoma is a tumour of the kidney made up of oncocytes, epithelial cells with an excess amount of mitochondria.
Osteofibrous dysplasia is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of bone.
Epithelial-myoepithelial carcinoma of the lung is a very rare histologic form of malignant epithelial neoplasm ("carcinoma") arising from lung tissue.
Giant-cell carcinoma of the lung (GCCL) is a rare histological form of large-cell lung carcinoma, a subtype of undifferentiated lung cancer, traditionally classified within the non-small-cell lung carcinomas (NSCLC).
Odontogenic cyst are a group of jaw cysts that are formed from tissues involved in odontogenesis. Odontogenic cysts are closed sacs, and have a distinct membrane derived from rests of odontogenic epithelium. It may contain air, fluids, or semi-solid material. Intra-bony cysts are most common in the jaws, because the mandible and maxilla are the only bones with epithelial components. That odontogenic epithelium is critical in normal tooth development. However, epithelial rests may be the origin for the cyst lining later. Not all oral cysts are odontogenic cysts. For example, mucous cyst of the oral mucosa and nasolabial duct cyst are not of odontogenic origin.
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.
The ameloblastic fibro-odontoma (AFO) is essentially a benign tumor with the features characteristic of ameloblastic fibroma along with enamel and dentin. Though it is generally regarded as benign, there have been cases of its malignant transformation into ameloblastic fibrosarcoma and odontogenic sarcoma. Cahn LR and Blum T, believed in "maturation theory", which suggested that AFO was an intermediate stage and eventually developed during the period of tooth formation to a complex odontoma thus, being a hamartoma.