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Ceruminous adenoma are rare tumors, accounting for less than 1% of all external ear tumors. The patients will present with a mass, perhaps associated pain, and may have changes in hearing (usually a sensorineural or a conductive hearing loss). Some patients have tinnitus. Nerve paralysis is very uncommon.
Pain is the most common symptom, followed by either sensorineural or conductive hearing loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow growing.
This tumor only affects the outer 1/3 to 1/2 of the external auditory canal as a primary site. If this area is not involved, the diagnosis should be questioned. The most common tumor type is ceruminous adenoid cystic carcinoma and ceruminous adenocarcinoma, NOS.
Hemangiopericytoma located in the cerebral cavity is an aggressive tumor of the Mesenchyme with oval nuclei with scant cytoplasm. "There is dense intercellular reticulin staining. Tumor cells can be fibroblastic, myxoid, or pericytic. These tumors, in contrast to meningiomas, do not stain with epithelial membrane antigen. They have a grade 2 or 3 biological behavior, and need to be distinguished from benign meningiomas because of their high rate of recurrence (68.2%) and metastases (Maier et al. 1992; Kleihues et al. 1993 )."
A ceruminous adenoma (also known as adenoma of the ceruminous gland and ceruminoma) 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.
Glomus tumors are usually solitary and small lesions. The vast majority are found in the distal extremities, particularly in the hand, wrist, foot, and under the fingernails.
They are often painful, and the pain is reproduced when the lesion is placed in cold water.
These tumors tend to have a bluish discoloration, although a whitish appearance may also be noted. Elevation of the nail bed can occur.
In rare cases, the tumors may present in other body areas, such as the gastric antrum or glans penis. Treatment is essentially the same.
The exact incidence of glomus tumors is unknown. The multiple variant is rare, accounting for less than 10% of all cases. The probable misdiagnosis of many of these lesions as hemangiomas or venous malformations also makes an accurate assessment of incidence difficult.
- Sex:
Solitary glomus tumors, particularly subungual lesions, are more common in females than in males. Multiple lesions are slightly more common in males.
- Age:
Solitary glomus tumors are more frequent in adults than in others. Multiple glomus tumors develop 11–15 years earlier than single lesions; about one third of the cases of multiple tumors occur in those younger than 20 years. Congenital glomus tumors are rare; they are plaquelike in appearance and are considered a variant of multiple glomus tumors.
A hemangiopericytoma (HPC) is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with a special aggressive behavior. It was first characterized in 1942.
Malignant transformation to squamous cell carcinoma may occur, but is unusual.
Swelling is the most common presenting complaint; however, OKCs may be asymptomatic and found incidentally on dental X-rays.
This lesion has been called a fetal lipoma, lipoma of embryonic fat or a lipoma of immature fat.
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). Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.
Benign osteofibrous dysplasia may be a precursor of adamantinoma or a regressive phase of adamantinoma.
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.
Patients present with a slow-growing, painless, solitary mass, usually of the subcutaneous tissues. It is much less frequently noted in the intramuscular tissue. It is not uncommon for symptoms to be present for years.
Benign neoplasm with "BROWN FAT" is noted.
Children affected by pilocytic astrocytoma can present with different symptoms that might include failure to thrive (lack of appropriate weight gain/ weight loss), headache, nausea, vomiting, irritability, torticollis (tilt neck or wry neck) difficulty to coordinate movements and visual complaints (including nystagmus). The complaints may vary depending on the location and size of the neoplasm. The most common symptoms are associated with increased intracranial pressure due to the size of the neoplasm.
A glomus tumor (also known as a "solitary glomus tumor," "solid glomus tumor," or glomangioma) is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot. They account for less than 2% of all soft tissue tumors. The majority of glomus tumors are benign, but they can also show malignant features. Glomus tumors were first described by Hoyer in 1877 while the first complete clinical description was given by Masson in 1924.
Histologically, glomus tumors are made up of an afferent arteriole, anastomotic vessel, and collecting venule. Glomus tumors are modified smooth muscle cells that control the thermoregulatory function of dermal glomus bodies. As stated above, these lesions should not be confused with paragangliomas, which were formerly also called glomus tumors in now-antiquated clinical usage. Glomus tumors do not arise from glomus cells, but paragangliomas do.
Familial glomangiomas have been associated with a variety of deletions in the GLMN (glomulin) gene, and are inherited in an autosomal dominant manner, with incomplete penetrance.
Typically patients present with progressive pain, often long standing and/or bony swelling and restricted range of movement in affected limb 3,12. The latter is most often the case in bones with little overlying soft tissues (e.g. short tubular bones of the hands and feet).
Most chondromyxoid fibromas are located in the metaphyseal region of long bones (60%), and may extend to the epiphyseal line and even rarely abut the articular surface 3,12. They are almost never just epiphyseal 3. The classical site is the upper 1/3rd of tibia ( which accounts for 25% of all cases) with the small tubular bones of the foot, the distal femur and pelvis being other relatively common locations 12.
Rarely occur in the skull or skull base.
Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of parenchymatous glandular 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 (variable appearance) seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which describes its pleomorphic appearance as opposed to its dual origin from epithelial and myoepithelial elements.
Considered part of the PTEN hamartoma tumor syndrome (PHTS), which also includes Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome, and Proteus-like syndrome, Cowden syndrome is a serious genetic disorder characterized by multiple hamartomas. Usually skin hamartomas exist, and commonly (in about 66% of cases) hamartoma of the thyroid gland exists. Additional growths can form in many parts of the body, especially in bones, CNS, the eyes, the genitourinary tract, the GI tract, and mucosa. The hamartomas themselves may cause symptoms or even death, but morbidity is more often associated with increased occurrence of malignancies, usually in the breast or thyroid.
The tumor is usually solitary and presents as a slow growing, painless, firm single nodular mass. Isolated nodules are generally outgrowths of the main nodule rather than a multinodular presentation. It is usually mobile unless found in the palate and can cause atrophy of the mandibular ramus when located in the parotid gland. When found in the parotid tail, it may present as an eversion of the ear lobe. Though it is classified as a benign tumor, pleomorphic adenomas have the capacity to grow to large proportions and may undergo malignant transformation, to form carcinoma ex-pleomorphic adenoma, a risk that increases with time (9.5% chance to convert into malignancy in 15 years). Although it is "benign" the tumor is aneuploid, it can recur after resection, it invades normal adjacent tissue and distant metastases have been reported after long (+10 years) time intervals.
One general danger of hamartomas is that they may impinge into blood vessels, resulting in a risk of serious bleeding. Because a hamartoma typically lacks elastic tissue, it may lead to the formation of aneurysms and thus possible hemorrhage. Where a hamartoma impinges into a major blood vessel, such as the renal artery, hemorrhage must be considered life-threatening.
Angiomyolipoma of the kidney was previously considered to be a hamartoma or choristoma.
Hamartomas of the spleen are uncommon but can be dangerous. About 50% of such cases manifest abdominal pain, and they are often associated with hematologic abnormalities and spontaneous rupture.
Focal nodular hyperplasia's most recognizable gross feature is a central stellate scar seen in 60–70% of cases. Microscopically, a lobular proliferation of bland-appearing hepatocytes with a bile ductular proliferation and malformed vessels within the fibrous scar is the most common pattern. Other patterns include telangiectatic, hyperplastic-adenomatous, and lesions with focal large-cell dysplasia. Rarely, these lesions may be multiple or can occur as part of a syndrome with hemangiomas, epithelioid hemangioendothelioma, hepatic adenomas, fibrolamellar hepatocellular carcinoma, vascular malformations of the brain, meningiomas, and/or astrocytomas.
The majority of cases occur in the second and third decades, with approximately 75% of cases occurring before the age of 30 years 1,12-15. There is no recognised gender predilection. Examples have however been seen in patients up to the age of 75 years. In some series there is a male predilection 12 whilst in others no such distribution is found 2
Small tumors (e.g., < 2.0 cm) usually are incidental findings at autopsy without having caused symptoms. Larger tumors may cause symptoms, depending on the size and location.
- Focal seizures may be caused by meningiomas that overlie the cerebrum.
- Progressive spastic weakness in legs and incontinence may be caused by tumors that overlie the parasagittal frontoparietal region.
- Tumors of the Sylvian aqueduct may cause myriad motor, sensory, aphasic, and seizure symptoms, depending on the location.
- Increased intracranial pressure eventually occurs, but is less frequent than in gliomas.
- Diplopia (Double vision) or uneven pupil size may be symptoms if related pressure causes a third and/or sixth nerve palsy.
The treatment is simple excision and exclusion of a malignant neoplasm.
Osteoblastoma is an uncommon osteoid tissue-forming primary neoplasm of the bone.
It has clinical and histologic manifestations similar to those of osteoid osteoma; therefore, some consider the two tumors to be variants of the same disease, with osteoblastoma representing a giant osteoid osteoma. However, an aggressive type of osteoblastoma has been recognized, making the relationship less clear.
Although similar to osteoid osteoma, it is larger (between 2 and 6 cm).
Focal nodular hyperplasia (FNH) is a benign tumor of the liver (hepatic tumor), which is the second most prevalent tumor of the liver (the first is hepatic hemangioma). It is usually asymptomatic, rarely grows or bleeds, and has no malignant potential. This tumour was once often resected because it was difficult to distinguish from hepatic adenoma, but with modern multiphase imaging is usually now diagnosed by strict imaging criteria and not resected.