Most patients present clinically with progressive, one sided hearing loss, much more often of the sensorineural rather than conductive type. Patients may also experience tinnitus, vertigo, and loss of vestibular function (ataxia). Symptoms are usually present for a long time, which supports the slow growth of these tumors. Patients may also present with other symptoms related to von Hippel-Lindau syndrome in other anatomic sites, which will result in imaging evaluation of the head.

Imaging studies help to identify the tumor and the specific anatomic site of involvement. Magnetic resonance images show a hyperintensity (hypervascularity) of a heterogeneous mass by T1 weighted images. Computed tomography shows a multilocular, lytic destructive temporal bone mass, centered within the endolymphatic sac (between internal auditory canal and sigmoid sinus).

An odontogenic tumor is a neoplasm of the cells or tissues that initiate odontogenic processes.

Examples include:

- Adenomatoid odontogenic tumor

- Ameloblastoma, a type of odontogenic tumor involving ameloblasts

- Calcifying epithelial odontogenic tumor

- Keratocystic odontogenic tumor

- Odontogenic myxoma

- Odontoma

Malignant transformation to squamous cell carcinoma may occur, but is unusual.

This uncommon tumor accounts for less than 2% of all ear tumors. While patients present with symptoms related to the middle ear cavity location of the tumor, the tumor may expand into the adjacent structures (external auditory canal, mastoid bone, and eustachian tube). Patients come to clinical attention with unilateral (one sided) hearing loss, usually associated with decreased auditory acuity, and particularly conductive hearing loss if the ossicular bone chain (middle ear bones) is involved. Tinnitus (ringing), otitis media, pressure or occasionally ear discharge are seen. At the time of otoscopic exam, the tympanic membrane is usually intact, with a fluid level or mass noted behind the ear drum. Even though this is a "neuroendocrine" type tumor, there is almost never evidence of neuroendocrine function clinically or by laboratory examination.

A gonadal tissue neoplasm is a tumor having any histology characteristic of cells or tissues giving rise to the gonads. These tissues arise from the sex cord and stromal cells. The tumor may be derived from these tissues, or produce them.

Although the tumor is composed of gonadal tissue, it is not necessarily located in an ovary or testicle.

A gonadal tissue neoplasm should not be confused with a urogenital neoplasm, though the two topics are often studied together. The embryology of the gonads is only indirectly related to the embryology of the external genitals and urinary system.

Swelling is the most common presenting complaint; however, OKCs may be asymptomatic and found incidentally on dental X-rays.

A urogenital neoplasm is a tumor of the urogenital system.

Types include:

- Cancer of the breast and female genital organs: (Breast cancer, Vulvar cancer, Vaginal cancer, Cervical cancer, Uterine cancer, Endometrial cancer, Ovarian cancer)

- Cancer of the male genital organs (Carcinoma of the penis, Prostate cancer, Testicular cancer)

- Cancer of the urinary organs (Renal cell carcinoma, Bladder cancer)

Pathologists classify serous cystic neoplasms into two broad groups. Those that are benign, that have not spread to other organs, are designated "serous cystadenoma". Serous cystadenomas can be further sub-typed into microcystic, oligocystic (or macrocystic), solid, mixed serous-endocrine neoplasm, and VHL-associated serous cystic neoplasm. This latter classification scheme is useful because it highlights the range of appearances and the clinical associations of these neoplasms. Serous cystic neoplasms that have spread ("metastasized") to another organ are considered malignant and are designated "serous cystadenocarcinoma".

The treatment is simple excision and exclusion of a malignant neoplasm.

Neuroendocrine adenoma of the middle ear (NAME) is a tumor which arises from a specific anatomic site: middle ear. NAME is a benign glandular neoplasm of middle ear showing histologic and immunohistochemical neuroendocrine and mucin-secreting differentiation (biphasic or dual differentiation).

Adenomatoid tumor is a benign mesothelial tumor, which arises from the lining of organs. It generally presents in the genital tract, in regions such as the testis and epididymis. It is the second most common extratesticular scrotal mass, after lipoma, and accounts for 30% of these masses. It also has been found in the pancreas.

In the female, it has been found in the body of the uterus and the fallopian tube.

Pancreatic serous cystadenoma, also known as serous cystadenoma of the pancreas and serous microcystic adenoma, a benign tumour of pancreas. It is usually found in the head of the pancreas, and may be associated with von Hippel-Lindau syndrome.

In contrast to some of the other cyst-forming tumors of the pancreas (such as the intraductal papillary mucinous neoplasm and the mucinous cystic neoplasm), serous cystic neoplasms are almost always entirely benign. There are some exceptions; rare case reports have described isolated malignant serous cystadenocarcinomas. In addition, serous cystic neoplasms slowly grow, and if they grow large enough they can press on adjacent organs and cause symptoms.

Glandular and epithelial neoplasm is a grouping of tumors arising from the glands and epithelium.

An example is adenoma.

Sebaceous lymphadenoma is a tissue diagnosis, e.g. salivary gland biopsy.

It may be confused with a number of benign and malignant neoplasms, including Warthin tumour, mucoepidermoid carcinoma and sebaceous lymphadenocarcinoma.

This lesion has been called a fetal lipoma, lipoma of embryonic fat or a lipoma of immature fat.

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.

An muscle tissue neoplasm is a neoplasm derived from muscle.

An example is myoma.

Intraductal papillary mucinous neoplasm (IPMN) is a type of tumor that can occur within the cells of the pancreatic duct. IPMN tumors produce mucus, and this mucus can form pancreatic cysts. Although intraductal papillary mucinous neoplasms are benign tumors, they can progress to pancreatic cancer. As such IPMN is viewed as a precancerous condition. Once an intraductal papillary mucinous neoplasm has been found, the management options include close monitoring and pre-emptive surgery.

Proliferating trichilemmal cysts (also known as a "Pilar tumor", "Proliferating follicular cystic neoplasm", "Proliferating pilar tumor", and "Proliferating trichilemmal tumor") are a cutaneous condition characterized by proliferations of squamous cells forming scroll-like structures.

Pathologists classify intraductal papillary mucinous neoplasms (IPMNs) into two broad groups - those that are associated with an invasive cancer and those that are not associated with an invasive cancer. This separation has critical prognostic significance. Patients with a surgically resected intraductal papillary mucinous neoplasm without an associated invasive cancer have an excellent prognosis (>95% will be cured), while patients with a surgically resected intraductal papillary mucinous neoplasm with an associated invasive cancer have a worse prognosis. Intraductal papillary mucinous neoplasms without an associated invasive cancer can be further subcategorized into three groups. They are IPMN with low-grade dysplasia, IPMN with moderate dysplasia, and IPMN with high-grade dysplasia. This categorization is less important than the separation of IPMNs with an associated cancer from IPMNs without an associated invasive cancer, but this categorization is useful as IPMNs are believed to progress from low-grade dysplasia to moderate dysplasia to high-grade dysplasia to an IPMN with an associated invasive cancer.

These terms are related since they represent the steps of the progression toward cancer:

- Dysplasia is the earliest form of precancerous lesion recognizable in a biopsy. Dysplasia can be low-grade or high-grade. High-grade dysplasia may also be referred to as carcinoma "in situ".

- Invasive carcinoma, usually simply called cancer, has the potential to invade and spread to surrounding tissues and structures, and may eventually be lethal.

An adipose tissue neoplasm is a neoplasm derived from adipose tissue.

An example is lipoma.

Carcinoma "in situ (CIS), also known as in situ" neoplasm, is a group of abnormal cells. While they are a form of neoplasm there is disagreement over whether CIS should be classified as cancer. This controversy also depends on the exact CIS in question (i.e. cervical, skin, breast). Some authors do not classify them as cancer, however, recognizing that they can potentially become cancer. Others classify certain types as a non-invasive form of cancer. The term "pre-cancer" has also been used.

These abnormal cells grow in their normal place, thus ""in situ"" (from Latin for "in its place"). For example, carcinoma "in situ" of the skin, also called Bowen's disease, is the accumulation of dysplastic epidermal cells within the epidermis only, that has failed to penetrate into the deeper dermis. For this reason, CIS will usually not form a tumor. Rather, the lesion is flat (in the skin, cervix, etc.) or follows the existing architecture of the organ (in the breast, lung, etc.). Exceptions include CIS of the colon (polyps), the bladder (preinvasive papillary cancer), or the breast (ductal carcinoma "in situ" or lobular carcinoma "in situ").

Many forms of CIS have a high probability of progression into cancer, and therefore removal may be recommended; however, progression of CIS is known to be highly variable and not all CIS becomes invasive cancer.

In the TNM classification, carcinoma "in situ" is reported as TisN0M0 (stage 0).

Two thirds of cases are located in the anterior maxilla, and one third are present in the anterior mandible.

Two thirds of the cases are associated with an impacted tooth (usually being the canine).

On radiographs, the adenomatoid odontogenic tumor presents as a radiolucency (dark area) around an unerupted tooth extending past the cementoenamel junction.

It should be differentially diagnosed from a dentigerous cyst and the main difference is that the radiolucency in case of AOT extends apically beyond the cementoenamel junction.

Radiographs will exhibit faint flecks of radiopacities surrounded by a radiolucent zone.

It is sometimes misdiagnosed as a cyst.