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).

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 nervous system neoplasm is a tumor affecting the nervous system. Types include:

- Nerve sheath tumor

- Brain tumor

- Arachnoid cyst

- Optic nerve glioma

A cementoblastoma in a radiograph appears as a well-defined, markedly radiopaque mass, with a radiolucent peripheral line, which overlies and obliterates the tooth root. it is described as having a rounded or sunburst appearance. There is usually apparent external resorption of the root where the tumor and the root join. Severe hypercementosis and chronic focal sclerosing osteomyelitis are lesions to consider in the differential diagnosis of this lesion.

Cementoblastoma usually occurs in people under the age of 25, particularly males. It usually involves the permanent mandibular molars or premolars. The involved tooth usually has a vital pulp. It is attached to the tooth root and may cause its resorption, may involve the pulp canal, grows slowly, tends to expand the overlying cortical plates, and, except for the enlargement produced, is usually asymptomatic. This involves the buccal and lingual aspects of the alveolar ridges. But may be associated with diffuse pain and tooth mobility, but the tooth is still vital.

Since a cementoblastoma is a benign neoplasm, it grossly forms a mass of cementum-like tissue as an irregular or round mass attached to the roots of a tooth, usually the permanent mandibular first molar.

Ameloblastomas are often associated with the presence of unerupted teeth. Symptoms include painless swelling, facial deformity if severe enough, pain if the swelling impinges on other structures, loose teeth, ulcers, and periodontal (gum) disease. Lesions will occur in the mandible and maxilla, although 75% occur in the ascending ramus area and will result in extensive and grotesque deformities of the mandible and maxilla. In the maxilla it can extend into the maxillary sinus and floor of the nose. The lesion has a tendency to expand the bony cortices because slow growth rate of the lesion allows time for periosteum to develop thin shell of bone ahead of the expanding lesion. This shell of bone cracks when palpated and this phenomenon is referred to as "Egg Shell Cracking" or crepitus, an important diagnostic feature. Ameloblastoma is tentatively diagnosed through radiographic examination and must be confirmed by histological examination (e.g., biopsy).

There are three main clinical subtypes of ameloblastoma: unicystic, multicystic, peripheral. The peripheral subtype composes 2% of all ameloblastomas. Of all ameloblastomas in younger patients, unicystic ameloblastomas represent 6% of the cases. A fourth subtype, malignant, has been considered by some oncologic specialists, however, this form of the tumor is rare and may be simply a manifestation of one of the three main subtypes.

Ameloblastoma also occurs in long bones, and another variant is craniopharyngioma (Rathke's pouch tumour, pituitary ameloblastoma).

Vascular tissue neoplasms, like neoplasms of all tissues, are classified to benign and malignant ones, according to their biological behavior.

Pilocytic astrocytoma or juvenile pilocytic astrocytoma or cystic cerebellar astrocytoma (and its variant juvenile pilomyxoid astrocytoma) is a brain tumor that occurs more often in children and young adults (in the first 20 years of life). They usually arise in the cerebellum, near the brainstem, in the hypothalamic region, or the optic chiasm, but they may occur in any area where astrocytes are present, including the cerebral hemispheres and the spinal cord. These tumors are usually slow growing and benign. The neoplasms are associated with the formation of a single (or multiple) cyst(s), and can become very large.

Pilocytic astrocytomas are often cystic, and, if solid, tend to be well-circumscribed. It is characteristically easily seen on Computed tomography (CT scans) and Magnetic Resonance Imaging (MRI).

Juvenile pilocytic astrocytoma is associated with neurofibromatosis type 1 (NF1), and optic gliomas are among the most frequently encountered tumors in patients with this disorder. The majority of pilocytic astrocytomas have a unique KIAA1549-BRAF fusion gene.

Most malignant vascular tumors are considered sarcomas, a major histological group of tumors, arising from transformed cells of mesenchymal origin.

- Hemangiosarcoma

- Hemangiopericytoma

- Kaposi's Sarcoma

- Hemangioblastoma

- Lymphangiosarcoma

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

An example is adenoma.

An muscle tissue neoplasm is a neoplasm derived from muscle.

An example is myoma.

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".

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.

Solid pseudopapillary tumours are typically round, well-demarcated, measuring 2–17 cm in diameter (average 8 cm), with solid and cystic areas with hemorrhage on cut sections.

The histology of EST is variable, but usually includes malignant endodermal cells. These cells secrete alpha-fetoprotein (AFP), which can be detected in tumor tissue, serum, cerebrospinal fluid, urine and, in the rare case of fetal EST, in amniotic fluid. When there is incongruence between biopsy and AFP test results for EST, the result indicating presence of EST dictates treatment. This is because EST often occurs as small "malignant foci" within a larger tumor, usually teratoma, and biopsy is a sampling method; biopsy of the tumor may reveal only teratoma, whereas elevated AFP reveals that EST is also present. GATA-4, a transcription factor, also may be useful in the diagnosis of EST.

Diagnosis of EST in pregnant women and in infants is complicated by the extremely high levels of AFP in those two groups. Tumor surveillance by monitoring AFP requires accurate correction for gestational age in pregnant women, and age in infants. In pregnant women, this can be achieved simply by testing maternal serum AFP rather than tumor marker AFP. In infants, the tumor marker test is used, but must be interpreted using a reference table or graph of normal AFP in infants.

A solid pseudopapillary tumour (also known as solid pseudopapillary neoplasm or, more formally, solid pseudopapillary tumour/neoplasm of the pancreas) is a low-grade malignant neoplasm of the pancreas of architecture that typically afflicts young women.

A connective tissue neoplasm or connective tissue tumor is a neoplasm arising from the tissues of the connective tissue. (Not all tumors "in" the connective tissue are "of" the connective tissue.)

Endodermal sinus tumor (EST), also known as yolk sac tumor (YST), is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under 3, and is also known as infantile embryonal carcinoma. This age group has a very good prognosis. In contrast to the pure form typical of infants, adult endodermal sinus tumors are often found in combination with other kinds of germ cell tumor, particularly teratoma and embryonal carcinoma. While pure teratoma is usually benign, endodermal sinus tumor is malignant.

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

An example is lipoma.

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.

Poorly differentiated thyroid carcinoma (PDTC) is malignant neoplasm of follicular cell origin showing intermediate histopathological patterns between differentiated and undifferentiated thyroid cancers.

Brown tumours consist of fibrous tissue, woven bone and supporting vasculature, but no matrix. The osteoclasts consume the trabecular bone that osteoblasts lay down and this front of reparative bone deposition followed by additional resorption can expand beyond the usual shape of the bone, involving the periosteum thus causing bone pain. The characteristic brown coloration results from hemosiderin deposition into the osteolytic cysts. Hemosiderin deposition is not a distinctive feature of brown tumors; it may also be seen in giant cell tumors of the bone.

Brown tumors may be rarely associated with ectopic parathyroid adenomas or end stage renal osteodystrophy.

Squamous epithelial cells are not found in normal thyroid, thus the origin of SCTC is not clear. However, it might be a derived from the embryonic remnants such as thyroglossal duct or branchial clefts. Often SCTC is diagnosed in one of the thyroid lobes, but not in the pyramidal lobe. Another possible way of SCTC development can be through the squamous metaplasia of cells. However, that theory is also controversial, since the Hashimoto's thyroiditis and chronic lymphocytic thyroiditis (neoplasms to be showed squamous metaplasia) are not associated with SCTC. Primary STCT is usually diagnosed in both lobes of thyroid gland. The histopathology of STCT shows a squamous differentiation of tumor cells.