Treatment may include the following:

- Surgery with or without radiation

- Radiotherapy

Fast neutron therapy has been used successfully to treat salivary gland tumors, and has shown to be significantly more effective than photons in studies treating unresectable salivary gland tumors.

- Chemotherapy

These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question. Since these lesions do not have malignant potential, long-term observation is unnecessary. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy.

This is a very rare tumor, since only about 1 in 35,000 to 40,000 people have VHL, of whom about 10% have endolymphatic sac tumors. Patients usually present in the 4th to 5th decades without an gender predilection. The tumor involves the endolymphatic sac, a portion of the intraosseous inner ear of the posterior petrous bone.

Wide excision is the treatment of choice, although attempting to preserve hearing. Based on the anatomic site, it is difficult to completely remove, and so while there is a good prognosis, recurrences or persistence may be seen. There is no metastatic potential. Patients who succumb to the disease, usually do so because of other tumors within the von Hippel-Lindau complex rather than from this tumor.

Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).

Mucinous cystadenoma may arise in a number of locations; however, mucinous cystadenoma at different locations are not generally considered to be related to one another.

As metanephric adenomas are considered benign, they can be left in place, i.e. no treatment is needed.

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

An example is adenoma.

As the condition is quite rare, opinions among experts about how to treat OKCs differ.

Treatment options:

- Wide (local) surgical excision.

- Marsupialization - the surgical opening of the (OKC) cavity and a creation of a marsupial-like pouch, so that the cavity is in contact with the outside for an extended period, e.g. three months.

- Curettage (simple excision & scrape-out of cavity).

- Peripheral ostectomy after curettage and/or enucleation.

- Simple excision.

- Carnoy's solution - usually used in conjunction with excision.

- Enucleation and cryotherapy

Patients treated with complete surgical excision can expect an excellent long term outcome without any problems. Recurrences may be seen in tumors which are incompletely excised.

Wide, radical, complete surgical excision is the treatment of choice, with free surgical margins to achieve the best outcome and lowest chance of recurrence. Radiation is only used for palliation. In general, there is a good prognosis, although approximately 50% of patients die from disease within 3–10 years of presentation.

The treatment of choice for main-duct IPMNs is resection due to approximately 50% chance of malignancy. Side-branch IPMNs are occasionally monitored with regular CT or MRIs, but most are eventually resected, with a 30% rate of malignancy in these resected tumors. Survival 5 years after resection of an IPMN without malignancy is approximately 80%, 85% with malignancy but no lymph node spread and 0% with malignancy spreading to lymph nodes. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy or robotic surgery. A study using Surveillance, Epidemiology, and End Result Registry (SEER) data suggested that increased lymph node counts harvested during the surgery were associated with better survival in invasive IPMN patients.

Although the causes of craniopharyngioma is unknown, it can occur in both children and adults, with a peak in incidence at 9 to 14 years of age. There are approximately 120 cases diagnosed each year in the United States in patients under the age of 19 years old. In fact, more than 50% of all patients with craniopharyngioma are under the age of 18 years. There is no clear association of the tumor with a particular gender or race. It is not really known what causes craniopharyngiomas, but they do not appear to "run in families" or to be directly inherited from the parents.

A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour (MEST) and renal epithelial stromal tumour (REST), is a type of rare benign kidney tumour.

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.

While there is a wide age range at clinical presentation (12–85 years), most patients come to clinical attention at 55 years (mean). There is no gender difference.

Treatment generally consists of subfrontal or transsphenoidal excision. Surgery using the transsphenoidal route is often performed by a joint team of ENT and neurosurgeons. Because of the location of the craniopharyngioma near the brain and skullbase, a surgical navigation system might be used to verify the position of surgical tools during the operation.

Additional radiotherapy is also used if total removal is not possible. Due to the poor outcomes associated with damage to the pituitary and hypothalamus from surgical removal and radiation, experimental therapies using intracavitary phosphorus-32, yttrium, or bleomycin delivered via an external reservoir are sometimes employed, especially in young patients. The tumor, being in the pituitary gland, can cause secondary health problems. The immune system, thyroid levels, growth hormone levels and testosterone levels can be compromised from craniopharygioma. All of the before mentioned health problems can be treated with modern medicine. There is no high quality evidence looking at the use of bleomycin in this condition.

The most effective treatment 'package' for the malignant craniopharyngiomas described in literature is a combination 'gross total resective' surgery with adjuvant chemo radiotherapy. The chemotherapy drugs Paclitaxel and Carboplatin have shown a clinical (but not statistical) significance in increasing the survival rate in patients who've had gross total resections of their malignant tumours.

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.

Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal. This tumor is rare, with several names used in the past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous mucoepidermoid carcinoma.

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.

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

An example is lipoma.

Cystadenocarcinoma is a malignant form of a cystadenoma and is a malignant neoplasm derived from glandular epithelium, in which cystic accumulations of retained secretions are formed. The neoplastic cells manifest varying degrees of anaplasia and invasiveness, and local extension and metastases occur. Cystadenocarcinomas develop frequently in the ovaries, where pseudomucinous and serous types are recognized. Similar tumor histology has also been reported in the pancreas, although it is a considerably rarer entity.

It is the most common malignant ovarian tumor. Contains complex multi-loculated cyst but with exuberant solid areas in places. It usually presents with omental metastases which cause ascites.

Clear cell ovarian tumors are part of the surface epithelial-stromal tumor group of ovarian cancers, accounting for 6% of these cancers. Clear cell tumors are also associated with the pancreas and salivary glands.

An muscle tissue neoplasm is a neoplasm derived from muscle.

An example is myoma.

Metanephric adenoma (MA)is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma (Wilms tumours), or a papillary renal cell carcinoma.

It should not be confused with the pathologically unrelated, yet similar sounding, "mesonephric adenoma".

MCACL has a much more favorable prognosis than most other forms of adenocarcinoma and most other NSCLC's. Cases have been documented of continued growth of these lesions over a period of 10 years without symptoms or metastasis. The overall mortality rate appears to be somewhere in the vicinity of 18% to 27%, depending on the criteria that are used to define this entity.