The main treatment modalities are surgery, embolization and radiotherapy.

A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). Unlike other types of cancer, there is no test that determines benign from malignant tumors; long-term followup is therefore recommended for all individuals with paraganglioma. Approximately 50% of patients with recurrent disease experience distant metastasis. The five-year survival in the setting of metastatic disease is 40% to 45%.

Malignant triton tumor (MTT) is a relatively rare, aggressive tumor made up of both malignant schwannoma cells and malignant rhabdomyoblasts. It's classified as a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation.

The unusual name "triton" was first used in reference to observation of supernumerary limbs containing bone and muscle growing on the backs of triton salamanders after the implantation of sciatic nerve tissue.

Treatment for Nasopharyngeal angiofibroma (JNA) is primarily surgical. The tumor is primarily excised by external or endoscopic approach. Medical treatment and radiation therapy are only of historical interest.

External approaches:

- transpalatine approach

- transpalatine + sublabial (Sardana's) Approach

- infratemporal Approach

- nasal endoscopic Approach

- transmaxillary Approach

Endoscopic approach is an excellent tool in primary and recurrent JNA, it allows visualisation and precise removal of the lesion. Preoperative embolisation of tumour may be of some use in reducing intraoperative bleeding.

Direct visualization is not common.

Surgery, with as wide a margin of removal as possible, has generally been the most effective and preferred way to attack LMS. If surgical margins are narrow or not clear of tumor, however, or in some situations where tumor cells were left behind, chemotherapy or radiation has been shown to give a clear survival benefit. While LMS tends to be resistant to radiation and chemotherapy, each case is different and results can vary widely.

LMS of uterine origin do frequently, but not always respond to hormonal treatments.

Prognosis for nasopharyngeal angiofibroma is favorable. Because these tumors are benign, metastasis to distal sites does not occur. However, these tumors are highly vascularized and grow rapidly. Removal is important in preventing nasal obstruction and recurrent epistaxis. Mortality is not associated with nasopharyngeal angiofibroma.

The role of chemotherapy in DIPG remains unclear. Studies have shown little improvement in survival, although efforts (see below) through the Children's Oncology Group (COG), Paediatric Brain Tumour Consortium (PBTC), and others are underway to explore further the use of chemotherapy and other drugs. Drugs that increase the effect of radiotherapy (radiosensitizers) have shown no added benefit, but promising new agents are under investigation. Immunotherapy with beta-interferon and other drugs has also had little effect in trials. Intensive or high-dose chemotherapy with autologous bone marrow transplantation or peripheral blood stem cell rescue has not demonstrated any effectiveness in brain stem gliomas. Future clinical trials may involve medicines designed to interfere with cellular pathways (signal transfer inhibitors), or other approaches that alter the tumor or its environment.

Primary treatment for this cancer, regardless of body site, is surgical removal with clean margins. This surgery can prove challenging in the head and neck region due to this tumour's tendency to spread along nerve tracts. Adjuvant or palliative radiotherapy is commonly given following surgery. For advanced major and minor salivary gland tumors that are inoperable, recurrent, or exhibit gross residual disease after surgery, fast neutron therapy is widely regarded as the most effective form of treatment.

Chemotherapy is used for metastatic disease. Chemotherapy is considered on a case by case basis, as there is limited trial data on the positive effects of chemotherapy. Clinical studies are ongoing, however.

Conventional radiotherapy, limited to the involved area of tumour, is the mainstay of treatment for DIPG. A total radiation dosage ranging from 5400 to 6000 cGy, administered in daily fractions of 150 to 200 cGy over 6 weeks, is standard. Hyperfractionated (twice-daily) radiotherapy was used previously to deliver higher radiation dosages, but did not lead to improved survival. Radiosurgery (e.g., gamma knife or cyberknife) has no role in the treatment of DIPG.

A primary tumor is a tumor growing at the anatomical site where tumor progression began and proceeded to yield a cancerous mass. Most cancers develop at their primary site but then go on to metastasize or spread to other parts of the body. These further tumors are secondary tumors.

Most cancers continue to be called after their primary site, as in breast cancer or lung cancer for example, even after they have spread to other parts of the body. Cancer of unknown primary origin is where secondary tumors are found, but the original primary site cannot be decided.

There are three diagnostic criteria proposed:

1. the tumor arises along a peripheral nerve, or in a ganglioneuroma, or in a patient with neurofibromatosis type 1 (NF1), or has a metastatic character

2. the growth characteristics of the tumor is typical for a Schwann cell tumor

3. rhabdomyoblasts arise within the body of the tumor.

Leiomyosarcoma, also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. It is also important to note that while it has been believed that leiomyosarcomas do not arise from leiomyomas, there are leiomyoma variants for which classification is evolving.

About 1 person in 100,000 gets diagnosed with LMS each year. Leiomyosarcoma is one of the more common types of soft-tissue sarcoma, representing 10 percent to 20 percent of new cases. (Leiomyosarcoma of the bone is more rare.) Sarcoma is rare, consisting of only 1 percent of cancer cases in adults. Leiomyosarcomas can be very unpredictable. They can remain dormant for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to chemotherapy or radiation. The best outcomes occur when it can be removed surgically with wide margins early, while small and still in situ.

CUP sometimes runs in families. It has been associated with familial lung, kidney, and colorectal cancers, which suggests that these sites may often be the origin of unidentifiable CUP cancers.

Although metastasis is widely accepted to be the result of the tumor cells migration, there is a hypothesis saying that some metastases are the result of inflammatory processes by abnormal immune cells. The existence of metastatic cancers in the absence of primary tumors also suggests that metastasis is not always caused by malignant cells that leave primary tumors.

Adenoid cystic carcinoma (sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC or AdCC.) is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses.

It is the third most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma and polymorphous low grade adenocarcinoma). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years, reflecting deaths from late-occurring metastatic disease.

Most people with cancer of unknown primary origin have widely disseminated and incurable disease, although a few can be cured through treatment. With treatment, typical survival with CUP ranges from 6 to 16 months. Survival rates are lower in cases with visceral metastatic disease, ranging from 6 to 9 months. Survival rates are higher when the cancer is more limited to lymph nodes, pleura, or peritoneal metastasis, which ranges from 14 to 16 months. Long-term prognosis is somewhat better if a particular source of cancer is strongly suggested by clinical evidence.

Treatment and survival is determined, to a great extent, by whether or not a cancer remains localized or spreads to other locations in the body. If the cancer metastasizes to other tissues or organs it usually dramatically increases a patient's likelihood of death. Some cancers—such as some forms of leukemia, a cancer of the blood, or malignancies in the brain—can kill without spreading at all.

Once a cancer has metastasized it may still be treated with radiosurgery, chemotherapy, radiation therapy, biological therapy, hormone therapy, surgery, or a combination of these interventions ("multimodal therapy"). The choice of treatment depends on a large number of factors, including the type of primary cancer, the size and location of the metastases, the patient's age and general health, and the types of treatments used previously. In patients diagnosed with CUP it is often still possible to treat the disease even when the primary tumor cannot be located.

Current treatments are rarely able to cure metastatic cancer though some tumors, such as testicular cancer and thyroid cancer, are usually curable.

Palliative care, care aimed at improving the quality of life of people with major illness, has been recommended as part of management programs for metastasis.

One approach used for treatment is embolization. A six-vessel angiogram is employed to determine the vascular supply to the fistula. Detachable coils, liquid embolic agents like NBCA, and onyx, or combinations of both are injected into the blood vessel to occlude the DAVF. Preoperative embolization can also be used to supplement surgery.

Enucleation (surgical removal of the eye) is the treatment of choice for large ciliary body melanomas. Small or medium sized tumors may be treated by an "iridocyclectomy". Radiotherapy may be appropriate in selected cases.

Manual carotid self compression is a controversial treatment for DAVF. Patients using this method are told to compress the carotid with the opposite hand for approximately 10 minutes daily, and gradually increasing the frequency and duration of compression. Currently, it is unclear whether this method is an effective therapy.

A combination of lifestyle modifications and medications can be used for the treatment of dolichoectasias.

- Antihypertensive medications such as Thiazides, Beta Blocker, ACE Inhibitor

- Trental or other Pentoxifylline drugs

- Dietary changes

- Weight loss

- Regular exercise

Conservative (i.e. no treatment), or surgical . With surgical excision, recurrence is common, usually due to incomplete excision. Often, the tracts of the cyst will pass near important structures, such as the internal jugular vein, carotid artery, or facial nerve, making complete excision impractical.

Specific treatment for enchondroma is determined by a physician based on the age, overall health, and medical history of the patient. Other considerations include:

- extent of the disease

- tolerance for specific medications, procedures, or therapies

- expectations for the course of the disease

- opinion or preference of the patient

Treatment may include:

- surgery (in some cases, when bone weakening is present or fractures occur)

- bone grafting - a surgical procedure in which healthy bone is transplanted from another part of the patient's body into the affected area.

If there is no sign of bone weakening or growth of the tumor, observation only may be suggested. However, follow-up with repeat x-rays may be necessary. Some types of enchondromas can develop into malignant, or cancerous, bone tumors later. Careful follow-up with a physician may be recommended.

A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of cancer in the body, but unlike mass effect, is not due to the local presence of cancer cells. In contrast, these phenomena are mediated by humoral factors (such as hormones or cytokines) secreted by tumor cells or by an immune response against the tumor.

Paraneoplastic syndromes are typical among middle-aged to older patients, and they most commonly present with cancers of the lung, breast, ovaries, or lymphatic system (a lymphoma). Sometimes, the symptoms of paraneoplastic syndromes show before the diagnosis of a malignancy, which has been hypothesized to relate to the disease pathogenesis. In this paradigm, tumor cells express tissue-restricted antigens (e.g., neuronal proteins), triggering an anti-tumor immune response which may be partially or, rarely, completely effective in suppressing tumor growth and symptoms. Patients then come to clinical attention when this tumor immune response breaks immune tolerance and begins to attack the normal tissue expressing that (e.g., neuronal) protein.

The abbreviation PNS is sometimes used for paraneoplastic syndrome, although it is used more often to refer to the peripheral nervous system.

There is no known cure for FMD. However, treatment focuses on relieving symptoms associated with it. Medical management is the most common form of treatment. The best approach to medically managing these patients is constantly being reevaluated as more information is learned about the disease.