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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.
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
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.
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
The tumor must be removed with as complete a surgical excision as possible. In nearly all cases, the ossicular chain must be included if recurrences are to be avoided. Due to the anatomic site of involvement, facial nerve paralysis and/or paresthesias may be seen or develop; this is probably due to mass effect rather than nerve invasion. In a few cases, reconstructive surgery may be required. Since this is a benign tumor, no radiation is required. Patients experience an excellent long term outcome, although recurrences can be seen (up to 15%), especially if the ossicular chain is not removed. Although controversial, metastases are not seen in this tumor. There are reports of disease in the neck lymph nodes, but these patients have also had other diseases or multiple surgeries, such that it may represent iatrogenic disease.
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.
Complete surgical excision is the treatment of choice, associated with an excellent long term clinical outcome.
The main treatment modalities are surgery, embolization and radiotherapy.
Surgical excision of the lesion is done, and depending upon the clinical circumstances, this may or may not involve removal of the involved tooth. With incomplete removal, recurrence is common; some surgeons advocate curettage after extraction of teeth to decrease the overall rate of recurrence.
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.
All published findings on SNUC suggest that therapy that gives more than one kind of treatment (multimodality treatment) give SNUC patients the best possible chance for survival. Varying combinations of and length between surgery, radiation, and chemotherapy have been tested. Findings from Mendenhall et al. have suggest that surgery plus radiotherapy and concominant chemotherapy is most efficient rather thain radiotherapy combined with induced or maintenance chemotherapy.
Depending on the pet's unique condition, there are several treatment options, including surgery, chemotherapy and radiation therapy. Treating the pain adequately is also of crucial importance to improve the pet's quality of life, especially if amputation is not performed.
Radiation therapy has become the preferred treatment. Its advantage is that it treats the entire nasal cavity together with the affected bone and has shown the greatest improvement in survival. The radiation therapy is typically delivered in 10-18 treatment sessions over the course of 2–4 weeks.
Radiation therapy has a multitude of accompanying side effects and should be recommended on a case-by-case basis. Dogs in which nose bleeds are observed have an average life expectancy of 88 days. In instances where nosebleeds are not seen, the prognosis is slightly less grim. On average, a dog with nasal cancer has a life expectancy of 95 days.
The preferred treatment for esthesioneuroblastoma is surgery followed by radiotherapy to prevent reoccurrence of the tumor.
Radiotherapy alone is reserved only for small lesions not appropriate for either surgery or chemotherapy. Both photon and proton radiotherapy have been used effectively to treat esthesioneuroblastoma. Proton radiotherapy has recently been shown to be effective in a 10-person study with Kadish C tumors, while delivering less toxicity to the nervous system.
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.
The first line of treatment for nasal polyps is topical steroids. Steroids decrease the inflammation of the sinus mucosa to decrease the size of the polyps and improve symptoms. Topical preparations are preferred in the form of a nasal spray, but are often ineffective for people with many polyps. Steroids by mouth often provide drastic symptom relief, but should not be taken for long periods of time due to their side effects. Because steroids only shrink the size and swelling of the polyp, people often have recurrence of symptoms once the steroids are stopped. Decongestants do not shrink the polyps, but can decrease swelling and provide some relief. Antibiotics are only recommended if the person has a co-occurring bacterial infection.
In people with nasal polyps caused by aspirin or NSAIDs, avoidance of these medications will help with symptoms. Aspirin desensitization has also been shown to be beneficial.
Total resection of the tumour, followed by radiation therapy is the standard treatment modality. Medulloepithelioma of the ciliary body may necessitate enucleation of the eye. Radiation therapy alone may prolong survival. Aggressive chemotherapy with autologous bone marrow transplant is used for metastatic medulloepitheliomas.
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.
Endoscopic sinus surgery with removal of polyps is often very effective for most people providing rapid symptom relief. Endoscopic sinus surgery is minimally-invasive and is done entirely through the nostril with the help of a camera. Surgery should be considered for those with complete nasal obstruction, uncontrolled runny nose, nasal deformity caused by polyps or continued symptoms despite medical management. Surgery serves to remove the polyps as well as the surrounding inflamed mucosa, open obstructed nasal passages, and clear the sinuses. This not only removes the obstruction caused by the polyps themselves, but allows medications such as saline irrigations and topical steroids to become more effective.
Surgery lasts approximately 45 minutes to 1 hour and can be done under general or local anesthesia. Most patients tolerate the surgery without much pain, though this can vary from patient to patient. The patient should expect some discomfort, congestion, and drainage from the nose in the first few days after surgery, but this should be mild. Complications from endoscopic sinus surgery are rare, but can include bleeding and damage to other structures in the area including the eye or brain.
Many physicians recommend a course of oral steroids prior to surgery to reduce mucosal inflammation, decrease bleeding during surgery, and help with visualization of the polyps. Nasal steroid sprays should be used preventatively after surgery to delay or prevent recurrence. People often have recurrence of polyps even following surgery. Therefore, continued follow up with a combination of medical and surgical management is preferred for the treatment of nasal polyps.
Although surgery is the treatment of choice, it must be preceded by imaging studies to exclude an intracranial connection. Potential complications include meningitis and a cerebrospinal fluid leak. Recurrences or more correctly persistence may be seen in up to 30% of patients if not completely excised.
The most common form of treatment is having the tumor surgically removed however total resection is often not possible. The location could prohibit access to the neoplasm and lead to incomplete or no resection at all. Removal of the tumor will generally allow functional survival for many years. In particular for pilocytic astrocytomas (that are commonly indolent bodies that may permit normal neurologic function) surgeons may decide to monitor the neoplasm's evolution and postpone surgical intervention for some time. However, left unattended these tumors may eventually undergo neoplastic transformation.
If surgery is not possible, recommendations such as chemotherapy or radiation be suggested however side effects from these treatments can be extensive and long term.
Children with cerebellar pilocytic astrocytoma may experience side effects related to the tumor itself depending on the location and related to the treatment. Strabismus.
- Symptoms related to increased pressure in the brain often disappear after surgical removal of the tumor.
- Effects on coordination and balance improved and might progressively (to completely) disappear as recovery progresses.
- Steroid-treatment is often used to control tissue swelling that may occur pre- and post-operatively.
- Children Diagnosed can also suffer long term side effects due to the type of treatment they may receive.
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.
Medulloepithelioma carries a dismal prognosis with a median survival of 5 months.