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Likely, current chemotherapies are not effective. Antiprogestin agents have been used, but with variable results. A 2007 study of whether hydroxyurea has the capacity to shrink unresectable or recurrent meningiomas is being further evaluated.
Radiation therapy may include photon-beam or proton-beam treatment, or fractionated external beam radiation. Radiosurgery may be used in lieu of surgery in small tumors located away from critical structures. Fractionated external-beam radiation also can be used as primary treatment for tumors that are surgically unresectable or, for patients who are inoperable for medical reasons.
Radiation therapy often is considered for WHO grade I meningiomas after subtotal (incomplete) tumor resections. The clinical decision to irradiate after a subtotal resection is somewhat controversial, as no class I randomized, controlled trials exist on the subject. Numerous retrospective studies, however, have suggested strongly that the addition of postoperative radiation to incomplete resections improves both progression-free survival (i.e. prevents tumor recurrence) and improves overall survival.
In the case of a grade III meningioma, the current standard of care involves postoperative radiation treatment regardless of the degree of surgical resection. This is due to the proportionally higher rate of local recurrence for these higher-grade tumors. Grade II tumors may behave variably and there is no standard of whether to give radiotherapy following a gross total resection. Subtotally resected grade II tumors should be radiated.
The mainstay of treatment is surgical excision. Two adjuvant therapeutic strategies are Stereotactic surgery (SRS) and fractionated convention radiotherapy (FCRT). Both are highly effective means of treatment.
Chemotherapy is typically limited to patients with recurrent central neurocytoma. The course of chemotherapy used for CNC is one of two platinum-based regimes. The two regimes are:
- Carboplatin + VP-16 + ifosfamide
- cisplatin + VP-16 + cyclophosphamide
Because chemotherapy is used in rare cases there is still information to be gathered as to the efficacy of chemotherapy to treat benign CNC. Therefore, recommendations must be viewed as limited and preliminary.
Because ganglioneuromas are benign, treatment may not be necessary, as it would expose patients to more risk than leaving it alone. If there are symptoms or major physical deformity, treatment usually consists of surgery to remove the tumor.
The objective of irradiation is to halt the growth of the acoustic neuroma tumour, it does not excise it from the body, as the term 'radiosurgery' or 'gammaknife' implies. Radiosurgery is only suitable for small to medum size tumors.
Acoustic neuromas are managed by either surgery, radiation therapy, or observation with regular MRI scanning. With treatment, the likelihood of hearing preservation varies inversely with the size of the tumor; for large tumors, preservation of hearing is rare. Because acoustic neurmas, meningiomas and most other CPA tumors are benign, slow growing or non-growing, and non-invasive, observation is a viable management option.
Treatment is not needed in the asymptomatic patient. Symptomatic patients may benefit from surgical debulking of the tumor. Complete tumor removal is not usually needed and can be difficult due to the tumor location.
There are several different surgical techniques for the removal of acoustic neuroma. The choice of approach is determined by size of the tumour, hearing capability, and general clinical condition of the patient.
- The retrosigmoid approach offers some opportunity for the retention of hearing.
- The translabyrinthine approach will sacrifice hearing on that side, but will usually spare the facial nerve. Post-operative cerebrospinal fluid leaks are more common.
- The middle fossa approach is preferred for small tumours, and offers the highest probability of retention of hearing and vestibular function.
- Less invasive endoscopic techniques have been done outside of the United States for some time. Recovery times are reported to be faster. However, this technique is not yet mainstream among surgeons in the US.
Larger tumors can be treated by either the translabyrinthine approach or the retrosigmoid approach, depending upon the experience of the surgical team. With large tumors, the chance of hearing preservation is small with any approach. When hearing is already poor, the translabyrinthine approach may be used for even small tumors. Small, lateralized tumours in patients with good hearing should have the middle fossa approach. When the location of the tumour is more medial a retrosigmoid approach may be better.
Auditory canal decompression is another surgical technique that can prolong usable hearing when a vestibular schwannoma has grown too large to remove without damage to the cochlear nerve. In the IAC (internal auditory canal) decompression, a middle fossa approach is employed to expose the bony roof of the IAC without any attempt to remove the tumor. The bone overlying the acoustic nerve is removed, allowing the tumour to expand upward into the middle cranial fossa. In this way, pressure on the cochlear nerve is relieved, reducing the risk of further hearing loss from direct compression or obstruction of vascular supply to the nerve.
Radiosurgery is a conservative alternative to cranial base or other intracranial surgery. With conformal radiosurgical techniques, therapeutic radiation focused on the tumour, sparing exposure to surrounding normal tissues. Although radiosurgery can seldom completely destroy a tumor, it can often arrest its growth or reduce its size. While radiation is less immediately damaging than conventional surgery, it incurs a higher risk of subsequent malignant change in the irradiated tissues, and this risk in higher in NF2 than in sporadic (non-NF2) lesions.
Most ganglioneuromas are noncancerous, thus expected outcome is usually good. However, a ganglioneuroma may become cancerous and spread to other areas, or it may regrow after removal.
If the tumor has been present for a long time and has pressed on the spinal cord or caused other symptoms, it may have caused irreversible damage that cannot be corrected with the surgical removal of the tumor. Compression of the spinal cord may result in paralysis, especially if the cause is not detected promptly.
Supportive treatment focuses on relieving symptoms and improving the patient’s
neurologic function. The primary supportive agents are anticonvulsants and
corticosteroids.
- Historically, around 90% of patients with glioblastoma underwent anticonvulsant treatment, although it has been estimated that only approximately 40% of patients required this treatment. Recently, it has been recommended that neurosurgeons not administer anticonvulsants prophylactically, and should wait until a seizure occurs before prescribing this medication. Those receiving phenytoin concurrent with radiation may have serious skin reactions such as erythema multiforme and Stevens–Johnson syndrome.
- Corticosteroids, usually dexamethasone given 4 to 8 mg every 4 to 6 h, can reduce peritumoral edema (through rearrangement of the blood–brain barrier), diminishing mass effect and lowering intracranial pressure, with a decrease in headache or drowsiness.
A 2009 clinical trial at Massachusetts General Hospital used the cancer drug Bevacizumab (commercial name: Avastin) to treat 10 patients with neurofibromatosis type II. The result was published in "The New England Journal of Medicine". Of the ten patients treated with bevacizumab, tumours shrank in 9 of them, with the median best response rate of 26%. Hearing improved in some of the patients, but improvements were not strongly correlated with tumour shrinkage. Bevacizumab works by cutting the blood supply to the tumours and thus depriving them of their growth vector. Side effects during the study included alanine aminotransferase, proteinuria, and hypertension (elevated blood pressure) among others. A separate trial, published in "The Neuro-oncology Journal", show 40% tumour reduction in the two patients with NF2, along with significant hearing improvement.
Overall the researchers believed that bevacizumab showed clinically significant effects on NF-2 patients. However, more research is needed before the full effects of bevacizumab can be established in NF-2 patients.
Definitive treatment for ganglioglioma requires gross total surgical resection, and a good prognosis is generally expected when this is achieved. However, indistinct tumor margins and the desire to preserve normal spinal cord tissue, motor and sensory function may preclude complete resection of tumor. According to a series by Lang et al., reviewing several patients with resected spinal cord ganglioglioma, the 5- and 10-year survival rates after total resection were 89% and 83%, respectively. In that study, patients with spinal cord ganglioglioma had a 3.5-fold higher relative risk of tumor recurrence compared to patients with supratentorial ganglioglioma. It has been recognized that postoperative results correlate closely with preoperative neurological status as well as the ability to achieve complete resection.
With the exception of WHO grade III anaplastic ganglioglioma, radiation therapy is generally regarded to have no role in the treatment of ganglioglioma. In fact, radiation therapy may induce malignant transformation of a recurrent ganglioglioma several years later. Adjuvant chemotherapy is also typically reserved for anaplastic ganglioglioma, but has been used anecdotally in partially resected low grade spinal cord gangliogliomas which show evidence of disease progression.
Most studies show no benefit from the addition of chemotherapy. However, a large clinical trial of 575 participants randomized to standard radiation versus radiation plus temozolomide chemotherapy showed that the group receiving temozolomide survived a median of 14.6 months as opposed to 12.1 months for the group receiving radiation alone. This treatment regime is now standard for most cases of glioblastoma where the person is not enrolled in a clinical trial. Temozolomide seems to work by sensitizing the tumor cells to radiation.
High doses of temozolomide in high-grade gliomas yield low toxicity, but the results are comparable to the standard doses.
Antiangiogenic therapy with medications such as bevacizumab control symptoms but do not affect overall survival.
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.
Depending on the grade of the sarcoma, it is treated with surgery, chemotherapy and/or radiotherapy.
Because of the rarity of these tumors, there is still a lot of unknown information. There are many case studies that have been reported on patients who have been diagnosed with this specific type of tumor. Most of the above information comes from the findings resulting from case studies.
Since Papillary Tumors of the Pineal Region were first described in 2003, there have been seventy cases published in the English literature. Since there is such a small number of cases that have been reported, the treatment guidelines have not been established. A larger number of cases that contain a longer clinical follow-up are needed to optimize the management of patients with this rare disease.
Even though there is a general consensus on the morphology and the immunohistochemical characteristics that is required for the diagnosis, the histological grading criteria have yet to be fully defined and its biological behavior appears to be variable. This specific type of tumor appears to have a high potential for local recurrence with a high tumor bed recurrence rate during the five years after the initial surgery. This suggests the need for a tumor bed boost radiotherapy after surgical resection.
As stated above, the specific treatment guidelines have not yet been established, however, gross total resection of the tumor has been the only clinical factor associated overall and progression-free survival. The value of radiotherapy as well as chemotherapy on disease progression will need to be investigated in future trials. With this information, it will provide important insight into long-term management and may further our understanding of the histologic features of this tumor.
Because of the unclear pathogenesis and pathophysiology of Tarlov cysts, there is no consensus on the optimal treatment of symptomatic sacral perineural cysts. Patients often choose to pursue treatment when the progression of neurological deficits seriously impacts their quality of life.
Since cysts are innervated, microfenestration and surgical sleeving of the cysts to diminish the amount of accumulated cerebrospinal fluid and decrease compression of the spine and spinal nerves has been successful in a number of patients. The cysts are carefully separated enough from surrounding tissue to be wrapped with fatty tissue or pericardial biomaterial to excise the fluid from the cyst. If the cyst does not drain spontaneously, then it is drained and patched using a biosynthetic dural patch.
The use of this technique is done in the U.S. and is spreading in Europe but recovery is generally extensive. Microfenestration alone has been done with some success in Asia.
A biopolymer plate is also being used experimentally to strengthen a sacrum thinned by cystic erosion by Dr. Frank Feigenbaum.
The risks of CSF leakage are higher on patients that have bilateral cysts on the same spinal level or clusters of cysts along multiple vertebrae, but immediate recognition of the leakage and repair can mitigate that risk.
Various treatment methods have been tried in the past, including the extraction of cerebrospinal fluids from the cyst, fibrin glue injection and the complete or partial removal of cyst. Epidurals can provide temporary relief but are not generally recommended as they can cause cysts to enlarge. Extraction of fluid can provide limited or no relief depending on rate the cysts refill and the need to repeat the procedure. Removal of the cyst results in irreversible damage to the intersecting spinal nerve.
Although fibrin-glue therapy initially had been thought to be a promising therapy in the treatment of these cysts, there have been multiple problems associated with the fibrin glue therapy including seepage of fibrin. It is no longer recommended for use at present by the Health Department in some countries and neurosurgeons previously performing the procedures.
Nevertheless, all types of surgical treatment pose common risks, including neurological deficits, infection and inflammation, spinal headache, urinary disturbances, and leakage of cerebrospinal fluids.
Here is an article for treatment of meningeal diverticulum. Feigenbaum F1, Henderson FC. Giant sacral meningeal diverticula: surgical implications of the "thecal tip" sign. Report of two cases. J Neurosurg Spine. 2006 Nov;5(5):443-6.
MEM comprises a heterogeneous group of neoplasms believed to originate from the neural crest. First hints to this type of tumor were probably from Shuangshoti and Nestky (1971) and from Holimon and Rosenblum (1971) (2-3). Additional contributions were provided thereafter by Naka et al. (1975), Karcioglu et al. (1977), Cozzutto et al. (1982) and Kawamoto et al. (1987).
Kosem et al. collected 44 cases of MEM in a 2004 review and examined management data finding out that resection with pre- or post-surgery chemotherapy yielded the best results with one death only in 13. In the five cases reported by Mouton et al. an aggressive chemotherapy and adequate surgical excision granted a disease-free interval for 7 to 50 months. The attainability of radical surgical
ablation seems the most important prognostic factor (10).
Besides the frequent choice to leave the cyst in place, surgical treatments remain the primary elective option for treatment of ganglion cysts. The progression of ganglion surgery worldwide is to use an arthroscopic or mini-opening method. Alternatively, a hypodermic needle may be used to drain the fluid from the cyst (via aspiration) and a corticosteroid may be injected after the cyst is empty; however, if the fluid has thickened, owing to the passage of time, this treatment is not always effective.
There is a recurrence rate of approximately 50% following needle drainage (via aspiration) of ganglion cysts.
One common and traditional method of treatment for a ganglion cyst was to strike the lump with a large and heavy book, causing the cyst to rupture and drain into the surrounding tissues. Historically, a Bible was the largest or only book in any given household, and was often employed for this treatment. This led to the former nickname of "Bible bumps" or "Gideon's disease" for these cysts. This treatment risks injuring the patient.
The treatment of a Pancoast lung cancer may differ from that of other types of non-small cell lung cancer. Its position and close proximity to vital structures (such as nerves and spine) may make surgery difficult. As a result, and depending on the stage of the cancer, treatment may involve radiation and chemotherapy given prior to surgery (neoadjuvant treatment).
Surgery may consist of the removal of the upper lobe of a lung together with its associated structures (subclavian artery, vein, branches of the brachial plexus, ribs and vertebral bodies), as well as mediastinal lymphadenectomy. Surgical access may be via thoracotomy from the back or the front of the chest and modifications
Complications of treatment may include joint stiffness and scar formation. Recurrence of the lesion is more common following excision of a volar ganglion cyst in the wrist. Incomplete excision that fails to include the stalk or pedicle also may lead to recurrence, as will failing to execute a layered closure of the incision.
Treatment of THS includes immunosuppressives such as corticosteroids (often prednisolone) or steroid-sparing agents (such as methotrexate or azathioprine).
Radiotherapy has also been proposed.
Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults.
The prognosis of THS is usually considered good. Patients usually respond to corticosteroids, and spontaneous remission can occur, although movement of ocular muscles may remain damaged. Roughly 30–40% of patients who are treated for THS experience a relapse.