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The gold standard for treatment of umbilical cord prolapse in the setting of a viable pregnancy typically involves immediate delivery by the quickest and safest route possible. This usually requires cesarean section, especially if the woman is in early labor. Occasionally, vaginal delivery will be attempted if clinical judgment determines that is a safer or quicker method.
Other interventions during management of cord prolapse are typically used to decrease the chance of complications while preparations for delivery are being made. These interventions are focused on reducing pressure on the cord to prevent fetal complications from cord compression. The following maneuvers are among those used in clinical practice:
- manual elevation of the presenting fetal part
- repositioning of the mother to be head down with feet elevated
- filling of the bladder with a foley catheter, or tube through the urethra to elevate the presenting fetal part
- use of tocolytics (medications to suppress labor) have been proposed, usually in addition to bladder filling rather than a standalone intervention
If the mother is far from delivery, funic reduction (manually placing the cord back into the uterine cavity) has been attempted, with successful cases reported.
Retrospective data of over 182,000 births, with the statistical power to determine even mild associations, suggest that a single or multiple nuchal cords at the time of delivery is not associated with adverse perinatal outcomes, is associated with higher birthweights and fewer caesarean sections in births. Although some studies have found that a tight nuchal cord is associated with short term morbidity, it is unclear whether such outcomes are actually a result of the presence of the nuchal cord itself, or as a result of clamping and cutting the cord
Management of a presenting nuchal cord should be tailored to prevent umbilical cord compression whenever possible. Techniques to preserve an intact nuchal cord depend on how tightly the cord is wrapped around the infant’s neck. If the cord is loose, it can easily be slipped over the infant’s head. The infant can be delivered normally and placed on maternal abdomen as desired. If the cord is too tight to go over the infant’s head, the provider may be able to slip it over the infant’s shoulders and deliver the body through the cord. The cord can then be unwrapped from around the baby after birth. Finally, if the cord is too tight to slip back over the shoulders, one may use the somersault maneuver to allow the body to be delivered. The birth attendant may also choose to clamp and cut the umbilical cord to allow for vaginal delivery if other methods of nuchal cord management are not feasible.
Surgery is not always recommended for syringomyelia patients. For many patients, the main treatment is analgesia. Physicians specializing in pain management can develop a medication and treatment plan to ameliorate pain. Medications to combat any neuropathic pain symptoms such as shooting and stabbing pains (e.g. gabapentin or pregabalin) would be first-line choices. Opiates are usually prescribed for pain for management of this condition. Facet injections are not indicated for treatment of syringomyelia.
Drugs have no curative value as a treatment for syringomyelia. Radiation is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.
In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.
Since the natural history of syringomyelia is poorly understood, a conservative approach may be recommended. When surgery is not yet advised, patients should be carefully monitored. Periodic MRI's and physical evaluations should be scheduled at the recommendation of a qualified physician.
The first step after diagnosis is finding a neurosurgeon who is experienced in the treatment of syringomyelia. Surgery is the treatment for syringomyelia. Evaluation of the condition is necessary because syringomyelia can remain stationary for long periods of time, and in some cases progress rapidly.
Surgery of the spinal cord has certain characteristic risks associated with it, and the benefits of a surgical procedure on the spine have to be weighed against the possible complications associated with any procedure. Surgical treatment is aimed at correcting the condition that allowed the syrinx to form. It is vital to bear in mind that the drainage of a syrinx does not necessarily mean the elimination of the syrinx-related symptoms but rather is aimed at stopping progression. In cases involving an Arnold-Chiari malformation, the main goal of surgery is to provide more space for the cerebellum at the base of the skull and upper cervical spine without entering the brain or spinal cord. This often results in flattening or disappearance of the primary syrinx or cavity, over time, as the normal flow of cerebrospinal fluid is restored. If a tumor is causing syringomyelia, removal of the tumor is the treatment of choice, if this is considered to be safe.
Surgery results in stabilization or modest improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury. Recurrence of syringomyelia after surgery may make additional operations necessary; these may not be completely successful over the long term.
In some patients it may also be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. This system is also known as a shunt. Shunts are used in both the communicating and noncommunicating forms of the disorder. First, the surgeon must locate the syrinx. Then, the shunt is placed into it with the other end draining cerebrospinal fluid (CSF) into a cavity, usually the abdomen. This type of shunt is called a ventriculoperitoneal shunt and is particularly useful in cases involving hydrocephalus. By draining syrinx fluid, a shunt can arrest the progression of symptoms and relieve pain, headache, and tightness. Syringomyelia shunts are not always successful and can become blocked as with other central nervous system shunts.
The decision to use a shunt requires extensive discussion between doctor and patient, as this procedure carries with it greater risk of injury to the spinal cord, infection, blockage, or hemorrhage and may not necessarily work for all patients. Draining the syrinx more quickly does not produce better outcomes, but a shunt may be required if the fluid in the syrinx is otherwise unable to drain.
In the case of trauma-related syringomyelia, the surgeon operates at the level of the initial injury. The syrinx collapses at surgery, but a tube or shunt is usually necessary to prevent re-expansion.
Surgery
Surgical intervention is warranted in patients who present with new onset neurological signs and symptoms or have a history of progressive neurological manifestations which can be related to this abnormality. The surgical procedure required for the effective treatment of diastematomyelia includes decompression (surgery) of neural elements and removal of bony spur. This may be accomplished with or without resection and repair of the duplicated dural sacs. Resection and repair of the duplicated dural sacs is preferred since the dural abnormality may partly contribute to the "tethering" process responsible for the symptoms of this condition.
Post-myelographic CT scanning provides individualized detailed maps that enable surgical treatment of cervical diastematomyelia, first performed in 1983.
Observation
Asymptomatic patients do not require surgical treatment. These patients should have regular neurological examinations since it is known that the condition can deteriorate. If any progression is identified, then a resection should be performed.
Potential non-surgical treatments include:
- Education about the course of the condition and how to relieve symptoms
- Medicines to relieve pain and inflammation, such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs)
- Exercise, to maintain or achieve overall good health, aerobic exercise, such as riding a stationary bicycle, which allows for a forward lean, walking, or swimming can relieve symptoms
- Weight loss, to relieve symptoms and slow progression of the stenosis
- Physical therapy, to provide education, instruction, and support for self-care; physical therapy instructs on stretching and strength exercises that may lead to a decrease in pain and other symptoms
Potential surgical treatments include:
- Anterior cervical discectomy and fusion - A surgical treatment of nerve root or spinal cord compression by decompressing the spinal cord and nerve roots of the cervical spine with a discectomy in order to stabilize the corresponding vertebrae.
- Laminoplasty - A surgical procedure relieve pressure on the spinal cord by cutting the lamina on both sides of the affected vertebrae (cutting through on one side and merely cutting a groove on the other) and then "swinging" the freed flap of bone open.
- Laminectomy - A surgical procedure in which the lamina of the vertebra is removed or trimmed to widen the spinal canal and create more space for the spinal nerves and thecal sac.
Dexamethasone (a potent glucocorticoid) in doses of 16 mg/day may reduce edema around the lesion and protect the cord from injury. It may be given orally or intravenously for this indication.
Surgery is indicated in localised compression as long as there is some hope of regaining function. It is also occasionally indicated in patients with little hope of regaining function but with uncontrolled pain. Postoperative radiation is delivered within 2–3 weeks of surgical decompression. Emergency radiation therapy (usually 20 Gray in 5 fractions, 30 Gray in 10 fractions or 8 Gray in 1 fraction) is the mainstay of treatment for malignant spinal cord compression. It is very effective as pain control and local disease control. Some tumours are highly sensitive to chemotherapy (e.g. lymphomas, small-cell lung cancer) and may be treated with chemotherapy alone.
Once complete paralysis has been present for more than about 24 hours before treatment, the chances of useful recovery are greatly diminished, although slow recovery, sometimes months after radiotherapy, is well recognised.
The median survival of patients with metastatic spinal cord compression is about 12 weeks, reflecting the generally advanced nature of the underlying malignant disease.
There is no known treatment to reverse nerve damage due to myelomalacia. In some cases, surgery may slow or stop further damage. As motor function degenerates, muscle spasticity and atrophy may occur. Steroids may be prescribed to reduce swelling of the spinal cord, pain, and spasticity.
Research is underway to consider the potential of stem cells for treatment of neurodegenerative diseases. There are, however, no approved stem cell therapies for myelomalacia.
If left untreated, the pump twin will die in 50–75% of cases.
After diagnosis, ultrasound and amniocentesis are used to rule out genetic abnormalities in the pump twin. A procedure may then be performed which will stop the abnormal blood flow. The acardiac twin may be selectively removed. The umbilical cord of the acardiac twin may be surgically cut, separating it from the pump twin, a procedure called fetoscopic cord occlusion. Or a radio-frequency ablation needle may be used to coagulate the blood in the acardiac twin's umbilical cord. This last procedure is the least invasive. These procedures greatly increase the survival chances of the pump twin, to about 80%.
The pump twin will be monitored for signs of heart failure with echocardiograms. If the pump twin's condition deteriorates, the obstetrician may recommend early delivery. Otherwise, the pregnancy continues normally. Vaginal birth is possible unless the fetus is in distress, although it is recommended that the delivery take place at a hospital with NICU capabilities.
If there aren't neurological symptoms (such as difficulties moving, loss of sensation, confusion, etc.) and there is no evidence of pressure on the spinal cord, a conservative approach may be taken such as:
- Drugs, such as aspirin, without steroids to relieve inflammation
- Cervical traction, in which the neck is pulled along its length, thus relieving pressure on the spinal cord
- Using a neck collar or cervical-thoracic suit
If there is pressure on the spinal cord or life-threatening symptoms are present, surgery is recommended.
Initial care in the hospital, as in the prehospital setting, aims to ensure adequate airway, breathing, cardiovascular function, and spinal immobilization. Imaging of the spine to ascertain presence of SCI may need to wait if emergency surgery is needed to stabilize a life-threatening injury. Acute SCI merits treatment in an intensive care unit, especially injuries to the cervival spinal cord. Patients with SCI need repeated neurological assessments and treatment by neurosurgeons.
If the systolic blood pressure falls below 90 mmHg within days of the injury, blood supply to the spinal cord may be reduced, resulting in further damage. Thus it is important to maintain the blood pressure using a central venous catheter, intravenous fluids, and vasopressors, and to treat cases of shock. Mean arterial blood pressure is measured and kept at 85 to 90 mmHg for seven days after injury. The treatment for shock from blood loss (hypovolemic shock) is different from that for neurogenic shock, and could harm people with the latter type, so it is necessary to determine why someone is in shock. However it is also possible for both causes to exist at the same time. Another important aspect of care is prevention of hypoxia (insufficient oxygen in the bloodstream), which could deprive the spinal cord of much-needed oxygen. People with cervical injuries may experience a dangerously slowed heart rate; treatment to speed it up include atropine and electrical cardiac pacing.
Swelling can cause further damage to the spinal cord by reducing the blood supply and causing ischemia, which can give rise to an ischemic cascade with a release of toxins that damages neurons. Thus treatment is often geared toward limiting this secondary injury. People are sometimes treated with drugs to reduce swelling. The corticosteroid drug methylprednisolone is commonly used within eight hours of the injury, but its use is controversial because of side effects. Studies have shown high dose methylprednisolone may improve outcomes if given within 6 hours of injury. However, the improvement shown by clinical trials has been inconclusive, and comes at the cost of increased risk of serious infection or sepsis, gastrointestinal bleeding, and pneumonia. Thus organizations that set clinical guidelines have increasingly stopped recommending methylprednisolone in the treatment of acute SCI.
Surgery may be necessary, e.g. to relieve excess pressure on the cord, to stabilize the spine, or to put vertebrae back in their proper place. In cases involving instability or compression, failing to operate can lead to worsening of the condition. Surgery is also necessary when something is pressing on the cord, such as bone fragments, blood, material from ligaments or intervertebral discs, or a lodged object from a penetrating injury. Although the ideal timing of surgery is still debated, studies have found that earlier surgical intervention (within 24 hours of injury) is associated with better outcomes. Sometimes a patient has too many other injuries to be a surgical candidate this early. Surgery is controversial because it has potential complications (such as infection), so in cases where it is not clearly needed (e.g. the cord is being compressed), doctors must decide whether to perform surgery based on aspects of the patient's condition and their own beliefs about its risks and benefits.
In cases where a more conservative approach is chosen, bed rest, cervical collars, immobilizing devices, and optionally traction are used. Surgeons may opt to put traction on the spine to remove pressure from the spinal cord by putting dislocated vertebrae back into alignment, but herniation of intervertebral disks may prevent this technique from relieving pressure. "Gardner-Wells tongs" are one tool used to exert spinal traction to reduce a fracture or dislocation and to immobilize the affected areas.
Surgical intervention is usually given to those individuals who have increased instability of their cervical spine, which cannot be resolved by conservative management alone. Further indications for surgery include a neurological decline in spinal cord function in stable patients as well as those who require cervical spinal decompression.
A nuchal cord occurs when the umbilical cord becomes wrapped around the fetal neck 360 degrees. Nuchal cords are common, with prevalence rates of 6% to 37%. Up to half of nuchal cords resolve before delivery.
The primary concern with umbilical cord prolapse is inadequate blood supply, and thus oxygen, to the fetus if the cord becomes compressed. The cord can become compressed either due to mechanical pressure (usually from the presenting fetal part) or from sudden contraction of the vessels due to decreased temperatures in the vagina in comparison to the uterus. This can lead to death of the fetus or other complications.
Historically, the rate of fetal death in the setting of cord prolapse has been as high 40%. However, these estimates occurred in the context of home or births outside of the hospital. When considering cord prolapses that have occurred in inpatient labor and delivery settings, the rate drops to as low as 0-3%, though the mortality rate remains higher than for fetuses without cord prolapse. The reduction in mortality for hospital births is likely due to the ready availability of immediate cesarean section.
Many other fetal outcomes have been studied, including Apgar score (a quick assessment of a newborn's health status) at 5 minutes and length of hospitalization after delivery. While both measures are worse for newborns delivered after cord prolapse, it is unclear what effect this has in the long-term. Relatively large studies that have tried to quantify long-term effects of cord prolapse on children found that less than 1% (1 in 120 studied) suffered a major neurologic handicap, and less than 1% (110 in 16,675) had diagnosed cerebral palsy.
In many cases, individuals with CCS can experience a reduction in their neurological symptoms with conservative management. The first steps of these intervention strategies include admission to an intensive care unit (ICU) after initial injury. After entering the ICU, early immobilization of the cervical spine with a neck collar would be placed on the patient to limit the potential of further injury. Cervical spine restriction is maintained for approximately six weeks until the individual experiences a reduction in pain and neurological symptoms. Inpatient rehabilitation is initiated in the hospital setting, followed by outpatient physical therapy and occupational therapy to assist with recovery.
An individual with a spinal cord injury may have many goals for outpatient occupational and physiotherapy. Their level of independence, self-care, and mobility are dependent on their degree of neurological impairment. Rehabilitation organization and outcomes are also based on these impairments. The physiatrist, along with the rehabilitation team, work with the patient to develop specific, measurable, action-oriented, realistic, and time-centered goals.
With respect to physical therapy interventions, it has been determined that repetitive task-specific sensory input can improve motor output in patients with central cord syndrome. These activities enable the spinal cord to incorporate both supraspinal and afferent sensory information to help recover motor output. This occurrence is known as "activity dependent plasticity". Activity dependant plasticity is stimulated through such activities as: locomotor training, muscle strengthening, voluntary cycling, and functional electrical stimulation (FES) cycling
SCI patients often require extended treatment in specialized spinal unit or an intensive care unit. The rehabilitation process typically begins in the acute care setting. Usually the inpatient phase lasts 8–12 weeks and then the outpatient rehabilitation phase lasts 3–12 months after that, followed by yearly medical and functional evaluation. Physical therapists, occupational therapists, recreational therapists, nurses, social workers, psychologists and other health care professionals work as a team under the coordination of a physiatrist to decide on goals with the patient and develop a plan of discharge that is appropriate for the person’s condition.
In the acute phase physical therapists focus on the patient’s respiratory status, prevention of indirect complications (such as pressure ulcers), maintaining range of motion, and keeping available musculature active.
For people whose injuries are high enough to interfere with breathing, there is great emphasis on airway clearance during this stage of recovery. Weakness of respiratory muscles impairs the ability to cough effectively, allowing secretions to accumulate within the lungs. As SCI patients suffer from reduced total lung capacity and tidal volume, physical therapists teach them accessory breathing techniques (e.g. apical breathing, glossopharyngeal breathing) that typically are not taught to healthy individuals. Physical therapy treatment for airway clearance may include manual percussions and vibrations, postural drainage, respiratory muscle training, and assisted cough techniques. Patients are taught to increase their intra-abdominal pressure by leaning forward to induce cough and clear mild secretions. The quad cough technique is done lying on the back with the therapist applying pressure on the abdomen in the rhythm of the cough to maximize expiratory flow and mobilize secretions. Manual abdominal compression is another technique used to increase expiratory flow which later improves coughing. Other techniques used to manage respiratory dysfunction include respiratory muscle pacing, use of a constricting abdominal binder, ventilator-assisted speech, and mechanical ventilation.
The amount of functional recovery and independence achieved in terms of activities of daily living, recreational activities, and employment is affected by the level and severity of injury. The Functional Independence Measure (FIM) is an assessment tool that aims to evaluate the function of patients throughout the rehabilitation process following a spinal cord injury or other serious illness or injury. It can track a patient's progress and degree of independence during rehabilitation. People with SCI may need to use specialized devices and to make modifications to their environment in order to handle activities of daily living and to function independently. Weak joints can be stabilized with devices such as ankle-foot orthoses (AFOs) and knee-AFOs, but walking may still require a lot of effort. Increasing activity will increase chances of recovery.
The procedure of spine shortening via vertebral osteotomy (SSVO) for TCS is a surgical technique that avoids the complication with revision tethering. In this research a lateral retropleural approach was used for SSVO in recurrent TCS in a 21-year-old female. The patient presented with progressive lower extremity weakness, bowel and bladder incontinence, and back pain in the setting of childhood repair of mylomeningocele and two previous detethering procedures. The research on performing SSVO in this patient allowed the scientists to conclude that SSVO via lateral retropleural approach is a good treatment for the recurrence of TCS. This procedure is minimally invasive compared to the posterior approach which gives the advantages of having direct access to the vertebral body and disc while avoiding the need to have an operation near the spinal cord but further research is still needed.
A baby with a prenatally diagnosed cystic hygroma should be delivered in a major medical center equipped to deal with neonatal complications, such as a neonatal intensive care unit. An obstetrician usually decides the method of delivery. If the cystic hygroma is large, a cesarean section may be performed. After birth, infants with a persistent cystic hygroma must be monitored for airway obstruction. A thin needle may be used to reduce the volume of the cystic hygroma to prevent facial deformities and airway obstruction. Close observation of the baby by a neonatologist after birth is recommended. If resolution of the cystic hygroma does not occur before birth, a pediatric surgeon should be consulted.
Cystic hygromas that develop in the third trimester, after thirty weeks gestation, or in the postnatal period are usually not associated with chromosome abnormalities. There is a chance of recurrence after surgical removal of the cystic hygroma. The chance of recurrence depends on the extent of the cystic hygroma and whether its wall was able to be completely removed.
Treatments for removal of cystic hygroma are surgery or sclerosing agents which include:
- Bleomycin
- Doxycycline
- Ethanol (pure)
- Picibanil (OK-432)
- Sodium tetradecyl sulfate
The treatment and prognosis of myelopathy depends on the underlying cause: myelopathy caused by infection requires medical treatment with pathogen specific antibiotics. Similarly, specific treatments exist for multiple sclerosis, which may also present with myelopathy. As outlined above, the most common form of myelopathy is secondary to degeneration of the cervical spine. Newer findings have challenged the existing controversy with respect to surgery for cervical spondylotic myelopathy by demonstrating that patients benefit from surgery.
Because neurological deficits are generally irreversible, early surgery is recommended when symptoms begin to worsen. In children, early surgery is recommended to prevent further neurological deterioration, including but not limited to chronic urinary incontinence.
In adults, surgery to detether (free) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms. Although detethering is the common surgical approach to TCS, another surgical option for adults is a spine-shortening vertebral osteotomy. A vertebral osteotomy aims to indirectly relieve the excess tension on the spinal cord by removing a portion of the spine, shortening it. This procedure offers a unique benefit in that the spinal cord remains fixated to the spine, preventing retethering and spinal cord injury as possible surgical complications. However, its complexity and limited “track record” presently keeps vertebral osteotomies reserved as an option for patients who have failed in preventing retethering after detethering procedure(s).
Other treatment is symptomatic and supportive. Medications such as NSAIDs, opiates, synthetic opiates, COX-2 inhibitors, and off-label applications of tricyclic antidepressants combined with anti-seizure compounds have yet to prove they are of value in treatment of this affliction's pain manifestations. There is anecdotal evidence that TENS units may benefit some patients.
Treatment may be needed in adults who, while previously asymptomatic, begin to experience pain, lower back degeneration, scoliosis, neck and upper back problems and bladder control issues. Surgery on adults with minimal symptoms is somewhat controversial. For example, a website from the Columbia University Department of Neurosurgery says, "For the child that has reached adult height with minimal if any symptoms, some neurosurgeons would advocate careful observation only." However, surgery for those who have worsening symptoms is less controversial. If the only abnormality is a thickened, shortened filum, then a limited lumbosacral laminectomy with division of the filum may be sufficient to relieve the symptoms.
This syndrome was first noticed in the late 19th century. While information has been available for years, little widespread blind research has been done. More research has been called for, and doctors have conducted many studies with good results. There is a low morbidity rate, and no complications have been documented other than those typical of any type of back surgery. The association of this condition with others has been noticed, and needs further research to understand such relationships. TCS is causally linked to Chiari malformation and any affirmative diagnosis of TCS must be followed by screening for Chiari's several degrees. TCS may also be related to Ehlers-Danlos syndrome, or Klippel-Feil syndrome, which should also be screened for upon a positive TCS diagnosis. Spinal compression and the resulting relief is a known issue with this disorder. Like with the early-onset form, this disease form is linked to the Arnold-Chiari malformation, in which the brain is pulled or lowers into the top of the spine.
Systemic (intravenous or oral) chemotherapy and intrathecal chemotherapy: Intrathecal therapy is when injection is done directly to the spinal cord into the sub-arachnoid space to avoid the Blood-Brain-Barrier (BBB) and gain direct access to the CSF. Intrathecal Therapy is preferred since intravenous chemotherapy do not penetrate the BBB. The most common chemicals used are liposomal cytarabine (DepoCyte) and intrathecal methotrexate (MTX).
In combination, intrathecal chemotherapy most often comprises methotrexate, cytarabine, thiotepa and steroids. Ventriculoperitoneal shunts may also be applied with chemotherapy to avoid invasive surgery to gain access to the CSF.
An example of treatment:
Intrathecal MTX injection at a dose of 15 mg/day for 5 days every other week with hydrocortisone acetate injecting IT on day one to prevent arachnoiditis, the inflammation of the arachnoid. MTX administration is continued until neurological progression or relapse occurred. Systemic chemotherapy, radiotherapy, and surgery are performed depending on the need of the patient.
Risks of treatments:
Both Chemotherapy and Radiotherapy are harmful to the body and most definitely the brain. Caution must be utilized in treating patients with NM. Another factor that makes treatment difficult is that there is no suitable method to evaluate the disease progression.
Umbilical cord compression may be relieved by the mother switching to another position. In persistent severe signs of fetal distress, Cesarean section may be needed.
Treatment is directed at the pathology causing the paralysis. If it is because of trauma such as a gunshot or knife wound, there may be other life-threatening conditions such as bleeding or major organ damage which should be dealt with on an emergent basis. If the syndrome is caused by a spinal fracture, this should be identified and treated appropriately. Although steroids may be used to decrease cord swelling and inflammation, the usual therapy for spinal cord injury is expectant.
There is no standard treatment that has been established for NM thus treatments are almost always palliative.
Radiotherapy:
This method is used mostly for focal type of NM due to the nature of damage and success rate associated with the treatment. Radiotherapy targets and tumor and destroys the collective tissues of cancerous cells.