disponnect n.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
DisPONnect PowerPoint Presentation
Download Presentation
DisPONnect

Loading in 2 Seconds...

play fullscreen
1 / 128

DisPONnect - PowerPoint PPT Presentation


  • 122 Views
  • Uploaded on

DisPONnect. A Case of Pontine Glioma. The Medical City | Department of Pediatrics ASMPH Interns – Group 2. Outline. Patient Information and Data Approach to Diagnosis Course in the Wards Diagnostics Therapeutics Prognosis and Complications Biopsychosocial Aspect: Palliative Care.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'DisPONnect' - monet


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
disponnect

DisPONnect

A Case of Pontine Glioma

The Medical City | Department of Pediatrics

ASMPH Interns – Group 2

outline
Outline
  • Patient Information and Data
  • Approach to Diagnosis
  • Course in the Wards
  • Diagnostics
  • Therapeutics
  • Prognosis and Complications
  • Biopsychosocial Aspect: Palliative Care
disponnect1

DisPONnect

Patient History

A Case of Pontine Glioma

identifying information
Identifying Information

Patient Name: SA

Age: 8 years old

Nationality: Filipino

Religion: Roman Catholic

Handedness: Right

Admitted: November 15, 2013

Information: EC and RA, patient’s parents

Good Reliability

chief complaint

Chief Complaint

Inarticulation

(Nabubulol at Nagba-babytalk)

past medical history
Past Medical History
  • Past Illnesses
    • No previous history of cancer, stroke, seizures, eye correction, pneumonia, PTB, cardiac disease, hypertension, diabetes, asthma, kidney or thyroid disease
  • Hospitalizations
    • Previously admitted for Dengue Fever in 2012
  • Surgeries
    • No previous surgical procedures
  • Trauma
    • No history of trauma
  • Allergies
    • No allergies to food or medications
  • Medication
    • No current medication use
slide12

Family History

  • Patient is of Filipino descent from Maybunga, Pasig City
  • Bronchial Asthma in the maternal aunt
  • No family history of cancer, stroke, seizures, diabetes, hypertension, heart disease, allergies
  • Household Members:
    • Patient
    • Patient’s Siblings
    • Patient’s Parents
    • Patient’s maternal Aunt
history of birth and infancy
History of Birth and Infancy

Birth History

Nutritional History

Not breastfed

Due to low maternal milk production

Formula Milk: NAN HA, Gain, Lactum

Weaned at 6mo of age

Current Diet: meats, vegetables and fruits

Preferences: sour foods (e.g. sinigangisda)

  • Born full term via normal spontaneous delivery to a 37-year old G3P3 (3003)
  • Birth Weight: 3.08kg
  • Good Activity, Good Cry
  • Attended by: OB-GYN
  • No perinatal or neonatal complications
history of childhood
History of Childhood

Developmental History

Personal and Social History

Grade 3 Student

Above average performance (6th honor)

Favorite Subject: Science and English

Hobbies: Spend time with friends, singing and dancing

Has shown interest in the opposite sex, but has no crushes

  • Gross Motor
    • Able to do backward heel to toe walk
  • Fine Motor
    • Able to draw a complete person
    • Can write fairly well
  • Language
    • Can add and subtract
    • Can distinguish between left and right
  • Personal/Social
    • Can dress self completely
environmental history
Environmental History
  • Residence: 1-story cement structure
    • Maybunga, Pasig City
  • Electricity: Meralco
  • Water: Manila Water Company, Inc.
  • Near to major roads, but not near any factory
  • No exposure to tobacco, toxins or environmental hazards
  • Waste: Daily, not segregated
disponnect2

DisPONnect

Physical Examination

A Case of Pontine Glioma

physical examination
Physical Examination

Anthropometrics

Weight: 34.5kg

Z-score (0,2)

Height: 138cm

Z-score (0,2)

BMI: 18.11kg/m2

Vital Signs

BP: 118/76mmHg

HR: 82bpm

RR: 20cpm

Temperature: 36.5C

Pain: 0/10

General Survey

Awake, Alert

Not in CardioRespiratory Distress

GCS 15

physical examination1
Physical Examination
  • Eyes:
    • Anicteric sclerae, pink palpebral conjunctivae, no cataracts or discharge
  • Skin:
    • Fair color, no rashes, good skin turgor, hair evenly distributed, nails with no clubbing
  • Ears:
    • No visible mass or lesion, no discharge, no auricular tenderness, patent canal, intact tympanic membrane with cone of light
physical examination2
Physical Examination
  • Nose
    • No deformities, no nasal discharge, no nasal congestion
  • Throat
    • Lips moist and pink, no cleft lip or palate, no tonsillopharyngeal congestion
  • Neck
    • Flat neck veins, no cervical lymphadenopathy
physical examination3
Physical Examination
  • Chest/Lungs
    • Symmetric chest expansion, no retractions, clear breath sounds, no rales, no wheezes
  • Cardiovascular
    • Adynamic precordium, normal rate, regular rhythm, good S1/S2, no murmurs, heaves or thrills
  • Abdomen
    • Flat, no previous surgical scars, normoactive bowel sounds, no masses palpated, no organomegaly, no tenderness
physical examination4
Physical Examination
  • Genitalia
    • Grossly female genitalia, no discharge
  • Extremities
    • Full and equal pulses, no edema, no cyanosis, CRT <2 seconds
neurologic examination3
Neurologic Examination

++

++

++

++

++

++

No Flaccidity or Rigidity

No Atrophy or Hypertrophy

++

++

neurologic examination4
Neurologic Examination
  • Cerebellar
    • Dragging gait on the left
    • Dysdiadochokinesia: Left
    • Dysmetria: Left
  • Babinski: Bilateral
  • Meningeal signs
    • Negative Kernig’s and Brudzinski’s sign
    • No neck rigidity
salient features
Salient Features

SUBJECTIVE

OBJECTIVE

Stable VS, GCS 15

Shallow nasolabialfold, right

Dysarthria

Absent gag reflex

Left-sided motor weakness (4/5)

(+) Dysdiadochokinesia, dysmetria, left

(+) Dragging gait

(+) Babinski, bilateral

  • 8-year old female
  • No history of neurologic disease
  • 3 week history of right-sided facial weakness
  • 6 day history of drooling, dysphagia and slurred speech
  • Left-sided weakness
  • Unstable gait
disponnect3

DisPONnect

Approach to Diagnosis

A Case of Pontine Glioma

what is the lesion1

Arteriovenous Malformation

What is the Lesion?
  • Abnormal shunting of blood  expansion of vessels and a space-occupying effect or rupture of a vein and intracerebral bleeding
  • May remain asymptomatic throughout life but can rupture and bleed any time
  • History of ipsilateral seizures and migraine-like headaches
  • Ruptured AV malformation: severe headache, vomiting, nuchal rigidity, progressive hemiparesis, and seizure
what is the lesion2

Aneurysm

What is the Lesion?
  • Usually asymptomatic
  • Located at the carotid bifurcation or on the anterior and posterior cerebral arteries rather than the circle of Willis.
  • Results from a congenital weakness of the vessel
  • Ruptured aneurysms: intense headache, nuchal rigidity, coma, intracerebral hemorrhage and progressive hemiparesis
what is the lesion3

Meningitis

What is the Lesion?
  • Acute infection of the central nervous system (CNS)
  • May present acutely, subacutely and chronically (>1week)
  • Often preceded by fever, respiratory or gastrointestinal symptoms, followed by nonspecific signs of CNS infection such as increasing lethargy and irritability
  • Systemic infection + meningeal symptoms, seizures and altered mental status
what is the lesion4

Brain Lesion

What is the Lesion?
  • Most common in children 4 -8 years old
  • Causes: emboli, meningitis, chronic otitis media and mastoiditis, sinusitis, soft tissue infection of the face or scalp, orbital cellulitis, dental infections, penetrating head injuries, immunodeficiency states, and infection of ventriculoperitoneal shunts
  • 80% of abscesses are found in the frontal, parietal and temporal lobes
  • Clinical presentation: low grade fever, headache and lethargy  vomiting, severe headache, seizures, papilledema, focal neurologic signs (hemiparesis), coma
  • Cerebellar abscess: nystagmus, ipsilateral ataxia and dysmetria, vomiting, and headache
what is the lesion5

Primary CNS Lesion

  • Metastatic Lesion
What is the Lesion?
  • 2nd most frequent malignancy in childhood
  • Higher incidence in children >7 years
  • Progressive Symptoms
  • Brainstem tumor effects: motor weakness, cranial nerve deficits, cerebellar deficits, and/or signs of increased intracranial pressure
  • Uncommon
  • Primary neoplasia: ALL, lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, osteosarcoma, and clear cell sarcoma of the kidney
slide41

Baby Talk and Slurring of Speech Dysarthria

  • Dysarthria: disorders in articulating speech sounds
    • Vs. Dysphonia
    • Vs. Dysprosody
    • Vs. Dysphasia
  • Motor paralysis of organs of articulation
dysarthria
Dysarthria

Dysarthria

Cause of Dysarthria

  • Drooling + Dysphagia
    • Swallowing Problem
  • Absent Gag Reflex

CRANIAL NERVE IX and X Palsy

Dysarthria: http://trialx.com/curetalk/wp-content/blogs.dir/7/files/2011/05/diseases/Dysarthria-1.jpg

central facial nerve palsy right
Central Facial Nerve Palsy, Right
  • Possible Location of Lesion:
    • Left Corticobulbar Tract
    • Above the Facial Nucleus (located at the Pons)
left sided weakness
Left-Sided Weakness
  • Corticospinal Tract
    • Cerebral Cortex
    • Mesencephalon
    • Pons
    • Medulla
    • Spinal Cord
  • Contralateral lesion above decussation
cerebellar signs
Cerebellar Signs
  • Unsteady gait
  • Dysmetria, Left
  • Dysdiadochokinesia, Left
  • Possible Locations:
    • Cerebrum
    • Cerebellum
    • Midbrain
    • Pons
    • Midbrain

http://www.asn.org/neurographics/3/2/1/2.shtml

cranial nerves and the brainstem
Cranial Nerves and the Brainstem
  • CN involvement
    • Above Nucleus: Contralateral
    • At Nucleus and Below: Ipsilateral Manifestations
  • Corticospinal Tract
    • Contralateral weakness
pontine lesions
Pontine Lesions
  • Cranial Nerve Nuclei
    • Abducens nerve (CN VI)
    • Trigeminal nerve (CN V)
    • Cochlear and the lateral and superior vestibular (CN VIII)
    • The superior and inferior salivatory nuclei and the lacrimal nucleus (cranial nerves VII and IX)
  • Fiber Tracts
    • Corticospinal, corticobulbar, and corticopontine, spinocerebellar, spinothalamic, lateral tectospinal, rubrospinal, and corticopontocerebellartracts
disponnect4

DisPONnect

Course in the Wards: Diagnostic

A Case of Pontine Glioma

course in the wards diagnostic
Course in the Wards: Diagnostic
  • Patient was admitted in her 3rdweek of illness
  • S/O > Weakness of the left upper extremity (4/5), slurring of speech, drooling and shallow nasolabial fold, right
  • A> Vascular Insults probably right MCA, space-occupying lesions vs. Stroke in the Young
  • P> Referral to Pediatric Neurology; Neurovital signs to be monitored every 4 hours with strict aspiration precaution

Diagnostics

Laboratories: CBC with PC, serum electrolytes (Na, K, Cl, iCa), creatinine, ESR, PT and aPTT

Imaging: Cranial MRI plain and with IV contrast

disponnect5

DisPONnect

Patient’s Diagnostics

A Case of Pontine Glioma

diagnostics rationale1
Diagnostics: Rationale
  • Erythrocyte Sedimentation Rate
    • Rate at which RBC sediment in a period of 1 hour
    • Non-specific test used to detect conditions associated with acute and chronic inflammation (infection, cancers, autoimmune diseases)
    • A young stroke patient will often have signs of inflammation in the blood

Adams et al., 2003; European Stroke Initiative Executive Committee and the EUSI writing committee, 2003

diagnostics rationale2
Diagnostics: Rationale
  • Coagulation Tests (PT, aPTT)
    • Measure how quickly the blood clots
    • Abnormal results may either point to excessive bleeding or excessive clotting which present as risk factors to stroke (ischemic or hemorrhagic)

Adams et al., 2003; European Stroke Initiative Executive Committee and the EUSI writing committee, 2003

literature biopsy on brain glioma
Literature: Biopsy on Brain Glioma

Biopsy is seldom performed outside specialized biomedical research protocols for DIPG, unless the diagnosis of this tumor is in doubt.  Biopsy may be indicated for brain stem tumors that are focal or atypical, especially when the tumor is progressive or when surgical excision may be possible.

Childhood Brain Tumor Foundation

April 2010

If imaging findings are typical, biopsy is not usually necessary to confirm the diagnosis and should only be performed in the context of a formal clinical trial

Diffuse Pontine Glioma, UpToDate

Nov 2013

literature biopsy on brain glioma1
Literature: Biopsy on Brain Glioma

Stereotactic biopsy done for clarifying a diagnostic imaging in brainstem tumors is important in obtaining a definitive diagnosis with a low rate of complications.

Perez, et al. “Stereotactic biopsy for brainstem tumors in pediatric patients”, Jan 2010

Biopsy in children with MR findings of a diffuse intrinsic tumor is controversial and is not recommendedunless there is suspicion of another diagnosis, such as infection, demyelination, vascular malformation, multiple sclerosis, or metastatic tumors.

Nelson’s Textbook of Pediatrics

19th Ed.

literature radiologic imaging
Literature: Radiologic Imaging
  • MRI is the neuroimaging standard for primary brain tumors
    • Both diagnostic and prognostic
    • Help distinguishes between diffusely infiltrating and focal nodular tumors

Ropper and Brown. 2005. Adam and Victor’s Principles of Neurology. 8th ed. New York: McGraw-Hill

literature magnetic resonance imaging
Literature: Magnetic Resonance Imaging
  • Diffuse Type
    • More common
    • Mass effect with hypointense signal on T1 and heterogenously increased signal on T2
    • Asymmetric enlargement of the pons

Ropper and Brown. 2005. Adam and Victor’s Principles of Neurology. 8th ed. New York: McGraw-Hill

adjunctive diagnostics
Adjunctive Diagnostics
  • Tumors in the pituitary/suprasellar region, optic path, and infratentorium
    • Better delineation with MRI than with CT
  • Tumors of the midline and the pituitary/suprasellar/optic chiasmal region
    • Evaluation for neuroendocrine dysfunction
  • Tumors affecting the optic path
    • Formal ophthalmologic examination: oculomotor function, visual acuity, fields of vision

Kumar et. al. Nelson’s Textbook of Pediatrics, 19th Ed. New York: McGraw-Hill

adjunctive diagnostics1
Adjunctive Diagnostics

Adjunctive Diagnostics

  • Suprasellar and pineal regions
    • Preferential sites for germ cell tumors
    • Serum and CSF B-HCG and AFP
  • Tumors that spread to the leptomeninges
    • Medulloblastoma/PNET, ependymoma, and germ cell tumors
    • Lumbar puncture and cytologic analysis of the CSF

Kumar et. al. Nelson’s Textbook of Pediatrics, 19th Ed. New York: McGraw-Hill

patient s mri
Patient’s MRI
  • Impression:
    • Brainstem mass lesion with surrounding vasogenic edema. Consider a glioma.
    • No evidence of hydrocephalus or herniation noted at this time.
disponnect6

DisPONnect

Brainstem Glioma

Epidemiology, Etiology, Classification and Pathogenesis

A Case of Pontine Glioma

slide67

Epidemiology

  • BrainstemGliomas
    • 10-20% of all CNS tumors in children
    • More common in children than adults
  • Diffuse Intrinsic Pontine Glioma(DIPG)
    • Leading cause of brain tumor–related death in children
    • 15% of all childhood brain tumors
    • 58-75% of all brainstem tumors

Khatua, et al. 2011. Diffuse intrinsic pontineglioma – current status and future strategies. Child Nervous System Journal. 27: 1391-97. Springer-Verlag.

Jallo, G. 2005. Brainstem gliomas. Child Nervous System Journal. 22: 1-2. November 2005.

slide68

Epidemiology

  • Mean age at diagnosis is at 7 to 9 years
  • Males and females equally affected
  • USA: 200-300 children per year with this diagnosis of which 60-75% are DIPG
  • Incidence is greater in whites (18.52 per 100,000 person-years) than in blacks
  • Philippines and India – low incidence countries

Khatua, et al. 2011. Diffuse intrinsic pontineglioma – current status and future strategies. Child Nervous System Journal. 27: 1391-97. Springer-Verlag.

Jallo, G. 2005. Brainstem gliomas. Child Nervous System Journal. 22: 1-2. November 2005.

slide70

Classification

  • Localization
  • Diffuse
    • Diffuse Intrinsic Pontine
  • Non-Diffuse
    • Focal (e.g. Tectal)
    • Cervicomedullary
    • Dorsal Exophytic
slide71

Pons

  • Pontine tegmentum
  • Cranial nerves V, VI, VII VIII
slide72

Diffuse Intrinsic Pontine Gliomas

  • 80% of gliomas
  • Acute onset, high-grade
  • Rapid deterioration in 1-2 months
  • TRIAD:
    • Multiple cranial nerve deficits (CN VI, CN VII most common)
    • Long tract signs
    • Ataxia
  • Late stage: invasion of adjacent levels of brainstem and cerebellar peduncles
slide74

Focal Gliomas

  • 5% of Gliomas
  • Can be located anywhere in brainstem
  • Most common: tectum of midbrain
  • Well-defined margins
  • Indolent course

Tectal gliomas: OBSTRUCTIVE hydrocephalus

slide75

Dorsal ExophyticGliomas

  • 10-15% of gliomas
  • Arise from subependymal glial tissue in floor of 4th ventricle
  • Over 90%: Pilocytic Astrocytomas
  • Grow along path of least resistance (4th ventricle)
  • Long history of nonspecific headache and vomiting
  • Long tract signs usually not present
slide77

Medulla

  • CN IX, X, XI, XII
slide78

CervicomedullaryGliomas

  • 5-10% of brainstem gliomas
  • Arise from the lower medulla or the upper cervical spinal cord
  • Slow-growing, low-grade
  • Medulla: dysphagia, sleep apnea, dysarthria, recurrent URTI
  • Cervical cord: chronic neck pain, spasticity, weakness
  • Hydrocephalus: unusual in cervicomedullarygliomas.
slide79

Molecular Genetics of Brainstem Gliomas

  • Platelet-derived growth factor (PDGF) and its receptor (PDGFR): major driving forces of tumorigenesis
  • Gain Poly (ADP-ribose) polymerase (PARP-1)
  • Epidermal growth factor receptor (EGFR)
    • Expression indicates high grade tumor
  • p53 mutations
slide80

Clinical Presentation

  • Varied symptoms depending on the location of the lesion
  • Usually present with a short duration of symptoms (<3 months)
  • Common: abnormal or limited eye movements, diplopia, asymmetric smile, clumsiness, difficulty walking, loss of balance, weakness
slide81

Clinical Presentation

  • Classical Triad
    • Multiple cranial neuropathies (77%)
    • Long tract signs (53%)
    • Cerebellar signs (87%)
  • Cranial nerve palsies, long tract signs (e.g. hemiparesis) and ataxia
    • Over 50% of patients
  • Hydrocephalus with elevated ICP
    • Less than 10% of patients
  • Intratumoral hemorrhage
    • 6% of patients

Donaldson, et al. 2008. Advances toward an understanding of brainstem gliomas. Journal of Clinical Oncology. 24 (8): 1266-72. American Society of Clinical Oncology.

disponnect7

DisPONnect

Course in the Wards: Therapeutics

A Case of Pontine Glioma

slide83

Hospital Day 01

  • Subjective/Objective
    • Afebrile and with stable vital signs
    • Awake and comfortable with no symptoms of headache, vomiting, dizziness
    • Still presented with a shallow nasolabial fold, right and left-sided extremity weakness (4/5) with no deterioration
  • Assessment
    • Supratentorial Mass, r/o Malignancy
  • Plan
    • Monitored every 4 hours and maintained on strict aspiration precautions
slide84

Hospital Day 02: AM

  • Subjective/ Objective
    • Afebrile with stable vital signs
    • Awake and comfortable
    • Previously mentioned symptoms of right shallow nasolabial fold and extremity weakness were noted to have progressed, from a 4/5 to 1/5; intact extraocular muscles, (-) gag reflex
    • MRI results revealed brainstem mass lesion, possibly glioma
  • Assessment
    • t/c Brainstem Glioma
  • Plan
    • Referral to Oncology service
    • IVF D5NL (based on maintenance)
    • Dexamethasone 40mg/IV every eight hours
    • Additional monitoring through pulse oximetry
    • Stand-by intubation was ordered and a request for a family conference was initiated
slide85

Hospital Day 02: PM

  • Subjective/Objective
    • Patient had throbbing frontal headache, spontaneously resolving, with 2 episodes of vomiting, non-bilious, non-projectile
    • Patient was noted to have 94% oxygen saturation at room air
  • Assessment
    • t/c Brainstem Glioma
  • Plan
    • Oxygen support via nasal cannula at 2lpm
    • Mannitol 20% (50ml) was started every 8 hours and monitoring was changed to every 2 hours
    • Neurosurgery was called upon for further evaluation but was eventually deferred
slide86

Hospital Day 03

  • Subjective/Objective
    • Improvement in the severity of the headache, no recurrence of vomiting
    • Afebrile with stable vital signs
    • No progression of neurologic symptoms
  • Assessment
    • Diffuse Pontine Glioma
  • Plan
    • Family Conference
slide87

Family Conference

  • Attendees: Attending Physician, Pediatric Oncologist, Pediatric Resident, Patient’s Parents, Sister and two Aunts
  • Diagnosis and prognosis were disclosed to the family members and pertinent points discussed included the different options for the patient including surgery, radiation, chemotherapy and palliative care.
  • After discussion, patient’s family arrived at a consensus and opted to give the patient a good quality of life and asked for a referral to palliative care and subsequent home care.
slide88

Hospital Day 04

  • Subjective/Objective
    • Patient more comfortable
    • Able to tolerate feedings of soft, pureed foods with no difficulty in breathing
    • Still with left-sided extremity weakness, without progression
  • Assessment
    • Pontine Glioma
  • Plan
    • Further discussion and action with palliative care
    • Patient was deemed fit for discharge
disponnect8

DisPONnect

Therapeutic Options

A Case of Pontine Glioma

slide90

Surgical Resection

CONCLUSION:

Surgery is advocated for patients with well delineated, posteriorly, posterolaterally and ventrolaterally located tumors having slow progression and relative preservation of motor power

slide91

Chemotherapy

FINDINGS:

  • 43 patients with a defined clinico-radiological diagnosis of DIPG treated with radiotherapy + Temozolomide (75mg/m2), after which up to 12 courses of 21 days of adjuvant Temozolomide (75-100mg/m2) were given 4x weekly
slide92

Chemotherapy

FINDINGS:

  • Overall survival: 56%
  • Median survival: 9.5months
  • 2 year survivors: 5 (median age of 13.6years at diagnosis)
  • No survival benefit of the addition of dose dense temozolomide, to standard radiotherapy in children with classical DIPG.
  • Further exploration: Prolonged survival in adolescents
slide93

Chemotherapy

FINDINGS:

  • Time to tumor progression and survival times are longer than those previously reported in other DPG series
  • Arguments for therapeutic benefits:
    • Stable disease
    • Partial responses in DPG on MR imaging
    • Enhanced delivery of chemotherapy afforded by osmotic BBBD supports the further examination of this treatment modality for patients with DPG.
slide94

Immunology

FINDINGS:

  • VAE can be used as a novel virus-gene therapy strategy for glioma since it significantly inhabits GSC activity
    • Expression of exogenous Endo-Angio fusion gene can inhibit HBMEC proliferation.
slide95

Immunology

FINDINGS:

  • Amplification of the D-type cyclins and CDK4/6
  • Loss of Ink4a-ARF leading to aberrant cell proliferation
  • Targeting: cyclin-CDK-Retinoblastoma pathway in a genetically engineered PDGF-B-driven brainstem glioma mouse model
slide96

Immunology

FINDINGS:

  • 7-day treatment course with PD significantly prolonged survival by 12% in the PDGF-B; Ink4a-ARF deficient BSG model.
  • Furthermore, a single dose of 10 Gy radiation therapyfollowed by 7 days of treatment with PD increased the survival by 19% in comparison to RT alone.
slide97

Radiation Therapy

  • External Radiation Therapy
    • Uses a machine outside the body to send radiation toward the cancer
  • Internal Radiation Therapy
    • Uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer
slide98

Methods of Radiation

  • Conformal Radiation Therapy
    • Creates a 3D picture of the tumor and customizes radiation beams to fit the tumor, allowing precise and high dose radiation to reach its target
slide99

Methods of Radiation

  • Hyperfractionated Radiation Therapy
    • Total dose of radiation is divided into small doses and given more than once in a day.
slide100

Other Treatment Options

CONCLUSION:

We conclude that r beta IF plus hyperfractionated therapy can be tolerated by children with newly diagnosed brain stem gliomas, although there is occasional dose-limiting hepatic, blood, and central nervous system toxicity. This therapy did not result in a higher rate of disease control.

slide101

Other Treatment Options

CONCLUSION:

The major conclusion from this trial is that the hyperfractionated method of Rx 2 did not improve event-free survival (p = 0.96) nor did it improve survival (p = 0.65) over that of the conventional fractionation regimen of Rx 1, and that both treatments are associated with a poor disease-free and survival outcome.

slide102

Future Management

CONCLUSION:

IFN-beta gene therapy following tumor cell lysate-pulsed dendritic cellsimmunotherapy resulted in a significant prolongation in survival of the mice. Moreover, when this combination was performed twice, 50% of treated mice survived longer than 100 days. Considering these results, this combination therapy may be one promising candidate for glioma therapy in the near future.

disponnect9

DisPONnect

Brainstem Glioma

Complications, Prognosis, Prevention

A Case of Pontine Glioma

slide108

Prognosis

  • Survival period is shorter in children who presented with cranial nerve palsies (more likely to have malignant tumors)
  • Children with histologically malignant tumors had poorer outcomes
    • Best Survival Time: presence of Rosenthal fibers and calcification
    • Poor Survival Time: presence of mitoses
  • Decreased survival associated with two CT-Scan features:
    • Hypodense tumor prior to contrast
    • Tumor involving the entire brainstem

Albright et al. Prognostic factors in pediatric brainstem gliomas. Journal of Neurosurgery. 1986. 65 (5): 751-755

slide109

Prognosis

  • Median Survival Duration: 9-12 months even with treatment
disponnect10

DisPONnect

Brainstem Glioma

Biopsychosocial Impact: Palliative Care

A Case of Pontine Glioma

slide111

Factors that Place Families at High-Risk

  • Single parents or two parent families functioning at a single parent family
  • Preexisting chronic health or mental health problems
  • Economic problems: rural or urban poor: overextended middle-class families (debts): Job loss and minimal or no health insurance
  • Seperation, divorce
  • Chronic Unresolved Conflicts
  • Language difference: immigrant, foreign national, significantly different subculture
  • Families away from their cultural support network because of the child’s need for medical treatment
slide112

Initial Diagnostic Period:

A Time of Crisis

  • Family’s Reaction to Diagnosis
    • Shock, disbelief, guilt, anger, and fear
    • As the diagnosis is accepted, anger and guilt become significant emotions
slide113

Initial Diagnostic Period:

A Time of Crisis

  • Family’s Reaction to Diagnosis
    • An important task is to decide about telling the child about his or her diagnosis
slide114

Child’s Reaction to Diagnosis and Treatment

  • School-Age Children
    • Immediate concerns revolve around hospitalizaton, separation from parents, and fear of medical procedures
    • Constant reassurance is needed
    • Behavioral interventions may be necessary to gain cooperation
slide115

Child’s Reaction to Diagnosis and Treatment

  • School-Age Children
    • May have delayed or immediate reactions
      • Psychosomatic complaints
      • Nightmares
      • Labile emotions
      • Regressions
      • Stoic, adult-like acceptance
slide116

Child’s Reaction to Diagnosis and Treatment

  • School-Age Children
    • More likely to be verbal about their illness
    • Developmental period of vigorous inquiry
slide117

Child’s Reaction to Diagnosis and Treatment

  • School-Age Children
    • A special physician-child education session may be necessary
      • Can enhance compliance with procedures and treatment
    • Major activity outside the home is school, may help towards having some “normalcy”
slide118

Treatment and Adaptation Period

  • Normalcy is emphasized
  • “Burden of Normalcy” is created
    • The family has to reorganize itself but seem “normal”
slide119

Talking to the Dying Child

  • Two modes of Communicaton
    • Protective Approach
    • Open Approach
slide120

Talking to the Dying Child

  • “Understanding, acceptance, and convetance of permission to discuss any aspect of the illness decreases feelings of isolation and alienation from parents and other meaningful adults and gives the child the sense that his or her illness is not too terrible to discuss.”
slide121

Talking to the Dying Child

  • Common questions
    • “Am I going to die”
      • If yes, then
    • “When?”
    • “What will death be like?”
    • “What will happen to me after I die?”
    • “Will the “bad things” I have done or thought case me to be punished?”
    • “Will my parents be all right?”
    • “When can I be with my family again”
    • “Will it hurt when I die?”
slide122

End of Life Challenges: On Active and Passive Euthanasia

  • “Helping a child on his way” and “Ending a child’s suffering”
    • Open discussion does not yet warranted to chidren
  • Active euthanasia: Implies unwarranted assumption of infallibility on the part of the physician
  • Children should not be allowed to die in agony
slide123

Bereavement in the Family

  • “... More intense grief reactions of somatic types, greater deression, anger and guilt with accompanying feelings of despair”
slide124

Bereavement in the Family

  • Parents suffer from both the loss of the child and the loss of what the child represented to them.
slide125

Bereavement in the Family

  • Better adjustement if:
    • With Viable and ongoing “significant other”
    • Open and responsive communication with child during illness
    • Those with consistend life philosophies
slide126

Bereavement in the Family

  • Siblings should also be informed
    • Tailored to developmental ages
  • Follow-up counseling should be offered
disponnect11

DisPONnect

Update on AS

A Case of Pontine Glioma

disponnect12

DisPONnect

Thank You.

“Life is not measured by the breaths we take, but the moments that take our breath away.”

Hillary Cooper

A Case of Pontine Glioma