Surgery is the last resort
RADIOTHERAPEUTIC TREATMENT
Indications:
- Painful degenerative changes with no or minimal joint space narrowing
- Inflammatory synovitis
- Postoperative pain relief
- As an adjunct to conservative measures
Technique:
- Low energy X-rays or electrons
- Small field sizes (2-4 cm)
- Total dose 6-10 Gy in 3-5 fractions over 1-2 weeks
- Joint immobilization
Results:
- Pain relief in 60-80% patients lasting 3-6 months
- No disease modification
- Repeat treatment possible if pain recurs
2. IMPORTANT ASPECTS
Nonmalignant diseases can be successfully treated with ionizing radiation
These diseases can be classified but topography and morphology are more
relevant
Differences in clinical practice exist because of clinical traditions and
differences in organization and training
Criteria of evidence-based medicine are relevant for the use of
radiotherapy for non malignant diseases
4. JUSTIFICATION AND INDICATIONS
FOR RADIOTHERAPY
Invasive and aggressive growth (desmoids)
Cosmetic disfiguration and functional loss (keloids or endocrine orbitopathy
[EO])
Life-threatening complications (hepatic hemangioma [Kasabach-Merritt
Syndrome]) or juvenile angiofibroma of face
Nonmalignant diseases causing pain or other serious symptoms other
methods have failed, or may induce more side effects
5. LONG TERM RISK OF TUMOR INDUCTION
(UNSCEAR, BIER DATA)
Average lifetime risk men (9.5%) < women (11.5%)
The individual risk depends on:
individual sensitivity
eg) genetically predisposed diseases
anatomic site and
technical parameters
viz. single and total doses radiation protection
6. TYPES ABSOLUTE LIFETIME RISK
Skin (Basal cell
carcinoma)
0.1% for 100 cm2 field
Osteosarcoma <0.0001% for 1 Gy and 100 cm2 field
Leukemia 1% for 1 Gy TBI
Brain
tumor
0.2% after 20 Gy for endocrine orbitopathy
Thyroid carcinoma 1% per Gray for children < 10 years
Breast
carcinoma
5% for one breast, 1 Gy, age < 35 years
(<3% for age 25-35 years)
Lung
carcinoma
1% within 25 years after a mean lung dose of 1 Gy
7. As RT in most cases is an elective measure, a thorough risk-benefit analysis
is required
Organ-specific acute and chronic late effects including potential effects on
reproduction and possible induction of tumors and leukemia are potential
risks that have to be explained to patients within the INFORMED CONSENT
8. PRINCIPLES
Estimate the natural course of disease without therapy
Consider potential consequences of nontreatment
Review data about alternativetherapies and their therapeutic results
Conduct a risk-benefit analysis compared with other possible measures
Proof that the indication is justified:
• if conventional therapies have failed,
• if risks and consequences of other therapies are greater, and if
nontreatment has more dramatic consequences
than irradiation
9. Consider the individual potential long-term radiogenic risks
Inform patient about all details of radiotherapy:
target volume, single/total dose, duration of session and series,
relevant radiogenic risks, and side effects
Written consent of patient following thorough education
Assurance of long-term aftercare in order to document result
Request a competent second opinion in case of doubts and if the provided
patient data or treatment decision are uncertain
10. Outside Europe, the use of RT to treat benign disease is not well
established and often regarded with skepticism
The last written recommendations for the treatment of nonmalignant
disease in the United States were made by the Bureau of Radiologic
Health in 1977
11.
12.
13.
14. RADIOBIOLOGICAL ASPECTS
increase in capillary permeability and tissue perfusion (perfusion theory)
destruction of inflammatory cells and release of mediators,
cytokines, and proteolytic enzymes (fermentative theory)
impact on the autonomous nervous system (neuro regulatory theory)
impact on the composition of the tissue milieu (electrochemical theory)
preventing mitotic cells from proliferating (antiproliferative effect )
15. RADIATION THERAPY MECHANISMS OF ACTION
AND DOSE CONCEPTS
MECHANISMS OF ACTION SINGLE DOSE (Gy) TOTAL DOSE (Gy)
Cellular gene and protein
expression
(Eczemas)
< 2 < 2
Inhibition of inflammation in
lymphocytes (Pseudotumor
orbitae)
0.3 - 1 2 - 6
Inhibition of fibroblast
proliferation
(Keloids)
1.5 - 3 8 - 12
Inhibition of proliferation in
benign tumors
(Desmoids)
1.8 - 3 45 - 60
16. DISORDERS OF CONNECTIVE
TISSUES AND SKIN
DESMOID (AGGRESSIVE FIBROMATOSIS )
INDURATIO PENIS PLASTICA (MORBUS PEYRONIE)
MORBUS DUPUYTREN AND MORBUS LEDDERHOSE
KELOIDS AND HYPERTROPHIC SCARS
17. DESMOID (AGGRESSIVE FIBROMATOSIS)
Benign connective tissue tumors of deep muscular-aponeurotic structures in
the region of muscle fascias, aponeuroses, tendons, and scar tissue
Incidence 2-4 per 1 million per year
Female > Male (1:1.5 to 2.5)
Age group - Third and fourth decades of life, but children also
can be affected
May suspend spontaneously or grow fast
18. NON RADIOTHERAPEUTIC TREATMENT
Surgical removal with a safety margin of 2 to 5 cm is
the gold standard
R0 resection - no therapy is required
R1 resection - wait and treat the upcoming relapse
Tamoxifen and progesterone - growth inhibitory effects
NSAID’S, vitamin C and alkylating substances
Tested but not yet established
19. RADIOTHERAPEUTIC OPTIONS
Indications:
Inoperable cases
R2 resection
R1 resection if repeated surgery already applied
Dose:
Postoperatively 50 to 55 Gy ( 1.8 to 2.0 Gy / #)
Inoperable or recurrent desmoids 60 to 65 Gy
Rest tumor or recurrence 50 to 55 Gy
RT is often used as adjuvant or primary treatment
31. Dose: 50 to 55 Gy in 1.8 to 2 Gy fractions/ one fraction per day
Recommendation: Generous margins
Cover entire operative area and/or area of gross disease to avoid
geographic miss
32. No standard cm of coverage that is recommended
Extent of coverage depends on
• tumor size
• number of prior recurrences
• extent of operative hematoma
• proximity of tumor to paths of little resistance
(e.g., along the neurovascular bundle)
Fields for aggressive fibromatosis may need to be even more extensive
than those for soft tissue sarcomas
33. PTV = CTV + 0.5 CM
GTV = PREOP GTV
CTV = GTV + 5 CM
MEDIOLATERALLY AND
CRANIOCAUDALLY
38. KELOIDS AND HYPERTROPHIC SCARS
Excessive tissue proliferation about scars after skin injury from surgery,
heat, chemical burns, inflammation (e.g., acne), or even spontaneous
proliferation
Infiltrative growth pattern
Causes local pain, inflammatory reactions and long-term progression
Differential Diagnosis - Hypertrophic scars
39. Location - Upper body
Sternum
Earlobes
Cause - unknown
Genetic and Race-specific
predisposition noted during
adolescence
Disturbed cosmesis may be
accompanied by pain, itching, and
dysfunction
42. Primary RT:
• Functional inoperability
• Actively proliferating disorders within about 6
months after the triggering trauma
Prophylactic RT:
(immediately after excision of the recurrence)
• Fully matured keloids
43. RT is initiated 24 hours after surgery
Radiation quality has to be adapted to the local conditions
• Conventional x-rays (70 to 150 kV)
• Electrons (<6 MeV)
• Iridium-192 brachytherapy with implants
• Strontium-90 dermal plates
44. Target volume
Scar plus a 1-cm deep safety
margin on both sides of the scar
Lead absorbers need to be prepared if required
Recommended total dose:
12 to 20 Gy
5 X 3 or 4 X 4 Gy within 1 week
Single-dose RT with 7.5 to 10 Gy is also effective
48. INDURATIO PENIS PLASTICA
( MORBUS PEYRONIE)
Chronic, mostly progressive, inflammation and tissue
proliferation of the penile tunica albuginea
Affects men at 40 to 60 years
Cause is unknown
Diabetes mellitus
Arterial and venous vascular disease risk factors
Genetic predisposition
49. Inflammatory changes at tunica albuginea
Connective tissue reactions and formation of hard plaques lumps, and
cords (100%)
Penile bending Pain at erection Impair cohabitation (80%)
(80%) (30% to 50%)
Slow progression over several months is typical,
Spontaneous remission may develop rarely
50. NONRADIOTHERAPEUTIC TREATMENT
SO FAR THERE IS NO SUCCESSFUL STANDARD TREATMENT
EARLY PHASE:
Vitamin E, para-aminobenzoate, and steroids
Local treatments but their evidence is small
Ultrasound
Shock waves
Corticoid, procaine, and hyaluronic acid injections
RT may delay induration
softens lumps and strands
reduces pain, bending, and functional problems
ADVANCED STAGES:
51. RADIOTHERAPEUTIC TREATMENT
Protect gonads and glans penis with lead apron or capsule
Orthovoltage machine - non erected penis/ dorsal field
LINAC - electrons upto 6 MeV with 5 to 10 mm bolus
Usual dose - 20Gy / 2Gy per fraction
Hypofractionation - 12 to 15 Gy // 2 to 4 Gy / #
2- 4 #per week
No response, repeat this regimen after 6 to 12 weeks up to a total
dose of 30 Gy
For extensive indurations, high dose rate brachytherapy is suitable
52. Within 1 to 2 years, RT improves symptoms in 2/3rd early-stage patients
Local pain and associated clinical symptoms decrease in up to 75%
Angulation (25% to 30%) and dysfunction of the penis (30% to 50%)
show less response because these symptoms often indicate that
the disease is already in a more advanced stage
54. MORBUS DUPUYTREN AND MORBUS
LEDDERHOSE
Connective tissue disorders that
affect the palmar or plantar
aponeurosis
Often bilateral
Hands (MD) > feet (ML)
Mostly, the fourth/fifth
phalanges of the hand (MD) or
the first/second toes of the foot
(ML) are affected
Age group 40 and 70 years
55. RISK FACTORS AND PATHOGENESIS
Familial disposition
Alcohol abuse
Diabetes mellitus
Epilepsy, and other conditions
Causes and pathogenesis are still not adequately determined
56.
57. NONRADIOTHERAPEUTIC TREATMENT
Early stage
• Steroids
• Allopurinol
• Nonsteroidal
antiphlogistics
• Enzymes
• Vitamin E and
• Softeners
Surgery –
• Functionally impairing
flexion contractions of
fingers
(>30 degrees)
• Strong pain while walking
Preventive strategies are more important
58. RADIOTHERAPEUTIC TREATMENT
Prophylactic EBRT during early stages is a good rationale
RT makes sense during the early stage (lumps, strands) with
moderate extension deficit ( < =10 degrees)
Target cells: proliferating and radiosensitive fibroblasts and
inflammatory cells
The goal of treatment is to avoid further progression or later
surgery
59. Orthovolt (100 to 150 kV photons) or linear accelerator with
electrons (up to 6 MeV) via stationary field used
Protection of unaffected areas (individually adapted lead shields)
Safety margin -1 cm laterally and 2 cm proximally and distally
Total dose - 20 to 30 Gy// 2 to 3 Gy/#
Repeat after 6 to 12 weeks up to a total dose of 30 Gy.
4 to 5 Gy / # every 1 to 2 months
60. RESULTS OF THERAPY
Many studies have shown good response to RT
• Stabilization of the disease (70% to 80%)
• Regression of nodules and strands (20% to 30%)
• Disease progression in spite of previous irradiation occurs in
20% to 25%
Existing extension deficits are normally not improved
Patients with extension deficits (stage I/II) have a significantly
increased rate of recurrence or progression
62. DISORDERS OF JOINTS AND
TENDONS
GENERAL ASPECTS
DEGENERATIVE ARTHRITIS
ROTATOR CUFF SYNDROME
TENNIS / GOLFER’S ELBOW
CALCANEODYNIA / ACHILLODYNIA
63. GENERAL ASPECTS
RT is a last-resort approach, but before surgery is indicated
Recommended RT concepts use:
Daily fraction of 0.5 - 3 Gy for acute inflammation
and
0.5 to 1.0 Gy are applied 2-3 times per week up to 6 Gy for chronic
inflammatory reactions
In case of minor response, a second series is delivered after 6 weeks
64. DEGENERATIVE ARTHRITIS
A painful joint process associated with cartilage destruction, bone
modification, and structural changes of capsule and synovia
Subjective symptoms and radiographic findings are often incongruent
Functional loss has an impact on professional and leisure activities and
reduces quality of life
65.
66.
67. NONRADIOTHERAPEUTIC TREATMENT
Prevention and early recognition are crucial
Pre arthrotic changes - corrective osteotomy
Noninvasive measures
Invasive measures:
Arthroscopic lavage
Debridement of inflammatory synovial changes
Autologous cartilage replacement - small joint areas
Partial or total joint replacement with an artificial implant is lat option
68. RADIOTHERAPEUTIC TREATMENT
Low-dose RT is indicated if noninvasive measures have failed
- Reduces pain and pain-related dysfunction
- Does not remove pathomorphologic changes
The affected joints of the upper (shoulder, elbow, thumb, fingers) and lower
(hip, knee, ankle) extremity are irradiated
Lateromedial or ventrodorsal opposing fields using the orthovolt (150 to 200
kV/20 mA, 4-mm Al filter) or linear accelerator low-energy photons (<6 MV)
69. Dose reference point:
Always located in the center of joint (for opposing field setup)
A pain record of >2 years and objective findings like joint grinding,
deformity, radiologic OA stage IV, are indicators of unfavorable prognosis
Joint replacement surgery might be delayed or completely avoided in
individual cases
71. TENDINITIS AND BURSITIS
Causes:
• Acute and chronic strain with macro- or microtrauma
Particularly affected joints:
• Shoulder (peritendinopathia humeroscapularis)
• Elbow (epicondylopathia humeri or tennis/golfer's elbow)
• Ankle joint (plantar fasciitis)
• Achilles tendon (achillodynia)
• Heel region (calcaneodynia)
72. GENERAL MANAGEMENT
Immobilization and protection of joint function
Local cold or heat
Analgesics and antiphlogistics
Local injections of anesthetics and corticosteroids
Physiotherapy (stretching/deep friction technique)
Shock wave therapy
Electrotherapy
Acupuncture
Surgical measures - chronic-recurrent cases
73. RADIOTHERAPEUTIC MANAGEMENT
Indication:
when conservative measures have failed and before surgery
Energy used:
Orthovlotage (100 to 300 kV)
LINAC (6 to 10 MV)
Local and systemic medication can be continued during RT
Local response to irradiation is often preceded by short-term
intensification of pain about the irradiated tendon or bursa
75. ROTATOR CUFF SYNDROME
Periarthropathia humeroscapularis / subacromial syndrome describes a condition of pain
and functional loss of the shoulder joint
All muscular structures, tendons, and bursae of the shoulder region can be affected
Numerous conditions may trigger this problem:
• Arthritis
• Capsulitis
• Bursitis
• Tendonitis
Local calcifications or ossifications are signs of a chronic reactive disease process
76. NONRADIOTHERAPEUTIC TREATMENT
Local injections (corticoids, anesthetics)
Oral antiphlogistics and analgesics
Physical therapy
Surgical measures indicated in the case of tendon rupture
Radiation therapy if conservative management fails
77. RADIOTHERAPEUTIC MANAGEMENT
Orthovolt machine (200 to 250 kV, 15 mA, 1-mm Cu filter) or linear
accelerator
Opposing fields - shoulder joint-related pain
Direct single fields - localized tendinopathies
Dose reference point :
• center of the affected joint (5 to 8 cm) or
• at the depth of the tendon attachment site (1 to 2 cm)
Field sizes range between 10 x 10 to 10 x 15 cm2
78. Pain relief and improved mobility - in up to 80% of patients
Pain record (>2 years) and the extent of joint modifications
(accompanying arthrosis) are determinants for prognosis
Short-term intensification of pain during the RT series is frequently a
favorable sign for clinical response
Residual pain and functional deficit after one RT series of 6 X 1Gy, a
second series of RT is indicated
The clinical improvement is often long-lasting
80. TENNIS / GOLFER’S ELBOW
A painful inflammation of the tendon attachment site of the finger
and hand muscles of the radial (lateral) or ulnar (medial) epicondylus
of the upper arm
Causes
• intensive fine and gross motor activity
• extreme strain of the arm or awkward movements during exercise
• traumatic
• mechanical irritation of the bursae
Genders are equally affected
Average age for either condition is 45 years
81. First symptoms appear during stress, later also during night and day as
constant pain, resting pain, and start up pain (in the morning)
Affected people are impaired in professional work and leisure activities
Radiographs rarely demonstrate clear findings
MRI may uncover typical soft tissue modifications (edema of muscles and
insertion zone)
Cervical spine syndrome has to be excluded
82. If noninvasive treatments have failed, RT is indicated
Orthovoltage photons (100 to 150 kV, 20 mA, 4-mm Al filter) or linear
accelerator low-energy photons (<6 MeV)
A stationary field is targeted directly to the affected lateral or medial
epicondylus
The dose reference point is calculated at a depth of 5 mm
83. Even for chronic pain, RT achieves good pain reduction in up to 80% of
cases
>12 months pain record, the response is worse than for early RT
Relapses after therapeutic success are rare (5%)
Disease record (>1 year), number of previous treatments, and long
immobilization are unfavorable prognostic results
If pain reappears after surgery, RT as salvage therapy
84. CALCANEODYNIA / ACHILLODYNIA
Pain syndromes of the heel region
Entire osseous and tendinous system of the foot and legs is impaired
- flatfoot, splayfoot, and skew foot deformities
- genu varum or valgum malposition
Chronic stress leads to plantar and dorsal osseous spurs of up to 20
mm in length
85. Short-term vigorous forces or malpositions and mini
trauma are triggering factors
Both genders equally affected
Incidence increases with age
Running and sports with sudden or impulsive movements
may lead to an earlier onset of disease
86.
87. NONRADIOTHERAPEUTIC TREATMENT
Potential anatomic malpositions are corrected to avoid further stress.
For Painful heel - special cushion inlays.
Local injections (with corticoids, anesthetics) and oral antiphlogistics or
analgesics are used for immediate pain relief
Physical therapy and physical measures (cold, ultrasound, microwaves,
shock waves)
Surgical incision of the plantar fascia is only done if conservative
therapy fails
88. RADIOTHERAPEUTIC TREATMENT
RT is indicated if noninvasive treatment fails.
Orthovolt photons (100 to 150 kV, 20 mA, 4-mm Al filter) or linear
accelerator with low-energy photons (<6 MeV)
A stationary 6 X 6 to 8 X 8 cm2 field (plantar) or opposing fields (dorsal) are
used
Dose of 3 to 6 X 0.5 to 1 Gy to the dose reference point located at a depth
of 5 mm (stationary field) or at the center of the joint (opposing
fields)
89.
90. DISORDERS OF BONY TISSUES
ANEURYSMAL BONE CYST
PIGMENTED VILLONODULAR SYNOVITIS
VERTEBRAL HEMANGIOMAS
HETEROTOPIC OSSIFICATION
91. ANEURYSMAL BONE CYST
Benign, vascular cystic lesions
Metaphysis of bones
Cause functional impairment,
pathologic fractures,
damage of neighboring structures and
Infiltration into surrounding soft tissue
Leads to bone destruction and evoke serious problems, which is why
treatment is recommended once a cyst has been diagnosed, eg)
vertebral column.
92. X RAY APPEARANCE: An eccentric well defined radiolucent area with
expansion of overlying cortex and trabeculation within the substance
of tumor involving upper end of tibia likely Aneurysmal Bone Cyst.
93. NONRADIOTHERAPEUTIC TREATMENT
Surgery (resection or curettage) is the gold standard
Following curettage, recurrence occurs in up to 60% of patients
After complete resection, there is normally no recurrence
94. RADOTHERAPEUTIC TREATMENT
Indications:
• If cysts cannot be treated by surgery
• If curettage is difficult because of size or site of the lesion
• Cyst progression or repeated recurrences
• Inaccessible cysts of the vertebral column and the pelvis
RT doses should be kept as low as possible.
10 – 20 Gy for 1 to 2 weeks - an adequate dose.
95. PIGMENTED VILLONODULAR SYNOVITIS
Rare proliferative disease affecting the synovia of joints and the tendon
sheaths.
Two types of disease:
the strictly localized and
the diffuse affection of synovial membranes
In the majority of cases, the lesion is restricted to one joint and can
spread to muscles, tendons, and skin membranes.
96. NONRADIOTHERAPEUTIC TREATMENT
Surgical excision normally consists of synovectomy,
Rarely complete, particularly in the large joints like the knee.
Therefore, recurrences occur with a frequency of up to 45%.
97. RADIOTHERAPEUTIC TREATMENT
RT applied with radionuclides for localized PVNS or with external-beam
RT for diffuse PVNS.
TORONTO STUDY - Most patients showed excellent functional results
with 30 to 50 Gy in 15 to 20 fractions of 1.8 to 2.5 Gy as standard
regimen for PVNS.
GERMAN MONOCENTRIC STUDY - concluded that combined
treatment should be considered for all patients with
suspected or proven residual D-PVNS.
98. Heyd et al , IJROBP, 2010
Radiation achieves a higher local rate in postoperative setting after
nonradical resection and a salvage treatment option for recurrent and refractory
disease.
99. VERTEBRAL HEMANGIOMAS
Benign proliferations of vessels lesions that can affect any tisuue and are
asymptomatic
Usually only one vertebral body is affected
Most lesions are small and rare and require no therapy
Spread of tumor into extradural space, hemorrhage, or compression
fracture can lead to bone marrow compression and severe
consequences
102. RADIOTHERAPEUTIC TREATMENT
IN MOST CASES POSTOPERATIVE IRRADIATION SHOULD BE GIVEN
although radiation alone has been tried
Recommended Radiation Dose: 36 to 40 Gy delivered in 2 Gy per fraction
Radiation provides pain relief
103. HETEROTOPIC OSSIFICATION
Develops after trauma or surgery of the hip
They consist of real bone located in the periarticular soft
tissue
HO jeopardizes functional outcome and impairs rehabilitation
104. PREDISPOSING FACTORS FOR HO
Skeletal diseases : Forestier disease, Bechterew disease
Severe trauma of the brain and spinal cord
Patients with I/L or C/L HO after a previous total hip arthroplasty
Patients with femoral or pelvic bone osteophytes >1 cm in length
After acetabular or pelvic fractures
Several interventions at the hip joint
Men > women
Ankylosing spondylitis
Disseminated idiopathic hyperostosis of skeleton
105. ETIOLOGY
The etiology of HO is not fully understood
Pluripotent mesenchymal stem cells present in periarticular soft tissue,
under certain conditions develop into osteoblastic stem cells that finally
form HO’s
In vivo experiments showed that differentiation of osteoblastic stem cells
reaches its maximum after 32 hours
111. NONRADIOTHERAPEUTIC TREATMENT
Target groups for prophylactic treatment
• Patients with symptomatic HO
• Those likely to develop clinically relevant postoperative HO
Ethylhydroxydiphosphonates (EHDP) has been used for prevention of HO,
but outcome was contradictory
Indomethacin is effective in patients at high risk. Administered in different
dosages immediately after surgery for about 3 to 6 weeks.
112. RADIOTHERAPEUTIC TREATMENT
Prophylactic RT employed since late 1970s.
Initial dose concepts use 20 Gy/10#
Now 6-8 Gy/#
RT should be started no later than day 4 after surgery
Keep the postoperative interval as short as possible
24 to 48 hours
Preoperative RT with single fraction of 7 to 8 Gy
has been successfully applied
113. Target volume should encompass typical localizations of periarticular HO
Cranial field border: App. 3 cm above the acetabulum and includes about
two thirds of the implant shaft
Usual field size is 14 x 14 cm
Dose reference point - central beam at the center of the target volume (at
about 8 to 12 cm deep)
HO prophylaxis can also be done in paraplegic patients:
• Knee
• Elbow
• Shoulder
• Jaw joints
118. MENINGIOMAS
Most common benign tumors of the CNS
Incidence peaks in seventh decade of life
Female-to-male: 2:1
Classification:
• WHO Grade I: >90%) are benign
• WHO grade II: Atypical, Clear cell, or Chordoid - Local
recurrence high
• WHO grade III: Malignant - Anaplastic, Rhabdoid,
Papillary- Rare
119. MENINGIOMAS: DIAGNOSIS
Most common presenting symptom: headache
Other localizing symptoms: depend on tumor location
Radiographic diagnosis: CT or MRI
Homogeneously and intensely enhancing extra- axial
mass with or without the presence of a dural tail
120. MENINGIOMAS: MANAGEMENT
SURGICAL RESECTION: treatment of choice
Primary goal: Maximal Safe Resection
Tumors in convexity and olfactory groove: Gross Total
Resection (Relapse rate 10%)
Relapse rate depends on Simpson classification
121. SIMPSON GRADE DESCRIPTION RECURRENCE RATE
I
Complete macroscopic tumor removal
with adherent dura as well as the
possibly affected part of cranial calotte 8.9%
II
Complete macroscopic tumor removal
with adherent dura via diathermy 15.8%
III
Complete macroscopic tumor removal
without adherent dura or possibly
additional extradural parts
29.2%
IV
Partial macroscopic tumor removal
while removing intradural tumor parts 39.2%
V
Simple decompressive and bioptic
removal of tumor 88.9%
122. MENINGIOMAS: MANAGEMENT
Meningiomas (highly vascularized tumors): In select patients,
preoperative embolization
• decreases blood loss and improve extent of resection
Asymptomatic meningiomas: Observe clinically
Tumor grows or symptoms develop: Surgery or radiation
therapy
123. SYSTEMIC THERAPY
Antihormonal therapy: upto 67% express progesterone or
androgen receptor and 10% express estrogen receptor
(response rates are lower)
Hydroxyurea: under investigation in recurrent disease (shown
little efficacy)
124. RADIOTHERAPY
Indications:
• Primary radiotherapy (RT):
tumors in locations in which complete resection is not
feasible (i.e., optic nerve, cavernous sinus, major venous sinus)
or for patients who are poor surgical candidates.
• Adjuvant RT: patients with subtotal resection (STR),
recurrent disease, or for WHO grade II or III
tumors.
125. RADIATION TECHNIQUES
Conventionally fractionated three-dimensional conformal
radiotherapy (3D-CRT)
Conventionally fractionated intensity-modulated radiation
therapy (IMRT)
Frame-based or LINAC–based fractionated stereotactic
radiotherapy (FSRT), stereotactic radiosurgery (SRS)
Protons and heavy ions
126.
127. RADIATION DOSE
Benign meningiomas: the typical dose prescription to the PTV
is 50 to 54 Gy given in 1.8- to 2-Gy daily fractions
For patients with more aggressive histology (WHO grade II or
III tumors): the GTV is expanded by at least 2 cm, with a
higher dose prescription in the range of 59.4 to 63 Gy
128.
129. (C) Postoperative axial scan showing complete resection of tumor. (D)
Gamma Knife treatment MRI shows recurrence of convexity tumor.
130. (E, F) Three months after treatment, the tumor is unchanged in size
but there is an increase in surrounding edema as seen on
FLAIR images.
132. RADIATION DOSE: SRS
Frame-based SRS: 12 to 16 Gy prescribed to the 50% isodose
line (IDL) and
Frameless SRS: 14 to 18 Gy prescribed to the 80% IDL.
Selected Perioptic tumors, including meningiomas: 24 to 30 Gy
in 3 to 5 fractions
133. (B) Treatment plan with shaped beans, 14 Gy, 90% isodose line, volume 1.07
cm3.
134. Treatment plan with shaped beans, 18 Gy, 90% isodose line, volume 0.65
cm3.
136. Patients in the above reports typically, but not exclusively, had either known or presumed
low-grade meningiomas. The follow-up and dose columns list the mean or median figures.
153. Conformal, image-based EBRT. MR image and isodoses for IMRT of a
Cerebellopontine angle meningioma treated to 54 Gy in 40 fractions,
Prescribed to the 90% isodose. The yellow line represents the 70% isodose,
green 50%, and sky-blue 30%. The “dural tail” is not
included within the PTV.
154. AVASCULAR MALFORMATIONS
Intracranial AVMs are congenital vessel abnormalities
consisting of widened arteries connected to the normal
capillary bed
Nidus of AVM = tangled arteries and veins that are connected
by one or more fistulas
Overall prevalence: 18 in 100,000 individuals
Age at presentation: typically between 20 and 40 years old
155. CLINICAL PRESENTATION
Clinical concern: bleeding risk, 2% to 4% per year.
Approximately 50% of patients present with hemorrhage and
50% present with nonfocal (headache, nausea) symptoms or
incidentally found focal neurologic deficits.
The risk of death per bleed is up to 10%
Approximately 30% have serious morbidity associated with
each bleed
156. INVESTIGATIONS
Diagnostic imaging includes angiography, which is invasive
but allows for full grading of the AVM according to the
Spetzler-Martin scale.
MRI, MR angiography, and CT angiography are noninvasive
and complementary studies that may be used to visualize the
AVM.
157. SPETZLER MARTIN GRADING SYSTEM
Predicts patient outcomes after surgical resection of AVMs.
Composed of three components (AVM size, location, and
pattern of venous drainage), this system has been validated.
Unfortunately, this grading scale does not appear to correlate
with successful AVM radiosurgery.
Spetzler RF, Martin NA. J Neurosurg 1986; 65:476–483.
158. Predicts patient outcomes after AVM radiosurgery
POLLOCK-FLICKINGER SCORE
Pollock BE, Flickinger JC, Lunsford LD, et al. Neurosurgery 1998
160. SURGERY
Goal of any therapy: Complete obliteration
Provides immediate cure but carries a risk of
intraoperative bleeding, ischemic cerebrovascular
accident, infection, and death
Surgery is particularly indicated for AVMs in superficial,
noneloquent regions of the brain
162. RADIOTHERAPY
SRS is the radiation modality of choice for the treatment of
AVMs.
SRS is indicated mostly for lesions in deep or eloquent
regions of brain and particularly <3 cm size.
The time to obliteration ranges from 1 to 4 years after SRS, so
the patient remains at a continued bleeding risk.
163. Based on the Flickinger et al, typical prescriptions for
treatment of AVM are 21 to 22 Gy prescribed to the 50% IDL
for frame-based radiosurgery.
The prescription should be lowered for AVMs near the
brainstem or larger lesions (>3 cm).
For linac-based SRS, prescriptions generally range from 16 to
24 Gy in a single fraction to 20 to 22 Gy in 2 fractions for
spinal AVMs.
164. Neuroimaging studies of a 26-year-old woman with an incidentally discovered left basal ganglia
AVM. (A) Lateral and (B) anterior-posterior (B) left internal carotid angiograms showing AVM on
the day of radiosurgery. The AVM volume was 6.5 cm3; the AVM margin dose was 15 Gy.
165. (C) CT performed 6 months after radiosurgery showing both intraparenchymal
and intraventricular hemorrhage.
166. (Left) Post-gadolinium and (right) long-TR MRIs performed 3 years after
radiosurgery of a left sylvian fissure AVM demonstrates persistent enhancement
and edema consistent with radiation necrosis.
167. TRIGEMINAL NEURALGIA
A common problem affecting approximately 15,000 patients
each year in the United States
Female to male: 1.5 to 1
168. TRIGEMINAL NEURALGIA
Type I TN: predominantly (>50%) sharp, lancinating, and
shock-like with pain-free intervals
Type II: TN predominantly experience burning, aching, or
throbbing pain
Prognostic significance: type I TN patients more likely to be
pain free and have longer disease control than patients with
type II TN after decompression.
169. TRIGEMINAL NEURALGIA
The classic clinical feature is recurrent episodes of sudden,
brief, severe, stabbing, or lancinating pain in the area of the
trigeminal nerve sensory distribution
It is most commonly unilateral, but some cases are bilateral.
Common triggers for attacks include talking, “chewing,
brushing teeth, and cold air.
173. SURGERY
Medically refractory disease:
Microvascular decompression is the treatment of
choice
Other options include:
Rhizotomy with either radiofrequency ablation,
Glycerol injection, or
Balloon compression
174. STEREOTACTIC RADIOSURGERY
Treatment planning
• CT-MRI fusion
• Target: Root entry zone of the trigeminal nerve as it enters
the pons to the semilunar ganglion
• Typical doses using a frame-based radiosurgery platform
are 70 to 90 Gy, prescribed to the 50% IDL
• Higher rates of pain relief in the high-dose arm (72% vs. 9%
for patients treated with ≥70 Gy vs. <70 Gy)
• Median time to pain relief: 1 month
• Main concern is delayed onset of facial numbness.
175. Gamma Knife treatment plan for a patient with right
trigeminal neuralgia. Isodose coverage of the nerve is seen in the right
upper panel. The 20% line touches the brain stem, and the 10% line
cuts into it.
176. Beam shaping is accomplished by plugging 32 beams as
shown in the left upper panel. The result, seen in the right upper panel,
is a flattened 10% and 20% isodose line such that the brain stem
receives 10% only along the edge.
177. STEREOTACTIC RADIOSURGERY
Flickinger et al. who randomized patients to 75 Gy targeted to
a shorter (1 isocenter) or longer (2 isocenters) segment of the
trigeminal nerve.
The rates of pain relief were surprisingly identical between
the two groups.
There was a trend toward a higher incidence of numbness or
paresthesias in the 2-isocenter patients.
178. STEREOTACTIC RADIOSURGERY
Adler et al: frameless robotic radiosurgery platform to a 6-
mm segment of the trigeminal nerve with a mean marginal
prescription dose of 58.3 Gy and mean maximal dose of 73.5
Gy.
85% experienced a complete response and
96% reported excellent or good outcomes at a mean follow-
up of 15 months
Only 15% of patients experienced ipsilateral facial numbness
179.
180.
181. VESTIBULAR SCHWANOMMA
Acusticus neurinomas are benign neuroectodermal tumors
originating from Schwann cells of the neurilemma at the
vestibulocochlear nerve (CN IV)
Make upto 5% of primary brain tumors.
With an incidence of 1 in 100,000, 5% of cases are affected by
patients with neurofibromatosis type II (M. Recklinghausen).
182. VESTIBULAR SCHWANOMMAS
Growth in the cerebellopontine angle exerts pressure on the
vestibularis and cochlear nerves, causing hearing impairment,
tinnitus, and vertigo.
Later facialis paresis (CN VII), trigeminus neuropathy (CN V),
and brainstem symptoms occur.
Diagnosis is made via high-resolution CT and MRI which show
intra- or extrameatal location and size.
184. NON RADIOTHERAPEUTIC TREATMENT
Complete tumor resection is the standard therapy,
particularly for large tumors (>25 mm).
Nerve injury can be avoided intraoperatively by
electrophysiological monitoring
185. RADIOTHERAPEUTIC TREATMENT
Indication: Progressive and symptomatic primary or recurrent
acusticus neurinoma up to a size of 25 mm (Tos grade 0 to 2).
Stereotactic single-dose RT with gamma knife or modified
linear accelerator is the preferred RT technique.
Besides direct damage of proliferating tumor cells, tumor
vessels may slowly be occluded
40% to 70% of cases will develop a tumor remission.
186. RADIOTHERAPEUTIC TREATMENT
Aims of RT:
• disappearance of tumor and symptoms,
• prevention of tumor progression, and
• maintenance of the remaining hearing ability.
187. RADIATION DOSE
With single doses of 12 to 14 Gy at tumor edge (depending on
reference isodose 15 to 25 Gy centrally), local control reaches
up to 95%.
In order to decrease side effects, FSRT at linear accelerators
has been preferred recently: RT concepts are 5 × 5 Gy, 10 × 3
Gy, 25 × 2 Gy, and 30 × 1.8 Gy.
188.
189.
190. CRANIOPHARYNGIOMA
6% to 10% of pediatric CNS tumors
Median age of diagnosis: 5 to 10 years, second peak >40
years old
They arise from remnants of Rathke’s pouch (hypophyseal-
pharyngeal duct)
Most commonly suprasellar, may be found in sella proper
191. CRANIOPHARYNGIOMA
Generally abuts hypothalamus and third ventricle.
Histologically, divided into the adamantinomatous and
squamous subtypes.
The adamantinomatous subtype is characterized by a solid
and cystic pattern with the well-known description of
“machine oil-like” cystic fluid.”
192. CRANIOPHARYNGIOMA
Presenting signs and symptoms include
• Headache
• nausea and vomiting
• bitemporal hemianopsia
• endocrine dysfunction (diabetes insipidus, dwarfism, fat tissue
disturbance, adrenal cortical insufficiency)
The most common hormone deficiency is lack of GH.
193. INVESTIGATIVE WORKUP
Similar to that of pituitary adenoma
Includes H&P,
pituitary hormone levels, and
brain MRI with thin slices through the sella
194. SURGERY
Primary goal: Complete resection.
GTR associated with high rates of neurologic sequelae,
including visual impairment and panhypopituitarism.
Most patients are treated with maximal safe resection
followed by adjuvant RT.
Intralesional bleomycin directly into cyst decreases cyst
recurrence.
195. RADIOTHERAPY
Often used in adjuvant setting.
In select patients (<3 years old), RT at the time of relapse
(“salvage” RT)
196. RADIOTHERAPY TECHNIQUE
RT techniques include
3D-CRT, IMRT, FSRT, proton therapy, and intralesional
RT with β-emitting isotopes (yttrium-90,
phosphorous-32).
Postoperative MRI fused with treatment planning CT scan.
197. IMRT: VOLUMES AND DOSE
GTV: postoperative residual tumor volume, including the cyst
wall, if present.
PTV: A margin of 1 to 1.5 cm is added to the PTV
Dose prescriptions for 3D-CRT and IMRT are typically 54 Gy
given in 1.8-Gy daily fractions.
198. IMAGE GUIDED RADIOTHERAPY
Allows for convenient monitoring of cyst regrowth with cone-
beam CT scans while patients are on the treatment table.
199. FRACTIONATED RADIOTHERAPY:
LOMA LINDA SERIES
Fractionated proton radiotherapy has demonstrated excellent
results.
Loma Linda series: 15 patients treated to a total dose of 50.4
to 59.4 GyE given in 1.8-GyE daily fractions.
Local control was achieved in 14 of 15 patients, with few
long-term complications.
200. No failures were seen in 24 patients who received
fractionated proton radiotherapy to a total dose of 52.2 to 54
GyE in 1.8 GyE per fraction.
MASSACHUSETTS GENERAL HOSPITAL
201. STANFORD SERIES
Stanford Study: frameless robotic platform, postoperative
doses of 18 to 38 Gy given over 3 to 10 fractions prescribed to
mean IDL of 75%.
Local control was 91% with no visual or neuroendocrine
complications.
202.
203. INTRALESIONAL INJECTIONS
Cystic craniopharyngiomas: Intralesional radioactive isotope
injection using a β-emitter.
Typical prescriptions: 200 to 250 Gy prescribed to the cyst
wall.
Optimal results are seen in patients whose tumors have one
cyst and lack a large solid component.
204. EPILEPSY
Alternative to surgery in medically refractory epilepsy for
patients who are not surgical candidates.
Intractable mesial temporal lobe epilepsy: 24 to 25 Gy in a
single fraction with Gamma Knife.
205. At a follow-up of 2 years, 65% of the patients were seizure
free.
However, there was a 1-year lag between treatment and
maximal effect
There was a transient increase in seizures before the seizures
started to diminish.