MANAGEMENT OF

CRANIOPHARYNGIO
MA
RES. NGUYEN QUANG VIET
 Epidemiology; pathology of craniopharyngiomas
(CP).

Diagnosis of craniopharygiomas: Clinical

manifestations; Imaging.
OBJECTIVE
Management of craniopharyngiomas.

Management of recurrent and residual

progression craniopharyngiomas; cystic expansion
EPIDEMIOLOGY OF CP
3 % of intracranial neoplasms.
Incidence: 0,13-0,18/100000
person-years (US).

Bimodal distribution by age:

late adulthood (65- 75 yrs)
and childhood ( 5 – 14 yr).
PATHOLOGY

2 subtypes:
• Adamantinomatous
CP (ACP): majority in
children.
• Papilary CP (PCP):
mainly occur in adults.
Hermann L. Müller in Nature Review Disease Primers 5(1):75; 2019.
HYPHOTHESIS OF
CRANIOPHARYNGIOM
AS FORMATION

AJNR Am J Neuroradiol 18:77–87, January 1997

CLINICAL MANIFESTATION
The clinical manifestations are multiple and depend on the location of the
tumor, its size, growth pattern, and relationship to adjacent cerebral structures.
In adults the most common presenting clinical symptoms are visual field deficits
and signs of hypopituitarism (Hoff and Patterson, 1972).
In children: the clinical manifestation at the time of diagnosis is often dominated
by nonspecific manifestations of intracranial hypertension. Futher leading
Further leading manifestations include visual impairment (62–84%) and
endocrine deficits (52–87%).
Youmans and Winn neurological surgery; 7 edittion
IMAGING MANIFESTIATIONS

Gabriel zada et al; Neurosurg Focus 28 (4):E4, 2010

IMAGING MANIFESTIATIONS

S H Choi et al, Clininal Radiology (2007) 62(5):453-62.

MANAGEMENT OF CP
Due to the lack of prospective randomized trials, it cannot be stated with
certainty which treatment is optimal.
Treatment plans must be individualized.
Treatment options:
-Surgery.
-Radiotherapy: radiosurgery; intracavitary radiation; conventional radiotherapy;
proton therapy.
-Chemotherapy/ systemic therapy.
THE ROLE OF SURGERY IN CP
Surgical intervention is the initial step in the management of
craniopharyngioma.
The aspects of surgery:
-Preoperative management.
-Goal of surgery: grosstotal resection (GTR) or subtotal resection (STR).
-Approach route.
-Postoperative management.
PREOPERATIVE MANAGEMENT
Imaging characteristics: features of tumor: size; calcification; expansion;
invasion; adherence; associated structures: vasculars; hypothalamus….
Endocrine evaluation: hypopituitarism; hypothalamic compromic.
Opthalmological evaluation:
Neuropsychological assessment.
CP CLASSIFICATIONS
Several surgical classifications have been described such as: Yasagil; Wang;
Hoffman; Kasam; Sami… => choose the approach routes. Puget’s classification =>
predict the hypothalamus injury.
CP CLASSIFICATIONS

Neurosurg Focus 41 (6):E10, 2016

 CP CLASSIFICATIONS

Classification base on adherence

Puget’s classification
GTR versus STR

Neurosurg Focus 28 (4):E5, 2010

 J Neurooncol (2012) 108:133–139

Journal of Clinical Neuroscience 19 (2012) 1005–1008

Þ The goal of complete
resection and avoidance of
iatrogenic morbidity, even at
the expense of total
resection, is reasonable.
Youmans neurological surgery - 6th Edition -2012
Surgical approach:
endoscopic or transcranial?
• In general, the best approach should be
able to provide the maximal surgical
exposure and the most direct trajectory
to the tumor limiting brain retraction
and minimizing manipulation of
neurovascular structures.
• The choice of the approach will depend
on many factors: size; extension of
tumors; associated structured… and last,
but not least, the preferences and
experience of the surgical team
TRANSCRANIAL SURGICAL
APPROACH
POSTOPERATIVE MANAGEMENT
Clinical and endocrinological follow-up
Opthalmological follow-up.
Neurocognitive functions evaluation.
Imaging follow- up.
IMAGING FOLLOW - UP
RADIOTHERAPY IN CP
TIMING OF RADIOTHERAPY
W F Regine (1993): a potentially worse outcome for patients treated for recurrence than those
patients undergoing immediate adjuvant treatment.
Moon SH (2005): control rates did not depend upon the timing of radiation (ie, adjuvant vs
salvage therapy), but there were higher rates of diabetes insipidus; loss of visual field and acuity
in the delayed radiotherapy group.
Eisenstat (2001): , it may be best to treat some children at the time of recurrence.
OTHER TREATMENT
Intracavitary bleomycin.
Intracavitary interferon.
Target therapy.
 RECURRENCES AND RESIDUAL
PROGRESSION
• Options: watch and wait strategy,
second surgery, radiotherapy,
intracystic chemotherapy,
immunotherapy or target.
• The timing to perform: a matter
Examples:
of debate. • Small calcified residual tumor is present, showing no growth at
• The tumor progression history follow-up image???
• Rapidly growing residual tumors???
is the key factor to consider the
best tailored appropriate
treatment option for the patient,
and the management plan
should be based on a
multidisciplinary discussion
 SECOND SURGERY
• More difficult than primary
surgeries.
• The rates of GTR are
significantly inferior to
results obtained after
primary surgery while the
morbidity and mortality rate
is considerably high.
CYSTIC EXPANSION
When residual tissue exists, there is a propensity for further cyst development
and expansion during and after radiation therapy.
This can result in obstructive hydrocephalus, visual changes, and/or clinical
decline.
In the majority of patients, cyst expansion is a self-limiting process and does not
confer a worse outcome.
Options: cyst fenestration and aspiration and/or intracystic injectable agents;
image-guided radiation therapy;
John Varlotto ,Neuro-Oncology Practice 3(3), 173–187, 2016
 Craniopharyngioma is a a great challenge in management with
high recurrence rate.

Conclusive treatment remains a matter of debate.

CONCLUSION
When GTR is not an option, STR combined with radiotherapy
becomes the therapeutic option of choice.

Most importantly, the treatment of craniopharyngiomas is

complicated both surgically and medically, necessitating a
multidisciplinary approach involving neurosurgery, neurology,
endocrinology, ophthalmology and neuropsychology.