Case Report MINIMAL INVASIVE TREATMENT OF CYSTIC CRANIOPHARYNGIOMAS Shyam Sunder and Rajendra Prasad From the Department of Neurosurgery, Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India. Correspondence to: Dr. Rajendra Prasad, 268/Sec15A. Noida 201301, India. E-mail: [email protected]The management of craniopharyngiomas have been controversial for years. More conservative approaches in the management of craniopharyngioma are reasonable alternatives for treatment. Similar rates of disease control are observed with less morbidity and better quality of survival. We present a case report of a 36 year old male who underwent stereotactic placement of an ommaya catheter with repeated aspiration of cystic portion followed by radiation for a recurrent craniopharyngioma. He has been under followup for 68 months with no reccurance. A 36 year old male who presented with headache, decreased vison and excessive tiredness of one month duration. On examination he had bitemporal hemianopia, vison- R(6/6), L(6/9). There was no motor deficit. He was hypothyroid and had decreased serum testosterone level. MRI brain done revealed a sellar / suprasellar lesion with solid and cystic components measuring 5.6 × 4cm compressing the chiasm. The patient underwent a left pterional craniotomy and decompression of the largely cystic tumor. Small parts of the capsaule adherent to the chiasm were left behind. He was followed up . He developed recurrence of symptoms after 4 months of surgery and MRI showed a recurrence of craniopharyngioma cyst. He underwent stereotactic placement of fine catheter into the cyst connected to an ommaya reservoir from which with repeted aspirations were necessary. Post operatively he recieved stereotactic radiotherapy with X-Knife. He has been on regular followup since then with no recurrence at 68 months. Minimal invasive treatment of the craniopharyngioma cyst wall may be a better alternative than attempting total removal which is associated with a high morbidity.More conservative approaches in the management of largely cystic craniopharyngiomas are reasonable alternatives for treatment. Similar rates of disease control are observed with less morbidity and better quality of survival. Key words: Craniopharyngiomas, Ommaya reservoir placement. INTRODUCTION CRANIOPHARYNGIOMAS constitute 1-13% of intracranial neoplasms. They are the most frequent sellar tumors of childhood representing 10% of CNS tumors in children. The peak incidence is from 5-20 years. A second smaller peak is seen at 6th decade of life [1]. Craniopharyngiomas are epithelial tumors derived from the Rathke’s cleft, which is the embryonal precursor to the adenohypophysis. The craniopharyngeal duct is the embryonal structure along which the eventual adenohypophysis and infundibulum migrate.Some craniopharyngioma may arise within the third ventricle [2]. Infrasellar craniopharyngiomas are rare. From an embryological point of view, this unusual localisation can be explained by Erdheim’s theory that these tumours can arise anywhere along the craniopharyngeal canal (CPC). 15% of these tumor are intrasellar. Most are usually >1 cm [2]. They are usually well circumscribed tumors but may infiltrate into the brain. The tumor cyst contains a machine oil or black sludge cystic fluid with cholesterol crystals and calcifications. Headache is the most common presenting symptom, followed by endocrine deficiencies and visual disturbances. Headache is related to hydrocephalus, which occurs in 15-30% of patients and may need emergent treatment. Ninety-three percent of children experience growth failure. Adults have more varied presentations and may develop sexual or menstrual dysfunction. Eighty-eight percent of men experience decreased sex drive, while 82% of women have amenorrhea. Large tumors in adults can cause psychiatric symptoms, memory loss, apathy, incontinence, depression, and hypersomnia. Long-standing mentation deficits and profound memory loss have been reported and suggest a worse prognosis. Visual deficits are caused by compression of the optic chiasm from suprasellar tumor growth. The most common visual disturbance is a bitemporal hemianopsia. In children, hypothalamic and endocrine dysfunction may develop before visual defects are noticed. Obesity and lethargy are common in children with craniopharyngiomas. Enlarged or eroded sella turcica is seen in 50% of cases. Suprasellar calcifcation is seen in 50% of these cases. They are cystic in 85% of cases [3]. They do not undergo malignant transformation but difficulty in cure makes them malignant in behaviour [4]. Apollo Medicine, Vol. 3, No. 2, June 2006 246
Transcript
Case Report
MINIMAL INVASIVE TREATMENT OF CYSTIC CRANIOPHARYNGIOMAS
Shyam Sunder and Rajendra PrasadFrom the Department of Neurosurgery, Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India.
Correspondence to: Dr. Rajendra Prasad, 268/Sec15A. Noida 201301, India.E-mail: [email protected]
The management of craniopharyngiomas have been controversial for years. More conservative approaches inthe management of craniopharyngioma are reasonable alternatives for treatment. Similar rates of diseasecontrol are observed with less morbidity and better quality of survival. We present a case report of a 36 year oldmale who underwent stereotactic placement of an ommaya catheter with repeated aspiration of cystic portionfollowed by radiation for a recurrent craniopharyngioma. He has been under followup for 68 months with noreccurance. A 36 year old male who presented with headache, decreased vison and excessive tiredness ofone month duration. On examination he had bitemporal hemianopia, vison- R(6/6), L(6/9). There was no motordeficit. He was hypothyroid and had decreased serum testosterone level. MRI brain done revealed a sellar /suprasellar lesion with solid and cystic components measuring 5.6 × 4cm compressing the chiasm. Thepatient underwent a left pterional craniotomy and decompression of the largely cystic tumor. Small parts of thecapsaule adherent to the chiasm were left behind. He was followed up . He developed recurrence of symptomsafter 4 months of surgery and MRI showed a recurrence of craniopharyngioma cyst. He underwentstereotactic placement of fine catheter into the cyst connected to an ommaya reservoir from which withrepeted aspirations were necessary. Post operatively he recieved stereotactic radiotherapy with X-Knife. Hehas been on regular followup since then with no recurrence at 68 months. Minimal invasive treatment of thecraniopharyngioma cyst wall may be a better alternative than attempting total removal which is associatedwith a high morbidity.More conservative approaches in the management of largely cystic craniopharyngiomasare reasonable alternatives for treatment. Similar rates of disease control are observed with less morbidity andbetter quality of survival.
CRANIOPHARYNGIOMAS constitute 1-13% of intracranialneoplasms. They are the most frequent sellar tumors ofchildhood representing 10% of CNS tumors in children.The peak incidence is from 5-20 years. A second smallerpeak is seen at 6th decade of life [1]. Craniopharyngiomasare epithelial tumors derived from the Rathke’s cleft, whichis the embryonal precursor to the adenohypophysis. Thecraniopharyngeal duct is the embryonal structure alongwhich the eventual adenohypophysis and infundibulummigrate.Some craniopharyngioma may arise within thethird ventricle [2]. Infrasellar craniopharyngiomas are rare.From an embryological point of view, this unusuallocalisation can be explained by Erdheim’s theory that thesetumours can arise anywhere along the craniopharyngealcanal (CPC). 15% of these tumor are intrasellar. Most areusually >1 cm [2]. They are usually well circumscribedtumors but may infiltrate into the brain. The tumor cystcontains a machine oil or black sludge cystic fluid withcholesterol crystals and calcifications. Headache is the mostcommon presenting symptom, followed by endocrine
deficiencies and visual disturbances. Headache is relatedto hydrocephalus, which occurs in 15-30% of patients andmay need emergent treatment. Ninety-three percent ofchildren experience growth failure. Adults have morevaried presentations and may develop sexual or menstrualdysfunction. Eighty-eight percent of men experiencedecreased sex drive, while 82% of women haveamenorrhea. Large tumors in adults can cause psychiatricsymptoms, memory loss, apathy, incontinence, depression,and hypersomnia. Long-standing mentation deficits andprofound memory loss have been reported and suggest aworse prognosis. Visual deficits are caused bycompression of the optic chiasm from suprasellar tumorgrowth. The most common visual disturbance is abitemporal hemianopsia. In children, hypothalamic andendocrine dysfunction may develop before visual defectsare noticed. Obesity and lethargy are common in childrenwith craniopharyngiomas. Enlarged or eroded sella turcicais seen in 50% of cases. Suprasellar calcifcation is seen in50% of these cases. They are cystic in 85% of cases [3].They do not undergo malignant transformation butdifficulty in cure makes them malignant in behaviour [4].
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Untreated, these tumors are invasive and may extend intothe hypothalamus and third ventricle resulting inobstructive hydrocephalus. Various surgical optionsavailable include total excison, partial excison, drainageof cystic portion and insertion of ommaya resvoir. The10-40% recurrence rate is usually because of incompletesurgical removal [5]. Case reports of malignant trans-formation to squamous cell carcinoma and glioblastomaafter repeated recurrences over 35 years and 8 years postradiotherapy have been described [6].
CASE HISTORY
A 36-year-old male presented with headache, decreasedvison and excessive tiredness of one month duration. Onexamination he had bitemporal hemianopia with, visonof R(6/6), L(6/9). He had left sided primary opticatrophy.There was no motor deficit. Complete endo-crinological evaluation was done. This included Growthhormone (GH) and insulin growth factor (IGF-1), Cortisol(8 am, 6 pm), Prolactin, Serum testosterone, Thyroid-stimulating hormone [TSH], Free Triiodothyronine (T3)and Free Thyroxine (T4).
He was hypothyroid with decreased serumtestosterone level. MRI brain done revealed a sellar/suprasellar lesion with solid and cystic componentsmeasuring 5.6 × 4cm compressing the chiasm (Fig.1).There was no hydrocephalus..Corticosteroid and thyroidhormone replacement was given preoperatively. Heunderwent Left pterional craniotomy and decompressionof cystic portion. Post operatively CT scan revealeddecompression of cystic portion (Fig.2). He developedrecurrence of symptoms after 4 months (Fig. 3). CT scanshowed a recurrence of tumor cyst. He then underwentstereotactic placement of an ommaya catheter and severalaspirations of cyst were performed postoperatively throughthe ommaya reservoir (Fig. 4). He also receivedstereotactic radiotherapy (SRT) to the lesion by X-Knife.He was followed up for 68 months with no clinical orradiological reccurance (Figs. 5-10).
DISCUSSION
The dilemas that exsist in the management ofcraniopharyngiomas are(i) Should the tumor be totally excised?(ii) Is subtotal excison safer and preferable in the long
run?(iii) Between these two alternatives in which procedure is
hormonal disturbance minimal.(iv) What are the chances of recurrences after subtotal
removal?(v) Does radiotherapy minimise recurrence and minimise
hormonal disturbance?
Fig. 1. Preop image.
Fig. 2. Post op.
Fig.3. Recurrence.
Fig. 4. Stereotactic catheter placement.
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Though total excision should be the aim ofmanagement, the proximity and adherence of the lesion tooptic pathways, hypothalamus and adjacent neurovascularstructures often makes total excison hazardous. Acraniopharyngioma that is small or prechiasmatic inlocation can be excised totally. When the tumor is large,multicompartmental or retrochiasmatic, its total excisonbecomes problematic [7]. Finger like processes of thetumor may burrow into the hypothalamus and this oftenmakes total excison hazardous. Kobayashi et al [8] haveshown that these finger like processes of tumor cells doinfiltrate the hypothalamus and that reactive gliosis oftenprevents total excison of the tumor without hypothalamicdamage. In some cases the tumor does not have its owncapsule and that the cyst wall is formed by thickened
fibrous tunica vasculosa or arachnoidea and that the tumormay invade tunica vasculosa and invade the brain.Themortality is higher and morbidity much greater when totalexcison is attempted for recurrent craniopharyngiomas.The mortality for planned subtotal excison is less than thatfor total excison. This is because of less hypothalamic andvascular injury. It has been shown that the microsurgicaltotal excison of tumors has not always been total and thatsome of them have recurred 10-12 years after surgery [9].This is due to flake of calcium lying in the sella or a smallclustures of cell left behind. The 10-40% recurrence rateis usually because of incomplete surgical removal [10]. Aclose followup of such patients is therefore essential. Acontrast enhanced CT or MRI 6-8 weeks after surgeryshould be done to pick up a residual lesion that may be
Fig. 10. Sixty eight months after radiation.
Fig. 5. One month post radiation.
Fig. 6. Five months post radiation.
Fig. 7. Sixteen months after radiation.
Fig.8. Two years after radiation.
Fig. 9. Forty-two months after radiation.
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tackeled straightaway before it becomes large and developsfirm adhesions to adjacent neurovascular structures.Insertion of catheter with ommaya reservoir is indicatedin cystic lesions [11].
(i) Before radiation therapy to drain cyst.(ii) To treat cyst recurrance with isotope instillation.(iii) Buy time in children until growth is completed.
This modality of treatment is not effective if cyst wallis resected during first surgery or if the tumor is walled offby growth of fibrous tissue. Factors associated with pooroutcome are–lethargy at presentation, visual deterioration,papilledema, tumor calcification, hydrocephalus, tumoradhesiveness at surgery [12]. Craniopharyngiomas withintrasellar components should be followed cautiously andthe necessity for regular follow-up should be emphasized,even when the tumor is “totally resected”.
The 5-year recurrence-free survival rate was 39% forthose who had an intrasellar tumor component and 81%for those who did not [13]. Stereotactic radiosurgery withCT guided stereotactic intracavitory radiation usingphosphorus 32, Au 198, Yttrium 90 are the radioactiveisotopes that have been used for intracavitary irradiationof large cystic craniopharyngiomas [14]. Intracavitaryinstillation of bleomycin creates reactionary fibrosis withsome shrinkage of lesion and thickening of capsule, thatmakes its excision easier.Stereotactic delivery of ionisingradiation permits precise single treatment irradiation of thetumor either LINAC or gamma knife is effective indestroying small tumors or stopping their growth [15].Combined treatment with stereotactic intracavitaryirradiation and gamma knife surgery for cranio-pharyngiomas has recently been described as sole modalityof treatment [16]. Twelve cases of malignant glioma havebeen reported after radiation for craniopharyngioma. Themean latency period was 10.7 years (median 9.6 years).The shortest latency periods were found in patients whohad received GH therapy. The short latency periods forpatients treated with GH is remarkable. It was thereforesuspect that GH therapy may accelerate the developmentof a secondary brain tumour. GH therapy should beavoided in conventionally irradiated craniopharyngiomapatients [17]. Craniopharyngiomas originate from the samecells as squamous cell skin carcinoma, which can betreated successfully with interferon-alpha (IFNalpha)-2a[18].
CONCLUSION
In conclusion a policy of attempted total resection ofsolid craniopharyngiomas where possible and subtotalremoval along with adjuvant radiation in cases where totalresection is deemed unsafe, may be recommended as a
safer and more effective mode of therapy than aggressivetotal resection. In case of largely cystic cranio-pharyngiomas a less aggressive approach may offer longterm control of the disease with less morbidity andmortality. This involves insertion of an ommaya reservoirwith catheter in the cyst for repeated aspiration whennecessary. The cyst and solid component is then irradiatedby SRS or SRT (as in our case) using the X-Knife orGamma Knife. Long term prospective studies will benecessary to see if this conservative, less traumaticapproach is better in term of conserving vison and causingless hormonal deficiency.
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