J Neurosurg 74:653-655, 1991

Postoperative ectopic craniopharyngioma Case report ASHVIN T. RAGOOWANSI, M.D., AND DAVID G. PIEPGRAS, M.D.

Department of Neurologic Surgery, Mayo Clinic', Rochester, Minnesota ~" The case of an ectopic craniopharyngiomaarising from a seed of tissue deposited along the operativetrack is reported. The uniqueness of this lesion is addressed. Ideal therapy and controversies regarding radiation therapy of craniopharyngiomasare discussed in light of this new variation in recurrence. KEy WORDS 9 craniopharyngioma 9 ectopic tumor surgical seeding

N 1904, Erdheim ~ carefully detailed the histological features of craniopharyngiomas and postulated that they originated from embryonic squamous cell rests of an incompletely involuted hypophyseal-pharyngeal duct) Since then, these tumors have challenged the skill of neurosurgeons and have stimulated controversy regarding the ideal therapeutic approach. While total removal of the tumor is the most desirable operative goal, unacceptable morbidity and poor quality of survival, especially for patients with large tumors treated in the premicrosurgical era, have caused even the most skilled neurosurgeons to rethink the overall benefits of radical surgery versus subtotal removal and postoperative radiotherapy. The problem of treatment is compounded by the variability of rate and possible directions of growth. We present a case that adds an additional variable in consideration of the ideal therapy for craniopharyngiomas.

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mography showed a suprasellar mass extending into the hypothalamus (Fig. 1). Stereotactic biopsy confirmed a craniopharyngioma. The patient underwent a right frontotemporal craniotomy with gross total removal through the sylvian fissure and lamina terminalis. No postoperative radiation therapy was given. The patient did well on replacement hormonal therapy.

Case Report This 47-year-old man initially presented in September, 1987, with symptoms of decreased visual acuity, retro-orbital headaches, progressive fatigue, and decreased libido. He denied any change in levels of thirst, temperature regulation, or body weight. Endocrinological studies revealed a decreased testosterone value and an increased cortisol level. Visual field studies showed an incongruous right homonymous hemianopsia. Magnetic resonance (MR) imaging and computerized to-

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FIG. 1. Computerized tomography scan obtained in September, 1987, in preparation for stereotaetic biopsy, demonstrating the suprasellar tumor mass. 653

A. T. Ragoowansi and D. G. Piepgras In December, 1988, an MR image with contrasl enhancement was obtained as part of a routine followup evaluation and showed no evidence of recurrent tumor in the suprasellar region (Fig. 2 /tJ?). On reevaluation 1 year later, the patient continued to do well, with improved visual fields and no neurological symptoms; a repeat contrast-enhanced MR image, obtained in December, 1989, revealed a 2.5 • 1.5-cm nodule in the anterior aspect of the sylvian fissure (Fig. 2 right). In retrospect, a smaller lesion approximately 0.75 cm in diameter was present at the same site on the image taken the previous year (Fig. 2). The appearance was consistent with a craniopharyngioma, but no extension to the old tumor bed could be identified. Examination. Neurological examination was normal except for an incomplete, incongruous right homonymous hemianopsia, which was a residual deficit from the original tumor. Operation. The previous right frontotemporal craniotomy was reopened. The anterior portion of the sylvian fissure was split, and the lesion was identified. A partially cystic nodule was circumferentially dissected from the branches of the middle cerebral artery and removed in its entirety. Further dissection down the sylvian fissure confirmed no extension of this nodule into the area of the previous tumor resection in the suprasellar cistern. Gross and light microscopic examination of the nodule confirmed it to be a craniopharyngioma. Postoperative Course. The patient did very well and was discharged from the hospital on the 6th postoperative day. He was seen 3 weeks postoperatively, at which time radiation therapy was considered but rejected by the patient's neurosurgeon and neuro-oncologist, inasmuch as a gross total removal of the tumor at the secondary site had been accomplished. The patient continues to be followed by clinical as well as serial MR studies.

Discussion

While growth characteristics of craniopharyngiomas vary considerably, these tumors usually arise in the limited suprasellar area. The most common sites of craniopharyngiomas are immediately suprasellar either anterior to, posterior to, or beneath the optic chiasm. A small percentage of these tumors are intrasellar. 7 There are many reports in the literature concerning the large size that craniopharyngiomas can achieve, with extension in a number of directions. Cysts have been known to extend into the posterior fossa, with the posterior communicating artery, as far as the foramen magnumfl They can also extend laterally into the sylvian fissure or anteriorly into the frontal lobes. Given such an expansive tumor bed and the often densely adherent nature of the tumor, recurrence is a problem even in the hands of experienced neurosurgeons. The role of radiation therapy after subtotal removal of craniopharyngiomas has been advocated by a number of authors. :6 Survival rates for patients treated with subtotal resection and irradiation are better than for those with subtotal resection alone? However, the complications of radiation therapy must not be underestimated. Radiation necrosis, endocrine deficiency, optic neuritis, and dementia are all reported complications. Tumors secondary to radiation therapy, including gliomas, sarcomas, and meningiomas, have also been reported. 4 Owing to the benign nature of the epithelial cells comprising a craniopharyngioma, tumor recurrence usually occurs at the primary site or in contiguous areas rather than at metastatic sites. Cerebrospinal fluid seeding or distal metastasis have not been reported. There is one report of frontal lobe implantation of craniopharyngioma by repeated needle aspirations. ~This was postulated to occur secondary to seeding of epithelial cells along the needle track. Our case report demonstrates an "ectopic" craniopharyngioma in the sylvian

FIG. 2. Magnetic resonance images with contrast administration. Left: Image obtained in December, 1988, showing a small nodule of the ectopic craniopharyngioma (arrow) in the right anterior sylvian fissure. Right Image obtained in December, 1989, showing the enlarged (2.5 • 1.5 cm) partially cystic noduLe of craniopharyngioma (arrow) in the right anterior sylvian fissure. 654

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Postoperative ectopic craniopharyngioma fissure that was physically separate from the primary site. This is presumably due to tumor growth from a small "seed" dropped in the sylvian fissure during the first operation, Our review of the literature did not disclose a similar proven recurrence of tumor along the operative track and outside the original tumor bed. This case demonstrates a unique scenario of recurrent craniopharyngioma in an ectopic location, While it is possible that postoperative radiation therapy might have prevented the growth of this implant, the inherent risks and side effects of such treatment are a deterrent to its routine use. Long-term follow-up studies of patients treated by gross total microscopic removal of craniopharyngioma, who have had no radiation therapy, may cause this unusual mode of tumor recurrence to be increasingly recognized. References 1. Barloon TJ, Yuh W, Sato Y, et al: Frontal lobe implantation of craniopharyngioma. AJNR 9:406-407, 1988 2. Baskin DS, Wilson CB: Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg 65: 22-27, 1986 3. Carmel P: Craniopharyngiomas, in Wilkins RH, Ren-

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4. 5. 6.

7. 8. 9.

gachary SS (eds): Neurosurgery. New York: McGrawHill, 1985, Vol 1, pp 905-916 Carmel PW, Antunes JL, Chang CH: Craniopharyngiomas in children. Neurosurgery 11:382-389, 1982 Erdheim J: Uber Hypophysengangsreshwulste und Hirncholesteatome. Sitzungsber Akad Wiss 113: 537-726, 1904 Kramer S: Craniopharyngioma: the best treatment is conservative surgery and postoperative radiation therapy, in Morley TP (ed): Current Controversies in Neurosurgery. Philadelphia: WB Saunders, I976, pp 336-343 Northfield DWC: Rathke-pouch tumors. Brain 80:293, 1957 Sung DI, Chang CH, Harisiadis L, et al: Treatment results of craniopharyngioma. Cancer 47:847-852, 1981 Sweet WH: Craniopharyngiomas (with a note of Rathke's cleft or epithelial cysts and on suprasellar cysts), in Schmidek HH, Swee~ WH (eds): Operative Neurosurgieai Techniques. Indications, Methods and Results, ed 2. Orlando, Fla: Grune & Stratton, 1988, Vol 1, pp 349-380

Manuscript received May 31, 1990. Accepted in final form September 24, 1990. Address reprint requests to. Ashvin T. Ragoowansi, M.D., Department of Neurologic Surgery, 5-224 E. Joseph, St. Mary's Hospital, Rochester, Minnesota 55902,

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Postoperative ectopic craniopharyngioma. Case report.

The case of an ectopic craniopharyngioma arising from a seed of tissue deposited along the operative track is reported. The uniqueness of this lesion ...
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