Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 51, Num. 1, 2003, pp. 133-134

Neurology India, Vol. 51, No. 1, Jan-Mar, 2003, pp. 133-134

Letter to Editor

Craniopharyngioma in an 82-year-old male

S. K. Jain, S. Chopra, P. P. S. Mathur

Department of Neurosurgery, S. M. S. Medical College, Jaipur, India.

Accepted on 14.02.2002.

Code Number: ni03050

Sir

An 82-year-old male presented with behavioral abnormalities of 6 months and loss of memory of 3 months. There was no history of urinary incontinence, headache, loss of consciousness, seizures or visual difficulties. On examination he showed signs of frontal lobe dysfunction with normal fundi. Rest of the neurological examination was unremarkable. CT scan of the brain revealed a non-enhancing hypodense nodular mass in the suprasellar area in the midline, extending towards the left side (Figure 1). A right frontal craniotomy was done and through subfrontal approach, a radical tumor resection was carried out. The lesion was partly cystic, containing machine oil-like thick fluid. Histopathological examination of the specimen was consistent with a craniopharyngioma. It revealed cystic spaces lined by flattened to stratified epithelium and peripheral pallisading of nuclei. Calcification, areas of extensive fibrosis and xanthomatous changes were also seen at places. Focal areas showed ghost appearing squamous cells (Figure 2).

The name craniopharyngioma was introduced by Cushing in 1932.¹ Craniopharyngiomas are epithelial neoplasms arising in the hypothalamic and pituitary region. Although unconfirmed, they are derived from ectopic remnants of pharyngeal epithelium following the embryological evagination of Rathke's pouch during development of adenohypophysis.¹ An alternative hypothesis assumes a metaplastic origin from epithelium in the adenohypophysis and anterior infundibulum.² Craniopharyngioma accounts for approximately 2.5% to 4% of all intracranial neoplasms.¹

Sung et al 1982³ reported tumoral preponderance in childhood years. The oldest patient in his series was 71 years old. The tumor occurs with equal frequency in both the sexes throughout the life, although a male preponderance in childhood has been reported.¹ Russel and Penny in 1961 reported 27% of their patients above 40 years but all patients were under 70 years. The most common symptoms of headache and visual difficulties presented in 75% of the cases.¹ Less frequent complaints include mental changes, nausea, vomiting and endocrinological symptoms like oligo/amenorrhea and hyperprolactinemia.

Although most cases of craniopharyngiomas remain localized to the suprasellar region, they can extend in all directions, anteriorly to the frontal fossa (2%-5%), posteriorly to the posterior fossa (1%-4%) and laterally to the middle fossa (2%). The majority of the craniopharyngiomas are densely calcified within suprasellar cisterns. The predominantly solid tumor or solid element in a cystic tumor are evident as areas of density slightly higher or equal to that of surrounding normal tissue. Cystic tumor may contain thick, highly proteinaceous material with a density of 6 to 18 Hounsfield unit or cholesterol-like material with a density of as low as 40 Hounsfield units.

The commonly accepted microscopic features of craniopharyngiomas are squamous cell epithelium, often stratified and with epidermoid features; different stages of keratinous masses and stacks; a fibro-collagenous matrix, with calcification in the collagen or the keratin; and infiltrated brain tissue with glial cell reaction. Often, important features include presence of concentrically arranged cells undergoing coagulative necrosis known as epithelial pearls. Calcification is common and bone formation may occur. Szeifert et al⁴ showed secretory activity in the craniopharyngiomas and reported immunohistochemical positivity for pituitary hormones in scattered tumor cell groups. The presence of progesterone receptor (PR) and estrogen receptor (ER) messenger RNA has also been demonstrated. Zumkeller W et al 1991⁵ demonstrated insulin-like growth factors I, II and heterogeneous insulin growth factor binding proteins in the cystic fluid of patients with craniopharyngiomas.

Despite the histopathological benign nature, treatment of craniopharyngioma can be difficult. Radical surgical removal is often hindered by their adverse location and adherence to crucial intracranial structures and aggressive surgical procedures are associated with high risk. Moreover, when they are partially removed craniopharyngiomas are prone to recur.² Radiotherapy is beneficial but 5-year recurrence rate is still as high as 25%. Honegger J et al 1997² advocated that sex steroids might affect craniopharyngioma growth as peak incidence occurs at puberty.

S. K. Jain, S. Chopra, P. P. S. Mathur

Department of Neurosurgery, S. M. S. Medical College, Jaipur, India.

References

1. Pang D. Craniopharyngiomas, In: Rengachary SS, Wilkins RH, eds. Principles of Neurosurgery, London: Wolfe; 1994. pp. 2-26.
2. Honegger J, Renner C, Fahlbuarsh R, et al. Progesterone receptor gene expression in Craniopharyngiomas and evidence for biological activity. Neurosurgery 1997;41:1359-63.
3. Sung DI, Chang CH, Harisiadis L, et al. Treatment results of Craniopharyngiomas. Cancer 1981;47:847-52.
4. Szeifert G T, Pasztor I. Could Craniopharyngiomas produce pituitary hormones? Neurol Res 1993;15:68-9.
5. Zumkeller W, Saaf M, Rahn T, et al. Demonstration of insulin like growth factors I, II and heterogeneous insulin like growth factors binding proteins in the cyst fluid of patients with Craniopharyngioma. Neuroendocrinology 1991;54:196-201.

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