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Coexistence of a cerebellopontine epidermoid cyst with a pituitary adenoma
Clinical Neurology and Neurosurgery 104 (2002) 364 /366 www.elsevier.com/locate/clineuro
Case report
Coexistence of a cerebellopontine epidermoid cyst with a pituitary adenoma Serdar Kemalog˘lu a,*, Umit Ozkan a, I˙brahim Ziyal b, Yas¸ar Bukte c, Adnan Ceviz a a b
Department of Neurosurgery, School of Medicine, Dicle University, Diyarbakir, Turkey Department of Neurosurgery, School of Medicine, Hacettepe University, Ankara, Turkey c Department of Radiology, School of Medicine, Dicle University, Diyarbakir, Turkey
Received 18 June 2001; received in revised form 10 January 2002; accepted 10 January 2002
Abstract We report on an 18-year-old girl with the coexistence of an epidermoid cyst in the cerebellopontine angle (CPA) with a pituitary adenoma. The clinical course and histopathological characteristics were reviewed. The patient presented with headache, vision loss and amenorea. Computerised tomographic (CT) scan and magnetic resonance imaging (MRI) examinations demonstrated a solid sellar lesion with supra-sellar extension. There was another mass lesion in the CPA with prepontine extension. The tumours were removed surgically. Histopathological examination revealed an epidermoid cyst in the prepontine area and a pituitary adenoma in the sellar region. This has been the second case to be reported in the literature with the coexistence of an epidermoid cyst and a pituitary adenoma. # 2002 Elsevier Science B.V. All rights reserved. Keywords: Pituitary adenoma; Epidermoid cyst; Multiple tumours
1. Introduction
2. Case report
Intracranial epidermoid cysts account for 0.2 /1% of all brain tumours. However, pituitary adenomas represent 10/20% of all intracranial neoplasms. Epidermoid cysts are most commonly located at the cerebellopontine angle (CPA) and in the parasellar region. They characteristically spread along the basal surface of the brain [1 /4]. Except for cases with Von Recklinghausen’s disease, multiple primary intracranial tumours of different cell types are relatively rare. The most frequent combination of multiple tumours is a meningioma with a glioma [4]. Here we present a unique case of a CPA epidermoid cyst coexisting with a pituitary adenoma and review the possible pathogenesis of those lesions.
An 18-year-old girl with a 2-year history of blurred vision was admitted to our hospital. She had been suffering from headache (blunt and persisting forehead pain) for the last 6 years. She had a slightly unsteady gait ataxia on the left side and had had amenorea for the last 3 months. Her neurological examination revealed left temporal hemianopsia and optic atrophy. The serum prolactine level was 470 mg/dl. Other laboratory data were normal. A skull X-ray revealed grade-3 sella according to Vezina classification. A computerised tomographic (CT) scan of the head showed an enhancing lobulated sellar tumour with suprasellar extension and a homogenous hypodense non-enhancing mass in front of the pons with extension to the left CPA (Fig. 1). On magnetic resonance imaging (MRI) there was an iso-intense lobulated mass (8 /5 /4 cm) with suprasellar extension on T1-weighed images (Fig. 2). This lesion showed nonhomogenous hyperintensity on T2-weighed images (Fig. 3). This sellar lesion with wide suprasellar extension was homogenously enhanced by gadolinium diethylene triamine penta acetic acid (Gd-DTPA). There was an
* Corresponding author. Present address: Faik Ali Sok, Rauf Bey Apt. No. 22 Ofis 21280, Diyarbakır, Turkey. Tel./fax: /90-412-2488182 E-mail address: [email protected] (S. Kemalog˘lu).
0303-8467/02/$ - see front matter # 2002 Elsevier Science B.V. All rights reserved. PII: S 0 3 0 3 - 8 4 6 7 ( 0 2 ) 0 0 0 0 3 - 3
S. Kemalog˘lu et al. / Clinical Neurology and Neurosurgery 104 (2002) 364 /366
Fig. 1. Non-contrast CT scans showing sellar enlargement and CPA hypodense mass with extension to the prepontine area and compressing the brain stem.
Fig. 2. T1-weighted axial MR image showing hypointense giant mass on CPA and sellar region occupied with hyperintense mass.
365
Fig. 4. Gadolinium enhanced T1-weighted sagittal image demonstrated heterogenous hypointense giant mass compressing brainstem, pons and cerebellum. Sellar and suprasellar region occupied with lobulated and enhanced mass, which is obliterated third ventricle.
A double-staged operation was planned. First, a left frontotemporal craniotomy was performed. Due to tumour invasion total resection of the pituitary tumour could not be achieved. Histological examination of the tumour revealed chromophobic cells in a diffuse pattern and spherical amyloid bodies. However, there were no psammomatous microcalcifications (Fig. 5). The pathological diagnosis was prolactinoma. At the second stage a left unilateral suboccipital craniectomy was performed. The tumour capsule was adherent to the brain stem. Falky white contents were removed. Histological examination of the surgical specimens revealed an epidermoid cyst (Fig. 6). After these two operations, her ataxia and hemiparesis improved, but headache and loss of vision persisted for 2 years. Residual prolactinoma has been treated with daily 12 mg doses of bromocriptine. The prolactine level was 48 ng/dl at 19 month follow-up.
Fig. 3. CPA mass revealed hyperintensity and sellar mass showed isointensity on T2-weighted axial image.
additional hypo-intense CPA mass extending to the prepontine area on T1-weighed images. This mass was slightly hyperintense on T2-weighed images, and was not enhancing by Gd-DTPA (Fig. 4).
Fig. 5. Photomicrograph showing prolactinoma having chromophobic cells with aciner pattern and spherical amyloid body (H&E, /40).
366
S. Kemalog˘lu et al. / Clinical Neurology and Neurosurgery 104 (2002) 364 /366
Fig. 6. Photomicrograph showing stratified squamous epithelium with eosinophilic laminated keratinous horny material (H&E, /200).
relationship between epidermoid cysts of the sellar region and craniopharyngioma [1]. Epidermoid cysts may be found in distinct intracranial locations by contagious spread along the normal cleavage lines, and may extent through the tentorial incisura [2,10]. In the literature, the coexistence of tumours of different cell types, especially in the sellar area, has been reported more often than in any other region. To date, there is only one reported epidermoid case associated with pituitary adenoma in sellar area [11]. It seems possible that epidermoid cells originated from the sellar area and spread along the basal surface of the brain stem to the prepontine region. As far as we know, the case we report here, is the first one presenting masses with distinct pathological nature in both supra- and infratentorial regions.
3. Discussion Acknowledgements Simultaneous occurrences of primary intracranial tumours of different cell types have been rarely documented [4]. There are some reports on the coexistence of pituitary adenoma and temporal lobe glioma, prolactinoma and craniopharyngioma, meningioma and pituitary adenoma, mixed germ-cell tumour and craniopharyngioma [4 /7]. Coexistence of meningioma and glioma was the most common combination of multiple tumours in the central nervous system [4]. Due to the complex embryological development of the sellar region, the simultaneous occurrence of primary intracranial tumours of different cell types in this region is considerable. Epidermoid cysts are slowly growing congenital neoplasms believed to arise from the germinal layer [2,3,8,9]. Epithelial components of the cyst wall develop between the 3rd and 5th weeks of embryonic life and are generally assumed to stem from the inclusion of ectoderm elements at the time of closure of the neural groove or during the formation of the cerebral vesicles [1,3,8]. Separation of neuroectoderm from its cutaneous counterpart occurs dorsally along the midline and easily explains the median location of many of these tumours. The lateral preference of the most epidermoid tumours is possibly due to proliferation of multipotential embryonic cell rests or transplantation of epithelial cell rests carried laterally with migrating otic vesicles or developing neurovasculathre. The parapituitary stalk lesions are attributed to the proliferation focus of the squamous cell rests in the anterior hypophysis [1]. Metaplasia of anterior hypophysial cells in assumed to be the source of the epidermoid cysts in this region [1,9]. It has also been proposed that there might be a new
The authors would you like to thank Elif Kemalog˘lu for editing and Murat Ag˘ar for typing the manuscript.
References [1] Berger MS, Wilson CB. Epidermoid cysts of posterior fossa. J Neurosurg 1984;62:214 /9. [2] Yas¸argil MG, Abernatey CD, Sarialioglu AC ¸ . Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561 /7. [3] Yoshizato K, Kai Y, Kuratsu J, Ushio Y. Intramedullary epidermoid cyst in the brain stem: case report. Surg Neurol 1996;45:537 /40. [4] Asari J, Yamanobe K, Sasaki T, Yamao N, Kodama N. A case of prolactinoma associated with craniopharyngioma. No Shinkei Geka 1987;12:1313 /8. [5] Gyori E, Gregg PA. Invasive pituitary adenoma co-existing with infiltrating temporal lobe glioma. South Med J 1992;85:1134 /7. [6] Laun A, Lenzen J, Hildebrandt G, Schachenmayr W. Tuberculum sellae meningioma and hypophyseal adenoma in a woman. Zentralbl Neurochir 1993;54(3):119 /24. [7] Nada R, Mohan H, Dhir SP, Mukherjee KK, Kak VK. Suprasellar malignant mixed germ cell tumour presenting as craniopharengioma. Neurol India 2000;48(4):381 /4. [8] Obana GW, Wilson CB. Epidermoid cysts of the brain stem. J Neurosurg 1991;74:123 /8. [9] Yamakava K, Shitara N, Genka S, Manaka S, Takakura K. Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 1989;24:568 /73. [10] Palmucci L, Liboni W, Favero M. Unusual extension of an epidermoid cyst. Zentrabl Neurochir 1977;38(3):299 /303. [11] Kaspera W, Macyszyn GB, Majchrzak H. A case of parasellar tumor with double histological texture: chromophobe pituitary adenoma and epidermoid cyst. Neurol Neurochir Pol 1998;32(4):987 /96.
Case report
Coexistence of a cerebellopontine epidermoid cyst with a pituitary adenoma Serdar Kemalog˘lu a,*, Umit Ozkan a, I˙brahim Ziyal b, Yas¸ar Bukte c, Adnan Ceviz a a b
Department of Neurosurgery, School of Medicine, Dicle University, Diyarbakir, Turkey Department of Neurosurgery, School of Medicine, Hacettepe University, Ankara, Turkey c Department of Radiology, School of Medicine, Dicle University, Diyarbakir, Turkey
Received 18 June 2001; received in revised form 10 January 2002; accepted 10 January 2002
Abstract We report on an 18-year-old girl with the coexistence of an epidermoid cyst in the cerebellopontine angle (CPA) with a pituitary adenoma. The clinical course and histopathological characteristics were reviewed. The patient presented with headache, vision loss and amenorea. Computerised tomographic (CT) scan and magnetic resonance imaging (MRI) examinations demonstrated a solid sellar lesion with supra-sellar extension. There was another mass lesion in the CPA with prepontine extension. The tumours were removed surgically. Histopathological examination revealed an epidermoid cyst in the prepontine area and a pituitary adenoma in the sellar region. This has been the second case to be reported in the literature with the coexistence of an epidermoid cyst and a pituitary adenoma. # 2002 Elsevier Science B.V. All rights reserved. Keywords: Pituitary adenoma; Epidermoid cyst; Multiple tumours
1. Introduction
2. Case report
Intracranial epidermoid cysts account for 0.2 /1% of all brain tumours. However, pituitary adenomas represent 10/20% of all intracranial neoplasms. Epidermoid cysts are most commonly located at the cerebellopontine angle (CPA) and in the parasellar region. They characteristically spread along the basal surface of the brain [1 /4]. Except for cases with Von Recklinghausen’s disease, multiple primary intracranial tumours of different cell types are relatively rare. The most frequent combination of multiple tumours is a meningioma with a glioma [4]. Here we present a unique case of a CPA epidermoid cyst coexisting with a pituitary adenoma and review the possible pathogenesis of those lesions.
An 18-year-old girl with a 2-year history of blurred vision was admitted to our hospital. She had been suffering from headache (blunt and persisting forehead pain) for the last 6 years. She had a slightly unsteady gait ataxia on the left side and had had amenorea for the last 3 months. Her neurological examination revealed left temporal hemianopsia and optic atrophy. The serum prolactine level was 470 mg/dl. Other laboratory data were normal. A skull X-ray revealed grade-3 sella according to Vezina classification. A computerised tomographic (CT) scan of the head showed an enhancing lobulated sellar tumour with suprasellar extension and a homogenous hypodense non-enhancing mass in front of the pons with extension to the left CPA (Fig. 1). On magnetic resonance imaging (MRI) there was an iso-intense lobulated mass (8 /5 /4 cm) with suprasellar extension on T1-weighed images (Fig. 2). This lesion showed nonhomogenous hyperintensity on T2-weighed images (Fig. 3). This sellar lesion with wide suprasellar extension was homogenously enhanced by gadolinium diethylene triamine penta acetic acid (Gd-DTPA). There was an
* Corresponding author. Present address: Faik Ali Sok, Rauf Bey Apt. No. 22 Ofis 21280, Diyarbakır, Turkey. Tel./fax: /90-412-2488182 E-mail address: [email protected] (S. Kemalog˘lu).
0303-8467/02/$ - see front matter # 2002 Elsevier Science B.V. All rights reserved. PII: S 0 3 0 3 - 8 4 6 7 ( 0 2 ) 0 0 0 0 3 - 3
S. Kemalog˘lu et al. / Clinical Neurology and Neurosurgery 104 (2002) 364 /366
Fig. 1. Non-contrast CT scans showing sellar enlargement and CPA hypodense mass with extension to the prepontine area and compressing the brain stem.
Fig. 2. T1-weighted axial MR image showing hypointense giant mass on CPA and sellar region occupied with hyperintense mass.
365
Fig. 4. Gadolinium enhanced T1-weighted sagittal image demonstrated heterogenous hypointense giant mass compressing brainstem, pons and cerebellum. Sellar and suprasellar region occupied with lobulated and enhanced mass, which is obliterated third ventricle.
A double-staged operation was planned. First, a left frontotemporal craniotomy was performed. Due to tumour invasion total resection of the pituitary tumour could not be achieved. Histological examination of the tumour revealed chromophobic cells in a diffuse pattern and spherical amyloid bodies. However, there were no psammomatous microcalcifications (Fig. 5). The pathological diagnosis was prolactinoma. At the second stage a left unilateral suboccipital craniectomy was performed. The tumour capsule was adherent to the brain stem. Falky white contents were removed. Histological examination of the surgical specimens revealed an epidermoid cyst (Fig. 6). After these two operations, her ataxia and hemiparesis improved, but headache and loss of vision persisted for 2 years. Residual prolactinoma has been treated with daily 12 mg doses of bromocriptine. The prolactine level was 48 ng/dl at 19 month follow-up.
Fig. 3. CPA mass revealed hyperintensity and sellar mass showed isointensity on T2-weighted axial image.
additional hypo-intense CPA mass extending to the prepontine area on T1-weighed images. This mass was slightly hyperintense on T2-weighed images, and was not enhancing by Gd-DTPA (Fig. 4).
Fig. 5. Photomicrograph showing prolactinoma having chromophobic cells with aciner pattern and spherical amyloid body (H&E, /40).
366
S. Kemalog˘lu et al. / Clinical Neurology and Neurosurgery 104 (2002) 364 /366
Fig. 6. Photomicrograph showing stratified squamous epithelium with eosinophilic laminated keratinous horny material (H&E, /200).
relationship between epidermoid cysts of the sellar region and craniopharyngioma [1]. Epidermoid cysts may be found in distinct intracranial locations by contagious spread along the normal cleavage lines, and may extent through the tentorial incisura [2,10]. In the literature, the coexistence of tumours of different cell types, especially in the sellar area, has been reported more often than in any other region. To date, there is only one reported epidermoid case associated with pituitary adenoma in sellar area [11]. It seems possible that epidermoid cells originated from the sellar area and spread along the basal surface of the brain stem to the prepontine region. As far as we know, the case we report here, is the first one presenting masses with distinct pathological nature in both supra- and infratentorial regions.
3. Discussion Acknowledgements Simultaneous occurrences of primary intracranial tumours of different cell types have been rarely documented [4]. There are some reports on the coexistence of pituitary adenoma and temporal lobe glioma, prolactinoma and craniopharyngioma, meningioma and pituitary adenoma, mixed germ-cell tumour and craniopharyngioma [4 /7]. Coexistence of meningioma and glioma was the most common combination of multiple tumours in the central nervous system [4]. Due to the complex embryological development of the sellar region, the simultaneous occurrence of primary intracranial tumours of different cell types in this region is considerable. Epidermoid cysts are slowly growing congenital neoplasms believed to arise from the germinal layer [2,3,8,9]. Epithelial components of the cyst wall develop between the 3rd and 5th weeks of embryonic life and are generally assumed to stem from the inclusion of ectoderm elements at the time of closure of the neural groove or during the formation of the cerebral vesicles [1,3,8]. Separation of neuroectoderm from its cutaneous counterpart occurs dorsally along the midline and easily explains the median location of many of these tumours. The lateral preference of the most epidermoid tumours is possibly due to proliferation of multipotential embryonic cell rests or transplantation of epithelial cell rests carried laterally with migrating otic vesicles or developing neurovasculathre. The parapituitary stalk lesions are attributed to the proliferation focus of the squamous cell rests in the anterior hypophysis [1]. Metaplasia of anterior hypophysial cells in assumed to be the source of the epidermoid cysts in this region [1,9]. It has also been proposed that there might be a new
The authors would you like to thank Elif Kemalog˘lu for editing and Murat Ag˘ar for typing the manuscript.
References [1] Berger MS, Wilson CB. Epidermoid cysts of posterior fossa. J Neurosurg 1984;62:214 /9. [2] Yas¸argil MG, Abernatey CD, Sarialioglu AC ¸ . Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561 /7. [3] Yoshizato K, Kai Y, Kuratsu J, Ushio Y. Intramedullary epidermoid cyst in the brain stem: case report. Surg Neurol 1996;45:537 /40. [4] Asari J, Yamanobe K, Sasaki T, Yamao N, Kodama N. A case of prolactinoma associated with craniopharyngioma. No Shinkei Geka 1987;12:1313 /8. [5] Gyori E, Gregg PA. Invasive pituitary adenoma co-existing with infiltrating temporal lobe glioma. South Med J 1992;85:1134 /7. [6] Laun A, Lenzen J, Hildebrandt G, Schachenmayr W. Tuberculum sellae meningioma and hypophyseal adenoma in a woman. Zentralbl Neurochir 1993;54(3):119 /24. [7] Nada R, Mohan H, Dhir SP, Mukherjee KK, Kak VK. Suprasellar malignant mixed germ cell tumour presenting as craniopharengioma. Neurol India 2000;48(4):381 /4. [8] Obana GW, Wilson CB. Epidermoid cysts of the brain stem. J Neurosurg 1991;74:123 /8. [9] Yamakava K, Shitara N, Genka S, Manaka S, Takakura K. Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 1989;24:568 /73. [10] Palmucci L, Liboni W, Favero M. Unusual extension of an epidermoid cyst. Zentrabl Neurochir 1977;38(3):299 /303. [11] Kaspera W, Macyszyn GB, Majchrzak H. A case of parasellar tumor with double histological texture: chromophobe pituitary adenoma and epidermoid cyst. Neurol Neurochir Pol 1998;32(4):987 /96.