55
Mucous-Secreting Choroid Plexus Adenoma Case Report and Review of the Literature By M.
s. M. Eljamel and R. V.]effreys
The Mersey Regional Department of Neurosciences, Walton Hospital, Liverpool L9 1AE, Great Britain
Abstract
Keywords Choroid plexus - Mucous-secreting adenoma - Mucous-secreting adenocarcinoma - Lateral ventrieIe
Introduclion Choroid plexus tumours represent 0.4-0.7 % of verified intracranial tumours (2, 3, 6, 7, 9, 13, 14). Mucousseereting choroid plexus adenomas are even rarer and only three other cases have been reported in the literature (1, 5, 8), we add another case.
Fig. 1 a Cranial CT with contrast showing a large left ventricular cyst with a thin capsule.
Case report A 3 1/2-year-old girl was presented in April 1987 with seizures. The seizures consisted of stereotyped myoclonic movements of bizarre nature. There was no family history of seizures and she had a normal perinatal history with no significant past medical history apart from childhood asthma. The abnormal physical signs were bulging of the skull in the left occipital region and minimal dysphasia. An EEG showed persistent slow wave discharge from the left temporal region. She had been treated with carbamazepine and Sodiumvalproate but the seizures persisted and she became irritable and hyperkinetic. Cranial Computed Tomogram (CT) showed a large left ventricular cyst with minimal hydrocephalus (Fig. 1 a). She underwent aspiration of the cyst and 30 mls of golden yellow fluid was obtained. The fluid contained: 3.3 g% protein, 4.7% mmoVI glucose and 12 cells/cmm mainly degenerative ependymal cells. A few days later a repeat CT showed that the cyst is smaller but the capsule was more obviously enhancing with contrast (Fig. 1b). 1t was therefore decided to exReceived May 29, 1989; accepted July 4, 1989 Neuropediatrics 21 (1990) 55-56 © Hippokrates Verlag Stuttgart
Fig. 1 b Cranial CT with contrast after cyst aspiration showing the cyst is smaller and the capsule is more obvious.
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Choroid plexus tumours are rare, and mucous-secreting choroid plexus adenomas are even rarer. The authors report the first case of mucous-secreting choroid plexus adenoma in childhood and review the relevant literature.
N europediatrics 21 (1990)
M. S. M. Eljamel et al
plore the lesion and attempt excision. Through a left occipital craniotomy the tumour was totally excised using a carbon dioxide Laser. The tumour was a multilocular with nodular surface and microscopically consisted of multiple cystic spaces lined by cuboidal to columnar epithelium containing amorphous material staining positive for mucin.
Discussion The overall incidence of choroid plexus tumoursvariesfrom 0.4-0.7% (2, 3, 6, 7, 9,13,14). Fortytofifty per cent of them appear under the age of 20 years (3, 9), and occur predominantly in the lateral ventricles (3, 4, 11, 14). Mucous-secreting choroid plexus adenomas (MSCPA) are extremely rare. From eleven choroid plexus papillomas (0.25 % of aIl verified intracranial tumours) surgically treated in the Mersey Regional Department of Neurosciences, only one was MSCPA. Only three other cases have been found in the literature (Table 1), and all were more than 22 years of age. The present case is different from the previously reported cases in that it was presented at 3 1/2 years of age, occupied the left lateral ventricle, manifested bizarre seizures and was totaIly removed with a good result. In addition there are three other reported cases of mucous-secreting choroid plexus adenocarcinomas (MSCPCa) (Table 1). It is essential to differentiate this malignant variety from the benign form. Lewis (10) laid down the light microscopic features of MSCPCa's as foIlows: invasion of the surrounding tissues, pleomorphism, hyperchromatism, loss of cell polarity, abnormal mitosis and extensive cerebro-spinal metastasis.
lable 1
Mucous-secreting choroid plexus tumours reported in the English literature.
Authorjyear
Ajir, F., et al
1982 (1)
Davis, R. L.,
1970 (5)
RLV
22 M
1967 (8)
IVV
68 F
et al**
Hoenig, E. M.,
Presenting features
Histology
difficulty in walking headachej vomiting syncope j ataxia
MSCPA MSCPA MSCPA
et al
Matsushima, T Shuangshot, S.,
1983 (12) CPA 1971 (15) RLV
46 F 47 F
et al
Tham, K. T, et al 1969 (16) LCPA
66 F
hydrocephalus CPACa headachej CPACa diplopia deafnessjfacial CPACa palsy
** The same case reported by Russel, D. S., and D. S. Rubinstein 1977 (14), RLV right lateral ventricle, IVV 4th ventricle, LCPA left cerebellopontine angle, CPA cerebellopontine angle, M male, F female, Age in years, MSCPA mucous-secreting choroid plexus adenoma and CPACa choroid plexus adenocarcinoma
Matsushima (12) reported the electron microscopic features of MSCPCa's particularly the presence of numerous intracytoplasmic secretory· vesicles and glycocalyx. The prolonged history and the absence of features of malignancy in the present case suggest a benign behavior. The child remained weIl and her hydrocephalus resolved without the requirement of CSF diversion. MSCPAs are so rare that their natural history is unknown, hence there is a need for further documentation of such tumours.
References Ajir, F., K. Chanbusarakum,]. C. Bolles: Acinar choroid plexus adenoma of the fourth ventricle. Surg. Neurol. 17 (1982) 290-292 2 Bailey, P.: Intracranial Tumours. Springfield, Charles C. Thomas, III, 1933 3 Bohm, E., R. Strang: Choroid plexus papillomas. J. Neurosurg. 18 (1961) 493-500 4 Boyd, M. C., P. Steinbock: Choroid plexus tumours: problems in diagnosis and management. J. Neurosurg. 66 (1987) 800-805 5 Davis, R. L., G. E. Fox: Acinar choroid plexus adenoma (case report). J. Neurosurg. 33 (1970) 587-590 6 Guidetti, B., A. Spallone: The surgical treatment of choroid plexus papillomas. The results of 27 years experience. Neurosurg. Rev. 4 (1981) 129-137 7 Hawkins,]. C. 111.: Treatment of choroid plexus papillomas in children: a brief analysis of twenty years experience. Neurosurgery 6 (1980) 380384 8 Hoenig, E. M., N. R. Ghatak, A. Hirano, H. M. Zimmerman: Multiloculated cystic tumour of the choroid plexus of the fourth ventricle (case report). J. Neurosurg. 27 (1967) 574-579 9 Kahn, E. A.,]. T. Luros: Hydrocephalus from overproduction of cerebrospinal fluid (and experiences with other papillomas of choroid plexus). J. Neurosurg. 9 (1952) 59-67 10 Lewis, P.: Carcinoma of the choroid plexus. Brain 90 (1960) 177-186 11 Mannoji, H., L. E. Becker: Ependymal and choroid plexus tumours, cytokeratin and GFAP expression. Cancer 61 (1988) 1377-1385 12 Matsushima, T.: Choroid plexus papillomas and human choroid plexus, a light and electron microscopic study. J. Neurosurg. 59 (1983) 10541062 13 Paraicz, E., 1. Szenazy: Brain tumours in infancy and childhood; areport of 843 treated cases. Acta Paediatr. Acad. Sei. Hung. 22 (1981) 251-258 14 Russel, D. S., L.]. Rubinstein: Pathology of Tumours of the Nervous System (ed.4). London, E. Arnold (1977) 220-224 15 Shuangshoti, S., P. Tangchai, G. Netsky: Primary adenocarcinoma of choroid plexus. Arch. Pathol. 91 (1971) 101-106 16 Tham, K. T., H. L. Wen, T. B. Teoh: A case of papillary adenocarcinoma of the choroid plexus. J. Pathol. 99 (1969) 321-324 1
M. S. M. Eljamel, FRCS1 The Mersey Regional Dept. of Neurosciences Walton Hospital Liverpool L9 1AE, Great Britain
Downloaded by: NYU. Copyrighted material.
56
Mucous-Secreting Choroid Plexus Adenoma Case Report and Review of the Literature By M.
s. M. Eljamel and R. V.]effreys
The Mersey Regional Department of Neurosciences, Walton Hospital, Liverpool L9 1AE, Great Britain
Abstract
Keywords Choroid plexus - Mucous-secreting adenoma - Mucous-secreting adenocarcinoma - Lateral ventrieIe
Introduclion Choroid plexus tumours represent 0.4-0.7 % of verified intracranial tumours (2, 3, 6, 7, 9, 13, 14). Mucousseereting choroid plexus adenomas are even rarer and only three other cases have been reported in the literature (1, 5, 8), we add another case.
Fig. 1 a Cranial CT with contrast showing a large left ventricular cyst with a thin capsule.
Case report A 3 1/2-year-old girl was presented in April 1987 with seizures. The seizures consisted of stereotyped myoclonic movements of bizarre nature. There was no family history of seizures and she had a normal perinatal history with no significant past medical history apart from childhood asthma. The abnormal physical signs were bulging of the skull in the left occipital region and minimal dysphasia. An EEG showed persistent slow wave discharge from the left temporal region. She had been treated with carbamazepine and Sodiumvalproate but the seizures persisted and she became irritable and hyperkinetic. Cranial Computed Tomogram (CT) showed a large left ventricular cyst with minimal hydrocephalus (Fig. 1 a). She underwent aspiration of the cyst and 30 mls of golden yellow fluid was obtained. The fluid contained: 3.3 g% protein, 4.7% mmoVI glucose and 12 cells/cmm mainly degenerative ependymal cells. A few days later a repeat CT showed that the cyst is smaller but the capsule was more obviously enhancing with contrast (Fig. 1b). 1t was therefore decided to exReceived May 29, 1989; accepted July 4, 1989 Neuropediatrics 21 (1990) 55-56 © Hippokrates Verlag Stuttgart
Fig. 1 b Cranial CT with contrast after cyst aspiration showing the cyst is smaller and the capsule is more obvious.
Downloaded by: NYU. Copyrighted material.
Choroid plexus tumours are rare, and mucous-secreting choroid plexus adenomas are even rarer. The authors report the first case of mucous-secreting choroid plexus adenoma in childhood and review the relevant literature.
N europediatrics 21 (1990)
M. S. M. Eljamel et al
plore the lesion and attempt excision. Through a left occipital craniotomy the tumour was totally excised using a carbon dioxide Laser. The tumour was a multilocular with nodular surface and microscopically consisted of multiple cystic spaces lined by cuboidal to columnar epithelium containing amorphous material staining positive for mucin.
Discussion The overall incidence of choroid plexus tumoursvariesfrom 0.4-0.7% (2, 3, 6, 7, 9,13,14). Fortytofifty per cent of them appear under the age of 20 years (3, 9), and occur predominantly in the lateral ventricles (3, 4, 11, 14). Mucous-secreting choroid plexus adenomas (MSCPA) are extremely rare. From eleven choroid plexus papillomas (0.25 % of aIl verified intracranial tumours) surgically treated in the Mersey Regional Department of Neurosciences, only one was MSCPA. Only three other cases have been found in the literature (Table 1), and all were more than 22 years of age. The present case is different from the previously reported cases in that it was presented at 3 1/2 years of age, occupied the left lateral ventricle, manifested bizarre seizures and was totaIly removed with a good result. In addition there are three other reported cases of mucous-secreting choroid plexus adenocarcinomas (MSCPCa) (Table 1). It is essential to differentiate this malignant variety from the benign form. Lewis (10) laid down the light microscopic features of MSCPCa's as foIlows: invasion of the surrounding tissues, pleomorphism, hyperchromatism, loss of cell polarity, abnormal mitosis and extensive cerebro-spinal metastasis.
lable 1
Mucous-secreting choroid plexus tumours reported in the English literature.
Authorjyear
Ajir, F., et al
1982 (1)
Davis, R. L.,
1970 (5)
RLV
22 M
1967 (8)
IVV
68 F
et al**
Hoenig, E. M.,
Presenting features
Histology
difficulty in walking headachej vomiting syncope j ataxia
MSCPA MSCPA MSCPA
et al
Matsushima, T Shuangshot, S.,
1983 (12) CPA 1971 (15) RLV
46 F 47 F
et al
Tham, K. T, et al 1969 (16) LCPA
66 F
hydrocephalus CPACa headachej CPACa diplopia deafnessjfacial CPACa palsy
** The same case reported by Russel, D. S., and D. S. Rubinstein 1977 (14), RLV right lateral ventricle, IVV 4th ventricle, LCPA left cerebellopontine angle, CPA cerebellopontine angle, M male, F female, Age in years, MSCPA mucous-secreting choroid plexus adenoma and CPACa choroid plexus adenocarcinoma
Matsushima (12) reported the electron microscopic features of MSCPCa's particularly the presence of numerous intracytoplasmic secretory· vesicles and glycocalyx. The prolonged history and the absence of features of malignancy in the present case suggest a benign behavior. The child remained weIl and her hydrocephalus resolved without the requirement of CSF diversion. MSCPAs are so rare that their natural history is unknown, hence there is a need for further documentation of such tumours.
References Ajir, F., K. Chanbusarakum,]. C. Bolles: Acinar choroid plexus adenoma of the fourth ventricle. Surg. Neurol. 17 (1982) 290-292 2 Bailey, P.: Intracranial Tumours. Springfield, Charles C. Thomas, III, 1933 3 Bohm, E., R. Strang: Choroid plexus papillomas. J. Neurosurg. 18 (1961) 493-500 4 Boyd, M. C., P. Steinbock: Choroid plexus tumours: problems in diagnosis and management. J. Neurosurg. 66 (1987) 800-805 5 Davis, R. L., G. E. Fox: Acinar choroid plexus adenoma (case report). J. Neurosurg. 33 (1970) 587-590 6 Guidetti, B., A. Spallone: The surgical treatment of choroid plexus papillomas. The results of 27 years experience. Neurosurg. Rev. 4 (1981) 129-137 7 Hawkins,]. C. 111.: Treatment of choroid plexus papillomas in children: a brief analysis of twenty years experience. Neurosurgery 6 (1980) 380384 8 Hoenig, E. M., N. R. Ghatak, A. Hirano, H. M. Zimmerman: Multiloculated cystic tumour of the choroid plexus of the fourth ventricle (case report). J. Neurosurg. 27 (1967) 574-579 9 Kahn, E. A.,]. T. Luros: Hydrocephalus from overproduction of cerebrospinal fluid (and experiences with other papillomas of choroid plexus). J. Neurosurg. 9 (1952) 59-67 10 Lewis, P.: Carcinoma of the choroid plexus. Brain 90 (1960) 177-186 11 Mannoji, H., L. E. Becker: Ependymal and choroid plexus tumours, cytokeratin and GFAP expression. Cancer 61 (1988) 1377-1385 12 Matsushima, T.: Choroid plexus papillomas and human choroid plexus, a light and electron microscopic study. J. Neurosurg. 59 (1983) 10541062 13 Paraicz, E., 1. Szenazy: Brain tumours in infancy and childhood; areport of 843 treated cases. Acta Paediatr. Acad. Sei. Hung. 22 (1981) 251-258 14 Russel, D. S., L.]. Rubinstein: Pathology of Tumours of the Nervous System (ed.4). London, E. Arnold (1977) 220-224 15 Shuangshoti, S., P. Tangchai, G. Netsky: Primary adenocarcinoma of choroid plexus. Arch. Pathol. 91 (1971) 101-106 16 Tham, K. T., H. L. Wen, T. B. Teoh: A case of papillary adenocarcinoma of the choroid plexus. J. Pathol. 99 (1969) 321-324 1
M. S. M. Eljamel, FRCS1 The Mersey Regional Dept. of Neurosciences Walton Hospital Liverpool L9 1AE, Great Britain
Downloaded by: NYU. Copyrighted material.
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