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Possible influence of cerebrospinal fluid pressure on the growth of a third ventricle choroid plexus papilloma
Surg Neurol 1984;22:243-8
243
Possible Influence of Cerebrospinal Fluid Pressure on the Growth of a Third Ventricle Choroid Plexus Papilloma Hossein Safdari G.H., M.D., Guy Bourbotte, M.D., Philippe Frerebeau, M.D., and Philippe Castan, M.D. Neurosurgical Service A and Neuroradiological Service, Centre Hospitalier Universitaire, Montpellier, France
Safdari H, Bourbotte G, Frerebeau P, Castan P. Possible influence of cerebrospinal fluid pressure on the growth of a third ventricle choroid plexus papilloma. Surg Neurol 1984;22:243-8.
A case of histologically proven benign choroid plexus papilloma of the third ventricle in a 4-month-old male child is reported. The c]iinical symptoms and the neuroradiologic findings were those of a supratentorial hydrocephalus. A shunting procedure was performed after refusal by the family c,f a direct surgical approach. Subsequently there was a four- to fivefold increase in volume of this tumor in les~ than 4 months under decreased cerebrospinal fluid pres,~ure. The purpose of this report is first to add a case to 27 cases of papilloma of the choroid plexus of third ventricle reported in the medical literature. Secondly to underline a possible influence of intraventricular pressure on the growth of a histologically benign intraventricular papilloma. Subsequently, the question of radiation therapy may be raised, if total removal of the papilloma is not obtained, especially in view of decreased cerebrospinal fluid pressure provided by the previous shunting procedure. KEY WORDS: Cerebrospinal fluid shunt; Cerebral ventricle neoplasm; Papilloma; Radiation therapy; Third ventricle; Tumor growth
Choroid plexus papilloma (CPP) is a rare intracranial neoplasm accounting fc,r 0 . 4 % - 0 . 6 % of all intracranial tumors in adults [1,3,~[1,13,15,22] and 3 % - 3 . 9 % of those in children [7,11,14,15,16]. Papillomas of the choroid plexus are located with predilection in the fourth ventricle o f adults with possible extention into the cerebellopontine angle [3,11,12,15,16], and in the lateral ventricle o f children 70% [3,29]. The approximate frequency o f their occurrence in the third ventricle, in all
Address reprint requests to: Hossein Safdari G.H.M.D. Service de Neurochirurgie A, Hopital Gui de Chauliac, CHU 34059 Montpellier, France.
© 1984 by Elsevier Science Publishing Co., Inc.
age groups, is as low as 1 0 % - 1 5 % of all papillomas [ 1,3,14,16,23,26,28,29]. Papillomas of the choroid plexus are commonly histologically benign and slowly growing tumors [3,13, 15,16,19,27 ], although malignant forms or their malignant transformation do exist, but are quite rare [7,15, 16,27]. Although bleeding from benign papillomas spontaneously or under decreased intraventricular pressure is observed [2,11,13,14,16,17,24], we have not been able to find a report, in the pertinent medical literature available to us, regarding the possible influence of intraventricular pressure on the growth of histologically proven benign papillomas. The precise preoperative diagnosis of papillomas of the third ventricle is still a difficult problem in spite of the use of modern neuroradiologic tools such as computed tomography [6,10,14,24]. Total removal of such papillomas is the treatment o f choice [1,13,17,24], and carries a lower morbidity and mortality with the proper use of microsurgical techniques [13,22,24,28,30].
Case Report A 4-month-old child with normal obstetrical course and growth was admitted to our neurosurgical service for progressive (within 10 days) hypotonia, drowsiness, and vomiting. The general physical examination was normal. The neurological examination revealed drowsiness, marked general hypotonia, and palsy of upward gaze of both eyes. Pupils were equal and reactive to light, and papilledema was found bilaterally. The head circumference was 45 cm and the anterior fontanel was bulging moderately. Routine laboratory findings were normal. The plain x-ray films of the skull were unremarkable for the age. An emergency external drainage of cerebrospinal fluid was performed by direct right lateral ventricular puncture. The intraventricular pressure was increased up to 450 m m H 2 0 . The cerebrospinal fluid was clear, with 600 red blood cells/mL and 4 white blood cells/mL. The 0090-3019/84/$3.00
244
Surg Neurol 1984;22:243-8
Safdari et al
cerebrospinal fluid protein was 64 mg/dL, sugar 35 mg/dL, and chloride 121 mEq/L. On July 14, 1980 a metrizamide ventriculogram demonstrated a severe degree of symmetrical supratentorial ventricular dilatation, and the third ventricle was partially filled with a papillary mass (Figure 1). The aqueduct o f Sylvius and the fourth ventricle were normal in size, not occluded and normally filled with contrast medium. A slight posterior displacement of the upper portion of the aqueduct and an anterior displacement of its lower portion and the fourth ventricle were present. A computed tomogram was then made after intraventricular injection o f metrizamide (Figure 2). This tomographic study showed a spherical, heterogenous, hypodense mass located within the anterior part o f the third ventricle (estimated at about 24.4 cm 3 in volume); both lateral ventricles were markedly dilated. The intraventricular catheter was connected to a reservoir through a flow control device, kept at 12 cm above the level of the head of the child for 48 hours, and was removed just before the shunting procedure. Five hundred milliliters of clear cerebrospinal fluid was drained during this period and this resulted in a drastic improvement o f his neurological state with the disappearance of the palsy of upward gaze of the eyes. The surgical removal of the tumor was categorically refused by the family and only a shunting procedure was accepted.
Figure 1. The metrizamide ventriculography showed a huge dilatation of the supraventricular cavities, and the third ventricle is partially filled with a papillary tumor.
Figure 2. (A) Computed metrizamide ventriculography showing a spherical, heterogenous, and hypodense mass within the third ventricle. A striking enlargement of both lateral ventricles with marked cerebral atrophy is best seen on the posterior right. (B) Lower level of the same computed tomography scanning showing the lateral displacement of the cerebralpeduncles, caused by the tumor in the third ventricle.
Choroid Plexus Papilloma
On July 16, 1980 a ventriculo-peritoneal shunt connected to a medium pressure Hakim pediatric valve (60-80) was then inst~tlled. Postoperatively, the child was less drowsy, the palsy of upward gaze disappeared, and the anterior fontanel was slightly depressed, but the general hypotonia persJtsted. During a routine postoperative observation one month later, the neurological examination was unchanged from the early postoperative examination: the head circumference remained at 45 cm and severe diffuse hypotonia including both extremities and a psychomotor retardation were noted. There was no spontaneous movement of the patient's extremities. The shunting system was functioning normally. A direct surgical approach was still refused by the family. At the age of 8 months, the child was readmitted to our neurosurgical service for vomiting, elevated temperature (38°C), and moderate painless abdominal tenderness. The examination showed important axial hypotonia, mild spasticity of the upper and lower extremities bilaterally, palsy of upward gaze in both eyes, and bulging anterior fontanel. The head circumference was increased to 47 cm, no papilledema was found, but the optic nerves were pale and atrophic on both sides. The blood pressure was 80 mmHg, the cardiac rate at 92/min, and the temperature was 37.8°C. The shunting system was functioning normally. Shortly after admission he developed a drop of l~lood pressure with diffuse cyanosis. Despite intensive cardiorespiratory resuscitation associated with a high Close of corticosteroid, he expired 5 hours after the admission. A post-mortem examination of the brain only was obtained.
Figure 3. Sagittal midline section of the brain showing a large, granular, and irregular solid tumor filling the third ventricle, without macroscopic evidence of invasion of the surrounding brain structures by the tumor.
Surg Neurol 1984;22:243-8
245
Neuropathological Findings Externally the brain was unremarkable except for a mildly bulging mass pushing down the floor of the third ventricle. The sagittal and serial axial sections of the brain showed a marked cerebral parenchymal atrophy due to a huge solid, papillary but not necrotic tumor (estimated at about 113 cm ~ in volume). This tumor arose from the choroid plexus of the third ventricle, and filled the anterior portion of both lateral ventricles symmetrically, through the dilated foramen of Monro (Figures 3 and 4). No evidence of invasion of the brain parenchyma was found at any point. Both lateral ventricles were dilated and the infratentorial structures of the brainstem and cerebellum appeared normal. Paraffin sections from different parts of the tumor were stained by methods appropriate for the demonstration of general tissue structure, cellular architecture, nuclei, connective tissue, and vessels, and they were examined microscopically. The tumor consisted of a typical solid and benign papillary formation with a single layer of cuboidal benign epithelial cells resting on a vascular connective tissue stroma (Figure 5). There was no evidence of malignancy, swelling of interstitial tissues, or unusual congestion of blood vessels.
Comment The choroid plexus epithelium originates from primitive medullary epithelium, much like the ependymal cells. However, the tumors of the ependymoma group are morphologically quite different from papillomas of the
246
Surg Neurol 1984;22:243-8
Figure 4. (A) Axial sections of the brain at different levels showing the tumor arising from the choroid plexus of the third ventricle, and filling both anterior parts of the lateral ventricles. (B) Small cystic cavitation is seen in the center of the upper section of the tumor. Both lateral ventricles are dilated.
choroid plexus. The latter bears a striking resemblance to the surface epithelium o f mucosal cover of other parts of the body [15,16,25,27]. Papillomas of the choroid plexus are commonly benign, slowly growing tumors [3,13,15,16,19,27 ], but their malignant transformation may quite rarely occur [3, 16,17,27]. Several controversial reports of the total removal o f intraventricular papillomas followed by clearing of hydrocephalus have demonstrated that they may produce hydrocephalus either by overproduction of cerebrospinal fluid, or by the obstruction o f its pathways, or both [3,8,12,16-18,20,29]. Furthermore, this case may demonstrate the possibility of an inhibitory influence of intraventricular pressure on the growth of papillomas, in that our histologically proven benign tumor o f the third ventricle had a four- to fivefold increase in volume within less than 4
Safdari et al
months after the reduction of the cerebrospinal fluid pressure by the shunting procedure. Considering the fact that this child had no antitumor treatment, and showed no evidence of tumor malignancy or swelling, or of abnormal dilatation o f interpapillary spaces or cystic degeneration at the autopsy, we may reasonably assume in this case that there was only a rapid and benign hyperplasia of this tumor under reduced cerebrospinal fluid pressure. In spite of its benign histological appearance, however, a rapidly growing papilloma is not excluded, although this is very unusual for this type of neoplasm. This was a surprising finding that we have not encountered previously nor in the pertinent medical literature available to us. This feature suggests that a histologically benign papilloma o f the choroid plexus may have, under similar circumstances, a highly rapid growth in volume only, although still remaining histologically a totally benign tumor. Therefore, it may be suggested that this unusual pattern of growth is to be taken into consideration in the therapeutic management of such papillomas. Accordingly, the interval o f time between the shunting procedure, if needed, and total removal of the tumor should be as short as possible. In cases of partial removal of
Choroid Plexus Papilloma
Figure 5. Papillary structure is composed of a single layer of benign cuboidal epthelial cells resting upon a vascular connective tissue stroma (Hematoxylin & eosin: × 40).
these papillomas, a continuous observation with appropriate neuroradiologic investigation should include a precise evaluation of tumor regrowth and size. Although the radiosensitivity of benign papillomas of the choroid plexus is still a controversial question, when their total removal is not obtained the possibility of radiation therapy must be considered, especially when a shunting procedure is required. The effect of radiation therapy on the tumor cells is perhaps not very significant, but it may reduce the2ir vasculature and subsequently retard both the tumor regrowth and their oversecretion of cerebrospinal fluid [4,5,13,14,17,21].
References 1. Arseni C, Contantinescu A, Danaila L, Istrate C. The choroid plexus papillomas. Neurochirurgia 1974;17:121-9. 2. Bitoh S, Hasegawa H, Fujiwara M, Nataka M, Sakurai M. Cryptic malformation of the choroid plexus. Surg Neurol 1981;16:72-6. 3. Bohm E, Strang R. Choroid plexus papillomas. J Neurosurg 1961;18:493-500. 4. Carrea R, Polak M. Preoperative radiotherapy in the management of the posterior fossa choroid plexus papillomas. Child's Brain 1977;3:12-24. 5. Chan RC, Thompson GB, Durity FA. Primary choroid plexus papilloma of the cerebellopontine angle. Neurosurgery 1983;12:334-6. 6. Cuatico G, Castan P. Choroid plexus of the third ventricle. J Neurosurg 1962;19:1085-7.
Surg Neurol 1984;22:243-8
247
7. Dohrmann GJ, ColliasJC. Choroid plexus carcinoma: case report. J Neurosurg 1975;43:225-32. 8. Eisenberg HM, McComb JG, Lorenzo AV. Cerebrospinal ow erproduction associated with choroid plexus papilloma. J Neurosurg 1974;40:381-5. 9. Fortuna A, Celli P, Ferrante L, Turano C. A review of papillomas of the third ventricle: one case report. J Neurosurg Sci 1979;23:61-76. 10. Ganti SR, Antunes JL, Louis KM, Hilal SK. Computed tomography in the diagnosis of colloid cysts of the third ventricle. Radiology 1981;138:385-9l. 11. Gradin WC, Taylon C, Fruin H. Choroid plexus papilloma of the third ventricle: case report and review of the literature. Neurosurgery 1983;12:217-20. 12. Gudeman SK, Sullivan HG, Rosner JJ, Becker PD. Surgical removal of bilateral papillomas of the choroid plexus of the lateral ventricles with resolution of hydrocephalus: case report. J Neurosurg 1979;50:677-81. 13. Guidetti B, Spallone A. The surgical treatment of choroid plexus papillomas. Neurosurg Rev 1981;4:129-37. 14. Hawkins JC lII. Treatment of choroid plexus papillomas in children: a brief analysis of twenty years' experience. Neurosurg 1980;6:380-4. 15. Koos WT, Miller MH. Choroid plexus papillomas. In: Koos WT, Miller MH, eds. Intracranial tumors of infants and children. Stuttgart: Georg Thieme Verlag, 1971:239-44, 335-6. 16. Laurence KM. The biology of choroid plexus papilloma and carcinoma of the lateral ventricle. In Vinken PJ, Bruyen GW, eds. Handbook of clinical neurology. Vol 17. New York: Elsevier, 1974:555-97. 17. Matson DD, Crofton FD. Papilloma of the choroid plexus in childhood. J Neurosurg 1960;17:1002-27. 18. McDonald JV. Persistent hydrocephalus following removal of papillomas of the choroid plexus of the lateral ventricles. J Neurosurg 1969;30:736-40. 19. Milhorat TH, Davis DA, Hammock MK. Choroid plexus papilloma. Child's Brain 1976;2:290-303.
248
Surg N e u r o l 1984;22:243-8
20. Milhorat TH, Hammock M, Davis DA, Fenstermacher JD. Choroid plexus papilloma: proof of cerebrospinal fluid overproduction. Child's Brain 1976;2:273-89. 21. Naguib MG, Chou SN, Mastri A. Radiation therapy of a choroid plexus papilloma of the cerebellopontine angle with bone involvement. J Neurosurg 1981;54:245-7. 22. Nassar SI, Mount LA. Papillomas ofchoroid plexus. J Neurosurg 1968;29:73-7. 23. Pecker J, Ferrand B,Javalet A. Tumeurs du troisieme ventricule. Neurochirurgie 1966; 12:9-121. 24. Raimondi AJ, Guiterrez FA. Diagnosis and surgical treatment of choroid plexus papillomas. Child's Brain 1975;1:81-115. 25. Ringertz N, Reymond A. Ependymomas and choroid plexus papillomas. J Neuropathol Exp Neurol 1949;8:355-80.
Safdari et al
26. Rovit RL, Schechter MM, ChodroffP. Choroid plexus papillomas. Observations on radiographic diagnosis. Am J Roentgenol 1970;110:608-17. 27. Russell DS, Rubinstein LJ. Papillomas of the choroid plexus. In: Russell DS, Rubinstein LJ, eds. Pathology of tumors of the nervous system. Baltimore: Williams and Wilkins, 1977:220-4. 28. Tomasello F, Albanese V, Bernini FP, Picozzi P. Choroid plexus papilloma of the third ventricle. Surg Neurol 1981 ;16:69-71. 29. Turcotte JF, Copty M, Bedard F, Michaud J, Verret S. Lateral ventricle choroid plexus papilloma and communicating hydrocephalus. Surg Neurol 1980;13:143-6. 30. Winston KR, Cavazzuti V, Arkins T. Absence of neurological and behavioral abnormalities after anterior transcallosal operation for third ventricular lesions. Neurosurgery 1979;4:386-93.
243
Possible Influence of Cerebrospinal Fluid Pressure on the Growth of a Third Ventricle Choroid Plexus Papilloma Hossein Safdari G.H., M.D., Guy Bourbotte, M.D., Philippe Frerebeau, M.D., and Philippe Castan, M.D. Neurosurgical Service A and Neuroradiological Service, Centre Hospitalier Universitaire, Montpellier, France
Safdari H, Bourbotte G, Frerebeau P, Castan P. Possible influence of cerebrospinal fluid pressure on the growth of a third ventricle choroid plexus papilloma. Surg Neurol 1984;22:243-8.
A case of histologically proven benign choroid plexus papilloma of the third ventricle in a 4-month-old male child is reported. The c]iinical symptoms and the neuroradiologic findings were those of a supratentorial hydrocephalus. A shunting procedure was performed after refusal by the family c,f a direct surgical approach. Subsequently there was a four- to fivefold increase in volume of this tumor in les~ than 4 months under decreased cerebrospinal fluid pres,~ure. The purpose of this report is first to add a case to 27 cases of papilloma of the choroid plexus of third ventricle reported in the medical literature. Secondly to underline a possible influence of intraventricular pressure on the growth of a histologically benign intraventricular papilloma. Subsequently, the question of radiation therapy may be raised, if total removal of the papilloma is not obtained, especially in view of decreased cerebrospinal fluid pressure provided by the previous shunting procedure. KEY WORDS: Cerebrospinal fluid shunt; Cerebral ventricle neoplasm; Papilloma; Radiation therapy; Third ventricle; Tumor growth
Choroid plexus papilloma (CPP) is a rare intracranial neoplasm accounting fc,r 0 . 4 % - 0 . 6 % of all intracranial tumors in adults [1,3,~[1,13,15,22] and 3 % - 3 . 9 % of those in children [7,11,14,15,16]. Papillomas of the choroid plexus are located with predilection in the fourth ventricle o f adults with possible extention into the cerebellopontine angle [3,11,12,15,16], and in the lateral ventricle o f children 70% [3,29]. The approximate frequency o f their occurrence in the third ventricle, in all
Address reprint requests to: Hossein Safdari G.H.M.D. Service de Neurochirurgie A, Hopital Gui de Chauliac, CHU 34059 Montpellier, France.
© 1984 by Elsevier Science Publishing Co., Inc.
age groups, is as low as 1 0 % - 1 5 % of all papillomas [ 1,3,14,16,23,26,28,29]. Papillomas of the choroid plexus are commonly histologically benign and slowly growing tumors [3,13, 15,16,19,27 ], although malignant forms or their malignant transformation do exist, but are quite rare [7,15, 16,27]. Although bleeding from benign papillomas spontaneously or under decreased intraventricular pressure is observed [2,11,13,14,16,17,24], we have not been able to find a report, in the pertinent medical literature available to us, regarding the possible influence of intraventricular pressure on the growth of histologically proven benign papillomas. The precise preoperative diagnosis of papillomas of the third ventricle is still a difficult problem in spite of the use of modern neuroradiologic tools such as computed tomography [6,10,14,24]. Total removal of such papillomas is the treatment o f choice [1,13,17,24], and carries a lower morbidity and mortality with the proper use of microsurgical techniques [13,22,24,28,30].
Case Report A 4-month-old child with normal obstetrical course and growth was admitted to our neurosurgical service for progressive (within 10 days) hypotonia, drowsiness, and vomiting. The general physical examination was normal. The neurological examination revealed drowsiness, marked general hypotonia, and palsy of upward gaze of both eyes. Pupils were equal and reactive to light, and papilledema was found bilaterally. The head circumference was 45 cm and the anterior fontanel was bulging moderately. Routine laboratory findings were normal. The plain x-ray films of the skull were unremarkable for the age. An emergency external drainage of cerebrospinal fluid was performed by direct right lateral ventricular puncture. The intraventricular pressure was increased up to 450 m m H 2 0 . The cerebrospinal fluid was clear, with 600 red blood cells/mL and 4 white blood cells/mL. The 0090-3019/84/$3.00
244
Surg Neurol 1984;22:243-8
Safdari et al
cerebrospinal fluid protein was 64 mg/dL, sugar 35 mg/dL, and chloride 121 mEq/L. On July 14, 1980 a metrizamide ventriculogram demonstrated a severe degree of symmetrical supratentorial ventricular dilatation, and the third ventricle was partially filled with a papillary mass (Figure 1). The aqueduct o f Sylvius and the fourth ventricle were normal in size, not occluded and normally filled with contrast medium. A slight posterior displacement of the upper portion of the aqueduct and an anterior displacement of its lower portion and the fourth ventricle were present. A computed tomogram was then made after intraventricular injection o f metrizamide (Figure 2). This tomographic study showed a spherical, heterogenous, hypodense mass located within the anterior part o f the third ventricle (estimated at about 24.4 cm 3 in volume); both lateral ventricles were markedly dilated. The intraventricular catheter was connected to a reservoir through a flow control device, kept at 12 cm above the level of the head of the child for 48 hours, and was removed just before the shunting procedure. Five hundred milliliters of clear cerebrospinal fluid was drained during this period and this resulted in a drastic improvement o f his neurological state with the disappearance of the palsy of upward gaze of the eyes. The surgical removal of the tumor was categorically refused by the family and only a shunting procedure was accepted.
Figure 1. The metrizamide ventriculography showed a huge dilatation of the supraventricular cavities, and the third ventricle is partially filled with a papillary tumor.
Figure 2. (A) Computed metrizamide ventriculography showing a spherical, heterogenous, and hypodense mass within the third ventricle. A striking enlargement of both lateral ventricles with marked cerebral atrophy is best seen on the posterior right. (B) Lower level of the same computed tomography scanning showing the lateral displacement of the cerebralpeduncles, caused by the tumor in the third ventricle.
Choroid Plexus Papilloma
On July 16, 1980 a ventriculo-peritoneal shunt connected to a medium pressure Hakim pediatric valve (60-80) was then inst~tlled. Postoperatively, the child was less drowsy, the palsy of upward gaze disappeared, and the anterior fontanel was slightly depressed, but the general hypotonia persJtsted. During a routine postoperative observation one month later, the neurological examination was unchanged from the early postoperative examination: the head circumference remained at 45 cm and severe diffuse hypotonia including both extremities and a psychomotor retardation were noted. There was no spontaneous movement of the patient's extremities. The shunting system was functioning normally. A direct surgical approach was still refused by the family. At the age of 8 months, the child was readmitted to our neurosurgical service for vomiting, elevated temperature (38°C), and moderate painless abdominal tenderness. The examination showed important axial hypotonia, mild spasticity of the upper and lower extremities bilaterally, palsy of upward gaze in both eyes, and bulging anterior fontanel. The head circumference was increased to 47 cm, no papilledema was found, but the optic nerves were pale and atrophic on both sides. The blood pressure was 80 mmHg, the cardiac rate at 92/min, and the temperature was 37.8°C. The shunting system was functioning normally. Shortly after admission he developed a drop of l~lood pressure with diffuse cyanosis. Despite intensive cardiorespiratory resuscitation associated with a high Close of corticosteroid, he expired 5 hours after the admission. A post-mortem examination of the brain only was obtained.
Figure 3. Sagittal midline section of the brain showing a large, granular, and irregular solid tumor filling the third ventricle, without macroscopic evidence of invasion of the surrounding brain structures by the tumor.
Surg Neurol 1984;22:243-8
245
Neuropathological Findings Externally the brain was unremarkable except for a mildly bulging mass pushing down the floor of the third ventricle. The sagittal and serial axial sections of the brain showed a marked cerebral parenchymal atrophy due to a huge solid, papillary but not necrotic tumor (estimated at about 113 cm ~ in volume). This tumor arose from the choroid plexus of the third ventricle, and filled the anterior portion of both lateral ventricles symmetrically, through the dilated foramen of Monro (Figures 3 and 4). No evidence of invasion of the brain parenchyma was found at any point. Both lateral ventricles were dilated and the infratentorial structures of the brainstem and cerebellum appeared normal. Paraffin sections from different parts of the tumor were stained by methods appropriate for the demonstration of general tissue structure, cellular architecture, nuclei, connective tissue, and vessels, and they were examined microscopically. The tumor consisted of a typical solid and benign papillary formation with a single layer of cuboidal benign epithelial cells resting on a vascular connective tissue stroma (Figure 5). There was no evidence of malignancy, swelling of interstitial tissues, or unusual congestion of blood vessels.
Comment The choroid plexus epithelium originates from primitive medullary epithelium, much like the ependymal cells. However, the tumors of the ependymoma group are morphologically quite different from papillomas of the
246
Surg Neurol 1984;22:243-8
Figure 4. (A) Axial sections of the brain at different levels showing the tumor arising from the choroid plexus of the third ventricle, and filling both anterior parts of the lateral ventricles. (B) Small cystic cavitation is seen in the center of the upper section of the tumor. Both lateral ventricles are dilated.
choroid plexus. The latter bears a striking resemblance to the surface epithelium o f mucosal cover of other parts of the body [15,16,25,27]. Papillomas of the choroid plexus are commonly benign, slowly growing tumors [3,13,15,16,19,27 ], but their malignant transformation may quite rarely occur [3, 16,17,27]. Several controversial reports of the total removal o f intraventricular papillomas followed by clearing of hydrocephalus have demonstrated that they may produce hydrocephalus either by overproduction of cerebrospinal fluid, or by the obstruction o f its pathways, or both [3,8,12,16-18,20,29]. Furthermore, this case may demonstrate the possibility of an inhibitory influence of intraventricular pressure on the growth of papillomas, in that our histologically proven benign tumor o f the third ventricle had a four- to fivefold increase in volume within less than 4
Safdari et al
months after the reduction of the cerebrospinal fluid pressure by the shunting procedure. Considering the fact that this child had no antitumor treatment, and showed no evidence of tumor malignancy or swelling, or of abnormal dilatation o f interpapillary spaces or cystic degeneration at the autopsy, we may reasonably assume in this case that there was only a rapid and benign hyperplasia of this tumor under reduced cerebrospinal fluid pressure. In spite of its benign histological appearance, however, a rapidly growing papilloma is not excluded, although this is very unusual for this type of neoplasm. This was a surprising finding that we have not encountered previously nor in the pertinent medical literature available to us. This feature suggests that a histologically benign papilloma o f the choroid plexus may have, under similar circumstances, a highly rapid growth in volume only, although still remaining histologically a totally benign tumor. Therefore, it may be suggested that this unusual pattern of growth is to be taken into consideration in the therapeutic management of such papillomas. Accordingly, the interval o f time between the shunting procedure, if needed, and total removal of the tumor should be as short as possible. In cases of partial removal of
Choroid Plexus Papilloma
Figure 5. Papillary structure is composed of a single layer of benign cuboidal epthelial cells resting upon a vascular connective tissue stroma (Hematoxylin & eosin: × 40).
these papillomas, a continuous observation with appropriate neuroradiologic investigation should include a precise evaluation of tumor regrowth and size. Although the radiosensitivity of benign papillomas of the choroid plexus is still a controversial question, when their total removal is not obtained the possibility of radiation therapy must be considered, especially when a shunting procedure is required. The effect of radiation therapy on the tumor cells is perhaps not very significant, but it may reduce the2ir vasculature and subsequently retard both the tumor regrowth and their oversecretion of cerebrospinal fluid [4,5,13,14,17,21].
References 1. Arseni C, Contantinescu A, Danaila L, Istrate C. The choroid plexus papillomas. Neurochirurgia 1974;17:121-9. 2. Bitoh S, Hasegawa H, Fujiwara M, Nataka M, Sakurai M. Cryptic malformation of the choroid plexus. Surg Neurol 1981;16:72-6. 3. Bohm E, Strang R. Choroid plexus papillomas. J Neurosurg 1961;18:493-500. 4. Carrea R, Polak M. Preoperative radiotherapy in the management of the posterior fossa choroid plexus papillomas. Child's Brain 1977;3:12-24. 5. Chan RC, Thompson GB, Durity FA. Primary choroid plexus papilloma of the cerebellopontine angle. Neurosurgery 1983;12:334-6. 6. Cuatico G, Castan P. Choroid plexus of the third ventricle. J Neurosurg 1962;19:1085-7.
Surg Neurol 1984;22:243-8
247
7. Dohrmann GJ, ColliasJC. Choroid plexus carcinoma: case report. J Neurosurg 1975;43:225-32. 8. Eisenberg HM, McComb JG, Lorenzo AV. Cerebrospinal ow erproduction associated with choroid plexus papilloma. J Neurosurg 1974;40:381-5. 9. Fortuna A, Celli P, Ferrante L, Turano C. A review of papillomas of the third ventricle: one case report. J Neurosurg Sci 1979;23:61-76. 10. Ganti SR, Antunes JL, Louis KM, Hilal SK. Computed tomography in the diagnosis of colloid cysts of the third ventricle. Radiology 1981;138:385-9l. 11. Gradin WC, Taylon C, Fruin H. Choroid plexus papilloma of the third ventricle: case report and review of the literature. Neurosurgery 1983;12:217-20. 12. Gudeman SK, Sullivan HG, Rosner JJ, Becker PD. Surgical removal of bilateral papillomas of the choroid plexus of the lateral ventricles with resolution of hydrocephalus: case report. J Neurosurg 1979;50:677-81. 13. Guidetti B, Spallone A. The surgical treatment of choroid plexus papillomas. Neurosurg Rev 1981;4:129-37. 14. Hawkins JC lII. Treatment of choroid plexus papillomas in children: a brief analysis of twenty years' experience. Neurosurg 1980;6:380-4. 15. Koos WT, Miller MH. Choroid plexus papillomas. In: Koos WT, Miller MH, eds. Intracranial tumors of infants and children. Stuttgart: Georg Thieme Verlag, 1971:239-44, 335-6. 16. Laurence KM. The biology of choroid plexus papilloma and carcinoma of the lateral ventricle. In Vinken PJ, Bruyen GW, eds. Handbook of clinical neurology. Vol 17. New York: Elsevier, 1974:555-97. 17. Matson DD, Crofton FD. Papilloma of the choroid plexus in childhood. J Neurosurg 1960;17:1002-27. 18. McDonald JV. Persistent hydrocephalus following removal of papillomas of the choroid plexus of the lateral ventricles. J Neurosurg 1969;30:736-40. 19. Milhorat TH, Davis DA, Hammock MK. Choroid plexus papilloma. Child's Brain 1976;2:290-303.
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