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Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri
Retropharyngeal Rhabdomyosarcoma Mimicking Pseudotumor Cerebri Masahiro Kikuchi, MD*, Sumio Kudo, MD†, Mitsuru Wada, MD†, Toshiro Kamoshida, MD‡, and Hisao Hoshino, MD* A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. Cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension. © 1999 by Elsevier Science Inc. All rights reserved. Kikuchi M, Kudo S, Wada M, Kamoshida T, Hoshino H. Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri. Pediatr Neurol 1999;21:496-499.
Introduction Pseudotumor cerebri (PTC) is a syndrome of elevated intracranial pressure with no evidence of intracranial mass lesion or abnormal ventricular size [1,2]. Characteristic clinical features include headache and papilledema, without focal neurologic signs. PTC includes distinct primary disorders and secondary intracranial hypertension [1]; thus, differential diagnosis must be extensive to detect underlying disorders, such as tumors [3-7] and arteriovenous malformations [8]. Described is a patient with
From the Departments of *Pediatrics and †Neurosurgery; and the ‡ Institute of Pathological Anatomy; Hitachi General Hospital; Hitachi, Japan.
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retropharyngeal rhabdomyosarcoma that obstructed the cerebral venous outflow at the level of the jugular foramen. His clinical presentation and radiologic findings suggested an initial diagnosis of PTC.
Case Report A 6-year-old male was admitted to the hospital with complaints of daily headaches for 4 months associated with transient visual obscuration and binocular diplopia. Headache was pulsatile in nature, holocranial, and usually ceased with vomiting. He did not notice decreased hearing or tinnitus. General ophthalmologic examination revealed bilateral optic disc edema, but ocular movements were normal. No other abnormal neurologic findings were found. Plain skull x-ray revealed only maxillary sinusitis. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) revealed no intracranial mass lesion, brain edema, or ventricular dilatation (Fig 1). Lumbar puncture demonstrated an opening pressure of 230 mm H2O, with normal cerebrospinal fluid cell count, protein, and cytology. Prednisolone was administered after the diagnosis of PTC. Headaches improved but papilledema remained. To re-evaluate intracranial lesions, he was referred to the authors’ hospital 3 months later. A slightly enlarged right superior ophthalmic vein on the venous phase of right carotid angiography indicated stagnant cerebral venous outflow, which was thought to result from the association of maxillary sinusitis and cavernous sinus thrombosis. He was asymptomatic until 1 month later when he returned to the hospital. He again experienced headaches and complained of a husky voice. Physical examination demonstrated no tumors in the head or neck. Blood pressure was 100/66 mm Hg. Neurologic examination revealed insufficient elevation of the right soft palate, with a slight reduction of the right gag reflex. Weakness in rotating the head to the left was observed. The tongue protruded to the right. The first through eighth cranial nerves seemed to be intact. These findings indicated that the patient had right ninth, tenth, eleventh, and twelfth cranial nerve palsies (Collet-Sicard syndrome). The papilledema was still present. A lumbar puncture revealed a spinal fluid pressure of 280 mm H2O, and the protein was 12 mg/dL, with normal cytology. MRI revealed no intracranial lesion but visualized a retropharyngeal mass enhanced with gadolinium (Fig 2). The mass involved the right jugular vein and extended into the jugular foramen, but no intracranial extensions were observed. The venous phase of right internal carotid angiography revealed occlusion of the right jugular system at the level of jugular foramen (Fig 3). The left transverse sinus was patent but hypoplastic. Cranial venous drainage mainly occurred through the collateral veins. It was considered that the interruption of the dominant transverse sinus drainage accounted for the characteristics of PTC. Transcervical surgery revealed a hard and encapsulated tumor. It was partially resected because sacrificing the right jugular system may have caused marked intracranial hypertension. Histologic examination disclosed round tumor cells with eosinophilic cytoplasm, which indicated rhabdomyosarcoma of an embryonal type (Fig 4). Immunohistochemistry demonstrated that the tumor cells were positive for vimentin, muscle actin, and myoglobin. Radiation therapy and chemotherapy with vincristine, actinomycin D, and cyclophosphamide was initiated. The headaches resolved with reduction of the tumor.
Communications should be addressed to: Dr. Kikuchi; Department of Pediatrics; Hitachi General Hospital; 2-1-1, Jonan-cho; Hitachi, Ibaraki, 317-0077, Japan. Received September 28, 1998; accepted January 1, 1999.
© 1999 by Elsevier Science Inc. All rights reserved. PII S0887-8994(99)00015-6 ● 0887-8994/99/$20.00
Figure 1. Initial T1-weighted MRI of the brain (TR 5 500 ms; TE 5 30 ms) did not demonstrate ventricular dilatation or any intracranial mass lesions.
Discussion The initial manifestations of the patient were consistent with PTC. Lumbar puncture demonstrated an elevated intracranial pressure, and computed tomography and MRI did not detect any intracranial mass lesions. There were no focal neurologic signs at the first examination. PTC is a term that described intracranial hypertension without intracranial mass lesions [1,2]. It includes distinct primary disorders and secondary intracranial hypertension caused by other underlying disorders. Cranial venous obstructions cause PTC [9], and it results in superior sagittal sinus hypertension. Sinus hypertension impairs the outflow of cerebrospinal fluid through the arachnoid granulation system thus increasing the intracranial pressure [4]. In the preantibiotic era, acute or chronic otitis media with infectious extension into the sigmoid sinuses was a frequently encountered cause of venous sinus
Figure 2. (A) MRI 5 months after the initial presentation. Axial T1-weighted image with gadolinium (TR 5 400 ms; TE 5 30 ms) demonstrates a hyperintense cervical mass lesion anterior to the right longus capitis muscle. (B) On coronal image the lesion compresses and occludes the right jugular vein (TR 5 300 ms; TE 5 30 ms).
occlusion and PTC [10]. In addition, PTC is reported in cases of sinus involvement with meningioma [4,5], venous thrombosis [9], arteriovenous malformations [8], and glomus jugulare tumors [6,7] and as a complication of jugular vein and radical neck ligations [9]. In this patient, retropharyngeal rhabdomyosarcoma involving the right jugular vein impaired the cerebral venous drainage at the level of the jugular foramen, resulting in PTC. Because the dural venous system is anatomically asymmetric and has many individual variations, any unilateral occlusion involving the more developed side may impair cerebral venous outflow. The occluded right jugular system was revealed to be the dominant side of cerebral venous outflow by angiography. Transient improvement of his symptoms despite continuous growth of the tumor may have been partly the result of the medical treatment but mainly because of the development of collateral venous drainage. Rhabdomyosarcoma is a common tumor in children, with the head and neck being the most frequent sites of
Kikuchi et al: Rhabdomyosarcoma and Pseudotumor Cerebri 497
Figure 3. The venous phase of right carotid angiography reveals occlusion of the jugular bulb and enlarged collateral veins (arrow). The left transverse sinus is hypoplastic (arrowhead).
involvement. It is rarely associated with cranial nerve palsies, including extraocular palsy or ptosis [11-13]. PTC or typical Collet-Sicard syndrome associated with rhabdomyosarcoma has not been reported. Glomus jugulare tumor and neurinoma, the most common neoplasms involving the jugular foramen, demonstrate cerebellar dysfunction and lower cranial nerve palsies, with the eighth nerve palsy being the most common [14-16]. Bilateral glomus jugulare tumors [6] or glomus jugulare tumor with arteriovenous shunting [7] may cause PTC. However, typical neurologic symptoms, such as Vernet’s syndrome or Collet-Siccard syndrome, observed in this patient are not common in these neoplasms [15]. In this patient the
correct diagnosis could not be made until the development of the focal neurologic signs. Although initial angiography revealed stagnant cerebral venous outflow, it was presumed to demonstrate infectious extension of maxillary sinusitis into the cavernous sinus. In conclusion, the differential diagnosis of PTC must be extensive and must include cervical tumors, as well as intracranial tumors or inflammatory diseases. In this particular case the coexistence of maxillary sinusitis delayed the correct diagnosis. This report emphasizes that PTC is a syndrome of exclusion, and patients must be carefully monitored with particular attention to the focal neurologic signs.
Figure 4. Histopathologic examination demonstrates small round cells with hyperchromatic nuclei and eosinophilic cytoplasm (3200).
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References [1] Martin TJ, Corbett JJ. Pseudotumor cerebri. In: Youmans JR, ed. Neurological surgery, Vol 5, 4th ed. Philadelphia: WB Saunders, 1996:2980-97. [2] Baker RS, Baumann RJ, Bucic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 1989;5:5-11. [3] Aroichane M, Miller NR, Eggenberger ER. Glioblastoma multiforme masquerading as pseudotumor cerebri. J Clin Neuro-Ophthalmol 1993;13:105-12. [4] Marr WG, Chambers JW. Occlusion of cerebral dural sinuses by tumor simulating pseudotumor cerebri. Am J Ophthalmol 1966;61:45-9. [5] Dung Truonug D, Holgate RC, Hsu CY, Scott T. Occlusion of the transverse sinus by meningioma simulating pseudotumor cerebri. Neuro-Ophthalmol 1987;7:113-7. [6] Angeli SI, Sato Y, Gantz BJ. Glomus jugulare tumors masquerading as benign intracranial hypertension. Arch Otolaryngol Head Neck Surg 1994;120:1277-80. [7] Beck DW, Kassell NF, Drake CG. Glomus jugulare tumor presenting with increased intracranial pressure. J Neurosurg 1979;50: 823-5. [8] van den Bergh R, Dralands G, Crolla D, van den Bergh P.
Pseudotumor cerebri due to intracranial arteriovenous malformation. Clin Neurol Neurosurg 1980;82:119-25. [9] Lam BL, Schatz NJ, Glaser JS, Bowen BC. Pseudotumor cerebri from cranial venous obstruction. Ophthalmology 1992;99:706-12. [10] Grant DN. Benign intracranial hypertension. Arch Dis Child 1971;46:651-5. [11] Months SR, Raney RB. Rhabdomyosarcoma of the head and neck in children: The experience at the children’s hospital of Philadelphia. Med Pediatr Oncol 1986;14:288-92. [12] Pratt CB, Smith JW, Woerner S, et al. Factors leading to delay in the diagnosis and affecting survival of children with head and neck. Pediatrics 1978;61:30-4. [13] Anderson GJ, Tom LWC, Womer RB, Handler SD, Wetmore RF, Potsic WP. Rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 1990;116:428-31. [14] Tan LC, Bordi L, Symon L, Cheesman AD. Jugular foramen neuromas: A review of 14 cases. Surg Neurol 1990;34:205-11. [15] Iwasaki K, Kondo A. Accessory nerve neurinoma manifesting with typical jugular foramen syndrome. Neurosurgery 1991;29:455-9. [16] Robertson JH, Brodkey JA. Glomus jugulare tumors. In: Youmans JR, ed. Neurological surgery, Vol 5, 4th ed. Philadelphia: WB Saunders, 1996;3084-101.
Kikuchi et al: Rhabdomyosarcoma and Pseudotumor Cerebri 499
Introduction Pseudotumor cerebri (PTC) is a syndrome of elevated intracranial pressure with no evidence of intracranial mass lesion or abnormal ventricular size [1,2]. Characteristic clinical features include headache and papilledema, without focal neurologic signs. PTC includes distinct primary disorders and secondary intracranial hypertension [1]; thus, differential diagnosis must be extensive to detect underlying disorders, such as tumors [3-7] and arteriovenous malformations [8]. Described is a patient with
From the Departments of *Pediatrics and †Neurosurgery; and the ‡ Institute of Pathological Anatomy; Hitachi General Hospital; Hitachi, Japan.
496
PEDIATRIC NEUROLOGY
Vol. 21 No. 1
retropharyngeal rhabdomyosarcoma that obstructed the cerebral venous outflow at the level of the jugular foramen. His clinical presentation and radiologic findings suggested an initial diagnosis of PTC.
Case Report A 6-year-old male was admitted to the hospital with complaints of daily headaches for 4 months associated with transient visual obscuration and binocular diplopia. Headache was pulsatile in nature, holocranial, and usually ceased with vomiting. He did not notice decreased hearing or tinnitus. General ophthalmologic examination revealed bilateral optic disc edema, but ocular movements were normal. No other abnormal neurologic findings were found. Plain skull x-ray revealed only maxillary sinusitis. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) revealed no intracranial mass lesion, brain edema, or ventricular dilatation (Fig 1). Lumbar puncture demonstrated an opening pressure of 230 mm H2O, with normal cerebrospinal fluid cell count, protein, and cytology. Prednisolone was administered after the diagnosis of PTC. Headaches improved but papilledema remained. To re-evaluate intracranial lesions, he was referred to the authors’ hospital 3 months later. A slightly enlarged right superior ophthalmic vein on the venous phase of right carotid angiography indicated stagnant cerebral venous outflow, which was thought to result from the association of maxillary sinusitis and cavernous sinus thrombosis. He was asymptomatic until 1 month later when he returned to the hospital. He again experienced headaches and complained of a husky voice. Physical examination demonstrated no tumors in the head or neck. Blood pressure was 100/66 mm Hg. Neurologic examination revealed insufficient elevation of the right soft palate, with a slight reduction of the right gag reflex. Weakness in rotating the head to the left was observed. The tongue protruded to the right. The first through eighth cranial nerves seemed to be intact. These findings indicated that the patient had right ninth, tenth, eleventh, and twelfth cranial nerve palsies (Collet-Sicard syndrome). The papilledema was still present. A lumbar puncture revealed a spinal fluid pressure of 280 mm H2O, and the protein was 12 mg/dL, with normal cytology. MRI revealed no intracranial lesion but visualized a retropharyngeal mass enhanced with gadolinium (Fig 2). The mass involved the right jugular vein and extended into the jugular foramen, but no intracranial extensions were observed. The venous phase of right internal carotid angiography revealed occlusion of the right jugular system at the level of jugular foramen (Fig 3). The left transverse sinus was patent but hypoplastic. Cranial venous drainage mainly occurred through the collateral veins. It was considered that the interruption of the dominant transverse sinus drainage accounted for the characteristics of PTC. Transcervical surgery revealed a hard and encapsulated tumor. It was partially resected because sacrificing the right jugular system may have caused marked intracranial hypertension. Histologic examination disclosed round tumor cells with eosinophilic cytoplasm, which indicated rhabdomyosarcoma of an embryonal type (Fig 4). Immunohistochemistry demonstrated that the tumor cells were positive for vimentin, muscle actin, and myoglobin. Radiation therapy and chemotherapy with vincristine, actinomycin D, and cyclophosphamide was initiated. The headaches resolved with reduction of the tumor.
Communications should be addressed to: Dr. Kikuchi; Department of Pediatrics; Hitachi General Hospital; 2-1-1, Jonan-cho; Hitachi, Ibaraki, 317-0077, Japan. Received September 28, 1998; accepted January 1, 1999.
© 1999 by Elsevier Science Inc. All rights reserved. PII S0887-8994(99)00015-6 ● 0887-8994/99/$20.00
Figure 1. Initial T1-weighted MRI of the brain (TR 5 500 ms; TE 5 30 ms) did not demonstrate ventricular dilatation or any intracranial mass lesions.
Discussion The initial manifestations of the patient were consistent with PTC. Lumbar puncture demonstrated an elevated intracranial pressure, and computed tomography and MRI did not detect any intracranial mass lesions. There were no focal neurologic signs at the first examination. PTC is a term that described intracranial hypertension without intracranial mass lesions [1,2]. It includes distinct primary disorders and secondary intracranial hypertension caused by other underlying disorders. Cranial venous obstructions cause PTC [9], and it results in superior sagittal sinus hypertension. Sinus hypertension impairs the outflow of cerebrospinal fluid through the arachnoid granulation system thus increasing the intracranial pressure [4]. In the preantibiotic era, acute or chronic otitis media with infectious extension into the sigmoid sinuses was a frequently encountered cause of venous sinus
Figure 2. (A) MRI 5 months after the initial presentation. Axial T1-weighted image with gadolinium (TR 5 400 ms; TE 5 30 ms) demonstrates a hyperintense cervical mass lesion anterior to the right longus capitis muscle. (B) On coronal image the lesion compresses and occludes the right jugular vein (TR 5 300 ms; TE 5 30 ms).
occlusion and PTC [10]. In addition, PTC is reported in cases of sinus involvement with meningioma [4,5], venous thrombosis [9], arteriovenous malformations [8], and glomus jugulare tumors [6,7] and as a complication of jugular vein and radical neck ligations [9]. In this patient, retropharyngeal rhabdomyosarcoma involving the right jugular vein impaired the cerebral venous drainage at the level of the jugular foramen, resulting in PTC. Because the dural venous system is anatomically asymmetric and has many individual variations, any unilateral occlusion involving the more developed side may impair cerebral venous outflow. The occluded right jugular system was revealed to be the dominant side of cerebral venous outflow by angiography. Transient improvement of his symptoms despite continuous growth of the tumor may have been partly the result of the medical treatment but mainly because of the development of collateral venous drainage. Rhabdomyosarcoma is a common tumor in children, with the head and neck being the most frequent sites of
Kikuchi et al: Rhabdomyosarcoma and Pseudotumor Cerebri 497
Figure 3. The venous phase of right carotid angiography reveals occlusion of the jugular bulb and enlarged collateral veins (arrow). The left transverse sinus is hypoplastic (arrowhead).
involvement. It is rarely associated with cranial nerve palsies, including extraocular palsy or ptosis [11-13]. PTC or typical Collet-Sicard syndrome associated with rhabdomyosarcoma has not been reported. Glomus jugulare tumor and neurinoma, the most common neoplasms involving the jugular foramen, demonstrate cerebellar dysfunction and lower cranial nerve palsies, with the eighth nerve palsy being the most common [14-16]. Bilateral glomus jugulare tumors [6] or glomus jugulare tumor with arteriovenous shunting [7] may cause PTC. However, typical neurologic symptoms, such as Vernet’s syndrome or Collet-Siccard syndrome, observed in this patient are not common in these neoplasms [15]. In this patient the
correct diagnosis could not be made until the development of the focal neurologic signs. Although initial angiography revealed stagnant cerebral venous outflow, it was presumed to demonstrate infectious extension of maxillary sinusitis into the cavernous sinus. In conclusion, the differential diagnosis of PTC must be extensive and must include cervical tumors, as well as intracranial tumors or inflammatory diseases. In this particular case the coexistence of maxillary sinusitis delayed the correct diagnosis. This report emphasizes that PTC is a syndrome of exclusion, and patients must be carefully monitored with particular attention to the focal neurologic signs.
Figure 4. Histopathologic examination demonstrates small round cells with hyperchromatic nuclei and eosinophilic cytoplasm (3200).
498
PEDIATRIC NEUROLOGY
Vol. 21 No. 1
References [1] Martin TJ, Corbett JJ. Pseudotumor cerebri. In: Youmans JR, ed. Neurological surgery, Vol 5, 4th ed. Philadelphia: WB Saunders, 1996:2980-97. [2] Baker RS, Baumann RJ, Bucic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 1989;5:5-11. [3] Aroichane M, Miller NR, Eggenberger ER. Glioblastoma multiforme masquerading as pseudotumor cerebri. J Clin Neuro-Ophthalmol 1993;13:105-12. [4] Marr WG, Chambers JW. Occlusion of cerebral dural sinuses by tumor simulating pseudotumor cerebri. Am J Ophthalmol 1966;61:45-9. [5] Dung Truonug D, Holgate RC, Hsu CY, Scott T. Occlusion of the transverse sinus by meningioma simulating pseudotumor cerebri. Neuro-Ophthalmol 1987;7:113-7. [6] Angeli SI, Sato Y, Gantz BJ. Glomus jugulare tumors masquerading as benign intracranial hypertension. Arch Otolaryngol Head Neck Surg 1994;120:1277-80. [7] Beck DW, Kassell NF, Drake CG. Glomus jugulare tumor presenting with increased intracranial pressure. J Neurosurg 1979;50: 823-5. [8] van den Bergh R, Dralands G, Crolla D, van den Bergh P.
Pseudotumor cerebri due to intracranial arteriovenous malformation. Clin Neurol Neurosurg 1980;82:119-25. [9] Lam BL, Schatz NJ, Glaser JS, Bowen BC. Pseudotumor cerebri from cranial venous obstruction. Ophthalmology 1992;99:706-12. [10] Grant DN. Benign intracranial hypertension. Arch Dis Child 1971;46:651-5. [11] Months SR, Raney RB. Rhabdomyosarcoma of the head and neck in children: The experience at the children’s hospital of Philadelphia. Med Pediatr Oncol 1986;14:288-92. [12] Pratt CB, Smith JW, Woerner S, et al. Factors leading to delay in the diagnosis and affecting survival of children with head and neck. Pediatrics 1978;61:30-4. [13] Anderson GJ, Tom LWC, Womer RB, Handler SD, Wetmore RF, Potsic WP. Rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 1990;116:428-31. [14] Tan LC, Bordi L, Symon L, Cheesman AD. Jugular foramen neuromas: A review of 14 cases. Surg Neurol 1990;34:205-11. [15] Iwasaki K, Kondo A. Accessory nerve neurinoma manifesting with typical jugular foramen syndrome. Neurosurgery 1991;29:455-9. [16] Robertson JH, Brodkey JA. Glomus jugulare tumors. In: Youmans JR, ed. Neurological surgery, Vol 5, 4th ed. Philadelphia: WB Saunders, 1996;3084-101.
Kikuchi et al: Rhabdomyosarcoma and Pseudotumor Cerebri 499