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Rhabdomyosarcoma presenting as Garcin's syndrome
Rhabdomyosarcoma Presenting As Garcin's Syndrome Toshihiro Takahashi, M.D.,* Tadao Murase, M.D.,* Yoshimasa Isayama, M.D.,* Norihiko Tamaki, M.D.,t Kiyoshi Fujiwara, M.D., t and Satoshi Matsumoto, M.D. t
A case of rhabdomyosarcoma of the head and neck demonstrating signs and symptoms of Garcin's syndrome is presented. Ocular findings are discussed clinicopathologically. Takahashi T, MuraseT, IsayamaY, TamakiN, FujiwaraK, MatsumotoS: Rhabdomyosarcoma presenting as Garcin's syndrome.SurgNeurol 17:269-272, 1982 Garcin's syndrome, also called hemi-basis syndrome, is characterized by unilateral paralyses of all cranial nerves, absence of cerebral signs, and symptoms such as disturbances of the motor and sensory systems, with no signs of increased intracranial pressure [1, 8]. Rhabdomyosarcoma is a prevalent malignant tumor in childhood and occurs frequently in the head and neck [2, 4, 5, 9]. However, to our knowledge, rhabdomyosarcoma presenting as Garcin's syndrome has not yet been reported.
Case Report A 6-year-old boy was referred to the Ophthalmology Department of Kobe University on February 4, 1974, because of diplopia, left facial nerve palsy, and tumor in the left retromandibular region. Neuroophthalmological examination revealed the corrected visual acuity was 20/15 on the right and 20/25 on the left. Lagophthalmos of the left eye due to facial nerve palsy and a sensory deficit in the left half of the face were observed. Lateral movement of the left eye was disturbed. The left pupil was miotic. Both pupils reacted promptly and completely to light. Ophthalmoscopy revealed no remarkable changes in either fundi. Goldmann's visual fields showed an inferior nasal quadrantanopia in both eyes (Fig. 1). The patient's clinical course was progressive and he was admitted to the Neurosurgery Department of Kobe University on February 19, 1974. From the *Department of Ophthalmologyand tDepartment of Neurosurgery, KobeUniversity,School of Medicine, Kobe,Japan. Address reprint requests to Dr. Toshihiro Takahashi. Department of Ophthalmology, Kobe University, School of Medicine, Kusunoki-cho, 7-chome, Chuo-ku, Kobe,Japan #650. Key words: Garcin's syndrome;Guillain-Alajouanine-Garcinsyndrome; hemi-basis syndrome;multiple cranial neuropathy; rhabdomyosarcoma; optic atrophy.
Neurological examination on admission showed paralyses of the left third through twelfth cranial nerves and disturbance of visual function in both eyes. However, no other neurological findings, such as cerebral signs and symptoms, were detected and cerebrospinal fluid studies were normal. Skull roentgenograms demonstrated destructive lesions in the base of the skull, indicated by enlargement of the optic canal and erosion of the middle cranial fossa, the anterior part of the petrous bone, and the jugular foramen (Fig. 2). Carotid and vertebral angiograms revealed occlusion of the left internal carotid artery and collateral circulation in the left cerebral hemisphere, supplied by the left external carotid, right internal carotid, and vertebral arteries (Figs. 3, 4). Biopsy of the tumor located in the left retromandibutar region was performed. This revealed rhabdomyosarcoma. The patient was treated with 5,100 rads of radiation. Thereafter, shrinkage of the tumor was observed but visual function remained unchanged. On November 18, 1974, the visual disturbance of the left eye rapidly deteriorated to 20/1000. The left pupil became mydriatic and the left optic disc appeared to be slightly atrophic, as observed on funduscopic examination. The patient's general condition deteriorated in spite of a further course of 3,000 fads and he died on February 14, 1975. At postmortem examination the tumor was found to be solid and yellowish white with nodular extensions; it was well demarcated from the surrounding tissue and measured 6 x 6 × 7 cm. The tumor was located chiefly in the left side of the tuberculum sellae, the middle cranial fossa, and the anterior portion of the foramen magnum, and furthermore, it partially extended over the midline of the clivus and tuberculum sellae (Fig. 5). The tumor extended into the epipharynx and the left retromandibular region through the parapharyngeal space. The left third through twelfth cranial nerves were completely buried in this tumor. Both intracranial optic nerves, particularly on the left, were markedly elevated and were atrophic due to compression by the tumor. Microscopically the tumor was composed mainly of elongated, ovoid, and plump cells with eosinophilic cytoplasm. These tumor cells were arranged in wavy and interlacing bundles, and cross-striations were visible on hematoxylin and eosin staining and phosphotungstic acid-hematoxylin
0090-3019/82/040269-04501.25 © 1981 by Little, Brown and Company (Inc.)
269
270 SurgicalNeurology Vol 17 No 4 April 1982
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Fig. 1. Bilateral inferi~rr nasal quadrantanopia was evident in the visual fields,
Fig. 2. Roentgenogram showing erosion of the left petrous bone.
Fig. 3. Angiogram in which occlusion of the left internal carotid artery is seen.
Takahashi et al: Rhabdomyosarcoma 271
Fig. 4. An~ogram showing collateral circulation supplied from the vertebrobasilar artery system on the left.
Fig. 5. At postmortem examination the tumor was found to be located mainly in the left hemi-basis.
staining. Cellular pleomorphism was marked (Figs. 6, 7). In the chiasmal region, the tumor invaded the pia-arachnoid and surrounded the meningeal vessels. In the optic nerve and chiasm, degenerative changes of the nerve fibers were observed. Discussion Rhabdomyosarcoma in the base of the skull produces signs and symptoms of cranial nerve palsies. The facial abducent nerves may be involved either prior to or concomitantly with other cranial nerves [3, 5-7, 9]. In our patient, the initial symptoms and signs were facial and abducent nerve palsies. In his clinical course, unilateral involvement of all cranial nerves, except for the olfactory nerve, was observed. Cerebral signs and symptoms and any signs of increased intracranial pressure, including papilledema and spinal fluid changes, were not found. Therefore, the present case fulfills the criteria of Garcin's syndrome. h is of greater interest that fair visual function in the left eye remained for a long period of time, in spite of complete occlusion of the left internal carotid artery. Carotid and
Fig. 6. Tumor cells with eosinophilic cytoplasm arranged in interlacing bundles. (H & E; ×100 before 30% reduction.)
272
SurgicalNeurology Vol 17 No 4 April 1982
Furthermore, the occlusive changes of the left internal carotid artery caused by invasion of the tumor might have progressed comparatively slowly. These factors might be related to the prolonged preservation of visual function. Finally, the left miotic pupil observed at the initial stage might be attributed to Homer's syndrome.
Fig. 7. Elongated tumor cell with cross-striations. (Phosphotungstic acid-hematoxylin; ×250 before 30% reduction.)
vertebral angiograms demonstrated collateral circulation derived from the right internal carotid, left external carotid, and vertebral arteries. In particular, collateral circulation, supplied by the external carotid artery through the facial artery, the superficial temporal artery, and the maxillary artery to the left ophthalmic artery, was detected.
References I. Bignas B: Tumorsof the base of the skull, in Vinken PJ, BruynGW (eds): Handbookof Clinical Neurology.Amsterdam: North-Holland, 1974, Vol 16, pp 180-183 2. DanzigerJ, Handel SF, Jing BS, WallaceS: Computerizedtomography in rhabdomyosarcomaof the head and neck. Cancer44:463-467, 1979 3. DeutschM, LeenR, MercadoR Jr: Rhabdomyosarcomaof the middle cranial fossa. Cancer 31:1193-1196, 1973 4. Dim WR, Batsakis JG: Rhabdomyosarcomaof the head and neck. Arch Surg 84:112-117, 1962 5. FleischerAS, KoslowM, Rovit RL: Neurologicalmanifestationof pri. maryrhabdomyosarcomaof the head and neck in children. J Neurosurg 43:207-214, 1975 6. Jaffe BF, Fox JE, BatsakisJG: Rhabdomyosarcomaof the middle ear and mastoid. Cancer 27:29-37, 1971 7. MassonJK, SouleEH: Embryonalrhabdomyosarcomaof the head and neck. Am J Surg 110:585-591, 1965 8. SpiegelLA: Syndromeof Garcin--unilateral total involvementof the cranial nerveswith report of one case. Ann Otol 52:706-712, 1943 9. White A, Verma PL, BullimoreJ: Rhabdomyosarcomaof the nasophary~x. J LaryngolOtol 88:271-276, 1974
Book Review Craniofacial and Upper Cervical Arteries By Pierre L. Lasjaunias, M.D. Baltimore, London: Williams & Wilkins, 1981 199 pp., $57. O0 Reviewed by Richard H. Jackson, M.D., and Arthur L, Day, M.D., Gainesville, Florida This book presents a highly detailed anatomical analysis of arteries supplying the craniofacial and upper cervical region. Dr. Lasjaunias uses a unique approach to classify the several recognized normal anatomical variants, based on phylogenic and ontogenic principles plus territorial demands. High-quality subtraction angiograms constitute the major part of the book. The angiograms are accompanie d by text that describes in detail the smaller arterial branches off
the subclavian, external carotid, and extradural internal carotid arteries. Good-quality color photographs of injected cadaver dissections supplement the angiograms. Diagrams of various regions, such as the internal maxillary system, cavernous sinus, and petrous bone, illustrate normal and nonpathological variations in the arterial supply to these regions. Very few pathological studies are included and no cerebral vasculature is presented. The book provides a sound background for understanding the detailed normal angiographic and territorial arterial blood supply to this highly vascular region. As this book has minimal application to intracranial pathology or surgery, this book would not be recommended reading for most general neurological subspecialists, but it should be quite useful to specialized surgeons and interventional radiologists working in the region of the base of the skull, face, and neck.
A case of rhabdomyosarcoma of the head and neck demonstrating signs and symptoms of Garcin's syndrome is presented. Ocular findings are discussed clinicopathologically. Takahashi T, MuraseT, IsayamaY, TamakiN, FujiwaraK, MatsumotoS: Rhabdomyosarcoma presenting as Garcin's syndrome.SurgNeurol 17:269-272, 1982 Garcin's syndrome, also called hemi-basis syndrome, is characterized by unilateral paralyses of all cranial nerves, absence of cerebral signs, and symptoms such as disturbances of the motor and sensory systems, with no signs of increased intracranial pressure [1, 8]. Rhabdomyosarcoma is a prevalent malignant tumor in childhood and occurs frequently in the head and neck [2, 4, 5, 9]. However, to our knowledge, rhabdomyosarcoma presenting as Garcin's syndrome has not yet been reported.
Case Report A 6-year-old boy was referred to the Ophthalmology Department of Kobe University on February 4, 1974, because of diplopia, left facial nerve palsy, and tumor in the left retromandibular region. Neuroophthalmological examination revealed the corrected visual acuity was 20/15 on the right and 20/25 on the left. Lagophthalmos of the left eye due to facial nerve palsy and a sensory deficit in the left half of the face were observed. Lateral movement of the left eye was disturbed. The left pupil was miotic. Both pupils reacted promptly and completely to light. Ophthalmoscopy revealed no remarkable changes in either fundi. Goldmann's visual fields showed an inferior nasal quadrantanopia in both eyes (Fig. 1). The patient's clinical course was progressive and he was admitted to the Neurosurgery Department of Kobe University on February 19, 1974. From the *Department of Ophthalmologyand tDepartment of Neurosurgery, KobeUniversity,School of Medicine, Kobe,Japan. Address reprint requests to Dr. Toshihiro Takahashi. Department of Ophthalmology, Kobe University, School of Medicine, Kusunoki-cho, 7-chome, Chuo-ku, Kobe,Japan #650. Key words: Garcin's syndrome;Guillain-Alajouanine-Garcinsyndrome; hemi-basis syndrome;multiple cranial neuropathy; rhabdomyosarcoma; optic atrophy.
Neurological examination on admission showed paralyses of the left third through twelfth cranial nerves and disturbance of visual function in both eyes. However, no other neurological findings, such as cerebral signs and symptoms, were detected and cerebrospinal fluid studies were normal. Skull roentgenograms demonstrated destructive lesions in the base of the skull, indicated by enlargement of the optic canal and erosion of the middle cranial fossa, the anterior part of the petrous bone, and the jugular foramen (Fig. 2). Carotid and vertebral angiograms revealed occlusion of the left internal carotid artery and collateral circulation in the left cerebral hemisphere, supplied by the left external carotid, right internal carotid, and vertebral arteries (Figs. 3, 4). Biopsy of the tumor located in the left retromandibutar region was performed. This revealed rhabdomyosarcoma. The patient was treated with 5,100 rads of radiation. Thereafter, shrinkage of the tumor was observed but visual function remained unchanged. On November 18, 1974, the visual disturbance of the left eye rapidly deteriorated to 20/1000. The left pupil became mydriatic and the left optic disc appeared to be slightly atrophic, as observed on funduscopic examination. The patient's general condition deteriorated in spite of a further course of 3,000 fads and he died on February 14, 1975. At postmortem examination the tumor was found to be solid and yellowish white with nodular extensions; it was well demarcated from the surrounding tissue and measured 6 x 6 × 7 cm. The tumor was located chiefly in the left side of the tuberculum sellae, the middle cranial fossa, and the anterior portion of the foramen magnum, and furthermore, it partially extended over the midline of the clivus and tuberculum sellae (Fig. 5). The tumor extended into the epipharynx and the left retromandibular region through the parapharyngeal space. The left third through twelfth cranial nerves were completely buried in this tumor. Both intracranial optic nerves, particularly on the left, were markedly elevated and were atrophic due to compression by the tumor. Microscopically the tumor was composed mainly of elongated, ovoid, and plump cells with eosinophilic cytoplasm. These tumor cells were arranged in wavy and interlacing bundles, and cross-striations were visible on hematoxylin and eosin staining and phosphotungstic acid-hematoxylin
0090-3019/82/040269-04501.25 © 1981 by Little, Brown and Company (Inc.)
269
270 SurgicalNeurology Vol 17 No 4 April 1982
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Fig. 1. Bilateral inferi~rr nasal quadrantanopia was evident in the visual fields,
Fig. 2. Roentgenogram showing erosion of the left petrous bone.
Fig. 3. Angiogram in which occlusion of the left internal carotid artery is seen.
Takahashi et al: Rhabdomyosarcoma 271
Fig. 4. An~ogram showing collateral circulation supplied from the vertebrobasilar artery system on the left.
Fig. 5. At postmortem examination the tumor was found to be located mainly in the left hemi-basis.
staining. Cellular pleomorphism was marked (Figs. 6, 7). In the chiasmal region, the tumor invaded the pia-arachnoid and surrounded the meningeal vessels. In the optic nerve and chiasm, degenerative changes of the nerve fibers were observed. Discussion Rhabdomyosarcoma in the base of the skull produces signs and symptoms of cranial nerve palsies. The facial abducent nerves may be involved either prior to or concomitantly with other cranial nerves [3, 5-7, 9]. In our patient, the initial symptoms and signs were facial and abducent nerve palsies. In his clinical course, unilateral involvement of all cranial nerves, except for the olfactory nerve, was observed. Cerebral signs and symptoms and any signs of increased intracranial pressure, including papilledema and spinal fluid changes, were not found. Therefore, the present case fulfills the criteria of Garcin's syndrome. h is of greater interest that fair visual function in the left eye remained for a long period of time, in spite of complete occlusion of the left internal carotid artery. Carotid and
Fig. 6. Tumor cells with eosinophilic cytoplasm arranged in interlacing bundles. (H & E; ×100 before 30% reduction.)
272
SurgicalNeurology Vol 17 No 4 April 1982
Furthermore, the occlusive changes of the left internal carotid artery caused by invasion of the tumor might have progressed comparatively slowly. These factors might be related to the prolonged preservation of visual function. Finally, the left miotic pupil observed at the initial stage might be attributed to Homer's syndrome.
Fig. 7. Elongated tumor cell with cross-striations. (Phosphotungstic acid-hematoxylin; ×250 before 30% reduction.)
vertebral angiograms demonstrated collateral circulation derived from the right internal carotid, left external carotid, and vertebral arteries. In particular, collateral circulation, supplied by the external carotid artery through the facial artery, the superficial temporal artery, and the maxillary artery to the left ophthalmic artery, was detected.
References I. Bignas B: Tumorsof the base of the skull, in Vinken PJ, BruynGW (eds): Handbookof Clinical Neurology.Amsterdam: North-Holland, 1974, Vol 16, pp 180-183 2. DanzigerJ, Handel SF, Jing BS, WallaceS: Computerizedtomography in rhabdomyosarcomaof the head and neck. Cancer44:463-467, 1979 3. DeutschM, LeenR, MercadoR Jr: Rhabdomyosarcomaof the middle cranial fossa. Cancer 31:1193-1196, 1973 4. Dim WR, Batsakis JG: Rhabdomyosarcomaof the head and neck. Arch Surg 84:112-117, 1962 5. FleischerAS, KoslowM, Rovit RL: Neurologicalmanifestationof pri. maryrhabdomyosarcomaof the head and neck in children. J Neurosurg 43:207-214, 1975 6. Jaffe BF, Fox JE, BatsakisJG: Rhabdomyosarcomaof the middle ear and mastoid. Cancer 27:29-37, 1971 7. MassonJK, SouleEH: Embryonalrhabdomyosarcomaof the head and neck. Am J Surg 110:585-591, 1965 8. SpiegelLA: Syndromeof Garcin--unilateral total involvementof the cranial nerveswith report of one case. Ann Otol 52:706-712, 1943 9. White A, Verma PL, BullimoreJ: Rhabdomyosarcomaof the nasophary~x. J LaryngolOtol 88:271-276, 1974
Book Review Craniofacial and Upper Cervical Arteries By Pierre L. Lasjaunias, M.D. Baltimore, London: Williams & Wilkins, 1981 199 pp., $57. O0 Reviewed by Richard H. Jackson, M.D., and Arthur L, Day, M.D., Gainesville, Florida This book presents a highly detailed anatomical analysis of arteries supplying the craniofacial and upper cervical region. Dr. Lasjaunias uses a unique approach to classify the several recognized normal anatomical variants, based on phylogenic and ontogenic principles plus territorial demands. High-quality subtraction angiograms constitute the major part of the book. The angiograms are accompanie d by text that describes in detail the smaller arterial branches off
the subclavian, external carotid, and extradural internal carotid arteries. Good-quality color photographs of injected cadaver dissections supplement the angiograms. Diagrams of various regions, such as the internal maxillary system, cavernous sinus, and petrous bone, illustrate normal and nonpathological variations in the arterial supply to these regions. Very few pathological studies are included and no cerebral vasculature is presented. The book provides a sound background for understanding the detailed normal angiographic and territorial arterial blood supply to this highly vascular region. As this book has minimal application to intracranial pathology or surgery, this book would not be recommended reading for most general neurological subspecialists, but it should be quite useful to specialized surgeons and interventional radiologists working in the region of the base of the skull, face, and neck.