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Pediatr Neurosurg 19 9 1-9 2 :17:53-56
Clival Chondroma in a Child with Ollier’s Disease Case Report Z. Ghogawala, M. Moore, R. Strand, W.J. Kupsky, R.M. Scott The Section of Pediatric Neurosurgery, The Children's Hospital, Boston, Mass., USA
Key Words. Ollier’s disease • Enchondromatosis • Magnetic resonance imaging • Chondroma ■Brain tumor • Clivus A bstract. An 8-ycar-old female with Ollier’s disease (multiple enchondromatosis) developed an intracranial chon droma arising from the clivus, which was diagnosed by both computed tomography and magnetic resonance imaging. The clinical presentation, differential diagnosis, and management of this rare pediatric intracranial tumor are discussed.
Ollier’s disease (multiple enchondromatosis) is a noninherited dysplasia of cartilage which clinically manifests itself in multiple bone tumors and in secondary limb length inequalities and bone deformities. The long bones of the extremities and the pelvis constitute the most com mon sites of enchondromatosis and ribs, sternum, and skull are rarely involved. We report an unusual case of a clival chondroma in an 8-year-old child causing obstruc tive hydrocephalus and focal neurological deficit.
Case Report
Fig. 1. Axial CT. without enhancement, at the level of the temporal horns. The mass demonstrates high signal attenuation, and fills the su prasellar cistern and proximal sylvian fissure. The right cerebral pedun cle is displaced posteriorly and the right temporal horn is dilated. The tumor’s relationship to the petrous apex is well seen. The mass is delin eated by the arrowheads.
Computed tomography (CT) imaging (fig. 1) revealed a large (5 X 4 X 4 cm) high attenuation mass tilling the suprasellar cistern and prox imal sylvian fissure, displacing the right cerebral peduncle posteriorly and right temporal lobe laterally. The lateral ventricles were dilated, with partial obliteration of the third ventricle and a normal fourth ven tricle.
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The patient is an 8-year-old female in whom ihe diagnosis of Ol lier's disease had been made at age 6 months. She had undergone mul tiple orthopedic procedures for excision of enchondromas of the long bones. One year prior to admission the patient developed gradual onset of right temporal region headaches, weight gain, and double vision. Over the ensuing months, the patient had increasing episodes of uri nary frequency, urgency, and incontinence. Immediately prior to hospi talization she was noted to have difficulty with concentration and memory. The physical examination was remarkable for multiple well-healed scars on the extremities from prior surgery. The neurological examina tion revealed a right Bird cranial nerve palsy with preserved pupillary reactivity. The rest of the cranial nerve examination was normal. Motor examination showed a mild tremor of both upper extremities (left great er than the right), but strength and tone were normal throughout all limbs. Deep tendon reflexes were increased on the left arm and leg.
54
Ghogawala/Moore/Strand/Kupsky/Scott
2a
4
Tl-weighted (TE = 20. TR = 600) magnetic resonance imaging (MRI) in the sagittal and coronal planes (fig. 2) strikingly delineated the extra-axial mass which arose front the clivus at the basisphenoid-basio cciput junction and extended superiorly to involve structures at the cra nial base, including the right internal carotid artery, middle cerebral ar
tery and pituitary stalk. T2-weighled (TE=8(), TR=2,500) axial MR (fig. 3) confirmed the marked posterior displacement and deformity of the brain stem at the midbrain level. The skeletal radiographs were typical of enchondromatosis (fig. 4), with essentially all of the long bones of the patient affected.
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3
Fig. 2. Tl-weighted MR images in the paramedian sagittal (a) and coronal (b) planes, a The arrowhead demonstrates the probable region of origin of the tumor at the basisphenoid-basiocciput junction. The posterior and superior aspects of the mass displace the pons and the thalamus, as delineated by the arrows, b The eccentric intracranial pro jection of the tumor is well visualized in the coronal plane, with dis placement of the middle cerebral artery superiorly (vertical arrow) and partial obliteration and displacement of the third ventricle. Fig. 3. Axial T2-weighted MR image at the level of the undivided middle cerebral arteries and the midbrain. There is anterior and lateral displacement of the right middle cerebral artery and branches. At one site, the tumor appears to encase the vessel (arrowhead). There is gross deformity and displacement of the midbrain (arrow). The inhomogene ity of the tumor on this sequence is a reflection of the combination of cnchondromatous bone and cartilage. Fig. 4. Lower extremities. AP radiograph. The florid splaying and irregular ossification is apparent at the ends of all the long bones seen. This pattern is typical lor enchondromatosis.
Clivai Chondroma in Ollier’s Disease
55
Fig. 5. a Initial exposure of intracranial chondroma. The right anterior temporal lobe has been resected, and the tumor is seen as a rounded mass eroding bone at the base of the right middle cranial fossa. The plane between normal bone and dura and the chondroma is delineated by the arrow heads.!) Internal decompression of the tumor. 1:2 magnification, demonstrating the spiculated particles of cartilage within it.
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Fig. 6. Chondroma composed of irregular lobules of moderately cellular hyaline cartilage with very few blood vessels., a Bands of sparsely cellular matrix arc shown (arrows). HE. X 100. b Higher magnification of chondrocytes with a lobule. Most cells sit individually in lacunes. There is minimal nuclear X 400.
Postoperative Coarse At follow-up 2 years later, the patient's lllrd nerve palsy had im proved slightly with ptosis reduced and return of medial rectus function. There were subjective sensory changes in VI on the right (without sen sory loss on clinical examination) and hypalgesia in V2 on the right. A
mild right-sided hemiparesis was also appreciated. Follow-up CT and MR1 scans have demonstrated stability of the residual tumor and brain stem displacement. Histologic Examination Pathologic examination of the tumor revealed that the tissue con tained abnormal sparsely to moderately cellular hyaline cartilage ar ranged in irregular lobules separated by virtually acellular matrix material (fig. 6a). Blood vessels were sparse. Within the lobules, chondrocytes were scattered singly or in small clusters in an abundant, palely baso philic matrix. Most chondrocytes were contained singly within a lacunc (fig. 6b). The nuclei were small, with minimal pleomorphism, and there was abundant cytoplasm. No atypical cells or cells in mitosis were seen, and no areas of ossification were noted. The microscopic features were considered typical of a benign cartilaginous neoplasm (enchondroma).
Downloaded by: Thomas Jefferson University Scott Library 147.140.27.100 - 8/23/2018 8:39:58 AM
Operative Approach The patient underwent a right temporal craniotomy. The anterior temporal lobe was removed to gain medial exposure. The tumor extend ed from the petrous apex to the cavernous sinus region; it was extradu ral and had the consistency of cartilage. The tumor was debulked using the Cavitron at maximum settings. A complete resection was not pos sible because of encasement of the carotid and basilar arteries, but the brain stem was decompressed and the posterior cerebral artery and the proximal portion of the right third nerve were visualized (fig. 5).
Ghogawala/Moore/Strand/Kupsky/Scott
56
Involvement of the skull in Ollier's disease is extraor dinarily rare 11 ]. There are 5 reported cases in adults in the literature [2-6] and the early presentation in an 8-year-old child is quite unusual. In general, chondromas of the base of the skull occur in patients from the 2nd to the 5th de cade of life [7], Intracranial chondromas in the general population are quite rare as well. Sarwar et al. 16] found only 122 previ ously reported cases of intracranial chondromas; Cushing had only three of these tumors in his series of 2.033 [7]. Two thirds of these tumors are found at the skull base [8] and are probably derived from cartilaginous rests in basal synchondroses [3, 5]. Clinically, basal intracranial tumors frequently cause cranial nerve palsies resulting in diplopia and in impair ment of vision due to involvement of cranial nerves II through VI [6]. The symptoms usually progress gradually as these tumors slowly enlarge. Although the tumors are benign with few exceptions, complete excision is rarely possible [9|. Malignant degeneration of chondromas can occur in Ollier's disease although malignancy is found more often (15%) in patients with Maffucci’s syndrome in which multiple enchondromatosis occurs in association with subcutaneous hemangiomas [10] . In both syndromes, however, malignant change in an intracranial chondroma is exceedingly rare [11]. The long-term outlook for our pa tient is not clear, but regrowth of her residual tumor should be anticipated, necessitating additional surgery and/or ad juvant treatment. In patients with Ollier's disease or Maffucci’s syn drome, a calcified mass at the base of the skull is most likely a chondroma |6 | although chordoma or chondroid chordoma must also be considered. Depending on the ra diologic appearance, other diagnoses also can be consid ered including meningioma, acoustic neuroma, epider moid, craniopharyngioma, and metastatic disease [3, 6 ].
Acknowledgement Wo are grateful to Dr. Frederick Shapiro, Department of Orthope dies. Children's Hospital. Boston. Mass., for the referral of this patient.
References 1 Rawlings CE. Bullard DE. Burger PC. Friedman AH: A ease of Ol lier's disease associated with two intracranial gliomas. Neurosur gery 1987:21:400-403. 2 Pospiech J. Mehdorn HM, Reinhardt V, Grote W: Sellar chondroma in a case of Ollier’s disease. Neurochirurgia 1989:32:30-35. 3 Traflet RF. Babaria AR. Barolat G, Doan HT, Gonzalez C. Mishkin MM: Intracranial chondroma in a patient with Ollier's disease. J Neurosurg 1989;70:274-276. 4 Imagawa K. Toda I. Hayashi M. Asai A, Nomura T: A case of enchondroma of the skull base - Manifestation of generalized chon dromatosis (in Japanese). No Shinkei Geka 1977:5:457-463. 5 Reuter K. Weber AL: Parasellar chondrosarcoma in a patient with Ollier's disease. Neuroradiology 1981:22:151-154. 6 Sarwar M. Swischuk LE. Schechler MM: Intracranial chondromas. Am J Roentgenol 1976:127:973-977. 7 Bakdash H. Alksne JF. Rand RW: Osteochondroma of the base of the skull causing an isolated oculomotor nerve paralysis. J Neuro surg 1969;31:230-233. 8 Bingas B: Tumours of the Base of the Skull. Handbook of Clinical Neurology. Amsterdam. North-Holland. 1974. vol:l7, pp:136—233. 9 Falconer MA, Bailey IC. Duchen LW: Surgical treatment of chordo ma and chondroma of the skull base.J Neurosurg 1968:29:261-275. 10 Lewis RJ. Katcham AS: Maffucci’s syndrome: Functional and neo plastic significance. J Bone Joint Surg 1973;55:1465-1479. 11 List CL: Osteochondromas arising from the base of the kull. Sur gery Gynecol Obstet 1943;76:480-492.
R. Michael Scott. MD Section of Pediatric Neurosurgery Children's Hospital 300 Longwood Avenue Boston, MA 02115 (USA)
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Discussion
Pediatr Neurosurg 19 9 1-9 2 :17:53-56
Clival Chondroma in a Child with Ollier’s Disease Case Report Z. Ghogawala, M. Moore, R. Strand, W.J. Kupsky, R.M. Scott The Section of Pediatric Neurosurgery, The Children's Hospital, Boston, Mass., USA
Key Words. Ollier’s disease • Enchondromatosis • Magnetic resonance imaging • Chondroma ■Brain tumor • Clivus A bstract. An 8-ycar-old female with Ollier’s disease (multiple enchondromatosis) developed an intracranial chon droma arising from the clivus, which was diagnosed by both computed tomography and magnetic resonance imaging. The clinical presentation, differential diagnosis, and management of this rare pediatric intracranial tumor are discussed.
Ollier’s disease (multiple enchondromatosis) is a noninherited dysplasia of cartilage which clinically manifests itself in multiple bone tumors and in secondary limb length inequalities and bone deformities. The long bones of the extremities and the pelvis constitute the most com mon sites of enchondromatosis and ribs, sternum, and skull are rarely involved. We report an unusual case of a clival chondroma in an 8-year-old child causing obstruc tive hydrocephalus and focal neurological deficit.
Case Report
Fig. 1. Axial CT. without enhancement, at the level of the temporal horns. The mass demonstrates high signal attenuation, and fills the su prasellar cistern and proximal sylvian fissure. The right cerebral pedun cle is displaced posteriorly and the right temporal horn is dilated. The tumor’s relationship to the petrous apex is well seen. The mass is delin eated by the arrowheads.
Computed tomography (CT) imaging (fig. 1) revealed a large (5 X 4 X 4 cm) high attenuation mass tilling the suprasellar cistern and prox imal sylvian fissure, displacing the right cerebral peduncle posteriorly and right temporal lobe laterally. The lateral ventricles were dilated, with partial obliteration of the third ventricle and a normal fourth ven tricle.
Downloaded by: Thomas Jefferson University Scott Library 147.140.27.100 - 8/23/2018 8:39:58 AM
The patient is an 8-year-old female in whom ihe diagnosis of Ol lier's disease had been made at age 6 months. She had undergone mul tiple orthopedic procedures for excision of enchondromas of the long bones. One year prior to admission the patient developed gradual onset of right temporal region headaches, weight gain, and double vision. Over the ensuing months, the patient had increasing episodes of uri nary frequency, urgency, and incontinence. Immediately prior to hospi talization she was noted to have difficulty with concentration and memory. The physical examination was remarkable for multiple well-healed scars on the extremities from prior surgery. The neurological examina tion revealed a right Bird cranial nerve palsy with preserved pupillary reactivity. The rest of the cranial nerve examination was normal. Motor examination showed a mild tremor of both upper extremities (left great er than the right), but strength and tone were normal throughout all limbs. Deep tendon reflexes were increased on the left arm and leg.
54
Ghogawala/Moore/Strand/Kupsky/Scott
2a
4
Tl-weighted (TE = 20. TR = 600) magnetic resonance imaging (MRI) in the sagittal and coronal planes (fig. 2) strikingly delineated the extra-axial mass which arose front the clivus at the basisphenoid-basio cciput junction and extended superiorly to involve structures at the cra nial base, including the right internal carotid artery, middle cerebral ar
tery and pituitary stalk. T2-weighled (TE=8(), TR=2,500) axial MR (fig. 3) confirmed the marked posterior displacement and deformity of the brain stem at the midbrain level. The skeletal radiographs were typical of enchondromatosis (fig. 4), with essentially all of the long bones of the patient affected.
Downloaded by: Thomas Jefferson University Scott Library 147.140.27.100 - 8/23/2018 8:39:58 AM
3
Fig. 2. Tl-weighted MR images in the paramedian sagittal (a) and coronal (b) planes, a The arrowhead demonstrates the probable region of origin of the tumor at the basisphenoid-basiocciput junction. The posterior and superior aspects of the mass displace the pons and the thalamus, as delineated by the arrows, b The eccentric intracranial pro jection of the tumor is well visualized in the coronal plane, with dis placement of the middle cerebral artery superiorly (vertical arrow) and partial obliteration and displacement of the third ventricle. Fig. 3. Axial T2-weighted MR image at the level of the undivided middle cerebral arteries and the midbrain. There is anterior and lateral displacement of the right middle cerebral artery and branches. At one site, the tumor appears to encase the vessel (arrowhead). There is gross deformity and displacement of the midbrain (arrow). The inhomogene ity of the tumor on this sequence is a reflection of the combination of cnchondromatous bone and cartilage. Fig. 4. Lower extremities. AP radiograph. The florid splaying and irregular ossification is apparent at the ends of all the long bones seen. This pattern is typical lor enchondromatosis.
Clivai Chondroma in Ollier’s Disease
55
Fig. 5. a Initial exposure of intracranial chondroma. The right anterior temporal lobe has been resected, and the tumor is seen as a rounded mass eroding bone at the base of the right middle cranial fossa. The plane between normal bone and dura and the chondroma is delineated by the arrow heads.!) Internal decompression of the tumor. 1:2 magnification, demonstrating the spiculated particles of cartilage within it.
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Fig. 6. Chondroma composed of irregular lobules of moderately cellular hyaline cartilage with very few blood vessels., a Bands of sparsely cellular matrix arc shown (arrows). HE. X 100. b Higher magnification of chondrocytes with a lobule. Most cells sit individually in lacunes. There is minimal nuclear X 400.
Postoperative Coarse At follow-up 2 years later, the patient's lllrd nerve palsy had im proved slightly with ptosis reduced and return of medial rectus function. There were subjective sensory changes in VI on the right (without sen sory loss on clinical examination) and hypalgesia in V2 on the right. A
mild right-sided hemiparesis was also appreciated. Follow-up CT and MR1 scans have demonstrated stability of the residual tumor and brain stem displacement. Histologic Examination Pathologic examination of the tumor revealed that the tissue con tained abnormal sparsely to moderately cellular hyaline cartilage ar ranged in irregular lobules separated by virtually acellular matrix material (fig. 6a). Blood vessels were sparse. Within the lobules, chondrocytes were scattered singly or in small clusters in an abundant, palely baso philic matrix. Most chondrocytes were contained singly within a lacunc (fig. 6b). The nuclei were small, with minimal pleomorphism, and there was abundant cytoplasm. No atypical cells or cells in mitosis were seen, and no areas of ossification were noted. The microscopic features were considered typical of a benign cartilaginous neoplasm (enchondroma).
Downloaded by: Thomas Jefferson University Scott Library 147.140.27.100 - 8/23/2018 8:39:58 AM
Operative Approach The patient underwent a right temporal craniotomy. The anterior temporal lobe was removed to gain medial exposure. The tumor extend ed from the petrous apex to the cavernous sinus region; it was extradu ral and had the consistency of cartilage. The tumor was debulked using the Cavitron at maximum settings. A complete resection was not pos sible because of encasement of the carotid and basilar arteries, but the brain stem was decompressed and the posterior cerebral artery and the proximal portion of the right third nerve were visualized (fig. 5).
Ghogawala/Moore/Strand/Kupsky/Scott
56
Involvement of the skull in Ollier's disease is extraor dinarily rare 11 ]. There are 5 reported cases in adults in the literature [2-6] and the early presentation in an 8-year-old child is quite unusual. In general, chondromas of the base of the skull occur in patients from the 2nd to the 5th de cade of life [7], Intracranial chondromas in the general population are quite rare as well. Sarwar et al. 16] found only 122 previ ously reported cases of intracranial chondromas; Cushing had only three of these tumors in his series of 2.033 [7]. Two thirds of these tumors are found at the skull base [8] and are probably derived from cartilaginous rests in basal synchondroses [3, 5]. Clinically, basal intracranial tumors frequently cause cranial nerve palsies resulting in diplopia and in impair ment of vision due to involvement of cranial nerves II through VI [6]. The symptoms usually progress gradually as these tumors slowly enlarge. Although the tumors are benign with few exceptions, complete excision is rarely possible [9|. Malignant degeneration of chondromas can occur in Ollier's disease although malignancy is found more often (15%) in patients with Maffucci’s syndrome in which multiple enchondromatosis occurs in association with subcutaneous hemangiomas [10] . In both syndromes, however, malignant change in an intracranial chondroma is exceedingly rare [11]. The long-term outlook for our pa tient is not clear, but regrowth of her residual tumor should be anticipated, necessitating additional surgery and/or ad juvant treatment. In patients with Ollier's disease or Maffucci’s syn drome, a calcified mass at the base of the skull is most likely a chondroma |6 | although chordoma or chondroid chordoma must also be considered. Depending on the ra diologic appearance, other diagnoses also can be consid ered including meningioma, acoustic neuroma, epider moid, craniopharyngioma, and metastatic disease [3, 6 ].
Acknowledgement Wo are grateful to Dr. Frederick Shapiro, Department of Orthope dies. Children's Hospital. Boston. Mass., for the referral of this patient.
References 1 Rawlings CE. Bullard DE. Burger PC. Friedman AH: A ease of Ol lier's disease associated with two intracranial gliomas. Neurosur gery 1987:21:400-403. 2 Pospiech J. Mehdorn HM, Reinhardt V, Grote W: Sellar chondroma in a case of Ollier’s disease. Neurochirurgia 1989:32:30-35. 3 Traflet RF. Babaria AR. Barolat G, Doan HT, Gonzalez C. Mishkin MM: Intracranial chondroma in a patient with Ollier's disease. J Neurosurg 1989;70:274-276. 4 Imagawa K. Toda I. Hayashi M. Asai A, Nomura T: A case of enchondroma of the skull base - Manifestation of generalized chon dromatosis (in Japanese). No Shinkei Geka 1977:5:457-463. 5 Reuter K. Weber AL: Parasellar chondrosarcoma in a patient with Ollier's disease. Neuroradiology 1981:22:151-154. 6 Sarwar M. Swischuk LE. Schechler MM: Intracranial chondromas. Am J Roentgenol 1976:127:973-977. 7 Bakdash H. Alksne JF. Rand RW: Osteochondroma of the base of the skull causing an isolated oculomotor nerve paralysis. J Neuro surg 1969;31:230-233. 8 Bingas B: Tumours of the Base of the Skull. Handbook of Clinical Neurology. Amsterdam. North-Holland. 1974. vol:l7, pp:136—233. 9 Falconer MA, Bailey IC. Duchen LW: Surgical treatment of chordo ma and chondroma of the skull base.J Neurosurg 1968:29:261-275. 10 Lewis RJ. Katcham AS: Maffucci’s syndrome: Functional and neo plastic significance. J Bone Joint Surg 1973;55:1465-1479. 11 List CL: Osteochondromas arising from the base of the kull. Sur gery Gynecol Obstet 1943;76:480-492.
R. Michael Scott. MD Section of Pediatric Neurosurgery Children's Hospital 300 Longwood Avenue Boston, MA 02115 (USA)
Downloaded by: Thomas Jefferson University Scott Library 147.140.27.100 - 8/23/2018 8:39:58 AM
Discussion
