1991, The British Journal of Radiology, 64, 633-636 Case reports
other, may reflect predominance of medullary cystic changes early in the disease. This might explain the similarity with ARPK where the cystic change is also predominantly intramedullary. Although the incidence of neonatal renal ultrasound changes is not known, LMBS should be included in the differential diagnosis of bilateral hyperechoic large kidneys in early infancy. Acknowledgments We would like to thank Dr I. Gordon for reading the manuscript. We are also grateful to Professor T. M. Barratt for allowing us to report his patients. References CHURCHILL, D. N., MCMANAMON, P. & HURLEY, R. M., 1981.
W. & PARFREY, P. S., 1988. The spectrum of renal disease in Laurence-Moon-Biedl syndrome. New England Journal of Medicine, 319, 615-618. HURLEY, R. M., DERY, P., NOGRADY, M. B. & DRUMMOND,
K. N., 1975. The renal lesions of the Laurence-Moon-Biedl syndrome. Journal of Pediatrics, 87, 206-209. LINNE, T., WIKSTAD, I. & ZETTERSTROM, R.,
1986. Renal
involvement in the Laurence-Moon-Biedl syndrome. Ada Paediatrica Scandinavica, 75, 240-244. RITCHIE,
G.,
JEQUIER,
S. &
LUSSIER-LAZAROFF,
J.,
1988.
Prenatal renal ultrasound of Laurence-Moon-Biedl syndrome. Pediatric Radiology, 19, 65-66. SMITH, D. W., 1988. Recognizable Patterns of Human Malformation, 4th edn (W. B. Saunders, Philadelphia), pp. 530-531. TEIDER, M., LEVY, M., GUBLER, M. C , GAGNADOUX, J. R. &
BROYTER, M., 1982. Renal abnormalities in the Bardet-Biedl syndrome. International Journal of Pediatric Nephrology, 3, 199-203.
Renal disease: a sixth cardinal feature of the Laurence-Moon-Biedl syndrome. Clinical Nephrology, 16, 151-154.
WILLIAMS, B., JENKINS, D. & WALLS, J., 1988. Chronic renal
HARNETT, J. D., GREEN, J. S., CRAMER, B. C , JOHNSON, G., CHAFE, L., MCMANAMON, P., FARID, N. R., PRYSE-PHILIPS,
failure: an important feature of the Laurence-Moon-Biedl syndrome. Postgraduate Medical Journal, 64, 462-464.
Intracranial chondrosarcoma in a patient with Ollier's disease By A. G. Clifton, MA, MRCP, FRCR, B. E. Kendall, FRCP, FRCR, H. A. Crockard, FRCS and T. Hughes, FRCS The National Hospitals for Nervous Diseases, Maida Vale, London W9 1TL and University College Hospital, London WC1 E 6JJ, UK (Received May 1990) Keywords: Intracranial chondrosarcoma, Ollier's disease, Computed tomography
Cartilaginous intracranial tumours are rare. A case of intracranial chondrosarcoma is described in a patient with Ollier's disease. The patient also had evidence on computed tomography of enchondromata involving the ethmoid sinuses. Case report A 36-year-old woman with Ollier's disease diagnosed at the age of 13 months, involving mainly her right side and affecting hand, rib cage, right femur, and right toes, presented with a 10month history of intermittent diplopia on right gaze. Examination showed a mild right proptosis (2 mm) with restriction of right eye movements, particularly in abduction and elevation. The visual acuities, fields and the optic discs were normal. There was no demonstrable trigeminal nerve dysfunction. A chest radiograph revealed multiple enchondromata involving the anterior ends of the left 2nd and right 4th, 5th and 7th ribs, as well as the medial borders of both scapulae (Fig. 1). Computed tomography (CT) showed a large mass, isodense Address correspondence to: Dr A. G. Clifton, Department of Radiology, Atkinson Morley's Hospital, Wimbledon, London SW20 ONE. Vol. 64, No. 763
with brain and strongly enhancing in the medial half of the right middle fossa. It eroded the right side of the clivus, sella turcica and the greater wing of the sphenoid. Its anterior margin impinged on the superior orbital fissure and its outer margin was clearly defined against the temporal lobe. Multiple mixed density lesions with regions of bone density consistent with enchondromata were seen expanding the ethmoid labyrinths (Fig. 2). Selective right internal and external carotid angiography showed the large mass to be displacing the precavernous segment of the right internal carotid artery medially and anteriorly and the proximal part of the cavernous segment anteroinferiorly. There was a hypovascular region between the displaced segment and the vessels on the inferior surface of the temporal lobe (Fig. 3). A right pterional craniotomy and excision of the tumour was performed. Cytology was consistent with a chondroid tumour, its benign or malignant nature being indeterminate. Formal histology showed a low-grade chondrosarcoma. Discussion
Intracranial cartilaginous tumours (chondromas, chondrosarcomas, osteochondromas) are uncommon. In Cushing's series of 2023 tumours, only three were of cartilaginous origin (Cushing, 1932). They usually arise at the base of the skull, the most frequent site being the 633
Case reports
parasellar region (Reuter & Weber, 1981), other sites such as the dura, choroid plexus, leptomeninges or within the brain parenchyma being even more rare (Ozgen et al, 1984). They may be solitary or a component of multiple enchondromatosis (Ollier's disease) and probably arise from cartilaginous rests in the area of the basal synchondroses (Imagawa etal, 1977). Ollier's disease is a rare non-hereditary disorder characterized by masses of cartilage persisting in the metaphysis and diaphysis, subperiosteal deposition of cartilage, and asymmetric involvement of the limbs,
Figure 1. Postero-anterior chest radiograph showing multiple enchondromata involving the ribs and scapulae.
Figure 2. (a) CT scan (bone window: WW 1600, WL 309) showing erosion of the right side of the clivus and skull base, (b) CT scan after intravenous contrast medium (soft-tissue window: WW 100, WL 47). A strongly enhancing mass is seen in the medial half of the right middle fossa eroding the sella. Its lateral margin is well defined against the brain's substance, and its anterior margin impinges on the superior orbital fissure, (c) CT scan (bone window: WW 1600, WL 266) There is a mixed density mass in the left anterior ethmoids with regions of dense calcification consistent with an enchondroma.
634
The British Journal of Radiology, July 1991
Case reports
(b) Figure 3. (a, b) Subtraction angiograms after selective injection of the right internal carotid artery (see text).
develop a malignant lesion, most likely a chondrosarcoma (Oilier, 1900; Liu et al, 1987). In up to 60% of intracranial chondromas and chondrosarcomas, calcification is seen (Sarwar et al, 1976). Angiography in cranial chondromas and chondrosarcomas shows displacement of vessels around an avascular mass (as in our case) (Sarwar et al, 1976; Bahr & Gagler, 1977). Computed tomography is usually performed as the primary investigation and clearly shows the extradural location and extent of the lesion, presence of calcification and any bone destruction. Bone destruction suggests malignant change. Reports of enhancement after intravenous contrast medium at CT vary. Bahr et al (1977) report very slight enhancement of a single chondrosarcoma with contrast medium but marked enhancement was shown in a case reported by Reuter and Weber (1981). Grossman and Davis (1981) reviewed the CT findings of five chondrosarcomas of the skull base; high density on plain CT, contrast enhancement and osteolytic changes of the contiguous bone were highly suggestive of the diagnosis. The differential diagnosis for masses at the skull base include chordoma and meningioma. If the lesion is calcified a carotid aneurysm (Sarwar et al, 1976) may need to be excluded in parasellar lesions. It can be difficult to differentiate meningioma, chordoma and chondrosarcoma. Meningiomas tend to extend along the meninges; the other tumours are extradural in origin and tend to displace the meninges, which tend to give the mass a well defined edge. Meningiomas usually show marked enhancement on CT, but, as in this case, chondrosarcoma may show marked enhancement; chordoma also may show variable enhancement. Meningiomas usually cause hyperostosis; chondrosarcomas and chordomas are destructive. Chordomas usually involve the midline but may be very asymmetrical; chondrosarcomas are generally parasellar in location. At angiography, meningiomas are usually highly vascular and chondrosarcomas hypovascular. Enchondromata are very rare in the paranasal sinuses and nose. Those in the nose usually arise from the nasal septum and are found incidentally. The most common sinus to be involved is the ethmoid (Lloyd, 1988). Kilby and Ambegoakar (1977) on reviewing the literature found that 50% of reported tumours arose from the ethmoids and 17% from the septum. These tumours are also thought to arise from cell rests. When the ethmoid tumour is large, symptoms are of nasal obstruction or pressure effects and deformities. They may recur following excision and late sarcomatous change occurs. References BAHR,
with one side being exclusively or predominantly involved. In a patient with Ollier's disease a chondroid tumour should be considered the most likely diagnosis for a mass at the base of the skull. Chondrosarcoma can result from malignant degeneration of a chondromatous lesion and up to 30% of patients with Ollier's disease Vol. 64, No. 763
A.
L.
&
GAGLER,
B.
W.,
1977.
Cranial
chondrosarcomas. Radiology, 124, 151-156. CUSHING, H., 1932. Notes on a series of two thousand verified cases with surgical-mortality percentages pertaining thereto. In Intracranial Tumours. Ed. by H. Cushing (Thomas Springfield, Illinois), ch. 8, p. 133. GROSSMAN, R.I. & DAVIS, K. R., 1981. Cranial computed
tomographic appearance of chondrosarcoma of the base of the skull. Radiology, 141, 403-408. 635
Case reports IMAGAWA, K., TODA, I. & HAYASHI, M., 1977. A case of
enchondroma
of the skull
base—a
manifestation
of
OLLIER, L. L. X. E., 1900. Dyschondroplasie. Lyon Medicate,
93, 23-24.
generalized chondromatosis. No Shinkei Geka, 5, 457-463.
OZGEN, T., PAMIR, M., AKALAN, N., BERTAN, V. & BEHSAN, O.,
KILBY, D. & AMBEGOAKAR, A. G., 1977. The nasal chondroma. Two case reports and a survey of the literature. Journal of Laryngology and Otology, 91, 415-426. Liu, J., HUDKINS, P., SWEE, R. G. & UNWI, K., 1987. Bone sarcomas associated with Ollier's disease. Cancer, 59,
1984. Intracranial solitary chondroma. Journal of Neurosurgery, 61, 399-401. REUTER, K. & WEBER, A. L., 1981. Parasellar chondrosarcoma in a patient with Ollier's disease. Neuroradiology, 22, 151-154.
1376-1385.
LLOYD, G. A. S., 1988. Diagnostic Imaging of the Nose and Paranasal Sinuses. (Springer Verlag, Berlin), pp. 163-166.
636
SARWAR,
M.,
SWISCHUK,
L.
Intracranial chondromas. Roentgenology, 127, 973-977.
&
SCHECHTER,
M. M.,
American
Journal
1976.
of
The British Journal of Radiology, July 1991
other, may reflect predominance of medullary cystic changes early in the disease. This might explain the similarity with ARPK where the cystic change is also predominantly intramedullary. Although the incidence of neonatal renal ultrasound changes is not known, LMBS should be included in the differential diagnosis of bilateral hyperechoic large kidneys in early infancy. Acknowledgments We would like to thank Dr I. Gordon for reading the manuscript. We are also grateful to Professor T. M. Barratt for allowing us to report his patients. References CHURCHILL, D. N., MCMANAMON, P. & HURLEY, R. M., 1981.
W. & PARFREY, P. S., 1988. The spectrum of renal disease in Laurence-Moon-Biedl syndrome. New England Journal of Medicine, 319, 615-618. HURLEY, R. M., DERY, P., NOGRADY, M. B. & DRUMMOND,
K. N., 1975. The renal lesions of the Laurence-Moon-Biedl syndrome. Journal of Pediatrics, 87, 206-209. LINNE, T., WIKSTAD, I. & ZETTERSTROM, R.,
1986. Renal
involvement in the Laurence-Moon-Biedl syndrome. Ada Paediatrica Scandinavica, 75, 240-244. RITCHIE,
G.,
JEQUIER,
S. &
LUSSIER-LAZAROFF,
J.,
1988.
Prenatal renal ultrasound of Laurence-Moon-Biedl syndrome. Pediatric Radiology, 19, 65-66. SMITH, D. W., 1988. Recognizable Patterns of Human Malformation, 4th edn (W. B. Saunders, Philadelphia), pp. 530-531. TEIDER, M., LEVY, M., GUBLER, M. C , GAGNADOUX, J. R. &
BROYTER, M., 1982. Renal abnormalities in the Bardet-Biedl syndrome. International Journal of Pediatric Nephrology, 3, 199-203.
Renal disease: a sixth cardinal feature of the Laurence-Moon-Biedl syndrome. Clinical Nephrology, 16, 151-154.
WILLIAMS, B., JENKINS, D. & WALLS, J., 1988. Chronic renal
HARNETT, J. D., GREEN, J. S., CRAMER, B. C , JOHNSON, G., CHAFE, L., MCMANAMON, P., FARID, N. R., PRYSE-PHILIPS,
failure: an important feature of the Laurence-Moon-Biedl syndrome. Postgraduate Medical Journal, 64, 462-464.
Intracranial chondrosarcoma in a patient with Ollier's disease By A. G. Clifton, MA, MRCP, FRCR, B. E. Kendall, FRCP, FRCR, H. A. Crockard, FRCS and T. Hughes, FRCS The National Hospitals for Nervous Diseases, Maida Vale, London W9 1TL and University College Hospital, London WC1 E 6JJ, UK (Received May 1990) Keywords: Intracranial chondrosarcoma, Ollier's disease, Computed tomography
Cartilaginous intracranial tumours are rare. A case of intracranial chondrosarcoma is described in a patient with Ollier's disease. The patient also had evidence on computed tomography of enchondromata involving the ethmoid sinuses. Case report A 36-year-old woman with Ollier's disease diagnosed at the age of 13 months, involving mainly her right side and affecting hand, rib cage, right femur, and right toes, presented with a 10month history of intermittent diplopia on right gaze. Examination showed a mild right proptosis (2 mm) with restriction of right eye movements, particularly in abduction and elevation. The visual acuities, fields and the optic discs were normal. There was no demonstrable trigeminal nerve dysfunction. A chest radiograph revealed multiple enchondromata involving the anterior ends of the left 2nd and right 4th, 5th and 7th ribs, as well as the medial borders of both scapulae (Fig. 1). Computed tomography (CT) showed a large mass, isodense Address correspondence to: Dr A. G. Clifton, Department of Radiology, Atkinson Morley's Hospital, Wimbledon, London SW20 ONE. Vol. 64, No. 763
with brain and strongly enhancing in the medial half of the right middle fossa. It eroded the right side of the clivus, sella turcica and the greater wing of the sphenoid. Its anterior margin impinged on the superior orbital fissure and its outer margin was clearly defined against the temporal lobe. Multiple mixed density lesions with regions of bone density consistent with enchondromata were seen expanding the ethmoid labyrinths (Fig. 2). Selective right internal and external carotid angiography showed the large mass to be displacing the precavernous segment of the right internal carotid artery medially and anteriorly and the proximal part of the cavernous segment anteroinferiorly. There was a hypovascular region between the displaced segment and the vessels on the inferior surface of the temporal lobe (Fig. 3). A right pterional craniotomy and excision of the tumour was performed. Cytology was consistent with a chondroid tumour, its benign or malignant nature being indeterminate. Formal histology showed a low-grade chondrosarcoma. Discussion
Intracranial cartilaginous tumours (chondromas, chondrosarcomas, osteochondromas) are uncommon. In Cushing's series of 2023 tumours, only three were of cartilaginous origin (Cushing, 1932). They usually arise at the base of the skull, the most frequent site being the 633
Case reports
parasellar region (Reuter & Weber, 1981), other sites such as the dura, choroid plexus, leptomeninges or within the brain parenchyma being even more rare (Ozgen et al, 1984). They may be solitary or a component of multiple enchondromatosis (Ollier's disease) and probably arise from cartilaginous rests in the area of the basal synchondroses (Imagawa etal, 1977). Ollier's disease is a rare non-hereditary disorder characterized by masses of cartilage persisting in the metaphysis and diaphysis, subperiosteal deposition of cartilage, and asymmetric involvement of the limbs,
Figure 1. Postero-anterior chest radiograph showing multiple enchondromata involving the ribs and scapulae.
Figure 2. (a) CT scan (bone window: WW 1600, WL 309) showing erosion of the right side of the clivus and skull base, (b) CT scan after intravenous contrast medium (soft-tissue window: WW 100, WL 47). A strongly enhancing mass is seen in the medial half of the right middle fossa eroding the sella. Its lateral margin is well defined against the brain's substance, and its anterior margin impinges on the superior orbital fissure, (c) CT scan (bone window: WW 1600, WL 266) There is a mixed density mass in the left anterior ethmoids with regions of dense calcification consistent with an enchondroma.
634
The British Journal of Radiology, July 1991
Case reports
(b) Figure 3. (a, b) Subtraction angiograms after selective injection of the right internal carotid artery (see text).
develop a malignant lesion, most likely a chondrosarcoma (Oilier, 1900; Liu et al, 1987). In up to 60% of intracranial chondromas and chondrosarcomas, calcification is seen (Sarwar et al, 1976). Angiography in cranial chondromas and chondrosarcomas shows displacement of vessels around an avascular mass (as in our case) (Sarwar et al, 1976; Bahr & Gagler, 1977). Computed tomography is usually performed as the primary investigation and clearly shows the extradural location and extent of the lesion, presence of calcification and any bone destruction. Bone destruction suggests malignant change. Reports of enhancement after intravenous contrast medium at CT vary. Bahr et al (1977) report very slight enhancement of a single chondrosarcoma with contrast medium but marked enhancement was shown in a case reported by Reuter and Weber (1981). Grossman and Davis (1981) reviewed the CT findings of five chondrosarcomas of the skull base; high density on plain CT, contrast enhancement and osteolytic changes of the contiguous bone were highly suggestive of the diagnosis. The differential diagnosis for masses at the skull base include chordoma and meningioma. If the lesion is calcified a carotid aneurysm (Sarwar et al, 1976) may need to be excluded in parasellar lesions. It can be difficult to differentiate meningioma, chordoma and chondrosarcoma. Meningiomas tend to extend along the meninges; the other tumours are extradural in origin and tend to displace the meninges, which tend to give the mass a well defined edge. Meningiomas usually show marked enhancement on CT, but, as in this case, chondrosarcoma may show marked enhancement; chordoma also may show variable enhancement. Meningiomas usually cause hyperostosis; chondrosarcomas and chordomas are destructive. Chordomas usually involve the midline but may be very asymmetrical; chondrosarcomas are generally parasellar in location. At angiography, meningiomas are usually highly vascular and chondrosarcomas hypovascular. Enchondromata are very rare in the paranasal sinuses and nose. Those in the nose usually arise from the nasal septum and are found incidentally. The most common sinus to be involved is the ethmoid (Lloyd, 1988). Kilby and Ambegoakar (1977) on reviewing the literature found that 50% of reported tumours arose from the ethmoids and 17% from the septum. These tumours are also thought to arise from cell rests. When the ethmoid tumour is large, symptoms are of nasal obstruction or pressure effects and deformities. They may recur following excision and late sarcomatous change occurs. References BAHR,
with one side being exclusively or predominantly involved. In a patient with Ollier's disease a chondroid tumour should be considered the most likely diagnosis for a mass at the base of the skull. Chondrosarcoma can result from malignant degeneration of a chondromatous lesion and up to 30% of patients with Ollier's disease Vol. 64, No. 763
A.
L.
&
GAGLER,
B.
W.,
1977.
Cranial
chondrosarcomas. Radiology, 124, 151-156. CUSHING, H., 1932. Notes on a series of two thousand verified cases with surgical-mortality percentages pertaining thereto. In Intracranial Tumours. Ed. by H. Cushing (Thomas Springfield, Illinois), ch. 8, p. 133. GROSSMAN, R.I. & DAVIS, K. R., 1981. Cranial computed
tomographic appearance of chondrosarcoma of the base of the skull. Radiology, 141, 403-408. 635
Case reports IMAGAWA, K., TODA, I. & HAYASHI, M., 1977. A case of
enchondroma
of the skull
base—a
manifestation
of
OLLIER, L. L. X. E., 1900. Dyschondroplasie. Lyon Medicate,
93, 23-24.
generalized chondromatosis. No Shinkei Geka, 5, 457-463.
OZGEN, T., PAMIR, M., AKALAN, N., BERTAN, V. & BEHSAN, O.,
KILBY, D. & AMBEGOAKAR, A. G., 1977. The nasal chondroma. Two case reports and a survey of the literature. Journal of Laryngology and Otology, 91, 415-426. Liu, J., HUDKINS, P., SWEE, R. G. & UNWI, K., 1987. Bone sarcomas associated with Ollier's disease. Cancer, 59,
1984. Intracranial solitary chondroma. Journal of Neurosurgery, 61, 399-401. REUTER, K. & WEBER, A. L., 1981. Parasellar chondrosarcoma in a patient with Ollier's disease. Neuroradiology, 22, 151-154.
1376-1385.
LLOYD, G. A. S., 1988. Diagnostic Imaging of the Nose and Paranasal Sinuses. (Springer Verlag, Berlin), pp. 163-166.
636
SARWAR,
M.,
SWISCHUK,
L.
Intracranial chondromas. Roentgenology, 127, 973-977.
&
SCHECHTER,
M. M.,
American
Journal
1976.
of
The British Journal of Radiology, July 1991
