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De novo development of intraosseous cavernous hemangioma Ralf Buhl *, Harald Barth, Lutz Do¨rner, Arya Nabavi, Axel Rohr, H. Maximilian Mehdorn Department of Neurosurgery, University of Schleswig Holstein, Schittenhelmstr. 10, 24105 Campus Kiel, Germany Received 7 October 2005; accepted 1 December 2005
Abstract Intraosseous cavernous hemangiomas are rare and not often multifocal. De novo development of a skull cavernous hemangioma has not been described previously. We present a 20-year-old man who was operated upon for a skull cavernoma in the right frontal area and developed a new lesion 3 years later in the right occipital region. The first lesion was removed completely and the postoperative course was uneventful. Histology showed an intraosseous cavernous hemangioma. MRI follow-up revealed a new lesion in the right occipital region. At the time of the first operation this lesion was not seen on CT or MRI scan. Surgical removal was performed and histology again showed a cavernous hemangioma. The patient seems to be unique and it is important to keep young patients with the diagnosis of cavernous hemangioma under close follow-up. This supports the experience from parenchymatous cavernous hemangiomas that this malformation may become a dynamic disease. 2005 Elsevier Ltd. All rights reserved. Keywords: Intraosseous; Cavernous hemangioma; MRI
1. Introduction Intraosseous cavernous hemangiomas are rare and account for only 0.2% of all osseous neoplasms with involvement of the calvarium. They usually develop in the diploic space and become symptomatic with painful swelling. Differential diagnosis includes fibrous dysplasia, osteoma, eosinophilic granuloma, aneurysmal bone cyst and meningioma.1 CT usually shows a well-defined osteolytic lesion and on MRI these lesions are usually isointense on T1-weighted images and hyperintense on T2-weighted images. Although multifocal skull cavernomas are described in the literature,2–4 a de novo development of a skull cavernous hemangioma is rare. There are reports of de novo appearance in parenchymatous lesions,4–6 but de novo appearance of an intraosseous cavernoma is extraordinary. This case supports the experience from parenchymatous cavernous hemangiomas that this malformation may become a dynamic disease.5,7 2. Case A 20-year-old male patient noticed a painful swelling in the right frontal area of his skull over 2 months. CT and MRI showed an osteolytic lesion within the skull affecting the outer layer of the bone (Figs. 1, 2). There were no neurological deficits. *
Corresponding author. Tel.: +00494315974920. E-mail address:
[email protected] (R. Buhl).
Fig. 1. CT skull with bone windows demonstrating the osteolytic lesion with involvement of the outer layer of the bone.
The lesion was removed completely (Fig. 3) and the postoperative course was uneventful. Histology showed an intraosseous cavernous hemangioma (Fig. 4). There was no familial history of this disease and there was no cranial irradiation in his past medical history. Follow-up examinations were normal and the patient had no clinical symptoms.
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Fig. 4. Histology showing blood-filled sinusoidal channels lined with a single layer of flattened endothelial cells contained within bony trabeculae (Elastica van Gieson stain, ·125).
Fig. 2. Coronal T1-weighted MRI showing the lesion in the right frontal area.
Fig. 5. Axial T1-weighted MRI showing the right occipital lesion.
3. Discussion
Fig. 3. Macroscopic intraoperative specimen with bony changes.
Three years later he noticed a swelling in the right occipital region over 2 months. CT and MRI revealed a new intraosseous lesion in the right occipital region which was suspicious again for a cavernous hemangioma (Figs. 5, 6). Retrospectively the first CT and MRI were reviewed and no lesion in this area could be seen. Surgical removal was performed as well as refilling of the bone defect with polymethylmethacrylate (PMMA). Histology again showed a cavernous hemangioma.
Intraosseous cavernous hemangiomas are rare and account for only 0.2% of all osseous neoplasms with involvement of the calvarium.3,8,9 They usually develop in the diploic space and become symptomatic with slow-growing, painful swelling.10 Most frequent sites are the parietal and frontal bones.1 Orbital involvement is also possible.11–13 Males are more affected and these lesions are usually diagnosed in the fourth and fifth decade.1,3,12,14 Yoshida et al.14 described a cavernous hemangioma of the skull in a 4-month-old male neonate. He had a left parietal mass since birth. Pathological examination revealed a cavernous hemangioma developing within the diploic space adjacent to prior hemorrhages. Cervoni et al.15 described two young patients, 7- and 16-years-old. Follow-up was without complications or recurrences. Our patient was 20 years and 23 years at the time of operations. A slow-growing
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by mutations in a cytoskeletal-related protein that is likely integral to interendothelial cell connectivity and maturation of the vascular wall. Molecular genetic studies of CCM have identified three loci, CCM 1–3, that can lead to CCM when mutated. A gene contributing to the autosomal-dominant CCM phenotype, KRIT 1 (Krev Interaction Trapped 1) has been identified through linkage analysis and mutation screening. KRIT 1 may take an active part in normal angiogenesis. It is expressed during early angiogenesis by endothelial cells and may play a key role in vessel formation and/or development.19 De novo development of a skull cavernous hemangioma is extremely rare.4 The patient in our case report seems to be unique and it is important to keep young patients with the diagnosis of cavernous hemangioma under close follow-up. We would recommend clinical examination and MRI of the head annually because the experience from parenchymatous cavernous hemangiomas is similar, that this malformation may become a dynamic disease.
References Fig. 6. Sagittal T1-weighted MRI demonstrating the second cavernous hemangioma.
cavernous hemangioma of the orbit in a 75-year-old man, which was confirmed by biopsy, was published by Colombo et al.12 Most calvarial lesions are unifocal, but some are multifocal.12 Peterson et al. presented a 64-year-old male patient with frontal and occipital headaches. He had two radiolucent lesions on plain X-ray on the left fontal and occipital bone, and both were cavernous hemangiomas. Cervoni et al.15 described one patient with two similar simultaneous lesions in the right occipital and left parietal bones. Differential diagnosis includes fibrous dysplasia, osteoma, eosinophilic granuloma, aneurysmal bone cyst and meningioma.12 CT usually shows a well-defined osteolytic lesion. The outer table of the bone is always involved. On MRI these lesions are usually isointense on T1-weighted images and hyperintense on T2-weighted images. Angiography shows increased vascularity in the area of the lesion.16 Operating usually includes resection of the lesion with a margin of 1 cm and refilling of the defect with PMMA. Histology shows blood-filled sinusoidal channels lined with a single layer of flattened endothelial cells contained within bony trabeculae.15,17 It is well known from parenchymal lesions that de novo formation of cavernous hemangiomas is frequent after irradiation of the head in childhood and also in both familial and sporadic cavernomas.5–7 Expression of proliferating cell nuclear antigen (PCNA) has been detected in the endothelium of cavernomas, indicating a growth potential in these lesions.7 Gault et al.18 found that familial cerebral cavernous malformation (CCM) disease is in part caused
1. Heckl S, Aschoff A, Kunze S. Cavernomas of the skull: review of the literature 1975–2000. Neurosurg Rev 2002;25:56–67. 2. Kumar NA, Ranganadham P, Bhaskar G, et al. Multifocal cavernous hemangiomas. Case report and review of the literature. Neuroradiology 1996;38:83–5. 3. Peterson DL, Murk SE, Story JL. Multifocal cavernous hemangioma of the skull: report of a case and review of the literature. Neurosurgery 1992;30:778–82. 4. Rosahl SK, Vorkapic P, Eghbal R, et al. Ossified and de novo cavernous malformations in the same patient. Clin Neurol Neurosurg 1998;100:138–43. 5. Detwiler PW, Porter RW, Zabramski JM, et al. De novo formation of a central nervous system cavernous malformation: implications for predicting risk of hemorrhage. J Neurosurg 1997;87:629–32. 6. Pozzati E, Acciarri N, Tognetti F, et al. Growth, subsequent bleeding, and de novo appearance of cerebral cavernous angiomas. Neurosurgery 1996;38:662–70. 7. Sure U, Butz N, Schlegel J, et al. Endothelial proliferation, neoangiogenesis, and potential de novo generation of cerebrovascular malformations. J Neurosurg 2001;94:972–7. 8. Bizzozero L, Solaining-Talamonti C, Villa F. Cavernous hemangioma of the skull. Case report and review of the literature. J Neurosurg Sci 1997;41:419–21. 9. Khanam H, Lipper MH, Wolff CL, et al. Calvarial hemangiomas: report of two cases and review of the literature. Surg Neurol 2001;55:63–7. 10. Suzuki Y, Ikeda H, Matsumoto K. Neuroradiological features of intraosseous cavernous hemangioma – case report. Neurol Med Chir (Tokyo) 2001;41:279–82. 11. Banerji D, Inao S, Sugita K, et al. Primary intraosseous orbital hemangioma: a case report and review of the literature. Neurosurgery 1994;35:1131–4. 12. Colombo F, Cursiefen C, Hofmann-Rummelt C, et al. Primary intraosseous cavernous hemangioma of the orbit. Am J Ophthalmol 2001;131:151–2. 13. Slaba SG, Karam RH, Nehme JI, et al. Intraosseous orbitosphenoidal cavernous angioma. J Neurosurg 1999;91:1034–6. 14. Yoshida D, Sugisaki Y, Shimura T, et al. Cavernous hemangioma of the skull in a neonate. Childs Nerv Syst 1999;15: 351–3.
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15. Cervoni L, Artico M, Delfini R. Intraosseous cavernous hemangioma of the skull. Neurosurg Rev 1995;18:61–4. 16. Lobato RD, Lamas E, Amor T, et al. Primary calvarial hemangioma: angiographic study. Surg Neurol 1978;10:389–94. 17. Barnes L. Solitary hemangioma of bone. In: Barnes L, editor. Surgical Pathology of the Head and Neck, Vol. 1. New York: Dekker; 1985. p. 932–6.
18. Gault J, Sarin H, Awadallah NA, et al. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance. Neurosurgery 2004;55:1–17. 19. Guzeloglu-Kayisli O, Kayisli UA, Amankulor NM, et al. Krev1 interaction trapped-1/cerebral cavernous malformation-1 protein expression during early angiogenesis. J Neurosurg 2004;100 (Suppl Pediatrics):481–7.
doi:10.1016/j.jocn.2005.12.007
High grade glioma in a focal cortical dysplastic lesion Wasiq Ahmad Thiryayi
a,*
, Marcia Donaldson-Hugh a, Stuart Ross a, Aruna Chakrabarty
b
a b
Department of Neurosurgery, Leeds General Infirmary, Leeds, UK Department of Histopathology, Leeds General Infirmary, Leeds, UK Received 28 December 2005; accepted 21 February 2006
Abstract A 61-year-old woman presented in 1999 with a recent onset of left-sided focal clonic seizures with associated vague speech arrests. A computerised tomography (CT) scan revealed a hypoattenuated non-enhancing lesion within her right temporal lobe which was biopsied and the histopathological features were reported to be consistent with a hamartoma, reminiscent of tuberous sclerosis. Her seizures remained partially controlled with phenytoin and carbamazepine. She presented again 2 years later with an increase in the frequency of her seizures. A magnetic resonance scan of her brain demonstrated a homogenously enhancing mass lesion in her right temporo-parietal lobe. An initial lobectomy showed a hamartomatous lesion on histology but a subsequent sample obtained from a wide resection carried out when there was further worsening of her condition revealed a grade IV astrocytoma within a focal cortical dysplastic lesion. Our case highlights the importance of considering the possibility of a cortical dysplastic lesion harbouring a malignancy and the need for further research to explore the behaviour of these malformative lesions. 2006 Elsevier Ltd. All rights reserved. Keywords: Focal cortical dysplasia; Cerebral malformations; Malignant transformation; Hamartoma
1. Introduction
2. Clinical presentation
Since the original description by Taylor and Falconer in 1971, focal cortical dysplasia (FCD), the commonest malformation of cortical development characterised by a localised disruption of cerebral cortical architecture with or without the presence of dysmorphic neurons (balloon cells), is being increasingly recognised as a cause of intractable epilepsy in patients of all ages.1,2 Their clinical behaviour has hitherto been considered undeniably benign. We describe the case of a 61-year-old patient with a focal cortical dyplastic lesion who presented with intractable seizures and was subsequently found to have a glioblastoma multiforme within the lesion.
A 61-year-old woman presented to a regional neurosurgical unit with a recent onset of frequent left-sided focal motor seizures. Investigations had identified a lesion within her right temporal lobe which was biopsied and the histopathological features were reported to be consistent with a hamartoma, typical of those seen in tuberous sclerosis. However, she did not possess any of the characteristic cutaneous lesions seen in tuberous sclerosis. There was no family history of this condition and a genetic evaluation did not show either of the classical associated mutations. Her symptoms remained stable with the seizures partially controlled by phenytoin and carbamazepine. During this time, the patient moved to live in another region of the country. Two years later, she presented to our unit with an increase in the frequency of her seizures and a recent onset of leftsided weakness. On neurological examination, there was reduced power and mild hyperreflexia in the left arm and leg. Cranial nerve and fundal examination were normal. An
* Corresponding author. Present address: Department of Neurosurgery, Hope Hospital, 65 Vancouver Quay, Salford Quays, Salford, Manchester, Lancashire M503TU, UK. Tel./fax.: +44 161 8777633. E-mail address:
[email protected] (W.A. Thiryayi).
