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Cavernous angioma

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Incidence of Occurrence and Symptoms

Cavernous Angioma, also known as cerebral cavernous malformation (CCM), cavernous haemangioma, and cavernoma, is a vascular disorder of the central nervous system that may appear either sporadically or exhibit autosomal dominant inheritance. The incidence in the general population is between 0.1-0.5%, and clinical symptoms typically appear between 30 to 50 years of age. Once thought to be strictly congenital, these vascular lesions have been found to occur de novo.

This disease is characterized by grossly dilated blood vessels with a single layer of endothelium and an absence of neuronal tissue within the lesions. These thinly-walled vessels resemble sinusoidal cavities filled with stagnant blood. Blood vessels in patients with CCM can range from a few millimeters to several centimeters in diameter. CCM lesions commonly resemble raspberries in external structure.

Many patients live their whole life without knowing they have a cerebral cavernous malformation. Other patients can have severe symptoms like seizures, headaches, paralysis, bleeding in the brain (cerebral hemorrhage), and even death. The nature and severity of the symptoms depend on the lesion's location in the brain. Approximately 70% of these lesions occur in the supratentorial region of the brain; the remaining 30% occur in the infratentorial region.

Symptoms and Diagnosis

Clinical symptoms of this disease include recurrent headaches, focal neurological deficits, hemorrahagic stroke, and seizures, but CCM can also be asymptomatic. Diagnosis is most commonly made by magnetic resonance imaging MRI, but not all MRI exams are created equal. It's paramount that the patient request a gradient-echo MRI (aka T2-Flair) in order to unmask small or punctate lesions which may otherwise remain undetected. Sometimes quiescent CCMs can be revealed as incidental findings during MRI exams ordered for other reasons.

Sometimes the lesion appearance imaged by MRI remains inconclusive. Consequently neurosurgeons will order a cerebral angiogram or magnetic resonance angiogram (MRA). Since CCMs are low flow lesions (they are hooked into the venous side of the circulatory system), they will be angiographically occult (invisible). If a lesion is discernable via angiogram in the same location as in the MRI, then an arteriovenous malformation (AVM) becomes the primary concern.

CCMs & Venous Angiomas

Not infrequently a CCM is accompanied by a venous angioma, also known as a developmental venous anomaly (DVA). These lesions appear either as enhancing linear blood vessels or caput medusae--a radial orientation of small vessels that resemble the hair of Medusa from Greek Mythology. These lesions are thought to represent developmental anomalies of normal venous drainage. These lesions should not be removed, as reports of venous infarcts have been reported. When found in association with a CCM that needs resection, great care should be taken not to disrupt the angioma.

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Multi-organ cavernous hemangiomas: Case report
From Neurological Research, 1/1/03 by Erdogan, Bulent

Hemangiomas are rare benign tumors of vascular origin and multiple location of these tumors in different organs is extremely unusual. We report a case with multiple hemangiomas, characterized by calvarial, hepatic and suspicious costal involvement. Organ involvement and multiplicity of the neoplasm made our case very interesting and distinct. Our search of the English literature has revealed that no similar case has ever been published. [Neurol Res 2003; 25: 92-94]

Keywords: Cavernous hemangiomas; skull; multi-organ involvement

INTRODUCTION

Hemangiomas of the bone are rare vascular tumors, and account for only 0.7% of all osseous neoplasms1,2. Osseous hemangiomas are mostly detected in vertebral body or skull, and involvement of other bones is considerably rare. The most affected site is the thoracic spine3 and hemangiomas of the calvarial bones account for about 0.2% of all bone neoplasms2,3. In the skull, they are usually solitary lesions2,3,4, mostly affecting parietal and frontal bones, less frequently temporal and occipital . Their multiplicity in the calvarium is 4.7% in the Mayo Clinic review as reported by Peterson DL et al.2. Different system involvement with calvarial hemangiomas is extremely unusual.

CASE REPORT

A previously fit 40-year-old man presenting with headache and swelling on the right parietal area for nearly one year was admitted to our clinic. Medical and familial histories were noncontributory. He had no recent history of trauma or any chronic systemic disease. Local examination revealed a palpable and painful mass, approximately 3 x 2 cm in diameter, covered by normal skin in the right parietal region. There was no temperature differential or discoloration in the scalp. The remaining physical and neurological examination were normal. Routine laboratory studies yielded normal values. Plain skull X-ray films demonstrated a slight expansile lesion with radial sunburst pattern of radiolucencies in the right parietal bone. The lesion had nonsclerotic lobulated margins. Computed tomographic (CT) scan of the head, with and without intravenous contrast medium showed enhancing expansile mass invading the diploic space. Bone window images revealed bony matrix within the tumor (Figure 1). Both inner and outer tables were eroded. Additionally, there were multiple millimetric lesions in the right and left parietal bone, the largest measuring 2-3 mm. Lesions were isointense and hyperintense respectively on T1- and T2-weighted MR images. Enhancement of the matrix was intense after the intravenous administration of contrast medium. There were similar signal intensities on the other lesions. Abdominal ultrasonography and CT scanning showed a 62 x 58 mm in diameter hemangioma at the right hepatic lobe with peripheral enhancement (Figure 2). Whole body bone scintigraphic scans with ^sup 99m^technetium methylenediphosphonate (^sup 99m^Tc-MDP) showed an increased uptake in the right parietal region and 9th costa (Figure 3). Although these features together with the bone scintigraphic images suggested a metastatic lesion, radiating sunburst pattern on X-ray and CT were typical for cavernous hemangioma.

In the surgery, the mass with irregular-nodular surface was removed with a healthy margin in an en-bloc resection using a high-speed drill and followed by an alloplastic methyl metacrylate cranioplasty. On gross examination, outer and inner tables of the affected bone were eroded. The underlying dura appeared to be intact.

Microscopic examination revealed flattened endothelium-lined, large caliber vascular spaces filled with blood cells. Focal infiltration into bone tissue was also noted. Histopathologically the tumor was reported as cavernous hemangioma. The post-operative course was uneventful, and the patient was discharged on the 7th day post-surgery.

DISCUSSION

Calvarial hemangiomas are rare lesions and it is difficult to diagnose them pre-operatively. These tumors tend to grow slowly over many years with vague symptoms1,2,3,4. Cavernous hemangiomas of skull are commonly observed between the second and fourth decades, although they may be seen at any age2,5. Most series indicate a preponderance of women, being approximately in the ratio of three to two1,2,6. This neoplasm may affect any skull bones, frontal and parietal being the most preferential sites2,3. Other sites of occurrence include the orbita7, intrasellar region8, middle fossa-sphenoid bone9, petrous bone, cavernous sinus10 and Meckel's cave11.

Cavernous hemangiomas are rarely congenital or familial12,13,14. Although most of the cavernous hemangiomas are unifocal, congenital or familial forms may display multiplicity and/or multiple system involvement12,13,15.

Hepatic cavernous hemangioma is the most common benign liver tumor and its prevalence is in the range of 0.5% to 7.0%16. They are usually asymptomatic and can be found coincidentally by abdominal imaging techniques.

Ribs are an unusual location for the cavernous hemangioma, however the pre-operative diagnosis of the chest wall tumors by imaging techniques is very difficult17. In our case, costal involvement was suspicious and required no treatment.

Metastatic lesions of the skull originate mostly from lung, breast or prostate, and have some difficulties in the differential diagnosis18. In these cases, radiological findings may not always be useful in providing the basis of the most effective treatment strategy. Conventional radiographs and CT may be useful if they show the classical findings of bone destruction on the skull lesions. On the other hand, radiological aspects and the clinical manifestation of the skull lesions may not always be definitive. However, CT or X-ray findings such as sunburst appearance may be highly suggestive but not always be diagnostic of a cavernous hemangioma1,7. Primary bone tumors (osteoma, osteosarcoma chondrosarcoma etc.), epidermoid and dermoid tumors, aneurysmal bone cysts and metastasis should be considered in the differential diagnosis of calvarial lesions.

We recognized that the radionucleotide bone images are frequently sensitive but not specific, for these lesions cause the increased uptake of the bone imaging agent, and do not tell us the nature of the lesion.

Reviewing the literature, we noticed that there was no consensus on the terminology of similar multiple hemangiomas affecting different systems. In these complex situations, the terms 'diffuse hemangiomatosis'19, 'benign hemangiomatosis'20 or 'multiple hemangiomatosis'13,21 are used in the literature. But in our opinion, the coexistence of calvarial hemangiomas, especially when they are multifocal, and accompanied with similar vascular lesions in the other organs would be better named as 'multi-organ' or 'multi-system' hemangiomas.

CONCLUSION

Clinical and radiological pictures of the skull lesions may be confusing. Cavernous hemangiomas, especially when they involve different organs, may be misinterpreted as an ominous disease. The true incidence of this occurrence is actually not known, since the vast majority of patients with cavernous hemangiomas of the skull do not have systemic evaluations performed. Thus, the patients with cavernous hemangiomas should be evaluated systematically. In these situations, considering the complex radiological and clinical criteria, serious measures should be taken for the differential diagnosis and suitable treatment of the skull lesions. Cavernous hemangioma should be included in the differential diagnosis of calvarial lesions. Although there were classic radiological findings of hemangioma, multifocal and multiorgan involvement were very interesting and distinct in our case.

REFERENCES

1 Khanam H, Lipper MH, Wolff CL, Lopes MBS. Calvarial hemangiomas: Report of two cases and review of the literature. Surg Neurol 2001; 55: 63-67

2 Peterson DL, Murk SE, Story JL. Multifocal cavernous hemangioma of the skull: Report of a case and review of the literature. Neurosurgery 1992; 30: 778-782

3 Corr P. Multiple calvarial haemangiomas. Australian Radiology 2000; 44: 118-120

4 Kumar NA, Ranganadham P, Bhaskar G, Chowdhury AR, Kumar NA. Multiple calvarial haemangiomas: Case report and review of the literature. Neuroradiology 1996; 38: 83-85

5 Yoshida D, Sugisaki Y, Shimura T, Teramoto A. Cavernous hemangioma of the skull in a neonate. Child's Nerv Syst 1999; 15: 351-353

6 Bastug D, Ortiz O, Schochet SS. Hemangiomas in the calvaria: Imaging findings. AJR 1995; 164: 683-687

7 Sweet C, Silbergleit R, Mehta B. Primary intraosseous hemangioma of the orbit: CT and MR appearance. AJNR 1997; 18: 379-381

8 Cobbs CS, Wilson CB. Intrasellar cavernous hemangioma. Case report. J Neurosurg 2001; 94: 520-522

9 Dickins JR. Cavernous hemangioma of the sphenoid wing. Arch Otolaryngol 1978; 104: 58-60

10 Sepehrnia A, Tatasiba M, Brandis A, Samii M, Parwitz RH. Cavernous angioma of the cavernous sinus: Case report. Neurosurgery 1990; 27: 151-155

11 Fehlings MG, Tucker WS. Cavernous haemangioma of Meckel's cave. J Neurosurg 1998; 68: 645-647

12 Fobe JL, de Lima JB, de Buone ML, Coreea Neto J. Familial cavernous angioma. Report in 3 generations. Arq Neuropsiquiatr 1996; 54: 665-660

13 Smit LM, Barth PG, Stam FC, Valk J. Congenital multiple hemangiomatosis with brain involvement. Childs Brain 1981; 8: 461-467

14 Tateno A, Matsui A, Sakuragawa N, Nonaka I, Arima M. Two siblings with multiple intracranial hemangimatosis with calcification. J Neurol 1985; 232: 112-114

15 Russell DS, Rubinstein LJ. Pathology of Tumors of the Nervous System, edn 5, London: E. Arnold, 1989: pp. 730-736

16 Isselbacher KJ, Dienstag JL. Tumors of the liver. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, eds. Harrison's Principles of the Internal Medicine, Vol. 2, edn 13, New York: McGraw-Hill, 1994: pp. 1495-1498

17 Clement RH, Turnage RB, Tindal EC. Hemangioma of the rib: A rare diagnosis. Am Surg 1998; 64: 1027-1029

18 Constans JP, Donzelli R. Surgical features of cranial metastasis. Surg Neurol 1981; 15: 35-38

19 Langner C, Thonhofer R, Hegenbarth K, Trauner M. Diffuse hemangiomatosis of the liver and spleen in an adult. Pathologe 2001; 22: 424-428 (Ger)

20 Dyall-Smith D, Cowen P. Benign neonatal hemangiomatosis. Int J Dermatol 1992; 31: 336-338

21 Roncaroli F, Scheithauer BW, Deen HG Jr. Multiple hemangiomas (hemangiomatosis) of the cauda, equina and spinal cord. Case report. J Neurosurg Spine 2000; 92: 229-232

Bulent Erdogan, Orhan Sen, Volkan M. Aydin, Tulin Yildirim*, Sema Bircan[dagger] and Nur Altinors

Department of Neurosurgery, * Department of Radiology, [dagger] Department of Pathology, Baskent University, Adana, Turkey

Correspondence and reprint requests to: Bulent Erdogan, MD, Baskent University, Medical School, Department of Neurosurgery, Dadaloglu mah. 39.Sok. No:6 Yuregir, Adana, Turkey. [uberdogan@hotmail.com] Accepted for publication September 2002.

Copyright Forefront Publishing Group Jan 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

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