A unique case of multiple calvarial hemangiomas with one large symplastic hemangioma

Abstract

Background Symplastic hemangioma is a benign superficial abnormal buildup of blood vessels, with morphological features which can mimic a pseudo malignancy. A few cases have been reported in the literature. We report here, a unique case of calvarial symplastic hemangioma, which is the first case in the calvarial region. Case presentation A 29-year-old male patient, with a left occipital calvarial mass since childhood, that gradually increased in size with age, was associated with recurrent epileptic fits controlled by Levetiracetam (Keppra), with no history of trauma. He presented to the emergency room with a recent headache, vomiting, frequent epileptic fits and a decrease in the level of consciousness 1 day prior to admission. A CT scan showed three diploic, expansile, variable sized lytic lesions with a sunburst appearance; two that were biparietal, and one that was left occipital, which were all suggestive of calvarial hemangiomas. However, the large intracranial soft tissue content, within the hemorrhage of the occipital lesion was concerning. The patient had refused surgery over the years; however, after the last severe presentation, he finally agreed to treatment. The two adjacent, left parietal and occipital lesions were treated satisfactorily using preoperative embolization, surgical resection, and cranioplasty. Histopathology revealed cavernous hemangiomas, in addition to symplastic hemangioma (pseudo malignancy features) on top at the occipital lesion. The right parietal lesion was not within the surgical field; therefore, it was left untouched for follow-up. Conclusions Histopathology and radiology examinations confirmed the diagnosis as symplastic hemangioma, on top of a pre-existing cavernous hemangioma. To the best of our knowledge, this is the first case of a calvarial symplastic hemangioma, which we report here.

Calvarial hemangiomas: Series of 6 cases and review of literature

“…Excluding the papers that deal with radiological approach to diagnosis of calvarial lesions, skull base haemangiomas, other lesions mimicking these like haemangioendothelioma, angiosarcomas, epithelial hyperplasia's, subgaleal haemangiomas, intraosseous venous malformations, aneurysmal bone cysts and those not in the English language we collected a total of 49 reports of 61 cases that are enumerated in Table 2 . 1 , 2 , 3 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 The classical radiological appearance of CHs consists of an expansile lytic lesion on computed tomography (CT) scans. 3 , 5 , 13 , 14 , 15 , 17 However, there are reports of finding sclerotic lesions mimicking an osteoma as well and also lesions that expand the diploic space with a central sclerosis resembling osteoblastoma.…”

“…1 , 2 , 3 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 The classical radiological appearance of CHs consists of an expansile lytic lesion on computed tomography (CT) scans. 3 , 5 , 13 , 14 , 15 , 17 However, there are reports of finding sclerotic lesions mimicking an osteoma as well and also lesions that expand the diploic space with a central sclerosis resembling osteoblastoma. 21 We found expansile lytic lesions in two of our 3 patients who had preoperative CT imaging done.…”

“… 3 , 4 , 5 , 51 Sunburst appearance of CHs have been reported by 2 , 3 , 4 , 17 , 18 , 19 , 27 , 28 , 33 , 36 , 39 , 41 , 42 , 44 authors on imaging either in x rays or CT scans. While several authors state that the inner table is usually not violated and that growth occurs by erosion of the outer table, 3 , 5 , 9 there are reports describing not only breech of the inner table of the skull [5] but also the dura 10 , 15 , 16 , 27 , 40 , 51 In 2 of our cases we encountered inner table violation on preoperative CT imaging and in one another on magnetic resonance imaging (MRI) but there was no intradural extension of the tumour in any of our cases. Calcification is found in CT scans within CHs 11 , 35 and 3 types of the same have been described 35 – a nonspecific amorphous variant, phleboliths (which are more common) and rarely metaplastic ossification.…”

“…Progressively increasing painless swelling with or without local tenderness or mild pain is the commonest presentation reported in literature too. 6 , 12 , 19 , 22 , 24 , 25 , 33 , 38 , 39 , 45 However, there are reports of patients presenting with features of raised intracranial pressure 7 , 15 , 27 , 42 , 51 as well as emotional disturbances, 14 , 20 , 40 ataxia, 27 diplopia and proptosis 2 , 13 and seizures. 11 , 15 In our series one patient had no symptoms related to the CH nor any history of progression of the lesion but after an initial period of conservative treatment for 2 years her relatives wished that the lesion be removed.…”

“…In 4 cases we used polymethylmethacrylate (PMMA) bone cement while in 2 cases titanium mesh fixed with screws was employed to cover the skull defect. Materials for CP mentioned in literature vary from split calvarial graft 32 , 39 , 44 to titanium mesh 15 , 16 and bone cement 36 , 37 , 44 Most authors describe resection and CP at the same sitting. 12 , 14 , 15 , 20 , 33 , 39 , 45 However, there are reports of staged cranioplasty too 11 , 21 , 42 usually due to torrential haemorrhage or doubt regarding the nature of the lesion.…”