Concomitant giant purely intradiploic cavernous haemangioma and multifocal neurotoxoplasmosis
- 1 Experimental Biomedicine and Clinical Neurosciences, School of Medicine, Postgraduate Residency Program in Neurological Surgery, Neurosurgical Clinic, University of Palermo, Palermo, Italy
- 2 Department of Neurosurgery, Garibaldi Hospital, Catania, Italy
- Correspondence to Dr Gianluca Scalia; gianluca.scalia@outlook.it
Abstract
Purely calvarial or intradiploic cavernous haemangiomas (PICHs) are rare benign tumours accounting for 0.2% of all bone tumours and 10% of benign skull tumours. They are generally small, slow-growing and asymptomatic lesions. Here the authors described an immunocompromised patient with concomitant giant intradiploic ossified globular cavernous angioma and multifocal neurotoxoplasmosis that underwent a combined approach to treat both lesions with an en-bloc resection of the right parietal intradiploic lesion and biopsy of the left occipital subcortical lesion.
Indeed, it is essential to exclude the presence of metastases by making a timely differential diagnosis. En-bloc surgical resection of purely intradiploic ossified cavernous angioma is the gold standard treatment and the prognosis after a complete excision is usually excellent with rarer recurrence rate.
Background
Purely calvarial or intradiploic cavernous haemangiomas (PICHs) are rare benign tumours accounting for 0.2% of all bone tumours and 10% of benign skull tumours.1 Parietal and frontal bones of the skull are common site of occurrence. They are generally small and remain as slow-growing and asymptomatic lesions. They may become symptomatic if they grow and become palpable with swelling over the scalp. In case of a ‘globular’ pattern, cavernous angiomas may present as space-occupying lesions.2 Differential diagnosis includes eosinophilic granulomas, aneurysmal bone cysts, dermoid and epidermoid tumours, metastases, meningiomas, sarcomas and Langerhans cell histiocytosis. Here the authors described an immunocompromised patient with concomitant giant intradiploic ossified globular cavernous angioma and multifocal neurotoxoplasmosis, focusing on differential diagnosis.
Case presentation
A male patient in his 40s affected by AIDS HIV-related and hepatitis B virus (HBV) infection since the last 5 years, was admitted to our department because he presented a medium-grade cognitive impairment with anxious-depressive status and intense headache. Neurologic examination showed mild left facio-brachio-crural weakness. He also presented an exophytic lesion at the right parietal scalp (figure 1).
Three-dimensional reconstruction of giant calvarial lesion at the right parietal scalp.
Investigations
He underwent a brain CT scan which showed a giant calvarial lesion (7×7×0.5 cm) with purely intradiploic development and greater exophytic development at the right parietal scalp (figure 2) and magnetic resonance which showed multifocal left occipital and frontal, right parietal postgadolinium ring enhanced intracerebral lesions, highly suspected for neurotoxoplasmosis (figure 3).
Preoperative axial (A), sagittal (B) and coronal (C) brain CT scan images showed a calvarial lesion (7×7×0.5 cm) with purely intradiploic development and greater exophytic development at the right parietal scalp.
Preoperative brain MRI axial (A, B), coronal (C) and sagittal (D) gadolinium T1-weighted images showing multifocal left occipital and frontal, right parietal postgadolinium ring-enhanced intracerebral lesions.
Treatment
The patient underwent a combined approach: right parietal craniectomy, en-bloc resection of the right parietal intradiploic lesion and cranioplasty with custom-made titanium mesh (figure 4); and left occipital craniotomy, biopsy and culture examination of the left occipital subcortical lesion. Histologic examination confirmed diagnosis of toxoplasmosis and documented a purely intradiploic ossified cavernous angioma (figure 5).
Intraoperative findings showed en-bloc resection of the right parietal intradiploic lesion.
Single-line diagram showed preoperative location of the extra-axial and intra-axial lesions, surgical approach and histological examination (realised by Gianluca Scalia).
Outcome and follow-up
The patient was discharged on the postoperative day 5 in good clinical status and addressed to our infectious diseases unit for medication therapy assessment and management. Three-month follow-up postoperative brain CT scan showed a complete removal of the right parietal scalp lesion with a good cosmesis and multifocal perilesional oedema reduction (figure 6).
Postoperative brain CT scan showed a complete removal of the right parietal scalp lesion with a good cosmesis of the one-step titanium mesh cranioplasty.
Discussion
Cavernous haemangioma can be divided into three histological groups: cavernous, capillary and mixed. They are composed by clusters of dilated blood vessels, separated by fibrous septa. Toynbee first described and reported in 1845 a cavernous angioma arising in the context of parietal bone. PICH affects predominantly patients between the fourth and fifth decades. These vascular lesions are supplied mainly by the external carotid artery, including branches like middle and superficial temporal arteries in the diploic space. Their aetiopathogenesis remains still unknown. An autosomal recessive inheritance was postulated, but trauma represents to date the most important cause. A total of 93 PICH cases were reported from 1845 to 2021. Regarding their location, frontal bone is the most common site (44.1%), followed by temporal bone and parietal bone (12.9%), occipital bone (11.8%) and skull base (5.4%).1 Clinically in almost all cases PICH presents as a solid swelling skull lesion, painful or painless. Some patients may also refer headache or dizziness. The gold standard to evaluate this kind of lesion is represented by CT scan. It allows to better characterise cortical and trabecular bone and, moreover, skull plates infiltration, that can present a ‘honeycomb’ or ‘jail bars’ aspect.2 3 MRI is also a feasible radiological examination and can show the amount of venous stasis. Therefore, a differential diagnosis is mandatory and can be just achieved through a histopathological examination. The differential diagnosis generally associated with intradiploic lesions are dermoid tumours, metastatic diseases, meningiomas, sarcomas, Langerhans cell histiocytosis, eosinophilic granulomas, fibrous dysplasia, giant cell tumours, aneurysmal bone cysts, osteomas and Paget diseases. En-bloc resection is the gold standard, replacing the bone defect with different methods (titanium mesh, methylmethacrylate cement or other types of cranioplasty).1 4
Neurotoxoplasmosis is a common infection reported in patients with AIDS. Nevertheless, clinical symptoms are represented by headache, seizures and haemiparesis, mimicking other neurological diseases (eg, metastases, brain abscess, tubercoloma) and requiring an appropriate differential diagnosis. The ‘concentric target sign’, according to Mahadevan et al, might be considered more specific than the ‘eccentric target sign’, commonly found in other brain lesions.5–11
The concentric target sign is characterised by a concentric alternating zone of hypointensity and hyperintensity on T2-weighted imaging typically found in neurotoxoplasmosis.
Despite that, as happened in some affected patients, toxoplasma antibody titre does not report a sufficient antibody response, necessitating a brain biopsy to obtain an accurate diagnosis.6 12 As in the present case, a biopsy was performed confirming the diagnosis of toxoplasmosis in order to start as soon as possible a target antibiotic therapy. Indeed, the management of a patient in a severe condition, especially with AIDS and HBV infections, must be achieved rapidly. Our case showed how a combined approach allows an appropriate and fast diagnosis, safeguarding patient’s life. In case of concomitant intracerebral lesions such as neurotoxoplasmosis in the case described, it is essential to exclude the presence of metastases by making a timely differential diagnosis. En-bloc surgical resection of purely intradiploic ossified cavernous angioma is the gold standard treatment and the prognosis after a complete excision is usually excellent with rarer recurrence rate.
Learning points
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In an immunocompromised patient with multiple cerebral localisation a timely differential diagnosis is mandatory.
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Purely calvarial or intradiploic cavernous haemangiomas usually present as a solid swelling skull lesion, painful or painless.
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Purely intradiploic ossified cavernous angioma should be always surgically treated.
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Timing is essential to safeguard immunocompromised patients’ life.
Ethics statements
Patient consent for publication
Footnotes
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Contributors Study conceptualisation was done by GS and RC. GS was responsible for methodology. Resources were obtained by GS and RC. Data curation was done by RC. Writing original draft preparation was done by RC and GS. Review and editing were done by GS and RC. Supervision was done by DGI and GN. All authors have read and agreed to the published version of the manuscript.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
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