Abstract

Background: Primary intraosseous cavernous hemangiomas (PICH) of the skull are rare, benign, vascular tumors mainly found in adults’ calvarium. Affection of the clivus has been reported, with the most frequent clinical presentation being headaches followed by cranial nerve compromise. In the pediatric population, it has only been described once and was treated with surgery and radiation.

Case Description: A 14-year-old previously healthy female presented with episodes of right lingual fasciculations. The investigation of these signs with brain magnetic resonance imaging (MRI) revealed a large clival contrast-enhancing lesion. The patient underwent an endoscopic endonasal biopsy. The histologic findings were consistent with the diagnosis of cavernous hemangioma. She started treatment with propranolol, with serial MRIs showing lesion stability. The last follow-up MRI, 4 years after diagnosis, revealed a mild decrease in the volume of the lesion, and she remains asymptomatic.

Conclusion: We report the first clival PICH in a pediatric patient, managed without surgery and radiotherapy and treated with propranolol. We present neuro-imaging findings at diagnosis and during follow-up and a brief review of the literature on the topic.

Keywords: Clivus, Endoscopic endonasal biopsy, Intraosseous hemangioma, Propranolol

INTRODUCTION

Primary intraosseous cavernous hemangioma (PICH) of the skull is a rare, benign, vascular tumors mainly found in the cranial vault of adult patients. The affection of the skull base is exceedingly rare, with only seven reported cases of PICH affecting the clivus and only one in a pediatric patient. The management of this type of skull base tumor is not well established and differs significantly from those arising in the calvarium. We report a case of a clival PICH in a female adolescent confirmed by biopsy and treated with propranolol. It is the first reported case showing tumor control without surgery or radiotherapy.

CASE DESCRIPTION

History and imaging

A 14-year-old otherwise healthy female patient presented with 3 weeks of recurrent right lingual fasciculations. She described transient episodes triggered by head rotation movements and progressively worsening in duration and frequency.

Magnetic resonance imaging (MRI) of the brain revealed a large lesion of the clivus extending to the prepontine space and further to the right [ Figure 1 ]. The lesion was slightly hypointense with mottled high-intensity areas in T1-weighted imaging, heterogeneously hyperintense in T2-weighted imaging, and showed intense gadolinium enhancement. Computerized tomography (CT) showed an erosive lesion of the clivus, preserving the cortical bone.

Figure 1:

Neuro-imaging findings at diagnosis. (a) Axial bone Computerized Tomography (b) Brain magnetic resonance imaging (MRI): Axial T2 (c) Brain MRI: Axial T1 without contrast (d) Brain MRI: Axial T1 with Gadolinium (e) Brain MRI: Coronal T1 with Gadolinium (f) Brain MRI: Sagittal T1 with Gadolinium.

Based on the radiographic studies, the differential diagnosis included chordoma, chondrosarcoma, osteosarcoma, intraosseous meningioma, and hemangioma.

Operative details

After discussion in the pediatric oncology multidisciplinary tumor board, it was decided to perform a biopsy to obtain a histological diagnosis and plan the following treatment. The biopsy was performed through an endoscopic endonasal approach with intraoperative neuronavigation. A thirty-degree endoscope was employed to access the nasal cavity and to reach the sphenoid sinus through the left nostril. Multiple fragments of the clival lesion were collected. The mass was extremely hemorrhagic, leading to significant blood loss during the procedure. Bleeding was controlled with a hemostatic matrix and compression with cotonoides. Closure was performed with posterior absorbable nasal packing.

Surgery and the postoperative period underwent without complications. She was discharged on the 2^nd postoperative day, after performing a CT scan that ruled out complications.

During a reassessment in the Pediatric Neurosurgery and Ear, Nose, and Throat outpatient clinic, she did not present any surgical complications or neurological deficits.

Histopathology and postoperative details

Histological analysis showed multiple dilated thin-walled vessels lined by single-layer endothelial cells. The histopathological diagnosis was cavernous hemangioma [ Figure 2 ].

Figure 2:

Histopathology: Microphotographs with hematoxylineosin staining showing multiple dilated thin-walled vessels lined by single-layer endothelial cells (×100 augmentation).

The patient was rediscussed in the pediatric oncology multidisciplinary tumor board, and since the child was asymptomatic, treatment with propranolol was decided. She started treatment with propranolol 10 mg twice a day for a month, with good tolerance. After a month, the dose increased to 20 mg twice a day. She has maintained this therapeutic for 4 years without any adverse effects. The radiological surveillance was done initially with a brain MRI every 3 months. Once lesion stability was observed, follow-up MRIs were performed twice a year and then annually. The last MRI showed a small reduction in the tumor size with less compression of the brainstem [ Figure 3 ].

Figure 3:

Comparison of the brain magnetic resonance imaging at diagnosis and 4 years later (a) Brain magnetic resonance sagittal T1 with Gadolinium, May 2020 (b) Brain magnetic resonance sagittal T1 with Gadolinium, May 2024.

DISCUSSION

PICHs are rare, benign bone tumors most commonly found in the vertebral column. They represent 0.7% of all osseous neoplasms.[ 7 , 20 ] Less frequently, they are found in the skull, accounting for ~0.2% of all bone tumors and 10% of benign skull tumors,[ 26 , 31 ] and affect predominantly frontal and parietal bones.[ 13 ]

PICHs of the skull base are extremely rare tumors, and their management differs as, due to their deep location, they are associated with greater risks of neurovascular deficits, and total resection is difficult to achieve. To our knowledge, only seven clival PICH have been reported in the literature [ Table 1 ].[ 2 , 5 , 10 , 18 , 24 , 26 , 29 ] PICHs typically occur in women in their fourth and fifth decades of life.[ 13 ] Only one pediatric case of clival PICH has been reported in the literature, in an 11-year-old male treated surgically twice and with radiation after a second relapse.[ 2 ]

Table 1:

Clival PICH reported in the literature.

Clinical presentation

Cranial PICHs can be asymptomatic and diagnosed incidentally. When symptomatic, the clinical presentation depends on the location of the lesion. In the calvarium, they tend to manifest as a growing lump, sometimes associated with throbbing headaches. Less commonly, they can be found in craniomaxillofacial bones and present as painless facial swelling. When involving the orbital bones, patients can present local swelling, neuralgia, nasolacrimal obstruction, proptosis, blepharoptosis, diplopia, or even visual loss.[ 5 , 13 ]

Skull-based affection is very unusual. In 1921, Brandt reported the first case of a hemangioma arising from the petrous bone.[ 1 ] Beyond headaches, cranial neuropathies have been associated with skull base lesions [ 5 , 24 ], and there is one report of spontaneous epidural hemorrhage of a petrosal lesion.[ 8 ]

Neuro-imaging findings

The earliest description of radiographic characteristics of intraosseous hemangiomas was in 1930.[ 1 ] They are typically expansive lesions with a well-circumscribed area of bone rarefaction. The CT is the most helpful imaging technique, showing a sunburst pattern of trabeculations radiating from a common center or a honeycomb appearance. Hemangiomas usually respect the cortical bone layer, as opposed to radiological studies from bone malignancies that are more invasive.[ 13 ] MRI imaging is not specific, with heterogeneous signals in both T1- and T2-weighted sequences depending on the amount of venous flow and fatty transformation within the tumor.[ 5 , 21 , 26 ]

In our case, all these characteristics were present; they were not enough to make the diagnosis, that was why it was essential to confirm it with a biopsy.

Histology

PICHs have a vascular origin and can be divided into three types: cavernous, capillary, or mixed. In contrast to vertebral hemangiomas that are more frequently of the capillary type, most of the calvarial hemangiomas are cavernous. They arise from vessels of the diploic space and are supplied by the branches of the external carotid artery.[ 9 ]

Treatment

Indications for PICH surgical treatment include neurological compromise, recurrent headaches, mass effect, cosmetic deformity, or need for definitive diagnosis.[ 5 ] Most authors recommend “en bloc” resection when possible,[ 13 , 20 ] with a 1 cm wide margin resection of uninvolved bone to prevent recurrence.[ 7 ] Because of its vascular origin, hemorrhagic risk is substantial, so embolization before surgery has been described as helpful in preventing excessive bleeding.[ 14 ]

Nonetheless, surgical resection of a clival hemangioma can be particularly challenging and associated with higher morbidity due to their tendency to involve neurovascular structures during their growth.[ 24 ] In addition, the low incidence of PICH located deeply in the skull base, such as the clivus, makes it difficult to access and predict the clinical evolution of these tumors with or without surgery.[ 24 ]

Taking into consideration the risk of a surgical procedure, particularly bleeding, in an asymptomatic child, treatment with propranolol was decided. The patient has been on propranolol for 4 years following the same treatment protocol as for infantile hemangioma (IH). Propranolol has been used to treat IH for more than 15 years,[ 12 ] and it has been widely accepted as the first-line treatment with satisfactory results and fewer adverse effects.[ 22 ] Its regulatory roles in adipogenesis, angiogenesis, and endothelial cell apoptosis prevent growth and promote the reduction of hemangiomas.[ 3 , 6 , 11 , 23 , 30 , 33 ] Considering its safety profile,[ 15 ] it has been used for other indications such as hepatic hemangiomas and airway IHs.[ 16 , 17 , 34 ] Its use in PICH has been previously reported to manage back pain in children with vertebral hemangioma.[ 28 ] However, to our knowledge, this is the first report of a clival PICH treated with propranolol and exhibiting tumor reduction after 4 years of therapy. More studies are necessary to determine the role of propranolol in the treatment of PICH.

Despite sparse evidence in the literature, radiation has been described as an alternative or adjunct treatment to incompletely resected PICH to prevent tumor growth, but with no clear effect on tumor reduction.[ 20 , 25 , 32 ] However, there have been some concerns about the long-term side effects of radiation, including malignant transformation, secondary malignancy generation,[ 4 ] hypopituitarism, and cranial nerve deficit.[ 2 , 5 ] Considering that these tumors do not spontaneously involute[ 2 ] but usually grow slowly, some authors defend that it may be preferable to follow such lesions conservatively rather than subject a patient to radiation. In case of progression, fractionated stereotactic radiation therapy or radiosurgery may be considered.[ 13 , 19 , 27 ]

CONCLUSION

We report a case of a clival PICH in a pediatric patient. Even though the first treatment option regarding skull PICH is surgical resection, this case illustrates that in deep-sited lesions associated with high surgical risks, medical treatment with propranolol might be an option to stabilize the disease. It also opens the discussion about the use of propranolol for intraosseous hemangiomas, although further data will be needed.

Ethical approval:

The Institutional Review Board approval is not required.

Declaration of patient consent:

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship:

Nil.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology to assist in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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