Intracranial Infantile Hemangioma: Highlighting a Rare Presentation With a Case Report and Literature Review

Infantile hemangioma is a common benign vascular tumor in children, but it is very unusual to be found intracranially. Our literature review identified 44 reported cases. Presentation can vary from asymptomatic to a life-threatening presentation that necessitates urgent surgical removal. There is no general consensus on management of these rare lesions and until recently, treatment was limited to surgery or pharmacological management with steroids, propranolol or interferon. We present a case of a four-week-old male infant with history of vomiting and increase in head circumference since birth. MRI of the brain revealed a large complex cyst occupying the right frontoparietal region, with round soft tissue component that is isointense on T1 and hyperintense on T2 weighted images. Complete surgical resection with evacuation of the cyst was achieved. Histopathology of the mass showed infantile hemangioma with positive CD31 on immunohistochemistry. The patient achieved an excellent outcome following surgical resection.


Introduction
Infantile hemangioma is the most commonly encountered benign vascular tumor of childhood [1].They typically tend to occur in superficial layers of the body more than deep layers.About 83% of these lesions affect the head and neck areas, but other parts of the body can be involved [2].Intracranial involvement is very rare with incidence of 0.1%, especially when it is isolated from other organ involvement, with only scattered reports in literature [1,2].Most documented cases were located in the cerebellopontine angle, with fewer reports describing other intracranial locations.
Hemangiomas have the capacity of rapid growth secondary to the rapid proliferative phase in the first 12 months of life [3].The first five months of life are considered the peak for hemangioma growth, at which 80% of the final size has often reached, reaching the maximal size by nine months [3,4].This period is followed by a gradual involutional phase, in which 90% of hemangiomas have spontaneously involuted by the age of five years [3,4].Treatment is not required for hemangiomas in general.However, intracranial hemangiomas require intervention as the rapid growth may result in severe neurological complications.Different modalities were used to treat intracranial infantile hemangiomas, though literature is lacking solid information on standard treatment [4].
We performed a literature review using Google Scholar and Pubmed searching for keywords "intracranial infantile hemangioma/s", and identified 44 reported cases [5][6][7].Cases with asymptomatic small hemangiomas were managed with watchful waiting.Most of the cases were treated medically with oral prednisolone, oral propranolol, and intralesional triamcinolone, interferon alpha or thalidomide.However, only nine cases were treated surgically [5][6][7].The efficacy of these measures was variable, with significant side effects noted in some cases [5][6][7].additional complaints.Full physical exam showed a head circumference above the 95th percentile, asymmetric facial appearance with a bulge of the right frontoparietal area, and wide bulging anterior fontanel.No skin lesions were present or other associated anomalies.A more extensive neuroimaging modality was requested.Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain revealed a large 9.7 X 6.6 X 5.7 cm complex cyst occupying the right frontoparietal region, with a small round soft tissue component around 1 cm in diameter.It is isointense on T1 and hyperintense on T2 weighted images.Minimal micro bleed is identified within the septation (Figures 1, 2).Based on the images, the provisional diagnosis was a desmoplastic infantile ganglioglioma.
Management plan was conducted based on symptoms, huge mass size, significant compression of brain structures and resection of a suspicious brain tumor.Decompression of the intracranial tumor via osteoplastic craniotomy was done.Right fronto-parietal scalp incision and Mayo scissors were used to create the bone flap as the skull had been thinned out by the prolonged applied pressure.50cc of brownish fluid was aspirated and the complete resection of the solid component was performed.Frozen section of the cystic wall was sent and showed normal brain parenchyma.Histopathology confirmed the diagnosis of infantile hemangioma with positive CD31 on immunohistochemistry (Figure 3).The patient's vomiting ceased and he was stable postoperatively.He was discharged from the hospital within the first week after surgery.Upon clinic follow-up the head circumference was markedly reduced and he was doing well without any recurrent or new signs or symptoms.

Discussion
On patient admission in the described case, we considered a desmoplastic infantile ganglioglioma or a pilocytic astrocytoma as possible etiologies.The isolated intracranial involvement in our patient has precluded the possibility of infantile hemangioma.The occurrence in the frontoparietal region in our case is the first to be reported.
To our knowledge, there are only 44 cases published in the literature (Table 1) and our case presented here is the 45th.Most of the cases were females (66.7%).The age at presentation ranges from 0-36 months with a mean of 3.7 months.The presenting symptoms were variable from case to case.In the analysis of the 36 cases done by Kang et al. in 2016, it was found that the cerebellopontine angle had been the most common location of involvement.Other locations included the internal auditory canal, middle and temporal cranial fossa and fourth ventricle.Their association with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACES) was found in nine of the cases [8].Oral prednisolone was the most used pharmacological intervention in treatment and the mainstay of steroid therapy for this condition [5,9].In 2008, Léauté-Labrèze et al. described the efficacy of oral propranolol in the management of infantile hemangioma as it is also well tolerated by the patients [5,10].Surgical intervention is necessary in the presence of raised intracranial pressure or preoperative diagnosis is not confirmed and a more serious process is suspected [11].Surgical resection is associated with high risk of bleeding as the vascular nature of the hemangioma which can accompanied by high morbidity and mortality [11].In our case, surgical resection was done with minimal blood loss of 70cc without any complications; the patient's presenting symptoms markedly improved after surgery.
Surgery has been performed as a treatment for these benign lesions in 10 reported cases as their rapid continuous growth had caused neurological complications.None of these cases had PHACES or additional hemangiomas.Others were managed pharmacologically with steroids, interferon therapy and thalidomide and some were watchfully observed.

Conclusions
Infantile hemangioma is a benign disease entity that is separate from cavernous hemangioma and hemangioblastoma, and it should be considered in the differential diagnosis of patients coming with intracranial lesion during infancy.Our report represents a very rare case of an isolated infantile hemangioma which presented in a rare location.We did an extensive literature review to review the previously reported cases and update the current knowledge regarding recent modalities in the management of such cases.Surgical resection is associated with high morbidity and pharmacological intervention should be considered first if possible with the exception of the conditions that necessitate urgent surgical intervention as the raised intracranial pressure from the compressive effect of the hemangioma or rapid neurological deterioration.

Additional Information
Author Contributions

FIGURE 1 :
FIGURE 1: (A) axial T1-weighted MRI (B) sagittal T1-weighted MRI demonstrate a large intraparenchymal cystic mass with low signal intensity, located in the right frontoparietal region of the right cerebral hemisphere.

FIGURE 3 :
FIGURE 3: (A) The nodule in the cyst is formed by vascular neoplasm (arrow).The cyst wall (arrow's head) is formed by reactive glial tissue.(B) The neoplasm is formed by cellular, and back-to-back capillary-size vascular spaces.Plumb endothelial cells with no atypia line these spaces.Haematoxylin & Eosin stain.

FIGURE 4 :
FIGURE 4: Postoperative T1-weighted MRI brain Coronal (A) Axial (B) Sagittal (C) views shows significant decompression of the large right cerebral cystic mass with almost no mass effects identified in the current MRI examination and no midline shift with normal configuration of the supratentorial and infratentorial ventricular system.Resection of the previously visualized well-defined rounded solid mass with no residual identified in the current MRI examination.Multiple hemorrhagic spots, pneumocephalus and mild subdural fluid are identified related to operative interference.