Introduction

Hemangiomas are considered the most common benign tumor in children, usually involving the skin of the face and neck [11–13]. Intracranial involvement of hemangiomas is rare and usually appears along with cutaneous lesion [5, 13].

Most infantile and congenital hemangiomas seem to follow a benign course and spontaneously arrest or regress; however, some are at risk for developing complications and warrant treatment [8, 13]. Treatment options for hemangiomas include medical options, surgical options, or careful follow-up [5, 8]. Recent studies suggest the effectiveness of β-adrenergic receptor antagonists (β-blockers), such as propranolol, in treating infantile hemangiomas as well as other vascular lesions which express β2-adrenergic receptors [4, 7].

We report a case of a giant cranial hemangioma treated with propranolol with significant response.

Case

A 28-day-old baby girl was brought by her parents after they noticed significant growth of a lump on the back of her neck which was first noticed when she was a few days old.

Workup showed an uneventful pregnancy and uneventful term delivery. Physical exam demonstrated a large firm mass located at the left posterior suboccipital region. There were no cutaneous abnormalities over the mass. There were no cardiac, pulmonary, or GI-related abnormal findings. Neurological exam was unremarkable, without lethargy, focal neurological signs, or midline abnormalities. Neonatal reflexes were all intact, and fontanelle was soft.

Imaging showed an extra-axial lesion in the left posterior fossa. The lesion included extracranial and intracranial components, destroying the occipital bone in its course. The lesion was septated, with areas of calcifications and necrosis as well as heterogeneous enhancement. Owing to its large size, the lesion compressed the left cerebellar hemisphere and the fourth ventricle, without causing hydrocephalus (Fig. 1a, b).

Fig. 1
figure 1

a MRI at presentation. b CT at presentation

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The child underwent an open biopsy. At surgery, an extremely vascular lesion was found, with very few avascular regions; thus, only a small sample was obtained. Biopsy revealed the lesion to be a hemangioma. Immunostains for CD31 and CD34 markers were positive in the endothelial cells. S100 stain was negative, and there was no mitosis.

After the biopsy, treatment with propranolol was initiated (1 mg/kg twice per day). An MRI done 22 days after the biopsy showed a decrease in the impression of the tumor on the cerebellum, brain stem, and fourth ventricle (Fig. 2a). Medication was continued for the last year. An MRI done 1 year after the biopsy shows a significant reduction in the size of the hemangioma (Fig. 2b, c). The girl remained clinically intact throughout the follow-up.

Fig. 2
figure 2

T2 axial MRI images. a One month after biopsy and initiation of propranolol. b Six months after initiation of treatment. c One year after initiation of treatment

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Discussion

Hemangiomas in children are divided into congenital and infantile hemangiomas. Congenital hemangiomas, which are rare, are fully developed at birth and may be further divided into non-involuting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH) [11]. NICH does not involute after birth and grow in proportion to the child’s regular growth pattern, while RICH involutes shortly after birth [11]. Infantile hemangiomas, which are more common, present shortly after birth and begin growing after birth, reaching a steady phase and then involuting. The rate of each phase depends on the location of the lesion [11, 13].

Another way to differentiate between congenital and infantile hemangiomas is by using glucose transporter 1 (GLUT1), an erythrocyte-type glucose transporter, as a marker, since it is expressed in infantile but not in congenital hemangiomas [1, 10, 11]. The biopsy specimen in the current case was not tested for GLUT1 due to insufficient pathological specimen. However, based on the clinical course of the growing lesion in this case, one may assume that the tumor is of the infantile subtype.

Current management of intracranial hemangiomas in neonates consists of surgery and/or administration of corticosteroids, interferon-α, thalidomide, vincristine, β-blockers, or careful observation [7, 8, 13]. Surgical resection is usually performed to relieve mass effect and prevent adverse effects of compression of brain tissue [5, 8]. However, the hemorrhagic risk associated with resection of this highly vascular tumor, especially in a neonate, raises the need for medical alternatives.

In the current case, after pathological confirmation of a hemangioma, propranolol was administered as the first-line therapy. This resulted in fast shrinkage of the hemangioma. Note that while it is possible that the shrinkage was due to the hemangioma entering its involution phase, this is unlikely as natural shrinkage usually takes place at the age of a few years [13], while the hemangioma in our case responded within weeks.

Another group of lesions with rare cranial occurrence and similar external features are sarcomas [3, 9]. Microscopically, sarcomas and hemangiomas have a significant difference in appearance. While sarcomas are mainly composed of spindle cells [6], hemangiomas have lobular vascular proliferation and an appearance of microvessels [12]. Sarcomas tend to have more aggressive behavior and worse prognosis despite extensive resections and adjuvant therapy [2, 3, 6], and they do not respond to β-blockers.

Conclusion

Propranolol is a valid treatment for cranial hemangiomas, when there is no clinical urgency to warrant radical resection.