Cranial imaging was performed to reveal any lesion causative of the facial nerve palsy. Findings of laboratory studies for blood cell count and coagulation were not remarkable. Facial nerve palsy of the peripheral type was suspected. On neurologic examination, facial asymmetry involving the unilateral forehead and cheek was noted, particularly when he was smiling. He was vaccinated as scheduled, did not undergo any surgery, and was not admitted for other diseases. He had no intrauterine disease or perinatal problem. The baby had been born to healthy parents at 40 weeks of gestation by normal delivery. We present clinical and radiologic findings of a rapidly growing Kaposiform hemangioendothelioma in the internal auditory canal.Ī 6-month-old male infant presented with facial asymmetry associated with left facial nerve palsy. 3 To our knowledge, there has been no report of Kaposiform hemangioendothelioma appearing within the cranial vault or in the skull base. ![]() 3 Kaposiform hemangioendothelioma is mostly located in the retroperitoneum and skin, but it can occur occasionally in the head and neck regions, mediastinum, and deeper soft tissues of trunk and extremities, whereas other hemangioendotheliomas mostly involve the distal extremities or skin. 2 Concerning the histopathologic classification and biologic behavior of hemangioendothelioma, Kaposiform, retiform, and composite types of hemangioendothelioma are known to be intermediate malignant tumors, and epithelioid hemangioendothelioma, to be a highly malignant tumor. 1 It is often associated with Kasabach-Merritt syndrome, but not always. It remains unclear whether a non-surgical therapy such as radiation therapy is an alternative treatment option for growing choristomas.Kaposiform hemangioendothelioma is a rare vascular tumor found predominantly in infancy and early childhood. Due to a lack of access for biopsy, lesions in the IAM are often only diagnosed after surgical removal. Therefore, choristomas are usually misdiagnosed as the more common VS initially. However, choristomas cannot be distinguished from other tumors in the IAM such as VS. Osteomas are hypointense in all sequences and non-enhancing after contrast. Lipomas can usually be recognized by their hyperintense signal in T 1-weighted MRI and can be confirmed by fat suppression techniques. Vascular tumors like hemangiomas tend to be more hyperintense in MRI. Meningiomas can be distinguished from other tumors by the presence of calcification, dural tail and bone infiltration. Schwannomas of the facial nerve may extend into the labyrinthine facial nerve canal, and this feature could distinguish them from other tumors. The latter are less common in MRI at this location and include astrocytomas, ependymomas, papillomas, hemangioblastomas and metastases. Tumors in the IAM can be classified into 2 groups depending on their location: there are extra-axial lesions (outside the brain parenchyma) such as VS, meningiomas, epidermoid cysts and paragangliomas, and rare tumors such as schwannomas of the 5th, 6th, 7th, 9th, 10th, 11th or 12th cranial nerve, and vascular lesions and there are intra-axial lesions (within the brain parenchyma). How can choristomas be differentiated from other tumors in the IAM? Contrast-enhanced MRI may be helpful. Two of the patients with hearing loss presented additionally with tinnitus, 1 patient reported additionally the loss of balance and another patient tinnitus and the loss of balance. All patients suffered from a hearing loss with the exception of 1 patient with hyperacusis and 2 additional patients in whom the hearing status was not reported. ![]() In 4 of 11 cases published in a complete clinical review, the tumors contained also skeletal muscle cells which were not found in our case. All contained smooth muscle cells as a major component, connective tissue and nerve fibers. The reports did not say whether the choristomas were removed due to growth or due to the symptoms they caused. We found only 14 reports of cases of choristomas of the 8th cranial nerve, which were all removed surgically. Smooth muscle tissue does not represent a component of cranial nerves and we suggest that the correct classification for this lesion is neuromuscular choristoma and not hamartoma. The tumor in our report consisted of smooth muscle tissue, nerve fibers and adipose tissue. Neuromuscular choristomas in the IAM are exceedingly rare lesions.
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