What is a spongiform hemangioma?

  Spongiform hemangioma is a spongy abnormal vascular mass composed of numerous thin-walled vessels. It is classified as an occult vascular malformation because the abnormal vascular mass is often not detected during angiography. In reality, the disease is not a true tumor, but a vascular malformation lacking an arterial component. With the development of medical imaging, reports of this disease are increasing. The incidence in the population is estimated to be 0.5%-0.7%, accounting for 8%-15% of all cerebrovascular malformations. The exact population incidence is still unknown because some patients are asymptomatic. The incidence of spongiform hemangioma is usually in the age of 30-40 years, and there is no significant gender difference.  In recent years, studies have demonstrated that cavernous hemangioma is an autosomal dominant disease with incomplete epiphenomenon, and the gene is located on the q11q22 of the long arm of chromosome 7q.  2, acquired theory that conventional radiotherapy, viral infection, trauma, surgery, vascular reaction after bleeding can induce cavernous hemangioma.  The main clinical manifestations of cavernous hemangioma are epilepsy (35.8%), intracranial hemorrhage (25.4%), neurological dysfunction (20.2%) and headache (6.4%), in that order. No clinical symptoms were observed in 12.1% of patients, and some patients had more than one clinical manifestation. If bleeding occurs in the lesion, it causes the corresponding clinical symptoms. In some cases, cavernous hemangiomas gradually increase in size, resulting in progressive worsening of neurological dysfunction due to the occupying effect. The clinical course is highly variable, and there can be acute or chronic neurological dysfunction, which can occur in remission or progressive exacerbation.  1. Epilepsy Epilepsy is the most common symptom of cavernous hemangioma, almost twice as common as cerebral arteriovenous malformation. To date, the incidence of refractory epilepsy is unknown, but Casazza et al. have shown in a large number of cases that 40% of patients with supratentorial cavernous hemangioma clinically present with refractory epilepsy. The incidence of epilepsy is higher in patients with lesions located in the temporal lobe, with calcification or severe iron-containing heme deposits, and in male patients. It is generally believed that seizures are associated with compression and irritation of the surrounding brain tissue by the lesion or hemorrhage or gliosis of the brain parenchyma.  Intracranial hemorrhage Almost all patients with cavernous hemangioma have subclinical microhemorrhages. Due to the small size and low pressure of the blood supply vessels, there are relatively few hemorrhages causing obvious clinical symptoms, accounting for 8-37%. Women, especially pregnant women, children, and patients with a history of previous bleeding have a relatively high rate of bleeding. Compared to cerebral arteriovenous malformations, bleeding in this disease is mostly minor and rarely life-threatening unless located in a critical functional area. Recovery after hemorrhage is generally good even with conservative treatment.  Focal neurological symptoms depend on the location and volume of the lesion, and hemorrhage within the lesion or in the periphery of the lesion can be detected by MRI. Because of the dense nuclei and conduction tracts in the brainstem, lesions located in these areas often have neurological dysfunction.  4. No clinical symptoms: 11-44% of all cases. Mild headache may be the only complaint, and the disease is often detected by this or by physical examination for imaging.  Spongiotic hemangioma is composed of three components: 1. a vascular component, consisting of sinusoidal cavities containing slow-flowing blood. 2. a connective tissue septum.  2, connective tissue septa.  3, Surrounded by glial hyperplasia surrounding the lesion.  CT CT generally shows a well-defined round or round-like equal to slightly dense shadow, which may be combined with speckled calcification, with no surrounding edema in general, and mild edema in larger lesions. Acute hemorrhage in spongiform hemangioma may show more uniform high-density shadowing with mild edema around the foci. 70%-94% of lesions may have mild to moderate enhancement after contrast injection, and the degree of enhancement is related to thrombosis and calcification within the lesion, typically showing inhomogeneous speckle-like enhancement. Circumferential enhancement is seen in lesions with a cystic component. The density of contrast enhancement can be increased by delaying the CT scan. The glial proliferation zone around the lesion is hypointense and perifocal edema is generally not apparent. If the lesion is small or isointense, it may be missed.  MRI MRI has a high diagnostic specificity and sensitivity for the diagnosis of cavernous hemangioma. The repeated small amount of hemorrhage in the nest and the fresh thrombus containing dilute, free ortho-hemoglobin make it high signal in all sequences, and the lesion is divided by bands of long T1 and short T2 signals forming popcorn or grid-like mixed signal clusters surrounded by low signal bands (especially T2 images) are typical MRI manifestations of intracerebral cavernous hemangioma.  Digital subtraction angiography (DSA) Spongiform hemangioma is an occult vascular malformation, which is difficult to detect even with digital subtraction technique.  V. Treatment Spongiform hemangioma is a benign disease. The pros and cons of therapeutic measures should be carefully weighed against the potential risks of the natural course of the disease before making treatment decisions. Surgical excision of the lesion is an option, and the following conditions may be suitable for gamma knife treatment: 1, those with a history of bleeding or epilepsy; 2, those with neurological deficits due to occupying effects; 3, those whose lesions are not suitable for excisional surgery; 4, those who refuse surgery and request gamma knife treatment.