Spinal capillary hemangiomas: Two cases reports and review of the literature.

Hemangiomas have rarely been found in the spinal cord. A few cases of spinal capillary hemangioma have been reported since 1987. The authors reported the two cases of capillary hemangioma including the tumor at conus medullaris and the another mimicked von Hippel-Lindau disease. A 15-year-old man was presented with coccydynia and left leg pain. A magnetic resonance imaging (MRI) revealed an intradural extramedullary enhancing mass at conus medullaris. Another case, a 31-year-old man was presented with a history of familial history of brain tumor, retinal hemangioma both eyes, multiple pancreatic cyst and syringobulbia with syringohydromyelia. On MRI, a well-circumscribed intramedullary nodule was detected at C5-6 level and multiple subpial nodule along cervicothoracic spinal cord. All patients underwent surgery, and the histological diagnosis confirmed capillary hemangioma. Although rare and indistinguishable from other tumors, capillary hemangioma should be in the differential diagnosis of the spinal cord tumor.

Introduction

Spinal capillary hemangioma is a rare tumor. A few cases have been reported in the literature. This report describes the clinicopathological findings in two cases of spinal capillary hemangiomas, including a capillary hemangioma at the conus medullaris in an adolescent and the another mimickied von Hippel-Lindau disease (VHL) disease which have never been reported before in a middle-aged adult.

Case Reports

Case 1

A 15-year-old boy presented a 4 month history of cocycodynia, associated with left leg pain, both of which had been progressively worsening. The previous medical history and family history were unremarkable, and an a neurological examination was normal. There were no motor or sensory deficits, the deep-tendon reflexes were normal, and a straight leg raising test was negative on both sides. Bowel and bladder functions and digital rectal examination were all normal. Various laboratory tests showed no abnormalities, but magnetic resonance imaging (MRI) showed a round 2 cm intradural extramedullary mass at the conus medullaris. On T1-weighted images, the signal of the lesion was isointense, and T2-weighted images revealed a hyperintense and homogeneous, strong enhancement on contrast-enhanced T1-weighted images. A spinal angiography with embolization demonstrated a hypervascular mass at the L1 level, just right at the tip of the conus medullaris, arising from an enlarged anterior spinal artery [Figure 1].

Figure 1

Preoperative imaging from case 1. (a) Sagittal view, (b) Coronal view, (c) Axial view, (d) Preoperative spinal angiogram

After embolization, a T12-L1 laminectomy with total tumor removal was performed with insignificant blood loss. The tumor was found emerging from the anterior spinal artery and attached to the nerve root. After the operation, the patient's pain decrease, but the postoperative course was complicated by urinary retention. 3 month following the operation the patient recovered his ability to urinate normally. Grossly, the surgical specimen was a lobular, sharp bordered, reddish mass, measuring 2.0 cm in maximum diameter. Microscopically, the capillary hemangioma featured a lobular architecture, with the lobules being composed of tightly packed capillary-sized vessels lined by a single layer of endothelial cells. Diagnosis was confirmed by immunohistochemical staining that positive for CD34 and factor 8 [Figure 2].

Figure 2

The characteristic histological features were found from case 1. (a) Grossly, the surgical specimen was a lobular, sharp-bordered, reddish mass, (b) The microphotograph shows many well-circumscribed lobules, (c) A high power magnification microphotograph showing the lobules pattern of vessels, separated by a myxoid, occasionally hyalinized stroma. Composed of tightly packed blood-filled, variably dilated, well-formed capillaries lined by endothelial cells (H and E, ×400)

Case 2

A 31-year-old man presented with a 1-month history of left hemiparesis, with aspiration pneumonia associated with dysphagia. He had retinal hemangioma in both eyes and a familial history of his first-degree relatives dying from brain tumors. A neurologic examination revealed left hemiparesis and impairment of cerebellar signs on the left side. A computerized tomography (CT) scan of his brain found a cystic lesion at the fourth ventricle. From the pertinent data, the differential diagnosis in this case was cerebellar hemangioblastoma, syringobulbia and an arachnoid cyst. An MRI brain showed syringobulbia with syringohydromyelia at the upper cervical cord, without Chiari malformation, and a CT scan of the whole abdomen showed multiple pancreatic cysts. An MRI of the whole spine that showed multiple innumerable enhanced nodules along cervicothoracic spinal cord with syringobulbia with syringohydromyelia involving almost the entire spinal cord from the T7 level extending superiorly to involving the brainstem. The intramedullary lesion at C5-6 level evidently the cause of syringobulbia with syringohyromyelia [Figure 3].

Figure 3

Preoperative imaging from case 2. (a) T2-weighted sagittal magnetic resonance imaging (MRI) images of the whole spine showing extensive syringobulbia with syringohydromyelia, (b) Postcontrast T1-weighted sagittal MRI images of the whole spine showing multiple subpial tumor along cervicothoracic spinal cord and an intramedullary tumor at the C5-6 level, (c) Computerized tomography abdomen revealed multiple pancreatic cysts (arrow), (d) MRI brain showing an enhancing nodules at the both globes

Following these investigations, our preoperative diagnosis was VHL. A laminectomy of C4-6 was performed. Intradural exploration revealed a reddish tumor, which was subpial tumor at C4. A posterior midline myelotomy was performed at the C5-6 level which found an intramedullary tumor which was removed completely. After the operation, the patient's dysphagia improved. A postoperative MRI of the brain and whole spine revealed decreased syringobulbia and syringohydromyelia. Histological microscopy showed circumscribed vascular tumor with fibrous connective tissue capsule. The vessels were small, and dilated capillary lined by homogeneous endothelial cell. The vessels separated by fibrous tissue without stromal cell. In this case, a definite diagnosis was considered carefully because the preoperative presentations indicated probable spinal hemangioblastoma with VHL disease. Immunohistochemistry showed positive reactions for the endothelial cell markers. histology and immunohistochemical staining confirmed the diagnosis of capillary hemangioma in this case [Figure 4].

Figure 4

The characteristic histological features were found from case 2. (a) Intraoperative findings showed a reddish subpial hemangioma which derived its blood from the radicular vessels, (b) Intraoperative findings showed a lobular, sharp bordered, reddish intramedullary hemangioma, (c) Histological finding showed the tumor composed of thin and irregular capillary-sized blood vessels separated by connective tissue (H and E ×100), (d) Staining for the endothelial marker CD34 disclosed a strong positivity of endothelial cells, compatible with capillary hemangioma (CD34 ×100)

Discussion

Capillary hemangioma is a benign vascular lesion, most often found in the skin and other soft tissues of children. Within the spine, this tumor is commonly found in the vertebral bodies[1] but only a few cases of intradural capillary hemangioma have been reported [Table 1].[234567891011121314151617181920212223242526272829303132333435] The tumor's characteristics are common in males, located at the thoraco-lumbar level, and clustered in the forth to sixth decades of life [Table 2]. Thus, based on case series of 64 patients, authors propose a classification scheme for the spinal capillary hemangioma classification into five types [Figure 5].

Table 1

Review of cases of spinal capillary hemangiomas previously reported in the English literature

Table 2

Clinical characteristics of spinal capillary hemagniomas

Figure 5

The classification of spinal capillary hemangiomas (a) Pediatric type, (b) Epidural type, (c) Intradural extramedullary type, (d) Intramedullary type, (e) Hemangiomatosis

Pediatric type has the cutaneous hemangioma following to the intradural extramedullary mass via sinus tract and usually form to the intradural extramedullary mass. This type is found in 5%

Epidural type is extradural mass that usually extend into neural foramen. This type is found 8% and 80% of this type are dumbbell shaped tumors

Intradural extramedullary type is found in 70% that is the most common type of spinal capillary hemangioma

Intramedullary type is found in 14%

Hemangiomatosis is found in 3%. Roncaroli et al. defined “hemagiomatosis” since 2000.[10] This type is disseminated tumor along entire spinal cord including subpial nodules and intramedullary mass.

The pathogenesis of this disease is still poorly understood. Several hypotheses have been proposed many factors starting hemangioma growth. Some of the key angiogenic factors are highly specific for endothelial cells. Basic fibroblast growth factor and vascular endothelial growth factor (VEGF) are proangiogenic molecules and are often produced by tumor cells.[36] Concurrently, VHL disease is an autosomal dominantly inherited neoplastic syndrome which is associated with various vascular tumors and cysts. The most frequent tumors are retinal and central nervous system hemangioblastomas, clear cell renal cell carcinoma, pheochromocytoma, pancreatic islet tumors, and endolymphatic sac tumors. This syndrome associates with mutation of the VHL gene. The Inactivation of the VHL tumor suppressor protein with loss of function of the VHL protein, and Elongin B, C complex high level of hypoxia-inducible factors causes increased transcription of VEGF, platelet-derived growth factor and transforming growth factor-α which is an important step in the development of highly vascular tumors.[37] The hypothesis of VHL disease that associated with capillary hemangioma is a high level of VEGF. This signal protein is an angiogenic factor with critical roles in tumor formation disseminated in multiple organs.

The surgical goal should be total complete tumor removal to decrease proangiogenic factors. Surgical standard procedures consists of a posterior midline approach to the spine, a one- or two-level laminectomy or laminectomy in children, and a midline durotomy. In an intramedullary tumor, a posterior midline myelotomy can avoid a major neurological deficit. This spinal tumor has multiple feeding arteries. The devascularization of the feeding arteries may be required to tumor resection in some situations. Perioperative embolization is an option to avoid massive surgical blood loss, especially in children.[3839] After total resection, tumor recurrence has rarely been reported.[32] However, Abe et al. reported no recurrence at 13 years after complete resection.[19]

Conclusion

We experienced a rare spinal cord tumor with a curious presentation. These lesions pose a challenging diagnostic problem, because variable types of this tumor can mimic other spinal tumors. For over 25 years, the tumor has been reported since 1987. It's important to concern in the differential diagnosis of spinal cord tumor, and surgical resection is the treatment of choice for a definite diagnosis.