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Left: A photomicrograph demonstrating dense fibrous connective tissue with a spindle cell proliferation of venous vessels marked by thickened walls. Hematoxylin and eosin stain, original magnification ×100. Right: A Movat stain showed the venous nature of the back-to-back vessels in the specimen. Original magnification ×100.
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Although venous hemangiomas are one of the most common soft-tissue tumors, venous hemangiomas in the central nervous system are extremely rare. We present an unusual case of venous hemangioma originating from the interdural space of the tentorium. A 32-year-old woman was incidentally found to have extra-axial mass occupying the left ambient cistern...
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Hemangiomas are one of the common primary benign tumors of the intraosseous and soft tissue compartments in humans. Vertebral hemangiomas being the most common of all are seen in daily radiological practice. Hemangioma of the rib is rarely seen.
We reported on a case of a rib lesion which had a classic imaging pattern of hemangioma. We highlighted...
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... Since a venous malformation is histologically benign and usually well marginated, gross-total removal should be attempted during surgical exploration. 21,22,24,27,31 For patients with lesions growing around vital structures (such as the vascular tree, spinal cord, or within the pleural cavity), total resection may come at the risk of serious complications. 3 We resected the dorsal component of the venous malformation but did not attempt to resect the ventral component because retracting the spinal cord in this location carries a high risk of paralysis. An anterior approach had been ruled out by the general surgeons, who recommended sclerotherapy for the mediastinal component. ...
While spinal epidural arteriovenous malformations, fistulas, and shunts are well reported, the presence of a venous malformation in the spinal epidural space is a rare phenomenon. Herein, the authors report the clinical presentation, imaging findings, pathological features, and the outcome of surgical and percutaneous interventional management of a mediastinal and spinal epidural venous malformation in a young woman who presented clinically with neurogenic claudication from presumed venous hypertension precipitating the formation of a syrinx. The patient underwent a C6-T5 osteoplastic laminectomy for decompression of the spinal canal and subtotal resection of the epidural venous malformation, followed by percutaneous sclerotherapy of the mediastinal and residual anterior spinal venous malformation. She developed transient loss of dorsal column sensation, which returned to baseline within 3 weeks of the surgery. A 6-month postoperative MRI study revealed complete resolution of the syrinx and the mediastinal venous malformation. Twelve months after the surgery, the patient has had resolution of all neurological symptoms with the exception of her premorbid migraine headaches. A multidisciplinary approach with partial resection and the use of percutaneous sclerotherapy for the residual malformation can be used to successfully treat a complex venous malformation.
The International Society for the Study of Vascular Anomalies (ISSVA) has defined four vascular lesions in the central nervous system (CNS): arteriovenous malformations, cavernous angiomas (also known as cerebral cavernous malformations), venous malformations, and telangiectasias. From a retrospective central radiological and histopathological review of 202 CNS vascular lesions, we identified three cases of unclassified vascular lesions. Interestingly, they shared the same radiological and histopathological features evoking the cavernous subtype of angioleiomyomas described in the soft tissue. We grouped them together with four additional similar cases from our clinicopathological network and performed combined molecular analyses. In addition, cases were compared with a cohort of 5 soft tissue angioleiomyomas. Three out 6 CNS lesions presented the same p.Gly41Cys GJA4 mutation recently reported in hepatic hemangiomas and cutaneous venous malformations and found in 4/5 soft tissue angioleiomyomas of our cohort with available data. Most DNA methylation profiles were not classifiable using the CNS brain tumor (version 12.5), and sarcoma (version 12.2) classifiers. However, using unsupervised t-SNE analysis and hierarchical clustering analysis, 5 of the 6 lesions grouped together and formed a distinct epigenetic group, separated from the clusters of soft tissue angioleiomyomas, other vascular tumors, inflammatory myofibroblastic tumors and meningiomas. Our extensive literature review identified several cases similar to these lesions, with a wide variety of denominations. Based on radiological and histomolecular findings, we suggest the new terminology of “dural angioleiomyomas” (DALM) to designate these lesions characterized by a distinct DNA methylation pattern and frequent GJA4 mutations.
As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature, and it has not been previously reported in the pediatric age group.
In this paper, we report on a 3-year-old patient who presented with progressive weakness in his left upper limb. Spinal magnetic resonance imaging (MRI) revealed an epidural cystic mass at the C6-T2 level. The lesion was diagnosed as venous angioma after total removal with laminectomy. Postoperatively, the patient remained symptom free, and no tumor recurrence was confirmed based on MRI at the time of the 18-month follow-up. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all six reported cases were reviewed.
Venous angiomas should be included in the differential diagnosis of spinal cystic lesions in children. A definitive diagnosis is difficult based on MRI alone. This rare lesion is amenable to surgery, and gross total removal (GTR) is usually achievable due to a well-demarcated dissection plane. A good clinical outcome after GTR can be expected.
