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Multifocal osteolytic lesions of the skull: a primary cavernous hemangioma mimicking a neoplastic invasive lesion.

ABSTRACT: Intraosseous cavernous hemangioma is a rare cause of osteolytic lesions of the skull, and its multifocal type is even more infrequent. This tumor is difficult to accurately diagnose by imaging and can be confused with osteolytic Langerhan's cell histiocytosis or other neoplasms. Here we present a case of multifocal intraosseous cavernous hemangioma of the skull treated with surgical intervention in our hospital five years ago. A review of related literatures and case reports is also provided to help clarify the diagnosis and devise treatment regimens. In light of the difficulties of early diagnosis, early en bloc surgical removal is recommended.

SUBMITTER: Hsiao IH

PROVIDER: S-EPMC4502045 | biostudies-other | 2015 Jun

REPOSITORIES: biostudies-other

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Project description:BackgroundCavernous hemangiomas, more accurately defined as cavernous venous malformations, constitute the most common primary intraorbital tumors of adults comprising 4-9% of all tumors,[4] and the second most frequent cause of unilateral proptosis after thyroid-related orbitopathy.[3] Over 80% are located within the intraconal compartment, most commonly in the lateral aspect.[1] Surgical treatment for orbital cavernous hemangioma is generally required in symptomatic cases, optic nerve compression, and cosmetically disfiguring proptosis.[2] Transcranial approaches, the most familiar approaches for neurosurgeons, provide wide access to the entire superior and lateral orbit. They usually offer direct visualization, allowing for a safer dissection, while minimizing significant injury to the native neural and vascular anatomy of the orbit.[5] Although transcranial approaches continue to evolve, in many cases, they have been supplanted by endoscopic skull base approaches and modifications to deep lateral orbitotomy approaches.[5].Case descriptionA 62-year-old male patient presented with slowly expanding left proptosis, which he had first noticed 3 years before presentation. He was already blind in his right eye due to a history of traumatic amaurosis in childhood. The left eye examination revealed severe proptosis with restricted eye movement in all directions and significant visual impairment (visual acuity of 20/300, expressed by Snellen test, with no improvement on correction). MRI of the orbit showed a large left superolateral intraconal cavernous hemangioma compressing and displacing the optic nerve, with the typical feature of slow gradual irregular enhancement with delayed washout on contrast-enhanced image. A one-piece modified orbitozygomatic approach was performed and a total en block resection was achieved. The bone flap was fixed with titanium miniplates and screws, the temporal muscle and the skin were closed in a standard fashion. The patient did not present any new deficit in the postoperative period. The patient had good functional and cosmetic outcomes with resolution of proptosis, restoration of eye movements, and improvement of visual acuity in the 3-month follow-up. Postoperative MRI showed total resection.ConclusionThe orbitozygomatic approach for large orbital cavernous hemangioma provides satisfactory orbital decompression and large working space, reduces traction, and increases visualization and freedom to dissect small vessels and nerves that may be tightly attached to the tumor pseudocapsule.

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Multifocal osteolytic lesions of the skull: a primary cavernous hemangioma mimicking a neoplastic invasive lesion.

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