Abstract
Objectives
The aim of this study was to present a rare case of a venous malformation that occupied the ethmoid and sphenoid sinuses. Prior to resection, it was believed to be a hemangioma.
Methods
This study includes a case report and review of the literature.
Conclusion
There is often confusion between “hemangiomas” and “vascular malformations,” but they are important to differentiate because they have unique approaches to treatment. Venous malformations in the paranasal sinuses are very rare. To our knowledge, this is the first case report that explicitly describes a venous malformation in the ethmoid and sphenoid sinuses. It was treated using endoscopic sinus surgery with intraoperative computer-assisted stereotactic navigation.
1
Introduction
Vascular malformations by definition are developmental errors in vascular morphogenesis that arise in utero. Those primarily affecting the venous vasculature, termed venous malformations , have an overall incidence of 1:10,000 and are the third most common type of benign vascular mass following hemangiomas and lymphatic malformations . Approximately 40% occur in the head and neck region where they develop most frequently in the lips, tongue, submandibular triangle, muscles of mastication, and upper aerodigestive tract . Venous malformations of the paranasal sinuses are very rare with only a few case reports to date. They consist of aberrant, often thin-walled, veins that demonstrate progressive ectasia over time and are lined by mitotically quiescent endothelial cells .
In contrast, hemangiomas represent a heterogeneous group of benign vascular tumors that arise by cellular hyperplasia . One of the most common types of hemangiomas is the widely recognized cellular tumor of infancy, currently termed infantile hemangioma , that arises in the immediate perinatal period and then slowly involutes spontaneously. Together, vascular malformations and vascular tumors comprise the overarching category of vascular anomalies . Unfortunately, the generic term hemangioma is historically and is still often used inappropriately to describe a variety of clinicopathologically distinct lesions, including both vascular malformations and vascular tumors. Because of this long-standing problem in nomenclature, many vascular lesions reported as “hemangiomas” are actually vascular malformations, not vascular tumors, making it difficult to make meaningful comparisons between reported cases and to guide appropriate therapy.
Greater than 50% of benign vascular anomalies as a whole are found within the head and neck, usually in the parotid gland, tongue, larynx, orbits, and cutaneous surfaces of the face and scalp . Vascular anomalies of the nasal cavity are uncommon, most of which arise from the nasal septum or vestibule and a minority originating from the lateral wall . They are even rarer in the paranasal sinuses . We present a rare case of a large venous malformation that occupied the ethmoid and sphenoid sinuses and was originally diagnosed both radiologically and pathologically as a hemangioma. This is the first case described in the English literature of a venous malformation in this area.
2
Case report
A 60-year-old male presented to an outside hospital with a large ethmoid sinus mass that was found incidentally on CT after sustaining facial trauma during an altercation. The mass had caused remodeling of the bilateral orbital walls and cribriform plate ( Fig. 1 ). This patient had a distant history of a malignant chondrosarcoma of a left lower rib in 1992. He was a 45 pack-year smoker and had 3–4 alcoholic beverages per night. At the time of the original discovery, he was asymptomatic. On follow-up MRI, the mass was measured to be 3.8 × 5.2 × 4.3 cm and displayed elevated T2 signal intensity, low T1 signal with patchy heterogeneous enhancement throughout, and scattered punctate foci of susceptibility artifact, or bright and dark areas with spatial distortion, that were interpreted as intratumoral hemorrhages or calcifications. The biopsied mass was described histologically as a proliferation of large, dilated, “cavernous” blood vessels within a fibrous background, and interpreted at that time as “hemangioma.”
The patient was then referred to the Department of Otolaryngology and Communication Sciences at Froedtert and The Medical College of Wisconsin seven months after the initial discovery. By this time, he was experiencing left sinus pain, moderate nasal airway obstruction, congestion, and olfactory dysfunction. Additionally, he noted left retro-orbital pressure and a decrease in the peripheral half of vision in his left eye. He denied headaches and epistaxis. Physical examination of the head and neck was unremarkable. The lesion was visualized using nasal endoscopy and it appeared to be entirely covered by mucosa with some superficial crusting.
Another MRI was ordered to assess for interval changes and to better characterize its relationship to surrounding structures for surgical planning ( Figs. 2, 3 ). Presence of a large soft tissue mass occupying and obstructing the bilateral ethmoid and sphenoid sinuses was once again demonstrated, but it had expanded into the upper portion of the nasal cavity as well. There was a suspected deformity of the medial aspect of the orbits bilaterally, but greater on the left with deformity of the medial rectus and superior oblique muscles. The posterior aspect of the mass abutted the cavernous portion of the internal carotid arteries. The cribriform plate was bowed superiorly with pronounced deformation of the left olfactory tract and sulcus. Finally, the mass appeared to compress the pituitary fossa and gland. The radiology report described it as possessing “heterogeneous, speckled enhancement consistent with a hemangioma.”
The patient was taken to the interventional radiology suite for preoperative diagnostic angiogram and embolization. However, the study revealed only minimal early venous shunting through dysplastic-appearing vessels along the medial orbital walls and no evidence of external carotid artery feeding vessels. Thus no embolization intervention was made at that time. The following day, the patient was brought to the operating room for endoscopic resection of the mass with intraoperative computer-assisted stereotactic navigation. After septectomy, the mass was well visualized in the nasal cavity and appeared to originate from the superior aspect of the sphenoid sinus. There was extensive bony and sinus remodeling throughout. It was debulked using a 4-handed approach with a coblation wand and grasping instruments. On histological examination, the mass was described as containing abundant fibrous tissue infiltrated by numerous dilated, thin-walled, erythrocyte-containing veins with scant smooth muscle and no mitotic activity ( Fig. 4 ). These findings, upon specialist review, yielded a definitive diagnosis of venous malformation.
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