B-ENT, 2017, 13, 73-78

Mucosal cavernous hemangioma of the maxillary sinus: a series of 3 cases

N. Terlinden1, T. Van Becelaere1, C. De Dorlodot1, M.C. Nollevaux2 and Ph. Eloy1

1ENT Department, CHU UCL Namur, Site de Godinne, Yvoir, Belgium; 2Department of Histopathology, CHU UCL Namur, Site de Godinne, Yvoir, Belgium

Key-words. Non-osseous (mucosal) cavernous hemangioma, sinonasal tract, maxillary sinus, case series, endonasal surgery

Abstract. Mucosal cavernous hemangioma of the maxillary sinus: a series of 3 cases. Introduction: Soft-tissue hemangiomas are benign vascular tumors that are common in the head and neck region and rare in the sinonasal tract. Those originating in the sinus mucosa are extremely rare. We report three cases of non-osseous (mucosal) cavernous hemangioma (CH) originating in the maxillary sinus and successfully managed endonasally and endoscopically.Patients and methods: All patients were referred to the ENT outpatient department for a persistent unilateral pansinusitis that was resistant to broad-spectrum antibiotics for one year. All patients complained of unilateral persistent nasal obstruction, and one presented with nosebleeds. The sinus CT scan revealed complete opacification of the maxillary sinus extending into the ethmoid sinus. MR images depicted a heterogeneous signal on both T1- and T2-weighted sequences. In one case, the tumor was highly vascularized and required a preoperative selective arterial embolization. Complete resection via an endonasal endoscopic medial maxillectomy was performed successfully in all cases without severe intraoperative bleeding. The pathologist confirmed the diagnosis of CH.Conclusion: CH is rare in the sinonasal tract but must be considered in the differential diagnosis of benign and malignant sinonasal tumors in adults. An endonasal endoscopic medial maxillectomy enabled complete tumor removal with optimal control of its extensions and vascular supply. This approach is associated with less morbidity than an open approach. When nosebleeds are present, injection of a contrast agent and a preoperative arterial embolization are recommended.

Introduction

Hemangiomas are blood-containing lesions classified by the International Society for the Study of Vascular Anomalies as benign vascular tumors.1,2 They result from active endothelial cell proliferation and are clearly separated from vascular malformations, which are inborn defects in vascular morphogenesis. Hemangiomas can originate in the skin, mucosa, and deep structures such as bones, muscles, and glands.3,4

The two major types of hemangiomas, capillary and cavernous hemangiomas (CHs),5 are categorized according to the predominant vessel size on microscopy. Capillary hemangiomas, also called infantile hemangiomas,6,7 arise from small-diameter vascular channels, and over 80% occur in the head and neck region. They are the most common benign vascular lesions observed in childhood and develop more often in females. CHs are composed of large vascular spaces with abnormal media3-7 and are more common in adults, also with a female preponderance. These lesions are rare in the sinonasal tract, and those originating in the sinus mucosa are extremely rare.7-22 Here we report three cases of mucosal CHs of the maxillary

sinus successfully operated endoscopically and endonasally.

Case reports

All patients agreed to the publication of their medical history.

Case 1

The first case involved a 78-year-old man who had complained for more than one year of unilateral nasal obstruction, unilateral mucoid rhinorrhea, and right epiphora. He had no pain or nosebleeds and no relevant medical history. The nasal endoscopy showed bulging of the lateral wall of the nasal fossa, with some brown polypoid tissue extruding from the middle meatus. A sinus CT scan showed a complete opacity of the right maxillary and anterior ethmoid sinuses (Figure 1a). There was also an erosion of the medial wall of the maxillary sinus. MR imaging demonstrated necrotic areas with a heterogeneous background, isointense in T1 and hyperintense in T2 sequences, enhancing after gadolinium injection (Figure 1b).A medial maxillectomy was performed, and the tumor was completely resected in one piece. The

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microscopic examination revealed large vascular spaces filled with blood, surrounded by an endothelium with an irregular media (Figure 1c) consistent with the CH diagnosis. At one year of follow-up, the patient was free of disease (Figure 1d).

Case 2

In the second case, a 45-year-old woman was referred to the ENT department for management

of a huge mass involving the right maxillary and ethmoid sinuses. She had been complaining of right nasal obstruction and nosebleeds, and her past medical history was uneventful. The nasal endoscopy showed friable necrotic tissue in the middle meatus, with bleeding on contact. A sinus CT scan with contrast injection showed a high vascularization of the mass (Figure 2a), and MR images depicted the same characteristics as in the first case (Figure 2b). Pre-operative angiography

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DFigure 1

(a) Coronal CT, bone window: non-contrast CT scan, coronal cut; bone window showing the mass occupying the right maxillary sinus and involving the ipsilateral nasal cavity with widening of the ostiomeatal complex in the nasal cavity. (b) Coronal MR T1-weighted image with gadolinium showing a heterogeneous signal intensity mass (low T1, high T2 centrally) centered in the right maxillary sinus with peripheral enhancement suggesting thick corrugated mucosa. (c) Histology: photomicrograph of the tumor showing large dilated vessels with sinusoidal blood-filled intercommunicating cavities lined by a single layer of flattened endothelium (hematoxylin–eosin, 100×). (d) Coronal CT: postoperative view showing no residual tumor.

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Mucosal cavernous hemangioma of the maxillary sinus: a series of 3 cases 75

involving his right maxillary and ethmoid sinuses (Figure 3a–c). The patient reported having unilateral nasal obstruction and unilateral rhinorrhea for more than 6 months. The nasal endoscopy showed a friable reddish tissue in the right nasal cavity. The CT scan revealed a large expanding process involving the maxillary and ethmoid sinuses, and the MRI showed heterogeneous T1 and T2 signals. An endonasal endoscopic medial maxillectomy was performed, and subsequent histology confirmed the diagnosis of CH. The postoperative course was marked by the presence of MRSA. The patient remained tumor free per post-operative CT at 9 months (Figure 3d).

and selective arterial embolization of the right internal maxillary artery were done. An endonasal endoscopic medial maxillectomy was performed, with preservation of the inferior turbinate and without significant intraoperative bleeding. The definitive histological examination of the surgical specimen confirmed the diagnosis of CH (Figure 2c). A CT scan performed 13 months after the surgery showed no tumor recurrence (Figure 2d).

Case 3

The third patient was a 67-year-old man referred to our hospital for the management of a huge tumor

Figure 2

(a) Axial soft tissue CT scan with contrast showing the mass occupying the right maxillary sinus extending into the nasal cavity. (b) Coronal MR image in T2 sequences delineating the extension of the tumor. (c) Histology compatible with CH with histologic features similar to the first case. (d) Coronal CT: postoperative view demonstrating the absence of residual disease.

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Discussion

The mean age at presentation of CH is about 40 years. Although there is a female preponderance in CH in the head and neck region, this is not the case for CH arising in the sinonasal tract.8-10 In our small series, there was a male preponderance. Complaints of cavernous sinonasal hemangiomas are nonspecific.9-16 The main clinical features are chronic rhinorrhea, nasal obstruction, facial pain, persistent epistaxis, and central facial deformity. Our patients presented with unilateral nasal obstruction and chronic rhinorrhea. In one case, nosebleeds were noticed. Those tumors grow slowly and tend

to be locally destructive, secondary to a pressure effect. Nasal endoscopy may show a firm, painless, reddish, polypoid, or sessile mass extruding from the middle meatus and occluding the nasal cavity. This mass bleeds on touch or is covered with a few blood clots. Imaging is essential.4,17-22 On CT, the mucosal cavernous hemangioma presents as a highly vascularized, well-demarcated soft tissue mass that emerges from or grows into the maxillary sinus in most cases. As it grows, it can extend into the nasal cavity, ethmoid air cells, or sphenoid sinus, causing bulging of the medial wall of the maxilla, widening

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DFigure 3

(a) Non-contrast coronal CT, bone window: huge tumor located in the maxillary sinus and extending into the ethmoid sinus and the ipsilateral nasal cavity. (b) Coronal post-contrast T2-weighted MR imaging revealing a heterogeneous signal in the maxillary sinus (CH) and a high T2 signal in the ethmoid corresponding to the CH and retained secretion. (c) Histological pictures compatible with CH: photomicrograph of the tumor showing large dilated vessels with sinusoidal blood-filled intercommunicating cavities lined by a single layer of flattened endothelium. (d) Postoperative CT, coronal plane: absence of recurrence of the CH but persistence of hyperplasia of the mucosa of the maxillary sinus due to MRSA infection.

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of the ostiomeatal complex, involvement of the adjacent structures like the ethmoid and sphenoid sinus, and displacement of the nasal septum. The underlying bone can be remodeled or destroyed by the long-standing pressure of the expanding mass. These bony changes range from simple erosion to complete destruction. This feature may lead to an incorrect diagnosis of malignant tumor.4 The osseous type of hemangioma remains confined within the bony trabeculae. Injection of contrast agent shows a non-homogeneous mass with moderate and partial contrast enhancement; several enhancing portions are present, but so are large unenhanced areas, reflecting necrosis, fibrosis, vascular proliferation, and bleeding within the tumor. Phleboliths are specific findings, resulting from dystrophic calcifications following intravascular thrombosis. Calcifications, trabeculation, and/or radiation from a central point with an oval radiolucent area may be seen but may be more suggestive of intraosseous hemangioma.5,17,21,22

MR imaging with contrast administration shows isointense lesions on T1-weighted images and hyper intense on T2, with an overall heterogeneous background compatible with low-flow vascular structures. Angiography might be useful to delineate the vascular supply and for pre-operative embolization of the tumor. However, we report only one case with high vascularization requiring preoperative arterial embolization. The lesion appeared hypervascularized with dilated feeding arteries and draining veins, but presented with regular contours and pooling of the contrast agent in the lesion. A vascular blush was observed in late films. Differential diagnoses of sinonasal cavernous hemangiomas include long-standing sinonasal polyps, mucoceles, inverted papillomas, polypoid cystic masses, angiosarcoma, other vascular lesions, neuroma, neuroblastoma, or lymphoma. If there is associated bone destruction, a malignant tumor must be ruled out.3,16 Histological evaluation is key to the definitive diagnosis: CHs are composed of large dilated vessels with sinusoidal blood-filled intercommunicating cavities arranged in a lobular or diffuse pattern and lined by a single layer of flattened endothelium. The media of the endothelium has an irregular thickness; the vessels are separated by fibrous stroma, and respiratory epithelium is seen in the periphery.1-3,7,13,14

Surgical excision of the tumor is the gold standard treatment. The approaches can be open, endoscopic, or combined depending on the situation and extent of the lesion. An external approach, such as lateral rhinotomy or the Caldwell Luc procedure, has been reported in the literature. However, an endonasal approach is another viable alternative. In our cases, an endonasal endoscopic medial maxillectomy enabled us to perform a complete resection of the tumor with a wide exposure of the lesion and an optimal control of its feeding afferences. In our series, one patient required a preoperative arterial embolization, but no blood transfusion was necessary. Concerning follow-up, there is no definitive consensus in the literature. By comparison with other benign but aggressive tumors such as inverted papilloma or juvenile angiofibroma, we recommend four consultations during the first postoperative year followed by an annual or biannual endoscopic control during the next 4 years. Depending on the endoscopic findings and patient complaints, imaging can be ordered after the first- or second-year follow-up. The recurrence rate after an endonasal or even an open approach is not published in the literature. The reason is likely that most cases are published as case reports associated with complete tumor resection and successful outcome. Partial resection with persistence of the tumor is, of course, associated with recurrence.

Conclusion

CHs are true endothelial vascular tumors but are rare in the sinonasal tract. They must be considered in the differential diagnosis of any benign or malignant tumor of the paranasal sinus cavities. Imaging (CT and MR) is mandatory for diagnosis. Complaints are nonspecific, so the correct diagnosis is usually made late. Moreover, bone destruction is easily misinterpreted as a sign of malignancy. Therefore, it is important for the radiologist and primary clinician to consider this entity in the diagnostic work-up of sinonasal masses. A high index of suspicion is required in the setting of chronic nasal obstruction and repeated epistaxis. Complete surgical excision is the gold standard. An endonasal endoscopic medial maxillectomy is a viable alternative to an open approach with less morbidity and quicker rehabilitation. Preoperative embolization may be considered in some selective cases.

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14. Mussak E, Lin J, Prasad M. Cavernous hemangioma of the maxillary sinus with bone erosion. Ear Nose Throat. 2007;86(9):565-566.

15. Raboso E, Rosell A, Plaza G, Martinez-Vidal A. Haemangioma of the maxillary sinus. J Laryngol Otol. 1997; 111(7):638-640.

16. Dutta M, Kundu S, Barik S, Banerjee S, Mukhopadhyay S. Mucosal cavernous hemangioma of the maxillary sinus. Arch Iran Med. 2015;18(2):130-132.

17. Kim HJ, Kim JH, Kim JH, Hwang EG. Bone erosion caused by sinonasal cavernous hemangioma: CT findings in two patients. AJNR Am J Neuroradiol. 1995;16(5):1176-1178.

18. Dillon WP, Som PM, Rosenau W. Hemangioma of the nasal vault:MR and CT features. Radiology. 1991;180(3):761-765.

19. Itoh K, Nishimura K, Togashi K, Fujisawa I, Nakano Y, Itoh H, Torizuka K. MR imaging of cavernous hemangioma of the face and neck. J Comput Assist Tomogr. 1986;10(5):831-835.

20. Kanter WR, Brown WC, Noe JM. Nasal bone hemangiomas: a review of clinical, radiologic and operative exerience. Plast Reconstr Surg. 1985;76(5):774-776.

21. Bakhos D, Lescanne E, Legeais M, Beutter P, Morinière S. Cavernous hemangioma of the nasal cavity [in French]. Ann Otolaryngol Chir Cervicofac. 2008;125(2):94-97.

22. Jung WS, Yoo CY, Park Y-J, Ihn YK. Hemangioma of the Maxillary Sinus presenting as a mass: CT and MR features. Iran J Radiol. 2015;12(2):e6923.

Prof. Eloy PhilippeENT departmentCHU UCL Namur, Site de Godinne,Thérasse, 15530, YvoirBelgiumE-mail: philippe.eloy@uclouvain.beFax: 003281423703Tel.: 003281423705

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