A Rare Case of Sinonasal Cavernous Hemangioma Open Access

Hemangiomas are common, benign vascular neoplasms that can occur in nearly every tissue in the human body. Although these lesions are fairly common in the head and neck, they occur infrequently in the sinonasal cavity [1]. Hemangiomas are divided into capillary and cavernous types, depending on the dominant vessel size seen on microscopic examination [2, 3].

The majority of the sinonasal hemangiomas are of capillary variety, usually seen in childhood on the septum and vestibule, and the less common cavernous type, generally encountered in adults with a female preponderance, characteristically involves the lateral nasal wall [4]. These lesions are rare in the sinonasal tract, and those originating in the sinus mucosa are extremely rare [5].

The main clinical features are chronic rhinorrhea, nasal obstruction, facial pain, persistent epistaxis, and central facial deformity [6, 7]. 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 [7].

In imaging studies hemangiomas appear as nonhomogeneous masses [8], the imaging modalities of choice are computed tomography (CT) and MRI; however, none of these provide pathognomonic data for hemangiomas. They are especially of use to reveal the boundaries and the soft-tissue extension of the lesion [8].

Angiography is always warranted if there is any suspicion about the nature of these vascular tumors, not only because it can enable the correct diagnosis but also because, with the aid of transarterial embolization, undue hemorrhage during surgical intervention can be avoided [3]. Clinically and radiologically, they are difficult to diagnose, due to the scarcity of cases a comprehensive clinical picture is lacking, and the imaging result is often nonspecific, increasing the chances of misinterpretation with the more common sinonasal disorders [4]. Conservative surgical intervention is curative for cavernous hemangioma of the sinus cavity [1].

A 39-year-old male with 2 years history, left-sided nasal obstruction and a recent onset of left cheek fullness, presented to Emergency Department with epistaxis from left nostril. Patient used to have intermittent bleeding for the last few months, but recently became profuse so he had been to another facility, where he underwent anterior bilateral nasal packing for 24 h, but continuous oozing from left side brought the patient to our emergency. He had no history of trauma. No allergies and his past medical history were unremarkable.

On anterior rhinoscopy, the nasal cavity on the left side was obstructed by a large necrotic mass which bleeds on touch. To rule out malignancy, biopsy was taken from the mass, but it was inconclusive.

CT scan done showed left maxillary sinus mass occupying the whole left maxillary sinus, measuring about 7 × 5 × 6.5 infiltrating into the nasal cavity, ethmoid sinuses, and bilateral frontal sinuses associated with bony rarefaction and destruction. There is significant displacement of the nasal septum to the right side with significant thinning and rarefaction (shown in Fig. 1). Patient underwent combined endoscopic medial maxillectomy and external degloving approach and mass was excised in its entirety.

Intraoperative frozen-section pathology revealed a respiratory epithelium showing marked hemorrhage, necrosis, inflammation, and edema intermixed with proliferation of variable sized blood vessels, but final biopsy of the lesion came as cavernous hemangioma shown in Figures 2 and 3, and due to rarity of cavernous hemangioma presentation in paranasal sinus this case report was documented. Patient had smooth postoperative recovery and he was symptom free for 1 year without recurrence.

Hemangiomas of the head and neck are benign vascular lesions [2]. Although the face, scalp, orbit, and oral cavity are mostly affected in the head-neck region, involvement of the nasal cavity and paranasal sinuses is very rare [4].

Batsakis classified hemangiomas according to the predominant type of vasculature involved in the lesion and described them as capillary, cavernous, or mixed [9]. Capillary hemangioma is the more common type and is composed of capillary-sized vessels lined with flattened epithelium [2]. They are the most common benign vascular lesions observed in childhood and develop more often in females [7]. Cavernous hemangiomas are composed of large, endothelium-lined vascular spaces [2], and are more common in adults, also with a female preponderance [7].

Most nasal hemangiomas arise from the nasal septum or vestibule and are of capillary type [3, 10]. Only a few arise from the lateral wall of the nose, and these usually are cavernous. In the paranasal sinuses, hemangiomas are even rarer [3]. They are often asymptomatic or present as reddish, polypoid, or sessile mass causing nasal obstruction or recurrent epistaxis [1, 10].

Cavernous hemangioma of the maxillary sinus is rare [6]. It grows slowly and causes local destruction due to pressure effect. The main symptoms are recurrent epistaxis and nose obstruction, whereas gradual swelling of the face, sinus headaches, and loosing of the maxillary teeth may also be seen [8]. Other presenting symptoms could be rhinorrhea and bulging of the eye [4]. It has a slow course with a tendency for bone erosion due to its compressive effect ranging from simple erosion to complete destruction [6]. Dillon et al. [11] reported that capillary hemangiomas of the nose had benign appearing bone changes, consisting of remodeling or expansion. In our case, the mass caused infiltration into the nasal cavity, ethmoid sinuses, and bilateral frontal sinuses associated with bony rarefaction and destruction with significant displacement of the nasal septum to the right side and significant thinning and rarefaction. Kim et al. [3] reported two cases of cavernous hemangioma that caused erosion of the wall of the maxillary sinus, nasal turbinate, and orbit.

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 [7].

Histological evaluation is key to the definitive diagnosis, cavernous hemangiomas are composed of large, dilated vessels having sinusoidal blood-filled cavities arranged in lobular or diffuse pattern and lined by a single layer of flattened endothelium [4, 7]. In this case, features of dilated vessels, vascular channels lined by endothelial cells and blood-filled cavities as shown in Figures 2 and 3, were diagnostic for cavernous type of hemangioma. The distinction between the cavernous and the capillary types is not always well defined as the microscopic size of the predominant vessels can vary in different areas of the tumor [4]. The differential diagnosis of hemangioma includes inverted papilloma, pyogenic granuloma, mucocele, hematoma, polypoid cystic masses and bacillary angiomatosis, Kaposi’s sarcoma, and other vascular tumors of the paranasal sinuses, like angiosarcoma [6].

Contrast enhanced computerized tomography features of maxillary sinus hemangioma most commonly will be a highly vascularized, soft-tissue mass with variable enhancement, though there can be area of non-enhancement due to necrosis and hemorrhage within the mass. Hemangiomas often cause adjacent bony remodelling or expansion or erosion [5]. This feature may lead to an incorrect diagnosis of malignant tumor [7].

Cavernous hemangioma can present with intralesional calcification phleboliths [5] which 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 [7].

MRI demonstrates isointense lesion on T1-weighted sequences and hyperintense lesion on T2-weighted sequences, compatible with low-flow vascular structures [6]. Phleboliths show T I hypointensity and T2 hyperintensity on magnetic resonance imaging [1, 4]. In this case, MRI was not done and only CT scan was done. Weiss et al. [12] reported that amorphous or curvilinear calcification is a nonspecific finding, whereas phlebolith formation is a more specific finding of cavernous hemangioma.

In this case, CT scan (shown in Fig. 1) also showed a soft-tissue mass with medial displacement of the medial maxillary sinus wall showed that the mass was originating within the maxillary sinus, with calcification within the lesion and bone destruction which was considered as a sign of malignancy. Angiography and transarterial embolization for the management of hemangiomas are valuable because biopsy or surgical intervention can lead to sudden loss of large quantities of blood [3].

Kim et al. presented two cases where surgery did not result in severe bleeding. This is because hemangiomas are venous malformations supplied by small-to-medium sized vessels [3]. In this case, we also did not do preoperative embolization and the intraoperative bleeding was not severed.

In symptomatic patients, treatment is primarily surgical, ranging from complete excision to local resection sparing vital structures, the approaches could be open, endoscopic, or combined depending on the situation and extent of the lesion [4]. According to Pasquini et al. [13] endoscopy approach has been successful in excision of benign tumors such as hemangiomas and angiofibromas. In this case, combined approach was chosen in order to better visualize the interior of maxillary sinus, an endonasal endoscopic medial maxillectomy accompanied by an external degloving approach, enabled us to perform a complete resection of the tumor.

Sinonasal cavernous hemangioma is a rare tumor that can be mistaken for a locally aggressive neoplasm. Understanding the clinicopathological profile would help identify these benign neoplasms as one the chief differential diagnoses of sinonasal neoplastic mass that presents mostly in young patients with nasal obstruction and epistaxis. The CT scan is a useful diagnostic tool in evaluating the local tumor extension and the surgical procedure type to be chosen for the therapeutic management. Endoscopic sinus surgery alone or combined with external approaches are successful surgical techniques, which can be used in the management of these tumors.

We thank Dr. Badr Abdul Hamid from Histopathology department of Dubai Hospital for his efforts and cooperation.

This case report complying with the guidelines for human studies was conducted ethically in accordance with the World Medical Association Declaration of Helsinki. Ethical approval was not required for this case report in accordance with the Dubai Scientific Research Ethics Committee policies. Written informed consent for publication of the case report and any accompanied images was obtained from the patient.

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors.

Khulood Abashar Siddig: conceived of the content and wrote the manuscript, collect data, and get consent for publication; Omar Nazhat: reviewed and critically revised the manuscript, collect data, and get consent for publication; Iyad Said Hamadi: supervision, reviewed and revised the manuscript Abdelgalil Ali Ragab and Fatma AlBudoor: reviewed and revised the manuscript.

All data generated or analyzed during this case report are included in this article. They are available from the corresponding author upon reasonable request.