Ear, Nose & Throat Journal2023, Vol. 102(2) 117–120© The Author(s) 2021Article reuse guidelines:sagepub.com/journals-permissionsDOI: 10.1177/0145561321993597journals.sagepub.com/home/ear
Lobular capillary hemangioma (LCH) is a benign capillary proliferation with a microscopically distinctive lobular architecture, composed of small to medium-sized vessels of capillary type. Rarely, it originates from the mucosa of the nasal cavity, most frequently from the anterior nasal septum, turbinates, and nasal vestibule. Etiology is unclear, but previous investigations suggest that this lesion is associated with injury, hormonal factors, some viral infections, and therapy with some drugs. We present a case of LCH arising from the medial wall of the maxillary sinus in a 15-year-old boy, which was completely excised by preoperative embolization and endoscopic sinus surgery. To our knowledge, this is the second reported case of an LCH originating from the maxillary antrum. Etiology, pathogenesis, diagnosis, and treatment modalities of LCH were also discussed.
Keywords
endoscopy, epistaxis, hemangioma, maxillary sinus, nasal surgical procedures
Lobular capillary hemangioma (LCH) is a benign vascular proliferation, usually originating from the skin and mucosa of the oral cavity. Rarely, it originates from the mucosa of the nasal cavity, most frequently from the anterior nasal septum, followed by turbinates and nasal vestibule, as well as from the nasopharynx.1-4 It was previously known as ‘‘pyogenic granuloma,’’ which is a misnomer because it is not an infectious or granulomatous lesion.3,4 Typical clinical associations are male predilection (82%) when it arises in those under 18 years of age and pregnancy, skewing the demographics to a female predilection (86%) in the reproductive years.5 Etiology of LCH is unknown, but previous studies suggest that this lesion is associated with injury, hormonal factors (pregnancy and contraceptive use), viral infection, and therapy with some drugs (vemurafenib). The nasal obstruction and bleeding episodes are the usual symptoms.1-6
According to the available literature, in almost all cases of hemangioma arising from the maxillary sinus, the excised mass had histological characteristics of cavernous hemangioma (CH), not LCH.2,3,5,6 By literature review, we found only one reported case of patient with LCH arising from the maxillary sinus in a 37-year-old Japanese woman.7 Here, we present one more case of maxillary sinus LCH in a 15-year-old boy.
A 15-year-old male patient, referred from other institution, had presented in our ENT outpatient department with a 6-month history of right-sided nasal obstruction, mucopurulent and bloody nasal discharge, recurrent nasal bleeding, and cheek pain and pressure. Neurological and ophthalmological examinations revealed no abnormalities and the results of routine laboratory analyses were normal. Nasal endoscopy revealed a large reddish mass filling the entire left nasal cavity. A contrast-enhanced computed tomography (CT) scan revealed an intensely enhancing mass in the right maxillary sinus which extended into the left nasal cavity and appeared to be destroying its medial wall, measuring 31 × 18 × 19 mm (Figure 1A and B). A magnetic resonance imaging (MRI) showed a gadolinium-enhancing mass of heterogeneous density arising from the right maxillary sinus and extending to the nasal cavity through the ostiomeatal complex.
We decided to perform a tumor biopsy. The results of histopathological analysis were consistent with a benign vascular lesion. In order to avoid serious nasal bleeding, preoperative digital subtraction angiography and embolization of the sphenopalatine artery were performed. Following embolization, the lesion’s size markedly decreased, and it was found to originate from the medial wall of the maxillary sinus. The mass was excised completely under general anesthesia by endoscopic approach without serious bleeding. On macroscopic examination, the removed lesion comprised a reddish to brown colored smooth surface mass, with dimensions of 25 × 10 × 10 mm (Figure 2). Histopathological examination showed the vascular proliferation consisted of small to medium-sized capillary vessels with a microscopically distinctive lobular architecture, covered with transitional epithelium (Figure 3A). The proliferation is composed of either cellular-packed endothelial and spindled cells with minimal vascular lumen or more dilated typical capillary spaces with intraluminal thrombi (Figure 3B). Histopathological results were consistent with LCH. On follow-up after 6 years, the patient was asymptomatic without any sign of recurrence.
Hemangiomas of the sinonasal mucosa are much rarer than cutaneous hemangiomas. According to the results of a retrospective study presented by Kim and Kwon,5 sinonasal hemangiomas composed 12.2% of all head and neck hemangiomas. There are 2 subtypes of hemangioma, determined histologically according to dominant microscopic vessels. Lobular capillary hemangioma is composed of capillary sized vessels lined with flattened endothelium separated by a collagen stroma. Cavernous hemangioma is composed of large endothelium-lined vascular spaces.1-3 Regarding the age of patients, LCH predominates in children, but adult hemangiomas are more likely to be the CH.3 Tumor origin differed according to histologic type. Lobular capillary hemangioma occurred more commonly at the nasal septum and inferior turbinate, whereas CH more often affects the maxillary sinus and middle turbinate.5 The exact reason for that distribution is not known, but previous retrospective studies showed that CH is more likely to be found on the lateral nasal wall, excluding inferior turbinate.2,3,5 Only 2 cases of LCH, including our patient, have been presented to originate from the maxillary sinus. In a previous report by Kanazawa et al.7 Lobular capillary hemangioma originated from the posterior wall of the left maxillary sinus, and in our 15-year-old boy, the lesion originated from the medial wall of the right maxillary sinus.
The mechanisms for the development of LCH is still unknown. Trauma, hormonal influences, viral oncogenes, underlying microscopic arteriovenous malformations, and the overproduction of angiogenic growth factors have been postulated to play a role in the pathogenesis. Among the traumatic factors, habitual nasal picking and nasal packing are considered the most significant.1-3,5 The mucosal trauma could be explanation for the LCH in the anterior segment of the nasal cavity, but not for this lesion in the posterior nasal fossa and, especially, in the maxillary sinus. Increased levels of estrogen and progesterone have been indicated as the hallmark in the pathogenesis of a specific form of LCH of the nasal cavity in the pregnant women, called ‘‘pregnancy tumor.’’1-3 Therefore, a higher incidence of LCH has been linked to the use of oral contraceptives with greater progestinic activity.1-3 The role of viral oncogenes in the pathogenesis of LCH in unclear and study by Kirdar et al8 was the first demonstration of the positivity of human papillomavirus (HPV) infection in a patient with LCH. In previous studies, it has been revealed that some viruses and bacteria cause damage to the endothelium of bloodvessels.9,10 This finding suggests that the association of HPV infection and LCH should be investigated in many cases of patients with this lesion in the future.
Diagnostic procedures for LCH include detailed history and physical examination as well as imaging studies such as CT, MRI, and angiography.1-4 Diagnostic confirmation is achieved through histopathological examination.1-7 Preoperative biopsy is recommended in larger tumors, but was rarely undertaken, probably due to the fact that office-based biopsies for highly vascular tumors may trigger uncontrolled nasal bleeding.1-3 Preoperative angiography and embolization are not always necessary because the main vessel is an LCH is usually small.1-3 However, some reports have suggested that embolization of the feeder artery should be performed before the excision of larger lesions.3,7 In such cases, preoperative embolization can decrease the tumor size and decrease the risk of severe nasal bleeding.3,7 In our case, we did not obtain detailed data on the histological nature of the lesion on the basis of preoperative biopsy, so we decided to apply the angiography and embolization. In differential diagnosis, angiofibroma and hemangiopericytoma are the most important lesions that should be kept in mind.11,12 A dense fibrous stroma with spindled or stellate cells and variably sized, thin-walled vascular spaces are main histological characteristics of angiofibroma. In contrast to angiofibroma, LCH shows the lobulation and not-excessive stromal fibrosis. Also, anastomosing vessel architecture, that is very specific for LCH, is not found in angiofibroma.11 Immunohistochemical analysis may be very helpful for differentiation between LCH and sinonasal-type hemangiopericytoma.12 Sinonasal-type hemangiopericytoma is a rare vascular tumor, arising from pericytes surrounding capillaries, situated usually in the nasal cavity and paranasal sinuses region, originating in the most cases from nasal septal mucosa. An immunohistochemical analysis of this lesion shows negative staining for CD34.12 On the other hand, cells from LCH react with CD31, CD34, and factor VIII-related antigen.11 The other lesions that should be considered in the differential diagnosis are venous hemangioma, glomus tumor and malignant nasal cavity, and paranasal sinuses tumors.
Endoscopic excision of LCH is a safe and effective substitute for conventional open approach surgery. Corticosteroids and propranolol have been introduced as treatment for nasal hemangiomas without major side effects.13 However, the principal treatment for LCH is complete surgical removal with bleeding control. The recurrence rate of LCH can be as high 16% due to incomplete excision.13 The spontaneous regression of LCH is also possible. Some LCH in pregnant women regress after delivery. It is proposed that reduced level of vascular endothelial growth factor (VEGF) in blood may play important role in the spontaneous regression of LCH.12,13 The concentrations of VEGF in circulation rapidly decrease after delivery, leading to the apoptosis of endothelial cells and spontaneous regression of the tumor.12,13 However, Lee reported an unusual case of a spontaneous regression of a large nasal LCH in a middle-aged male patient, although it was incompletely excised.13
The patient’s permission was obtained.
This investigation was conducted as a part of scientific project of the Military Medical Academy Faculty of Medicine, Belgrade, Serbia (MFVMA02/19-21/).
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Aleksandar Perić https://orcid.org/0000-0002-8453-7272
1 Department of Otorhinolaryngology, Military Medical Academy, Faculty of Medicine, University of Defence, Belgrade, Serbia
2 University of Montenegro Faculty of Medicine, Podgorica, Montenegro
3 Army of Serbia, Serbia
4 Institute for Pathology, Military Medical Academy Faculty of Medicine, University of Defence, Belgrade, Serbia
Received: December 30, 2020; revised: January 13, 2021; accepted: January 20, 2021
Corresponding Author:
Aleksandar Perić, MD, PhD, Military Medical Academy, Faculty of Medicine, University of Defence, Department of Otorhinolaryngology, Crnotravska 17, 11000 Belgrade, Serbia.
Email: aleksandarperic1971@gmail.com
