LCH is a benign acquired vascular tumor that affects mainly the skin or the mucous membranes of the mouth and oropharynx. However, involvement of the nasal cavity occurs on rare occasions. The anterior part of the nasal septum is the commonest site involved in this lesion in comparison with other nasal parts. The exact pathogenesis of the LCH has not yet been recognized. However, there are four main mechanisms for the involvement of this rare tumor, including nasal trauma (surgical or accidental), hormonal changes such as pregnancy or contraceptive pills, viral oncogenes, and arteriovenous malformations [3, 6]. The LCH is still obscure to otolaryngologists regarding many aspects, particularly its occurrence, which remains underestimated. All the information in the literature comes from case reports or small case series [2, 4, 6, 9, 10]. To our best knowledge, the current study is the largest case series in the world of LCH involving the nasal cavity. Besides, the study examined the relationship between the possible causes of this rare tumor with certain factors and showed a significant correlation between the cause and gender and age.
This study was consistent with previous studies in that the LCH is more prevalent in females than males [3, 11]. However, a study by Puxeddu et al. revealed a slight male predominance (21 males out of 40 cases) . The female predominance in our study may be related to a considerable number of pregnant cases with LCH (n = 26, 31.7%). Moreover, the current study reported a highly statistically significant difference between the possible cause and gender (P-value = 0.000).
LCH could affect all age groups . However, the most vulnerable age group is between the third and fifth decades [12, 13], while the disease is extremely rare in the pediatric age group . Around 80% of our cases were in the age group of 18–40 years. Besides, our results reported that nasal trauma was a possible cause in children (5/5), pregnancy in the age group of 18–40 years (26/60), and no identified cause in the age group > 40 years (11/12) (Table 2). Therefore, the possible cause of the LCH might be determined by the age of the patient (P-value = 0.000).
Owing to the vascular nature of the tumor, epistaxis is the chief complaint in the majority of cases. However, nasal obstruction, rhinorrhea, facial pain, headache, and hyposmia might be the chief complaints either alone or associated with other features in different combinations . Moreover, on rare occasions, intranasal LCH could be found as an incidental finding on a routine nasal examination . The current study revealed that epistaxis was the presenting symptom in 75 cases, and 44 of them were associated with nasal obstruction. In general, the bleeding is usually slight and recurrent with a relatively long duration (month/s). But severe epistaxis which does not stop spontaneously due to LCH of the nasal cavity is also reported in the literature . Therefore, it is of utmost importance to manage the patient promptly, as well as the exclusion of other differentials (bleeding nasal polyp, sinonasal tumor, angiofibroma, and vascular malformation) is essential too.
When we reviewed the literature, intranasal LCH occurs in the vast majority of cases on one side. However, the study by Lopez et al. reported 2 of their 38 cases with bilateral involvement, one of them with bilateral lesions and the other one with a midline tumor arising from the posterior rim of a nasal septal perforation . The current study reported that the left side was involved in 60%, which was consistent with previous studies [4, 11]. Besides, the present study was consistent with other investigations regarding the nasal septum as the commonest site of LCH [2,3,4], while the study by Chi et al. found only 7 out of 15 cases were originating from the nasal septum . Furthermore, the study did not show a significant difference between the possible cause and the side and location of the LCH (P-value > 0.05).
The following case reports showed that the possible causes of LCH in the nasal cavity were due to anterior nasal packs , endoscopic transsphenoidal gonadotrophin-producing pituitary adenoma resection , and pregnancy [16, 17]. It is well-known that trauma and pregnancy are possible causes of LCH in the nasal cavity. Hormonal changes in pregnancy might be implicated as a possible cause of the increased incidence of intra-nasal LCH . Another possible reason for such an increment is hyper-dynamic status during pregnancy. However, only a few cases were due to those two causes (pregnancy and nasal trauma) in many previous case series studies [2, 3, 7, 11]. Although our study revealed that there were considerable cases due to either pregnancy or nasal trauma, there were 42.7% of unidentified causes. We think that the higher percentage of an idiopathic cause may be related to either the patients’ having forgotten trivial trauma or other general diseases or other still unknown causes. Therefore, a thorough history and proper physical examination, including systemic evaluation, are of utmost importance to search for possible causes of this rare entity of the nasal cavity. Knowing the possible causes of this rare tumor helps us more in understanding the mechanisms of tumor initiation and better management of the patients.
It was reported in the literature that the incidence of intranasal LCH in pregnant women is ranged from 2 to 5% . In our series, there was a much higher number of this tumor during pregnancy (n = 26, 31.7%) than in other case series studies [2,3,4, 7, 11]. This may be attributed to the fact that the pregnancy rate in Iraqi women is much higher than that in women from the countries of the abovementioned studies .
Surgical removal is an appropriate method of LCH treatment. There are different ways to achieve its excision, including removal by electrocautery, cryotherapy or laser, and excisional procedures either alone or after embolization [2, 11]. The operation is performed either under local or general anesthesia, depending on the size and site of the lesion . In all cases of the current study, the LCH was excised using a zero-degree endoscope as well as the removal of a few millimeters of cuff of mucosa surrounding the lesion. The recurrence rate in the literature varies greatly, ranging from 0 to 42% [2, 4, 11]. This variation depends on the sample size of the case series and the period of follow-up. Our study reported that the recurrence rate was 3.66% (3/82), and all three cases were excised under local anesthesia. However, there was no statistically significant difference between the recurrence rate and the type of anesthesia used (P-value = 0.192).
Many limitations accompanied this study. The study did not take into consideration the exact site and the size of the LCH. The relatively short period of follow-up (mean 9.94 ± 3.008) had an impact on the accurate assessment of the recurrence rate. Lastly, the retrospective nature of the study was considered another limitation.
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