Authors: Shrestha BL*, Shrestha I*, Amatya RCM**, Shrestha RM***
*Lecturer (Otorhinolaryngology), **Professor (Otorhinolaryngology), ***Assistant Professor (Pathology)
Institution: Department of Otorhinolaryngology & Department of Pathology, Kathmandu University School of medical sciences, Dhulikhel, Nepal
Corresponding Author:
Dr. Bikash Lal Shrestha.
Lecturer, Department of Otorhinolaryngology, Kathmandu University School of medical sciences, Dhulikhel, Nepal.
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Abstract:
Leiomyoma of the nasal septum is an extremely rare and unusual benign mass showing smooth muscle differentiation. We report a case of a 29 year old male patient with a left nasal septal leiomyoma. On anterior rhinoscopy and nasoendoscopy, there was a single grayish polypoidal nasal septal mass at the level of the inferior turbinate which was excised endoscopically. Nasal leiomyoma carries a good prognosis after complete excision.
Introduction:
Leiomyomas are benign myogenic neoplasms of the smooth muscle. The most common sites are the uterus, skin and gastrointestinal tract and constitute 1% of all benign tumors in these organs. They are rarely found in the nasal cavity.1 A review of the literature revealed only 28 case reports of nasal cavity leiomyoma, of which only 7 cases originated from the nasal septum.
Case Report:
A 29 year old male presented to the ENT out patient department of Kathmandu University Hospital, Dhulikhel, Nepal with a history of left-sided nasal obstruction and spontaneous, intermittent nasal bleeding for one and a half years. He had no other complaints. Anterior rhinoscopy and nasal endoscopy showed a grayish, globular mass attached to the left side of the septum lying at the level of the inferior turbinate which bled on touch. CT scan of the nose and paranasal sinuses showed a 3 by 3 cm homogenous opacity limited to the left nasal cavity. (Fig.1) The mass was excised in total endoscopically and sent for histopathological examination.
Grossly, the mass was grayish-white in color with a size of 3 by 3cm. Microscopically, sections showed the lesion was composed of spindle cells arranged in long fascicles spindled nuclei, fine chromatin and a moderate amount of eosinophilic cytoplasm. No necrosis/mitosis were seen. Overlying areas showed respiratory type lining epithelium. (Fig.2, 3)
Fig 1. CT Scan Showing a homogenous mass arising from the left nasal cavity.
Fig. 2 Shows respiratory epithelium with underlying spindle cells arranged in long fascicles spindled nuclei, fine chromatin and moderate amount of eosinophilic cytoplasm.
Fig.3 Shows spindle cells with fine chromatin and a moderate amount of eosinophilic cytoplasm.
Discussion:
Leiomyoma is a benign neoplasm of the smooth muscle which commonly arises in the genitalia, skin (mainly from pilo erector muscles) and the gastrointestinal tract. They are rare in the upper aerodigestive tract. This tumor was first described by Virchow in 1958.2
In addition, leiomyomas in the nasal cavity is even less commonly encountered and in the nasal septum it is very rare. Maesaka, et al, initially reported an intranasal leiomyoma in 1966.3 To the best of our knowledge, there had been previously only seven reported cases of leiomyoma involving the nasal septum.
The exact tissue of origin is not known although most authors feel this lesion originates in the nasal cavity from vascular smooth muscle. Hair erectile smooth muscle and undifferentiated mesenchyme have also been implicated as tissues of origin. Trauma, steroid therapy and hormonal imbalance (since sex steroid receptor for progesterone has been found) have also been implicated as etiological factors in the past.2,4 The study performed by Barr, et al.,5 and Llorente, et al.,6 found the origin of the nasal septal leiomyoma is from the smooth muscle component of blood vessels, because of the absence of other types of muscle. Paucity of smooth muscle in the nasal cavity probably contributes to the rarity of leiomyoma occurring in this site.2,4-7
The most common site of origin is the inferior turbinate. Leiomyomas have not been commonly reported from the nasal septum, vestibule, floor or sinus cavities. Barr, et al, hypothesized that this was due to the absences of significant contractile tissue in these structures.5 In our case, the tumor arose from the nasal septum. The World Health Organization had classified the leiomyomas mainly into three histological types. They are, respectively, solid leiomyoma, vascular leiomyoma (also known as angioleiomyoma) and epithelial leiomyoma.8
Leiomyomas which arise from the head and neck region are mainly of vascular origin from smooth muscle. They are categorized into two types either solid leiomyoma or vascular leiomyoma, also known as angiomyoma or angioleiomyoma. It has been postulated that there is progressive development of vascular leiomyoma into a solid type with the reduction in vascularity as in the case of the nasal cavity.9
The literature review showed that leiomyomas are most commonly seen in adults with a predilection towards middle age. Regarding sex distribution, there is significant controversy with some authors suggesting equal sex distribution whereas others suggests 2:1 female predilection and again other reports male: female ratio of 2:1.10-13
However, in our case, the patient was a 29 year old male with involvement of the left nasal cavity. A change in airflow may result in nasal crusting, nasal mucosa desiccation and epistaxis. The treatment of choice is complete excision of the tumor mass along with a margin of normal mucosa. Recurrence after excision is rare despite its vascular origin.4,7 In our case, follow up after nine months revealed no recurrence.
Histologically, the tumor must be differentiated from leiomyosarcoma.14 In our case, the diagnosis of leiomyosarcoma was excluded as the tumor was moderately cellular and lacked nuclear atypia, mitosis and necrosis. This points towards the usual solid type of leiomyoma. However, at times, immunohistochemical markers such as muscle specific desmin, actin, myoglobulin, S-100, vimentin may be required for definitive diagnosis.1,14
Conclusion:
Leiomyoma of the nasal septum is an extremely rare and unusual benign mass showing smooth muscle differentiation. We report a case of a 29 year old male patient with a left nasal septal leiomyoma. On anterior rhinoscopy and nasoendoscopy, there was a single grayish polypoidal nasal septal mass at the level of the inferior turbinate which was excised endoscopically. Nasal leiomyoma carries a good prognosis after complete excision.
References:
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