Angioleiomyoma in deep neck space: A rare case report

Santhosh Kumar Nochikattil; Bipin T Varghese; Anitha M Francis; Shaji Thomas

doi:10.4103/jhnps.jhnps_35_16

CASE REPORT

Year : 2017 | Volume : 5 | Issue : 1 | Page : 38-40

Angioleiomyoma in deep neck space: A rare case report

Santhosh Kumar Nochikattil¹, Bipin T Varghese², Anitha M Francis³, Shaji Thomas²
¹ Department of Surgical Oncology, MCC, Thalassery, Kerala, India
² Department of Surgical Oncology, RCC, Thiruvananthapuram, Kerala, India
³ Department of Pathology, RCC, Thiruvananthapuram, Kerala, India

Date of Web Publication

27-Jul-2017

Correspondence Address:
Santhosh Kumar Nochikattil
Department of Surgical Oncology, MCC, Thalassery, Kerala
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jhnps.jhnps_35_16

Abstract

Angioleiomyoma is a benign, slow-growing soft tissue tumor originating from vascular smooth muscles. Angioleiomyoma in head and neck region is extremely rare, and when it is present, it is more commonly seen in the auricle, nasal cavity, lip, and buccal mucosa. We present a case of asymptomatic angioleiomyoma involving parapharyngeal and prevertebral space.

Keywords: Angioleiomyoma, deep neck space, soft tissue tumor

How to cite this article:
Nochikattil SK, Varghese BT, Francis AM, Thomas S. Angioleiomyoma in deep neck space: A rare case report. J Head Neck Physicians Surg 2017;5:38-40

How to cite this URL:
Nochikattil SK, Varghese BT, Francis AM, Thomas S. Angioleiomyoma in deep neck space: A rare case report. J Head Neck Physicians Surg [serial online] 2017 [cited 2023 Jun 4];5:38-40. Available from: https://www.jhnps.org/text.asp?2017/5/1/38/211729

Introduction

Leiomyomas were first described by Virchow as a benign soft tissue neoplasm arising from the smooth muscle. Depending on the origin as well as based on their histology or anatomic site, it can be classified as piloleiomyomas, angioleiomyomas, and genital leiomyomas. World Health Organization has defined angioleiomyoma as “a frequently painful, benign subcutaneous, or deep dermal tumor composed of mature smooth muscle bundles which are surrounded and interlaced by vascular channels.” Angioleiomyoma is a benign soft tissue tumor originating from vascular smooth muscle. Only around 12% are found in areas such as the head, neck, and trunk regions. Morimoto classified angioleiomyoma as solid, cavernous, and venous type with solid type being the most common. The clinical examination and imaging alone are not sufficient to diagnose angioleiomyoma; usually a biopsy is mandatory.^[1],[2],[3]

Case Report

A 49-year-old male presented with complaints of swelling on left side of the neck, which was noticed incidentally while evaluating for fracture of the right maxilla following a road traffic accident. The patient never had any symptoms related to the swelling. On examination, there was a 4 cm × 3 cm firm swelling left side of the neck, below the angle of mandible. The swelling was nontender, mobile, and was not compressible nor transilluminant. There were no transmitted pulsations. A mucosa covered bulge was noted in the left lateral pharyngeal wall, and the left tonsil and soft palate were pushed medially. The bulge was about 2 cm above the level of pharyngoepiglottic fold. The larynx and hypopharynx were normal. There were no neurological deficits, and all the cranial nerve functions were normal.

Magnetic resonance imaging (MRI) was showing a well-defined lesion of size approximately 4.1 cm × 4 cm × 2.6 cm in the left parapharyngeal space [Figure 1]. The lesion was displacing medial pterygoid muscle laterally and was closely abutting left internal carotid artery and internal jugular vein posteriorly with intervening fat plane. Left internal jugular vein was compressed by the lesion. There was another ill-defined heterogeneously hyperintense lesion noted in left prevertebral space of size approximately 6 cm × 4.7 cm × 4.1 cm. Left prevertebral muscle was displaced anteriorly by the lesion, and it was extending into epidural space at C2 level through the interface between the odontoid process and left lateral mass of the atlas and the intervertebral foramina. There was no extension into or indentation of the thecal sac. The lesion was bulging into the nasopharynx and oropharynx; however, pharyngeal mucosa appears intact.

Figure 1: Magnetic resonance imaging showing a well-defined lesion in the left parapharyngeal space

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Another ill-defined lesion noted in posterior cervical space within the intermuscular plane medial and posterior to left sternocleidomastoid of size approximately 3.2 cm × 2.6 cm × 2.4 cm. No intracranial extension of any of the lesions was noted.

Excision of all the three lesions was done by a team of head and neck surgeons and neurosurgeons. These masses were firm, smooth, and well defined except where it was extending to the epidural space, and some residual tissues were left behind in this area. The postoperative period was uneventful.

The histopathology report was suggestive of vasoformative neoplasm with features of angioleiomyoma. The lesion was consisting of multiple vascular channels, variably sized with marked thickening of the walls by proliferated smooth muscle cells and lined by plump endothelium. These vessels were embedded in a myxoid stroma, focally showing smaller bundles of smooth muscle cells and fat cells. Immunohistochemical (IHC) with smooth muscle actin highlights the proliferated smooth muscle cells [Figure 2] and [Figure 3].

Figure 2: Low power image showing multiple vascular channels, variably sized with marked thickening of the walls by proliferated smooth muscle cells and lined by plump endothelium

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Figure 3: High power view showing immunohistochemical with smooth muscle actin highlights the proliferated smooth muscle cells

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The patient is followed up for more than 1 year and he is asymptomatic. However, recent MRI is showing a small residual lesion in the prevertebral space which is not compressing or infiltrating any vital structures. The plan is to keep the patient under close follow-up with clinical examination and radiology, if required.

Discussion

Angioleiomyoma is a rare, benign, solitary tumor that originates from the vascular smooth muscle. It is a slow-growing tumor that originates from the vascular smooth muscles. Histologically, angioleiomyoma can be subdivided into three types, depending on the vascular and smooth muscle components present: the solid or capillary type, the venous type, and the cavernous type. Morimoto classified the tumors as follows:^[3]

Solid - the tumor comprises closely compacted smooth muscle and abundant blood vessels, which are small and slit-like. The smooth muscle bundles surround the vessels and interdigitate with them
Venous - the tumor lacks compacted smooth muscle bundles, and the blood vessels have thick muscular walls of varying size
Cavernous - the tumor consists of numerous dilated vascular channels and smaller quantities of smooth muscle bundles, which are difficult to distinguish from the muscular walls of the vessel channels.^[3]

In a study of 562 cases of angioleiomyoma by Hachisuga et al. Three hundred and seventy-four cases (66%) were of solid type, 127 (23%) were venous type, and 61 were (11%) of the cavernous type. Wang reported that 14 of 21 (67%) of head and neck angioleiomyoma were solid type, 6 (28%) were cavernous type, and only 1 (5%) was venous type.

Angioleiomyoma can be found anywhere in the body, but it is more commonly found in the lower extremities. Angioleiomyomas are extremely rare in the head and neck area. Only <200 head and neck angioleiomyomas have been described so far. Hachisugha reported that the auricle and the oral and sinonasal cavities are the most common sites of angioleiomyoma in the head and neck region.^[4],[5]

Liu et al. reported that angioleiomyomas accounted for only 0.18% of the benign head and neck tumors in the patients presenting to their hospital over 34 years. The most common sites were the buccal mucosa, parotid gland, and the palate. The 21 patients included in their study included 10% of the cases reported in the published literature. It was more common in males, and the mean age at diagnosis was 42.5 years.^[6]

In a case series of 21 cases of head neck angioleiomyoma by Wang et al., auricle was involved in five cases, nasal cavity in three, external nose in three, neck in three, lip in three, inner canthus two, forehead one, and had palate one. The mean age of presentation was 48 years. Our patient presented at the age 49 years and he had the lesion involving deep neck spaces.^[5]

The most common presentation of angioleiomyoma is pain with or without tenderness. Pain and tenderness may be the presenting feature in 45%–70% of all cases. However, head and neck angioleiomyomas usually present as painless masses. The solid type is usually painful, and the venous or cavernous type is painless. In the head and neck, venous type is more common compared to painful solid variant. However, no patients in Wang et al. series complained of pain, despite most tumors being of the solid type and the three in the neck being unusually large. Toida et al. reported that while intermittent pain and size changes are two characteristic features of this tumor in nonoral sites, only seven of the ninety oral cases were painful. Our patient presented with a painless mass which was detected incidentally while evaluating for fracture of the maxilla following a road traffic accident.^[3],[5],[7]

Majority of these tumors are small, usually <2 cm in size. However, Liu et al. reported that more than half of the tumors (61.9%) were >2 cm in diameter, and according to Wang et al., all the three vascular leiomyomas of the neck included in their study were >2 cm in size. Our patient had a deep-seated tumor which was >4 cm in size.^[3],[5],[6]

It is difficult to diagnose angioleiomyomas clinically and radiologically. The differential diagnosis of angioleiomyoma includes vascular tumors such as hemangiomas and lymphangiomas, lipomas, schwannomas and neurofibromas, and pleomorphic adenomas. MRI has been used more frequently as an investigative tool in these scenarios as it is more sensitive. Characteristically, angioleiomyoma on MRI can be described as a well-demarcated, strongly enhancing mass with an isointense to slightly hyperintense signal compared with muscle on T-weighted images and hyperintense signals on T-weighted images.^[8]

According to Ling Yiu, preoperative fine needle aspiration cytology was not useful and most often misleading by giving diagnosis of hemangioma or schwannoma. A histopathological examination with confirmatory IHC evaluation following excision remains the most reliable method for diagnosis. Malignant variants of this neoplasm have been reported; however, they are rare. It appears that the absence of mitosis is the most useful histologic indicator of a benign lesion.

Surgical excision is the treatment of choice. The chance of recurrence is rare. In Wang et al. series, only one case recurred after surgical excision and only two cases recurred in Hachisuga et al. study.^{[3],[4],[5],[6]}

Conclusion

Angioleiomyoma in parapharyngeal and prevertebral space is very rare. In our patient, it presented as a painless swelling in neck. The preoperative imaging and cytology are not very helpful in a preoperative diagnosis. With more awareness among clinicians regarding the nature of presentation and behavior of this tumor, it could be included in the differential diagnosis of a painless mass in these regions, thus enabling a holistic approach in the treatment of the tumor.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3.	Morimoto N. Angiomyoma (vascular leiomyoma): A clinicopathological study. Med J Kagoshima Univ. 1973;24:663-83.
4.	Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126-30. [PUBMED]
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7.	Toida M, Koizumi H, Shimokawa K. Painful angiomyoma of the oral cavity: Report of a case and review of the literature. J Oral Maxillofac Surg 2000;58:450-3.
8.	Brooks JK, Nikitakis NG, Goodman NJ, Levy BA. Clinicopathologic characterization of oral angioleiomyomas. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:221-7. [PUBMED]