Indian Journal of Pathology and Microbiology

: 2017  |  Volume : 60  |  Issue : 3  |  Page : 393--395

Nasal angiomyolipoma: Report of two cases of an extremely rare entity

Mohammed Abdul Aleem1, Ather Fatima2, P Kumudachalam3, Ramya Priyadarshini4,  
1 Department of Pathology, Osmania Medical College, Koti, Hyderabad, Telangana, India
2 Department of Pathology, Government Medical College, Nizamabad, Telangana, India
3 Department of Pathology, Government ENT Hospital, Osmania Medical College, Koti, Hyderabad, Telangana, India
4 Department of Pathology, Government ENT Hospital, Koti, Hyderabad, Telangana, India

Correspondence Address:
Mohammed Abdul Aleem
H. No: 19-2-11/142/A, Bilal Nagar, Kala Pathar, Hyderabad - 500 053, Telangana


Nasal angiomyolipoma (AML) are extremely rare tumors and so far <15 cases have been reported in the literature, and this is the first instance that Nasal AML is reported from India. We report two cases of AML arising in the nasal cavity described in 60-year-old male and 50-year-old female patient. Grossly, they were well circumscribed, lobulated masses, and microscopically, they were composed of an intimate mixture of mature fat, smooth muscle cells, and thick-walled varying sized blood vessels. Immunoexpression pattern and histopathology were characteristic. Both the patients had complete resolution of symptoms after endoscopic excision of the tumor.

How to cite this article:
Aleem MA, Fatima A, Kumudachalam P, Priyadarshini R. Nasal angiomyolipoma: Report of two cases of an extremely rare entity.Indian J Pathol Microbiol 2017;60:393-395

How to cite this URL:
Aleem MA, Fatima A, Kumudachalam P, Priyadarshini R. Nasal angiomyolipoma: Report of two cases of an extremely rare entity. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Nov 27 ];60:393-395
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Nasal angiomyolipomas (AML) are extremely rare, recently recognized benign tumors, <15 cases have been reported in worldwide literature. The majority of the cases have been reported from Asian countries.[1],[2],[3],[4],[5],[6],[7],[8] Nasal AML, is an extrarenal AML differs from renal AML but shares common clinical, histological, and immunohistochemical characteristics with mucocutaneous AML. Smooth muscle cells found in mucocutaneous AML lack CD117 and HMB45 immunoreactivity in contrast to renal AML.[2] Nasal AML do not show any association with tuberous sclerosis.[3] We report two additional AML arising in the sinonasal cavity. These are the first reported cases of Nasal AML from India.

 Case Reports

Case 1

A 60-year-old male presented with spontaneous, recurrent epistaxis, and left-sided nasal obstruction for 5 months. Nasal endoscopy revealed, a globular, lobulated mass measuring 8 mm × 8 mm arising from the roof of the left nasal cavity. Computed tomography (CT) scan revealed a soft-tissue mass density arising from the roof and left lateral wall of nasal cavity, proximal to inferior turbinate, septum was uninvolved [Figure 1]a. The provisional clinical diagnosis was hemangioma, complete endoscopic-guided excision was performed. Grossly, the tumor was gray-yellow to gray-brown lobulated mass, on cut section, solid, and hemorrhagic areas were identified.{Figure 1}

Case 2

A 50-year-old female presented with left-sided nasal obstruction. On anterior rhinoscopy, a globular polypoidal mass measuring 20 mm × 15 × 10 mm was found in the left nasal cavity. On gross examination, the tumor was globular, firm, and gray-tan to gray-yellow, solid on cut section.

Microscopy in both the cases showed similar features. The sinonasal mucosa was lined by pseudostratified columnar ciliated surface epithelium with focal squamous metaplasia. Submucosa showed an unencapsulated well-circumscribed tumor tissue with admixture of smooth muscle cells, mature adipose tissue, and varying sized thick-walled blood vessels. Sparse lymphocytes and plasma cells were seen focally in the tumor tissue. Nests of mature fat were present in the tumor tissue. Only mature smooth muscle cells were present while smooth muscle cells with epithelioid morphology or nuclear atypia were not observed [Figure 1]b,[Figure 1]c,[Figure 1]d.

Immunohistochemistry was performed using antibodies against alphasmooth muscle actin (α-SMA), Desmin, HMB45, and CD117. SMA expression was strongly positive in smooth muscle cells within the walls of blood vessels and smooth muscle bundles between the blood vessels [Figure 2]a. Desmin positivity was seen mainly in the smooth muscle cells between the blood vessels and focally in the vessel wall [Figure 2]b. CD117 and HMB45 immunoreactivity were negative in the tumor tissue [Figure 2]c and [Figure 2]d. There were numerous mast cells within stroma which expressed CD117 [Figure 2]d and also showed metachromatic granules when stained with toluidine blue stain.{Figure 2}


Dawlatly in 1988 first described an unusual case of AML of the nasal cavity.[1] Nasal AMLs are exceedingly rare, <15 cases have been reported in the world literature.[1],[2],[3],[4],[5],[6],[7],[8] Nasal AML usually present as small tumors in middle-aged or older men with an age range of 34–88 years and male to female ratio of 3:1. The majority of the cases have been reported from Asian countries including middle-east,[1] Turkey,[3] Japan,[2] and Korea;[4],[6] followed by Spain [7],[8] and the USA.[5] Their histology is similar to that of the renal counterpart, but differ in the absence of epithelioid cells, the lack of HMB-45 immunoexpression and do not show any association with tuberous sclerosis.[3]

AMLs are distinctive benign mesenchymal neoplasms composed of a mixture of mature adipose tissue, bundles of smooth muscles, and small to medium-sized, tortuous, thick-walled blood vessels in varying proportions.[1],[2],[3] Extrarenal AML have been reported in liver, lung, mediastinum, retroperitoneum, heart, skin, oral cavity, parotid, larynx, and nasal cavity.[2],[4]

Watanabe and Suzuki proposed the term “Mucocutaneous AML,” to classify AML in the skin, oropharynx, and nasal cavity as these are distinct from the more common renal and hepatic AML. Mucocutaneous AML tends to occur in older men, is not found to be associated with tuberous sclerosis. Mucocutaneous AML is much smaller while renal and hepatic AML may be as large as 45 cm.[2],[9] Clinically, patients present usually with nonspecific symptoms such as nasal obstruction, nasal polyp, recurrent epistaxis, snoring, and sometimes incidentally detected during CT brain.[5],[6] First ever report of monotypic AML of the nasal cavity was described by Banerjee et al. in 2001.[7]

Mucocutaneous AML is histopathologically identical and distinct in appearance from that of AML found in the liver or kidney. Smooth muscle cells found in mucocutaneous AML lack HMB45 immunoreactivity in contrast to HMB45 positive epithelioid smooth muscle cells in renal AML.[2],[3] The lymphocytic aggregates are infrequent in renal and hepatic AML while they are found in nasal AML.[2],[3] Nasal AML expresses SMA, desmin but lack CD117and HMB45 immunoreactivity. In our cases, microscopy as well as expression of immunohistochemical markers was characteristic of mucocutaneous AML. Both of our cases expressed SMA and desmin positivity while CD117 and HMB45 were negative in the tumor tissue.

Renal AML associated with tuberous sclerosis complex is characterized by germ-line mutations in the TSC1 or TSC2 genes located on chromosomes 9q34 and 16p13.3[10] and 5q deletions with a common region of deletion spanning 5q33 to q34. However, literature on molecular and cytogenetic alterations in sinonasal AML is unavailable.[10]

Sinonasal AML pose diagnostic difficulties as radiological and clinical examination findings are nonspecific. Nasal AML usually behave such as benign lesions and do not recur or metastasize. Complete surgical resection is curative, histopathological examination, and immunomarkers are needed for definitive diagnosis.[8]


AML of the nasal cavity is an extremely rare benign tumor not associated with tuberous sclerosis. Nasal AML has similarities with cutaneous and other mucosal AML but lacks epithelioid smooth muscle cells, CD117, and HMB45 expression, thus differs from renal counterpart. AML should be considered as a differential diagnosis for unilateral polypoidal nasal mass as clinical and radiologic findings usually do not distinguish them from other soft-tissue tumors in nasal cavity.

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Conflicts of interest

There are no conflicts of interest.


1Dawlatly EE, Anim JT, el-Hassan AY. Angiomyolipoma of the nasal cavity. J Laryngol Otol 1988;102:1156-8.
2Watanabe K, Suzuki T. Mucocutaneous angiomyolipoma. A report of 2 cases arising in the nasal cavity. Arch Pathol Lab Med 1999;123:789-92.
3Erkiliç S, Koçer NE, Mumbuç S, Kanlikama M. Nasal angiomyolipoma. Acta Otolaryngol 2005;125:446-8.
4Ahn S, Lee Y, Kim C, Chang Y. Angiomyolipoma of the nasal cavity resected with preoperative angio-embolization. Korean J Otorhinolaryngol Head Neck Surg 2013;56:528-31.
5Weindling SM, Menke DM, Bolger WE. Maxillary sinus angiomyolipoma: A case report and overview. Ear Nose Throat J 2015;94:E1-4.
6Go J. Angiomyolipoma of the nasal cavity. Korean J Pathol 2005;39:284-6.
7Banerjee SS, Eyden B, Trenholm PW, Sheikh MY, Wakamatsu K, Ancans J, et al. Monotypic angiomyolipoma of the nasal cavity: A heretofore undescribed occurrence. Int J Surg Pathol 2001;9:309-15.
8Ibáñez Rodríguez JA, Segura Sánchez J, Gómez Galán MJ, García-Eloy Carrasco C. Nasal angiomyolipoma. Acta Otorrinolaringol Esp 2014;65:205-7.
9Nepple KG, Bockholt NA, Dahmoush L, Williams RD. Giant renal angiomyolipoma without fat density on CT scan: Case report and review of the literature. ScientificWorldJournal 2010;10:1334-8.
10Mete O, van der Kwast TH. Epithelioid angiomyolipoma: A morphologically distinct variant that mimics a variety of intra-abdominal neoplasms. Arch Pathol Lab Med 2011;135:665-70.