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Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 106-108
Monotypic angiomyolipoma of the nasal cavity: An extremely rare cause of nasal mass with recurrent epistaxis

1 Accura Health Care and Diagnostics, Kandivali, Mumbai, Maharashtra, India
2 Department of Pathology, HBT Medical College and Dr R N Cooper Hospital, Juhu, Mumbai, Maharashtra, India

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Date of Web Publication31-Jan-2020


Monotypic angiomyolipoma is usually found in the kidneys and is composed predominantly of epithelioid cells which show positivity for melanocyte and smooth muscle markers. It can pose a diagnostic challenge due to a range of differential diagnosis. We report the second case of monotypic angiomyolipoma of nasal cavity and first from India in a 54-year-old male who presented with a nasal polyp. Grossly the tumor was well circumscribed and un-encapsulated. Microscopy showed a large number of epithelioid cells mixed with a few spindle cells, varying sized blood vessels, and focal areas of adipose tissue. Immunohistochemistry was positive for smooth muscle actin (SMA) and human melanoma black (HMB-45) stains. It is important to identify this tumor as it can sometimes be mistaken for malignancy and only needs endoscopic resection.

Keywords: Epithelioid cells, HMB-45, monotypic angiomyolipoma, nose, smooth muscle actin

How to cite this article:
Pandey V, Khatib Y, Gupte P, Pandey R, Khare MS. Monotypic angiomyolipoma of the nasal cavity: An extremely rare cause of nasal mass with recurrent epistaxis. Indian J Pathol Microbiol 2020;63:106-8

How to cite this URL:
Pandey V, Khatib Y, Gupte P, Pandey R, Khare MS. Monotypic angiomyolipoma of the nasal cavity: An extremely rare cause of nasal mass with recurrent epistaxis. Indian J Pathol Microbiol [serial online] 2020 [cited 2022 Aug 11];63:106-8. Available from: https://www.ijpmonline.org/text.asp?2020/63/1/106/277397

   Introduction Top

Angiomyolipoma is a distinctive tumor composed of varying proportion of mature fat, smooth muscle cells, and thick walled blood vessels along with perivascular epithelioid cells (PECs) which co-express smooth muscle and melanocyte markers.[1] The kidney is the commonest site for these tumors with one third of the cases occurring in association with tuberous sclerosis. Many extra-renal sites have also been reported for this tumor; like liver, mediastinum, lung, heart, lymph nodes, uterus, vagina,  Fallopian tube More Details, skin, oral cavity, pharynx, and nasal cavity.[1],[2] However, the angiomyolipomas occurring at mucocutaneous sites differ from visceral tumors as they are not associated with tuberous sclerosis, present with smaller size and lack positivity for melanocyte markers.[2] There have been case reports of less than 20 cases of this type of angiomyolipoma in the nasal cavity.[3],[4],[5],[6],[7],[8],[9],[10] However, only one case of nasal monotypic angiomyolipoma has been reported with presence of PEC and human melanoma black (HMB-45) positivity which was similar to their renal counterpart.[11] We report the second case of monotypic angiomyolipoma in the nasal cavity and first from India in a 54-year-old male who presented with epistaxis and nasal polyp.

   Case History Top

A 54-year-old male patient presented to the ENT out-patient department with complaints of nasal obstruction and recurrent epistaxis since 6 months. On anterior rhinoscopy a polypoidal mass was seen obstructing the left nasal cavity with deviation of the nasal septum toward the right. No features suggestive of tuberous sclerosis were present in this patient. Computed tomography scan of the paranasal sinuses revealed a polypoidal mass lesion in the posterior part of the left nasal cavity measuring 17 mm × 14 mm compromising the nasal airway and abutting the nasal septum. On contrast imaging the lesion showed contrast enhancement suggestive of high vascularity [Figure 1]a.
Figure 1: (a) Computed tomography scan showing a polypoidal mass in the left nasal cavity abutting the nasal septum. (b) Gross appearance showing small yellowish white tumor with areas of congestion

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Polypectomy was done. Grossly, the mass was well circumscribed but un-encapsulated and measured 2 cm × 1.7 cm × 1 cm. The cut surface was yellowish white with areas of congestion [Figure 1]b. On microscopy the tumor showed an admixture of fascicles and nests of epithelioid and spindle smooth muscle cells, thick and thin walled blood vessels and a minor component of mature adipose tissue [Figure 2]a. The tumor predominantly comprised of plump epithelioid cells with eosinophilic and clear cytoplasm [Figure 2]b. These cells displayed bland cytological features with small uniform nuclei. There was no mitotic activity or necrosis seen. On immunohistochemistry, the tumor cells expressed smooth muscle actin (SMA) [Figure 2]c, desmin and HMB-45 [Figure 2]d and were negative for pan cytokeratin. In view of the histomorphology and HMB-45 positivity a final diagnosis of monotypic angiomyolipoma was given. The postoperative course was uneventful.
Figure 2: (a) Microphotograph showing tumor composed of a mixture of epithelioid cells, spindle cells, blood vessels and adipose tissue (H and E-100×). (b) Microphotograph showing predominance of epithelioid cells with abundant eosinophilic cytoplasm and bland nuclei (inset). (H and E-100×). (c) Tumor cells are immunoreactive with HMB-45 and (d) smooth muscle actin

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   Discussion Top

Angiomyolipoma is an uncommon benign tumor which was previously thought to be a hamartoma. Nasal angiomyolipoma was first reported by Dawlatly[4] in 1988 and since then less than 20 cases have been reported in world literature. Unlike renal angiomyolipomas these tumors were not associated with tuberous sclerosis, lacked the PEC and were HMB-45 negative.[2] Similar tumors were reported from skin, larynx and pharynx. Hence on the basis of site of occurrence, histological and immunohistochemical features Watanba and Suzuki[2] divided angiomyolipoma into visceral and mucocutaneous angiomyolipoma. Mucocutaneous angiomyolipoma are rarer and occur in the skin and mucosal sites such as nasal cavity, larynx, pharynx, etc., They usually occur in middle aged men, are small in size, never associated with tuberous sclerosis and are HMB-45 negative. Visceral angiomyolipomas are more common; kidney being the commonest site followed by liver. They often present as large masses, are associated with tuberous sclerosis and are strongly positive for HMB-45 immunostain. Hence these features are in contrast to mucocutaneous angiomyolipomas which differ from visceral ones in many aspects. In contrast to the earlier reported cases of nasal angiomyolipoma one case was reported by Banerjee et al.[11] as monotypic angiomyolipoma with features similar to renal angiomyolipoma with perivascular epitheloid cells and HMB-45 positivity. Our case is the second such case to be reported in the nasal cavity.

Monotypic angiomyolipomas are fat poor variant of angiomyolipomas, comprising predominantly of smooth muscle cells that have a distinct epithelioid morphology.[1],[11] The smooth muscle cells co-express melanocytic and smooth muscle antigens and are ultrastructurally found to have melanosome like cytoplasmic granules instead of myofilament bundles.[1],[11] Zamboni et al.[12] demonstrated their close relationship to tumors of the PEComa family and hence they are also termed as monotypic PEComas. Monotypic angiomyolipomas have been described only in the visceral location and their occurrence in mucocutaneous sites is exceptional.[1]

The only reported case of monotypic angiomyolipoma of nasal cavity presented as a nasal mass in a 34-year-old female patient. Imaging findings were nonspecific and the tumor was mistaken for a hemangioma due to its high vascularity. The tumor measured 2 cm in diameter and was non encapsulated but well circumscribed.[11] Our patient was a 54-year-old male who also presented as a nasal polyp and showed similar gross features.

Microscopically monotypic angiomyolipoma is composed of fascicles and nests of plump spindle to epithelioid cells with small nuclei and abundant clear to granular cytoplasm due to the presence of glycogen. The stroma is vascular and comprises of many thick walled blood vessels and unlike the typical angiomyolipoma this tumor is typically fat poor. The tumor displays a bland cytology with lack of mitosis and necrosis.[11]

On immunohistochemistry, these tumors co-express smooth muscle antigens like desmin, SMA as well as melanocytic antigen HMB-45.[1],[11] Therefore, they are also termed as myomelanocytic tumors. Desmin positivity is not a feature of monotypic angiomyolipoma and it is usually negative or only focally positive. The previous reported case, however, showed desmin positivity which is similar to the finding in our case.[11]

Due to strong HMB-45 positivity, paucity of fat and epithelioid morphology of tumor cells, this tumor can be easily confused with melanoma as in the case reported previously.[11] The other important differentials that can cause a potential diagnostic pitfall are smooth muscle tumors, clear cell variant of carcinoma and epithelioid rhabdomyosarcoma.[1] All of these can be ruled out by careful histological examination and immunohistochemistry. The presence of SMA and desmin positivity and negativity for nuclear S100 protein coupled with bland histology, presence of thick walled vessels, and adipocytic component favors angiomyolipoma.

   Conclusion Top

Monotypic angiomyolipoma of the nose is a benign tumor with no recurrence potential. Simple surgical excision is curative. Although extremely rare in the nasal cavity, it must be included in the differential diagnosis of nasal mass with epistaxis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Mete O, Van der Kwast TH. Epithelioid angiomyolipoma: A morphologically distinct variant that mimics a variety of intraabdominal neoplasms. Arch Pathol Lab Med 2011;135:665-70.  Back to cited text no. 1
Watanabe K, Suzuki T. Mucocutaneous angiomyolipoma: A report of 2 cases arising in the nasal cavity. Arch Pathol Lab Med 1999;123:789-92.  Back to cited text no. 2
Aleem MA, FatimaA, KumudachalamP, Priyadarshini R. Nasal angiomyolipoma: Report of two cases of an extremely rare entity. Indian J Pathol Microbiol 2017;60:393-5.  Back to cited text no. 3
[PUBMED]  [Full text]  
Moreira MD, Lessa MM, Lima CM, Lessa HA, Fonseca Júnior LE. Angiomyolipoma of the nasal cavity. Braz J Otorhinolaryngol 2011;77:269.  Back to cited text no. 4
Dawlatly EE, Anim JT, El-Hassan AY. Angiomyolipoma of the nasal cavity. J Laryngol Otol 1988;102:1156-8.  Back to cited text no. 5
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Güezmes A, Mazorra F, García-Mantilla F, Eizaguirre MJ, Ondiviela R. Nasal angiomyolipoma. Acta Otorrinolaringol Esp 1990;41:341-2.  Back to cited text no. 7
Gatalica Z, Lowry LD, Petersen RO. Angiomyolipoma of the nasal cavity: Case report and review of the literature. Head Neck 1994;16:278-81.  Back to cited text no. 8
Tardío JC, Martín-Fragueiro LM. Angiomyolipoma of the nasal cavity. Histopathology 2002;41:174-5.  Back to cited text no. 9
Park WK, Kang MS, Park SK. A case of angiomyolipoma of the nasal cavity. Korean J Otorhinolaryngol-Head Neck Surg 2013;56:383-5.  Back to cited text no. 10
Banerjee 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.  Back to cited text no. 11
Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E, et al. Clear cell “sugar” tumor of the pancreas: A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol 1996;20:722-30.  Back to cited text no. 12

Correspondence Address:
Prajakta Gupte
202, Sea Breeze CHS, Seven Bungalows, JP Road, Andheri (West), Mumbai - 400 061, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_681_18

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