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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 2  |  Page : 242-243
Cutaneous angiomyolipoma


1 Department of Pathology, Kasturba Medical College, Mangalore, India
2 Department of Plastic Surgery, Kasturba Medical College, Mangalore, India

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   Abstract 

Cutaneous angiomyolipomas are rare. We report a case in a 45-year-old male with a well circumscribed lesion located on the chin. This lesion, probably hamartomatous in nature, differs from renal angiomyolipoma in terms of nonassociation with tuberous sclerosis, circumscription, and male predominance. Another characteristic feature is the absence of epithelioid cells. Differential diagnosis includes angiolipoma, angioleiomyoma, hemangioma, and myolipoma. It is distinguished from the abovementioned entities by the presence of a combination of thick-walled blood vessels, smooth muscle, and fat.

Keywords: Angiomyolipoma, hamartoma, skin

How to cite this article:
Singh K, Pai RR, Kini H, Kini UA. Cutaneous angiomyolipoma. Indian J Pathol Microbiol 2009;52:242-3

How to cite this URL:
Singh K, Pai RR, Kini H, Kini UA. Cutaneous angiomyolipoma. Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Mar 30];52:242-3. Available from: http://www.ijpmonline.org/text.asp?2009/52/2/242/48932



   Introduction Top


Angiomyolipomas are uncommon lesions that are seen most often in the kidney but have also been described at many other sites including the skin. Angiomyolipomas belong to the family of perivascular epithelioid cell tumors and are characteristically positive for HMB-45. [1] Cutaneous angiomyolipoma is a very rare benign vascular tumor that consist of convoluted thick-walled blood vessels, adipose tissue and smooth muscle bundles. [2] It differs from renal angiomyolipoma in terms of sex predominance, clinical association, circumscription and HMB-45 immunoreactivity. [3]


   Case Report Top


A 45-year-old male presented with a painful nodule on the chin measuring 1 1cm in size. It was nontender with no local increase in temperature. The skin over the nodule was not pinchable and the nodule was firm with well defined borders. The patient had no signs of tuberous sclerosis. The nodule was excised and sent for histopathologic evaluation.

Pathological findings

On gross examination, it was a well circumscribed nodule measuring 1cm in diameter with a solid white appearance on the cut surface. Histologically, a circumscribed tumor was seen composed of ectatic thick-walled blood vessels lined by plump endothelial cells and surrounded by bundles of smooth muscle cells. Islands of adipose tissue were also seen. Compressed fibro-adipose tissue and blood vessels were seen outside the expanding margin of the tumor [Figure 1]. Based on the admixture of vascular component, smooth muscle bundles and adipose tissue, a diagnosis of angiomyolipoma was made.


   Discussion Top


Angiomyolipoma is probably a hamartomatous process which is seen mainly in the kidney. It is often seen in patients with tuberous sclerosis. It shows a female predominance and is composed of an admixture of small to medium-sized thick-walled blood vessels, smooth muscle cells and adipose tissue. A population of epithelioid or clear cells may also be seen, arranged around the blood vessels. [3] Angiomyolipomas are rarely seen at many other locations including skin, liver, spleen, lymph node, spermatic cord, penis, vagina, uterus,  Fallopian tube More Details, oral cavity, nasal cavity, lung, heart, colon, abdominal wall, retroperitoneum, mediastinum, epidurally in the spine, and subgalea aponeurotica. [3] Cutaneous angiomyolipomas are rare and differ from renal angiomyolipomas in that they are more common in males, 33 to 77 years of age without any association with tuberous sclerosis and are HMB-45 negative. [3] Histologically, they are composed of thick-walled blood vessels, smooth muscle cells and mature fat in variable proportions. Epithelioid cell component is usually absent in cutaneous angiomyolipomas, in contrast to renal angiomyolipomas, which may be responsible for HMB-45 negativity of the former tumour. [3]

Histologic differential diagnosis of cutaneous angiomyolipoma include angiolipoma, angioleiomyoma, hemangioma with partial involution and fatty replacement and myolipoma. [3] Smooth muscle bundles are not seen in angiolipoma, whereas angioleiomyoma lacks an adipose tissue component. Hemangiomas do not show a smooth muscle component. Angiomyolipoma is differentiated from myolipoma by the absence of thick-walled blood vessels in the latter. Cutaneous angiomyolipomas are rare and to the best of our knowledge, twelve cases have been published to date. [3] Our findings in this case correlate with other cases in terms of male predominance, lack of association with tuberous sclerosis and solitary nature of the tumor.

It differs from the other cases of cutaneous angiomyolipomas reported in a series in terms of location. [4] All cutaneous angiomyolipomas presented in the reported series were acral in location, while our case was observed on the chin. The exact relationship to the newly described family of perivascular epithelioid cell tumors (PEComas) is unclear in view of the absence of epithelioid/clear cells, and differences in clinical associations. Cutaneous angiomyolipoma is uncommon and should be considered in the differential diagnosis of any skin tumor with a combination of blood vessels, smooth muscle cells and adipose tissue.

 
   References Top

1.Ma L, Kowalski D, Javed K, Hui P. Atypical angiomyolipoma of kidney in a patient with tuberous sclerosis: A case report with p53 gene mutation analysis. Arch Pathol Lab Med 2005;129:676-9.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Argenyi ZB, Piette WW, Goeken JA. Cutaneous angiomyolipoma: A light-microscopic, immunohistochemical and electron-microscopic study. Am J Dermatopathol 1991;13:497-502.  Back to cited text no. 2  [PUBMED]  
3.Val-Bernal JF, Mira C. Cutaneous angiomyolipoma. J Cutan Pathol 1996;23:364-8.  Back to cited text no. 3  [PUBMED]  
4.Fitzpatrick JE, Mellete JR Jr, Hwang RJ, Golitz LE, Zaim MT, Clemons D. Cutaneous angiolipoleiomyoma. J Am Acad Dermatol 1990;23:1093-8.  Back to cited text no. 4    

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Correspondence Address:
Kulwant Singh
Pathology Department, Kasturba Medical College, P.O. Box 53, Mangalore - 575 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.48932

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    Figures

  [Figure 1]

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    Abstract
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