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CASE REPORT
Year : 2012  |  Volume : 3  |  Issue : 1  |  Page : 40-41  

Cutaneous angiomyolipoma


1 Department of Pathology, S. N. Medical College, Bagalkot, Karnataka, India
2 Department of Surgery, P. E. S. Institute of Medical Sciences and Research, Kuppam, Andhra Pradesh, India

Date of Web Publication3-Mar-2012

Correspondence Address:
Anand S Ammanagi
Department of Pathology, S. N. Medical College, Bagalkot, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.93501

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   Abstract 

We report a case of cutaneous angiomyolipoma (AML) found on the anterior abdominal wall of a 3-year-old female child. Histologic examination showed a well-circumscribed nodule in the dermis composed of an intimate mixture of convoluted thick-walled blood vessels, smooth muscle, and mature fat. This lesion differs from renal AML in terms of a lack of association with tuberous sclerosis, circumscription, absence of epithelioid cells, and male predominance. We concluded that the unique features of this lesion distinguish it from other lesions such as angiomyoma, angiolipoma, myolipoma, haemangioma, and other mixed mesenchymal tumors. This case report suggests that the features considered diagnostic of AML can occur in extrarenal sites and, therefore, this diagnosis cannot be excluded on the basis of site alone.

Keywords: Angiomyolipoma, extra, hamartoma, renal, skin


How to cite this article:
Ammanagi AS, Dombale VD, Shindholimath VV. Cutaneous angiomyolipoma. Indian Dermatol Online J 2012;3:40-1

How to cite this URL:
Ammanagi AS, Dombale VD, Shindholimath VV. Cutaneous angiomyolipoma. Indian Dermatol Online J [serial online] 2012 [cited 2019 Nov 22];3:40-1. Available from: http://www.idoj.in/text.asp?2012/3/1/40/93501


   Introduction Top


Angiomyolipoma (AML) is a hamartomatous lesion usually seen in the kidney of patients with tuberous sclerosis. [1] Extrarenal AML is uncommon and it is usually not associated with tuberous sclerosis. The correct histological diagnosis depends on the classical triad: mature adipose tissue, convoluted thick-walled blood vessels and irregularly arranged sheets, and interlacing fascicles of smooth muscle. Despite the histologic similarity observed in renal and extrarenal AMLs, they differ in several ways from each other. AML of the kidney unlike extrarenal variant can be invasive and may recur. The purpose of the present report is to describe the rare case of cutaneous AML located in the anterior abdominal wall below the umbilicus in a female child and discuss its histological features.


   Case Report Top


A 3-year-old female child was presented with an asymptomatic, single, nontender, subcutaneous swelling over the anterior abdominal wall near the umbilicus measuring 2.5 cm in size. There was no local increase in temperature. The nodule was firm with well defined borders. The patient had no signs of tuberous sclerosis. The nodule was completely excised without any complications. The specimen was sent for histopathologic evaluation [Figure 1].
Figure 1: Intraoperative photograph showing well circumscribed nodule

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Pathological finding

On gross examination, it was a well-circumscribed mass measuring 2.5 cm in diameter with a solid and cystic appearance on the cut surface [Figure 2]. Microscopically, it showed a well-circumscribed lesion surrounded by an incomplete capsule. It was composed of convolutions 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. These three components were arranged irregularly and intermingled with each other [Figure 3]. Compressed fibro-adipose tissue and blood vessels were seen outside the expanding margin of the tumor. Based on the admixture of mature adipose tissue, convoluted thick-walled blood vessels and irregularly arranged sheets, and interlacing fascicles of smooth muscle, a diagnosis of AML was made.
Figure 2: Gross appearance of cutaneous angiomyolipoma

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Figure 3: Shows variable-sized blood vessels, smooth muscle bundles, and mature adipose tissue (H and E, ×40)

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   Discussion Top


AML is a benign hamartomatous lesion usually observed in the kidney of patients with tuberous sclerosis. [1] It is uncommon outside of the kidney, and the liver is the second most common site. 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. [2] Cutaneous AML, which is also known as cutaneous angiolipoleiomyoma, is a rare benign mesenchymal tumor. [2] Fitzpatrick et al. provided the first report of a cutaneous AML, under the name cutaneous angiolipoleiomyoma. [3] To our knowledge, only 21 cases have been reported in English-language literature. These tumors are usually solitary, asymptomatic, noninvasive, located most commonly in acral skin or on the ear. [4] In 1990, Fitzpatrick et al. described eight cases of cutaneous angiolipoleiomyoma (AML). Clinically, the tumors were acquired, solitary, asymptomatic nodules that were always acral in location. [3] Patients' ages ranged from 33 to 77 years (median 52.6 years); the male/female ratio was 7:1. The most common clinical impression is that of a cyst, a lipoma, or a vascular tumor. [3],[5] Signs of tuberous sclerosis were absent. In contrast to the renal form, the cutaneous AML is a tumor differing in sex predominance, clinical associations, circumscription, solitariness, and HMB-45 immunoreactivity. Distinction from other mesenchymal lesions depends on recognition of traditional histologic criteria. [4] 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 AMLs, in contrast to renal AMLs, which may be responsible for HMB-45 negativity of the former tumor. [4] Our case differs from the other cases of cutaneous AMLs reported in terms of location and age. [3] All cutaneous AMLs reported were located either in head and neck region or extremities, and were mostly seen in the 33-77 years age group while in our case AML was observed on the anterior abdominal wall below the umbilicus in a 3-year-old girl. [2],[4]

This case report suggests that the features considered diagnostic of AML can present in extrarenal sites and, therefore, this diagnosis cannot be excluded on the basis of site alone. We conclude that cutaneous AML although uncommon should be considered in the differential diagnosis of any skin tumor with a combination of blood vessels, smooth muscle cells, and adipose tissue. [1]

 
   References Top

1.Singh K, Pai RR, Kini H, Kini UA. Cutaneous angiomyolipoma. Indian J Pathol Microbiol 2009;52:242-3.  Back to cited text no. 1
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2.Shin JU, Lee KY, Roh MR. A Case of a cutaneous angiomyolipoma. Ann Dermatol 2009;21:217-20.  Back to cited text no. 2
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3.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. 3
    
4.Val-Bernal JF, Mira C. Cutaneous angiomyolipoma. J Cutan Pathol 1996;23:364-8.  Back to cited text no. 4
[PUBMED]    
5.Hatori M, Watanabe M, Kokubun S. Angiomyolipoma in the knee: A case report. Ups J Med Sci 2005;110:245-9.  Back to cited text no. 5
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    Figures

  [Figure 1], [Figure 2], [Figure 3]


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