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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 340-341
Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage


1 Department of Histopathology, Safdarjung Hospital, New Delhi, India
2 Department of Pathology, Safdarjung Hospital, New Delhi, India

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Date of Web Publication12-Jun-2010
 

   Abstract 

Angiomyolipomata of the kidney are unusual lesions composed of abnormal thick walled blood vessels, smooth muscle and adipose elements. These are asymptomatic and occasionally present with flank pain, a palpable mass or gross hematuria. They may be associated with tuberous sclerosis. The risk of bleeding is increased with size, and lesions greater than 4 cm have more than 50% chance of significant bleeding. An unusual case of multifocal renal angiomyolipoma associated with tuberous sclerosis and presenting as massive intra abdominal hemorrhage is reported.

Keywords: Angiomyolipoma, benign tumor, multifocal, renal tumors

How to cite this article:
Kushwaha R, Dhawan I, Arora R, Gupta K, Dhupia J S. Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage. Indian J Pathol Microbiol 2010;53:340-1

How to cite this URL:
Kushwaha R, Dhawan I, Arora R, Gupta K, Dhupia J S. Multifocal renal angiomyolipoma presenting as massive intraabdominal hemorrhage. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Nov 17];53:340-1. Available from: http://www.ijpmonline.org/text.asp?2010/53/2/340/64330



   Introduction Top


Renal angiomyolipoma have an incidence of 0.3-3% and arise from the mesenchymal elements of the kidney. [1] They are mostly benign tumors composed of varying proportions of mature adipose tissue, smooth muscle and abnormal thick walled blood vessels. [2],[3]

Angiomyolipomata have been described in the literature either as hamartomas or choriostomas. As fat and smooth muscle are not normal constituents of the renal parenchyma, angiomyolipoma by definition should be considered as choriostoma.

Spontaneous rupture of the kidney is rare but is a well-recognized surgical emergency. Resulting hematoma is confined to either the subcapsular or perinephric space. [4]

We describe a male patient of multifocal renal angiomyolipoma with massive intraabdominal hemorrhage resulting probably due to rupture of pseudo aneurysm of renal artery branch.


   Case Report Top


A 35 year old man presented in casualty with acute abdominal pain associated with nausea and hematuria. He had adenoma sebaceum on face. On radiological investigations renomegaly and a hyperechoic lesion at mid-pole of right kidney was seen, left kidney appeared normal. There was perinephric collection compressing the inferior vena cava. Contrast enhanced computed tomography of abdomen revealed multiple right sided renal angiomyolipoma with Perinephric and Subcapsular hematoma secondary to rupture of one of these masses and pseudo aneurysm in mid-polar segmental renal artery. During surgery large right-sided renal mass was found measuring 15 x 15 x 10 cm, abutting first and second part of duodenum, right gonadal vessels, ascending colon, hepatic flexure, liver and right side of diaphragm. Four hundred millilitre of blood was present in peritoneum.

Grossly, renal shape was distorted. Capsule was thickened. On cut section multiple tumors were identified, the largest measuring 6 x 5 cm. The tumor mass extended to perirenal fat. It was yellow to tan grey in appearance with areas of hemorrhage and necrosis. The compressed renal parenchyma was seen in between tumor masses [Figure 1].

Microscopically, appearance was similar in all the tumors consisting of tortuous, thick walled blood vessels and bundles of smooth muscle that seemed to emanate from vessel walls. Mature adipose tissue was present focally. Areas of hemorrhage and necrosis were seen. No nuclear atypia was identified. The diagnosis of angiomyolipoma was made [Figure 2] and [Figure 3]


   Discussion Top


Angiomyolipomata may occur as an isolated phenomenon or as part of the syndrome associated with tuberous sclerosis. The overall female to male predominance is approximately 4 : 1 to 8 : 1, suggesting role of female hormones to tumor growth. [2] Isolated angiomyolipoma occur sporadically, account for 80% of cases and are usually solitary that occur in the fourth to fifth decade of life (mean age 43 years). [5] Angiomyolipoma associated with tuberous sclerosis are typically larger than isolated angiomyolipoma, have multifocal or bilateral disease and tend to occur in younger patients.

This relatively young male patient had adenoma sebaceum and associated multiple renal angiomyolipoma, thus fulfilling two major criteria for tuberous sclerosis, so we did not recommend CT/MRI as presence of two major criterias is sufficient to diagnose tuberous sclerosis. This tumor was greater than 4 cm leading to rupture. In a study done by Steiner MS et al.[1] it is described that multiple renal angiomyolipomas associated with tuberous sclerosis, occur at early age, are more symptomatic and grow rapidly. Although most angiomyolipoma are benign and asymptomatic, symptoms develop in 68-80% of patients when tumor size reaches 4 cm or more. Patients with ruptured angiomyolipoma often present with acute pain abdomen as a result of hemorrhage and up to 20% are in shock at the time of initial presentation. Therefore, it is important to predict the possibility of rupture in patients with angiomyolipoma. [6] In our patient, rupture of renal angiomyolipoma was associated with rupture of pseudoaneurysm of a renal artery branch.

Angiomyolipomas are composed of variable amounts of adipose tissue, smooth muscle and vasculature. Over 90% of the tumors contain at least focal areas of mature adipose tissue. The smooth muscle component ranges from fascicles of elongated spindle cells with cigar-shaped nuclei to sheets of epithelioid cells with abundant eosinophilic cytoplasm. Thickened and hyalinized vessels with eccentric lumens are seen in most cases. [7]

In conclusion, angiomyolipoma of kidney are unusual lesions that behave in benign fashion. Multifocal lesions associated with tuberous sclerosis grow at a rapid rate and are likely to undergo rupture if size exceeds 4 cm. The primary complication is intraabdominal hemorrhage. Hence this case report carries an important message to clinicians that tumor greater than 4 cm should be resected to avoid complications.

 
   References Top

1.Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993;150:1782-6.   Back to cited text no. 1  [PUBMED]    
2.Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002;168:1315-25.   Back to cited text no. 2  [PUBMED]    
3.Nonomura A, Minato H, Kurumaya H. Angiomyolipoma predominantly composed of smooth muscle cells: Problems in histological diagnosis. Histopathology 1998;33:20-7.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Peh WC, Yip KH, Tam PC. Spontaneous renal pseudoaneurysm ruptures presenting as acute intraabdominal hemorrhage. Br J Radiol 1997;70:1188-90.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Blute ML, Malek RS, Segura JW. Angiomyolipoma: Clinical metamorphosis and concerns. Urol 1988;139:20-4.  Back to cited text no. 5      
6.Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal Angiomyolipoma: Relationships between tumor Size, Aneurysm Formation, and Rupture. Radiology 2002;225:78-82.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Victor RE, Satish KT. Adult renal tumors. In: Stacey EM, editor. Sternbergs diagnostic surgical pathology. 4th ed. Lippincott: Williams and Wilkins; 2004. p. 1955-2000.  Back to cited text no. 7      

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Correspondence Address:
Rashmi Arora
13 Vikram Vihar Extension, Lajpat Nagar IV, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.64330

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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