Ascending cholangitis: rare presentation of a ruptured right-sided renal angiomyolipoma

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Abstract

We report the case of a 46-year-old female who presented to the Emergency Department with acute, painful obstructive jaundice, with evidence of secondary ascending cholangitis. Surprisingly, imaging revealed the clinical picture to be caused not by hepatobiliary pathology, but by external compression of the biliary tree from a ruptured renal angiomyolipoma (AML) of the right kidney. The patient remained haemodynamically stable and conservative management saw resolution of biliary obstruction. We believe this to be the first report of a renal AML presenting in this way. This report highlights the diverse spectrum of presentations of renal angiomyolipomas.

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Contemporary diagnosis and management of renal angiomyolipoma.

Caleb P Nelson, Martin G. Sanda (2002)

We present a comprehensive resource that summarizes contemporary advances relevant to the clinical management of renal angiomyolipoma. A MEDLINE search was done using the key words angiomyolipoma, hemangioma or lipoma and kidney neoplasm, and therapeutic embolization. References from these articles were reviewed to identify additional relevant source material. We identified 13 series published since 1986 comprising 336 patients as well as 24 reports of therapeutic embolization for angiomyolipoma, comprising 76 patients. To our knowledge we report the largest compilation series to date. Angiomyolipoma is generally benign, although an uncommon subtype (epithelioid angiomyolipoma) may behave more aggressively. Tuberous sclerosis associated angiomyolipoma tends to be larger, multiple and more likely to cause spontaneous hemorrhage than the sporadic entity. Tumors that hemorrhage tend to be larger. Computerized tomography or magnetic resonance is usually sufficient for diagnosis. Biopsy is rarely useful. Primary indications for intervention include symptoms such as pain or bleeding or suspicion of malignancy. Prophylactic intervention is justifiable for large tumors, in females of childbearing age or in patients in whom followup or access to emergency care may be inadequate. Recent advances that have affected management include improved understanding of tuberous sclerosis complex and angiomyolipoma genetics, the identification of molecular markers that facilitate histopathological diagnosis, and the refinement of embolization and partial nephrectomy techniques. Although some cases of angiomyolipoma may require complete nephrectomy, most can be managed by conservative nephron sparing approaches. Ongoing research into the molecular biology and clinical behavior of angiomyolipoma may improve our ability to manage these lesions.

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Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated.

John C Kingswood, Prasanna Sooriakumaran, James Gibbs … (2009)

To examine the presentation, management and outcomes of patients with renal angiomyolipoma (AML) over a period of 10 years, at St George's Hospital, London, UK. We assessed retrospectively 102 patients (median follow-up 4 years) at our centre; 70 had tuberous sclerosis complex (TSC; median tumour size 3.5 cm) and the other 32 were sporadic (median tumour size 1.2 cm). Data were gathered from several sources, including radiology and clinical genetics databases. The 77 patients with stable disease were followed up with surveillance imaging, and 25 received interventions, some more than one. Indications for intervention included spontaneous life-threatening haemorrhage, large AML (10-20 cm), pain and visceral compressive symptoms. Selective arterial embolization (SAE) was performed in 19 patients; 10 received operative management and four had a radiofrequency ablation (RFA). SAE was effective in controlling haemorrhage from AMLs in the acute setting (six) but some patients required further intervention (four) and there was a significant complication rate. The reduction in tumour volume was only modest (28%). No complications occurred after surgery (median follow-up 5.5 years) or RFA (median follow-up 9 months). One patient was entered into a trial and treated with sirolimus (rapamycin). The management of AML is both complex and challenging, especially in those with TSC, where tumours are usually larger and multiple. Although SAE was effective at controlling haemorrhage in the acute setting it was deemed to be of limited value in the longer term management of these tumours. Thus novel techniques such as focused ablation and pharmacological therapies including the use of anti-angiogenic molecules and mTOR inhibitors, which might prove to be safer and equally effective, should be further explored.

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Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.

Ghadeer Mokhtar, Wahib E Fahmy, S Kattan … (2008)

To evaluate the changing management of sporadic renal angiomyolipoma and renal angiomyolipoma associated with the tuberous sclerosis complex (TSC) during the past 16 years. We retrospectively reviewed the charts of 60 patients with angiomyolipoma seen at our institutions. The median age at presentation was 45 years (range 7-78). The presentation was pain in 30 patients and hematuria in 13; it was incidentally discovered in 17 patients. Of the 60 patients, 43 were females. TSC was present in 14 patients. The median tumor size was 4 cm (range 0.3-40, mean 6.5 +/- 1.1). Of the 60 patients, 31 were followed up expectantly. Surgery or intervention was needed for 29 patients to control hemorrhage or relieve pain or because of the suspicion of malignancy. Of these 29 patients, 12 underwent nephrectomy, 11 partial nephrectomy, and 6 embolization. The patients treated for hemorrhage had a median tumor diameter of 11 cm (range 2-21). Patients were followed up for a mean of 39.3 +/- 5.4 months. The lesions grew an average of 4.7 +/- 3.4 cm for TSC tumors and 0.6 +/- 0.2 cm for sporadic angiomyolipoma tumors. None of the patients developed renal impairment. Patients with TSC presented at a younger age, had larger and bilateral lesions, and were more symptomatic during follow-up. In the past 6 years, a significant trend was seen toward finding tumors in asymptomatic patients and toward the use of conservative or interventional (embolization) treatment. Renal angiomyolipoma has a slow growth rate. The preservation of renal function was noted in all our patients. A recent shift was noted toward finding smaller tumors in asymptomatic patients and the use of conservative and interventional treatment.

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Journal

Journal ID (nlm-ta): J Surg Case Rep

Journal ID (iso-abbrev): J Surg Case Rep

Journal ID (publisher-id): jscr

Title: Journal of Surgical Case Reports

Publisher: Oxford University Press

ISSN (Electronic): 2042-8812

Publication date Collection: October 2017

Publication date (Electronic): 07 October 2017

Publication date PMC-release: 07 October 2017

Volume: 2017

Issue: 10

Electronic Location Identifier: rjx191

Affiliations

[1 ] Department of Surgery, St. James’s Hospital, Dublin 8, Ireland

[2 ] Department of Urology, St. James’s Hospital, Dublin 8, Ireland

Author notes

[* ]Correspondence address. Tel: +353-1-4162160; Fax: +353-1-4284171; E-mail: croghans@ 123456tcd.ie

Article

Publisher ID: rjx191

DOI: 10.1093/jscr/rjx191

PMC ID: 5632343

SO-VID: 2701fb4b-b253-4638-aa93-ce255b1f47b0

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This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@ 123456oup.com

History

Date received : 28 May 2017

Date accepted : 05 September 2017

Page count

Pages: 3

Ascending cholangitis: rare presentation of a ruptured right-sided renal angiomyolipoma

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Most cited references 10

Contemporary diagnosis and management of renal angiomyolipoma.

Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated.

Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.

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