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Journal of Postgraduate Medicine, Vol. 49, No. 3, July-Sept, 2003, pp. 278-279 Images in Pathology Angiomyolipoma of Kidney as a Part of Tuberous Sclerosis Complex Chen CH, Tzeng CC, Cheng TC, Chiu AW
Division of Urology, Department of Surgery, Chi Mei
Medical Center, Tainan, Taiwan. Code Number: jp03075 A 27-year-old male was admitted to our hospital due to sudden onset of right abdominal pain. Reviewing his history, he had several episodes of epilepsy since the age of 3 years. At the age of 17 years, he had presented to our institution with projectile vomiting, right eye blindness and unstable gait. Multiple facial tumours and periungual fibromas were noted during examination. He had undergone excision of sub-ependymal giant cell astrocytoma in the right frontal lobe at the same institution when he was first admitted. He had two younger sisters who were healthy. He was sent to our emergency room because of acute right abdominal pain. He denied any history of trauma. On physical examination, the patient appeared in distress with pain. His abdomen was mildly distended and diffusely tender with evidence of rebound pain. There was marked right flank knocking pain. Urinalysis was essentially negative for haematuria and pyuria. Radiological examination of the chest revealed cardiomegaly. Radiography of the abdomen showed normal intestinal gas patterns with no evidence of pathological calcifications. Owing to intractable abdominal pain, a computed tomography of the abdomen and pelvis was performed. Bilateral renal tumours and right renal haematoma were found [Figure-1]. One hour after admission, the patient's systolic blood pressure fell to 80 mm Hg. Because of acute haemorrhage compromising his haemodynamics, selective arterial embolisation was performed emergently to stop right renal bleeding. His vital signs became stable after the procedure. Right radical nephrectomy and facial tumour biopsy were done four days later. The pathological diagnosis was angiomyolipoma of the right kidney [Figure-2] and angiofibroma of the facial skin, compatible with tuberous sclerosis complex (TSC) clinically. He was then diagnosed as TSC with spontaneous rupture of angiomyolipoma. The diagnosis of TSC was not established until this event occurred. DISCUSSION Tuberous sclerosis complex is an autosomal dominant neurocutaneous disorder characterised by seizures, mental retardation, cutaneous lesions and visceral harmatoma.[1] So it is necessary to trace the family history of such patients and to screen for undiscovered patients in order to treat them as early as possible. The diagnosis of TSC in our patient was not made early enough though his father also had facial tumours. If his visceral harmatoma had been found before it ruptured, selective surgery could have been performed. Epilepsy is the most common neurological feature, occurring in 96% of patients with TSC. Seizures often begin in the first months of life and are usually severe and intractable .[2] In our patient, the seizures began at the age of 3 and were possibly responsible for the interruption of his education. However, the diagnosis of TSC was not made then. Renal involvement in patients with TSC begins at infancy, and the most common lesion is angiomyolipoma.[3] This case illustrates a potentially life-threatening development due to the spontaneous rupture of angiomyolipoma in a patient with TSC. Both neurologists and urologists must be aware of the clinical features of TSC and all the organs that could be involved, such as the brain, skin, heart, eyes and kidneys, to ensure appropriate monitoring for complications. REFERENCES
Copyright 2003 - Journal of Postgraduate Medicine. Online full-text also available at http://www.jpgmonline.com/ The following images related to this document are available:Photo images[jp03075f2.jpg] [jp03075f1.jpg] |
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