Gigantisches, blutendes Angiomyolipom der Niere bei tuberöser Sklerose

Tuberous sclerosis complex (TSC) may be associated with hepatic angiomyolipoma. In contradistinction to renal angiomyolipoma this lesion is rare, and nontraumatic hemorrhage has not so far been described. This is the first report of a spontaneously ruptured, surgically treated and histologically verified, hepatic angiomyolipoma in TSC. Anomalies of the vessel walls and consequently the hazard of bleeding are known to increase with the diameter of the tumor in other localizations. It is concluded that in cases with rapid tumor growth and marked vessel anomalies elective surgery should be considered.

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“…Hepatic manifestations appear mainly in women [23]. These alterations seems to be marked in large tumors [4]. Fig.…”

Section: Incidencementioning

confidence: 99%

Ruptured hepatic angiolipoma in tuberous sclerosis complex

Huber

Treutner

Steinau

et al. 1996

Langenbecks Arch Chir

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“…Angiographische Kriterien sind gekna È uelte oder traubenfo È rmig verlaufende Gefa È ûe mit Distanzspru È ngen zwischen den Gefa È ûen; Kaliberspru È nge und arterioveno È se Shunts sind mo È glich [2]. Diese fragilen Gefa È ûstrukturen sind fu È r die Blutungsneigung der Hamartome verantwortlich.…”

Section: Zusammenfassungunclassified

Renale Angiomyolipome als seltene Ursache einer retroperitonealen Blutung

Gawenda

Erasmi

Lorenzen

et al. 1998

Chirurg

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Angiomyolipomas are hamartomas that may be found sporadically or associated with tuberous sclerosis (M. Bourneville-Pringle). Clinically, this long-term asymptomatic tumor becomes evident as an acute retroperitoneal hemorrhage or by symptoms of a flank mass. Due to the high percentage of fat components in this tumor type, computed tomography is far superior to other radiological procedures. In view of two of our own case reports, the therapeutic strategies are discussed, paying regard to the actual literature in this field.

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Renal involvement in tuberous sclerosis complex: a retrospective survey

et al. 1994

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In a retrospective survey performed in Germany and Switzerland, 207 patients (ranging in age from newborn to 70 years) were evaluated in order to establish the frequency, prognosis and diagnostic awareness of kidney involvement in the tuberous sclerosis complex. Renal manifestations were observed in 48% of patients: renal cysts (33 patients), renal angiomyolipoma (AML) (30 patients), a combination of both (8 patients), renal cell carcinoma (3 patients), life-threatening events such as haemorrhage (4 patients), hypertensive crisis (2 patients) and chronic renal failure (10 patients) were also documented. The diagnostic imaging techniques of ultrasonography, intravenous urography, computed tomography and magnetic resonance imaging (MRI) are important but do not always yield definitive information. Differentiation between AML and cysts can be achieved using special MRI techniques (RARE). The potential for renal involvement should be monitored in all patients with the tuberous sclerosis complex.

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Gigantisches, blutendes Angiomyolipom der Niere bei tuberöser Sklerose

Cited by 4 publications

References 0 publications

Ruptured hepatic angiolipoma in tuberous sclerosis complex

Ruptured hepatic angiolipoma in tuberous sclerosis complex

Renale Angiomyolipome als seltene Ursache einer retroperitonealen Blutung

Renal involvement in tuberous sclerosis complex: a retrospective survey

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