Angiomyolipoma causing life-threatening hematuria in a child with tuberous sclerosis

Azmy, Amir; Stephenson, John; Ziervogel, M A

doi:10.1016/s0022-3468(89)80574-3

Cited by 8 publications

(3 citation statements)

References 3 publications

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“…Some members of the PEComa family, specifically angiomyolipoma and lymphangioleiomyomatosis, occur in the context of tuberous sclerosis, an autosomal dominant disorder caused by germline mutations in TSC1 or TSC2 genes. The different PEComa family members vary in their clinicopathologic features and are rare in children, as summarized in Table 6 [245,248–295].…”

Section: Tumors Of Uncertain Histogenesismentioning

confidence: 99%

Soft Tissue Tumors of Uncertain Origin

2012

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Many soft tissue tumors of childhood lack obvious differentiation toward a defined mesenchymal tissue type or have a phenotype that does not correspond to any defined normal tissue. These challenging tumors are currently regarded as neoplasms of uncertain differentiation. Nonetheless, there have been great strides in the understanding of their pathologic and genetic features and biologic underpinnings. The application of new genetic information to the pathologic diagnosis among this group of tumors is an emerging area in diagnostic pediatric pathology. This article reviews the clinicopathologic features of tumors of uncertain and/or miscellaneous origin, with an emphasis on the unique aspects of these neoplasms in children and adolescents, use of diagnostic adjuncts, and differential diagnosis.

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Section: Tumors Of Uncertain Histogenesismentioning

confidence: 99%

Soft Tissue Tumors of Uncertain Origin

2012

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“…The most severe symptom is hematuria (spontaneous or as a result of mild traumas) [7] which is sometimes so massive that it becomes life-threatening [10] and necessitates demolitive surgery. For instance Hamburger et al [11] were obliged to conduct bilateral nephrectomy in a patient with bilaterally bleeding angiomyolipoma.…”

Section: Discussionmentioning

confidence: 99%

“…Surgery is necessary in cases of severe hemorrhage, but not without prior consideration of arterial embolization [13,16] or partial nephrectomy [10]. Nephrectomy should also be avoided if possible, given that renal involvement is usually bilateral [3].…”

Section: Discussionmentioning

confidence: 99%

Symptomatic Unilateral Renal Angiomyolipoma in a Child with Tuberous Sclerosis

et al. 2001

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We report a case of unilateral renal angiomyolipoma in a 10-year-old boy with tuberous sclerosis. The case is worthy of attention because the symptoms occurred at an early age and because the lesion was unilateral, the latter confirmed at 11-year follow-up. The presentation and treatment are discussed, and the pertinent literature is reviewed. The case demonstrates that it is of clinical importance to monitor renal involvement in children with tuberous sclerosis.

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Ruptured hepatic angiolipoma in tuberous sclerosis complex

Huber

Treutner

Steinau

et al. 1996

Langenbecks Arch Chir

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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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Angiomyolipoma causing life-threatening hematuria in a child with tuberous sclerosis

Cited by 8 publications

References 3 publications

Soft Tissue Tumors of Uncertain Origin

Soft Tissue Tumors of Uncertain Origin

Symptomatic Unilateral Renal Angiomyolipoma in a Child with Tuberous Sclerosis

Ruptured hepatic angiolipoma in tuberous sclerosis complex

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