Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis

Schneider-Monteiro, Edison Daniel; Lucon, Antônio Marmo; Figueiredo, André Avarese de; Rodrigues, Aldo Junqueira; Arap, Sami

doi:10.1590/s0041-87812003000200008

Cited by 20 publications

(31 citation statements)

References 21 publications

(23 reference statements)

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“…Most of these tumors appear in the kidney (renal parenchyma), however these can be found in other locations, such as the spleen, liver, uterus and fallopian tubes 14 . About 20% of the cases are associated with tuberous sclerosis, which is an autosomal dominant disease, rare, characterized by the growth of benign tumors in multiple organs (kidney, brain, heart, lungs and skin) [16][17][18] . Signs and symptoms depend on the affected organ and may have Spontaneous rupture of renal angiomyolipoma during pregnancy multiple levels of severity 19 .…”

Section: Discussionmentioning

confidence: 99%

Spontaneous rupture of renal angiomyolipoma during pregnancy

Santos¹,

Proença²,

Reis³

et al. 2014

Rev. Bras. Ginecol. Obstet.

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Renal angiomyolipoma is a benign tumor, composed of adipocytes, smooth muscle cells and blood vessels. The association with pregnancy is rare and related with an increased risk of complications, including rupture with massive retroperitoneal hemorrhage. The follow-up is controversial because of the lack of known cases, but the priorities are: timely diagnosis in urgent cases and a conservative treatment when possible. The mode of delivery is not consensual and should be individualized to each case. We report a case of a pregnant woman with 18 weeks of gestation admitted in the emergency room with an acute right low back pain with no other symptoms. The diagnosis of rupture of renal angiomyolipoma was established by ultrasound and, due to hemodinamically stability, conservative treatment with imaging and clinical monitoring was chosen. At 35 weeks of gestation, it was performed elective cesarean section without complications for both mother and fetus. ResumoO angiomiolipoma é um tumor benigno, constituído por adipócitos, células de músculo liso e vasos sanguíneos. Sua associação com a gravidez é rara e está relacionada com um aumento de complicações, nomeadamente rotura com hemorragia retroperitoneal maciça. O follow-up é controverso em razão do escasso número de casos descritos, no entanto as prioridades são: diagnóstico atempado nas situações urgentes e, sempre que possível, tratamento conservador. O tipo de parto não é consensual e deve ser individualizado caso a caso. Relatamos um caso de uma grávida com 18 semanas de gestação que recorreu ao serviço de urgência por lombalgia direita aguda, sem outros sintomas relevantes. Diagnosticou-se ecograficamente rotura de angiomiolipoma renal e, em decorrência da estabilidade hemodinâmica do quadro, procedeu-se ao tratamento conservador com monitorização imagiológica e clínica. Às 35 semanas de gestação, realizou-se uma cesariana eletiva que decorreu sem complicações maternas ou fetais.

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Section: Discussionmentioning

confidence: 99%

Spontaneous rupture of renal angiomyolipoma during pregnancy

Santos¹,

Proença²,

Reis³

et al. 2014

Rev. Bras. Ginecol. Obstet.

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“…[2][3][4]6 Renal angiomyolipoma is a benign tumor composed of three different types of tissue: fatty, smooth muscle and vascular. 17 Angiomyolipoma often occurs in association with tuberous sclerosis between the second and third decades of life in isolated form. 17,18 It primarily affects women between the fourth and seventh decade of life.…”

Section: Discussionmentioning

confidence: 99%

“…17 Angiomyolipoma often occurs in association with tuberous sclerosis between the second and third decades of life in isolated form. 17,18 It primarily affects women between the fourth and seventh decade of life. The most common signs and symptoms are abdominal pain, palpable abdominal mass, hematuria and other consequences of intra-tumoral hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

“…The most common signs and symptoms are abdominal pain, palpable abdominal mass, hematuria and other consequences of intra-tumoral hemorrhage. 17,19 The latter occurs in approximate 25%…”

Section: Discussionmentioning

confidence: 99%

“…According to the literature, the treatment depends on the lesion size. 17 Between 40% and 80% of individuals with TSC present renal angiomyolipoma with multiple bilateral asymptomatic tumors, which may be associated with cysts and, less frequently, with renal carcinoma. 17,18,20 …”

Section: A B Cmentioning

confidence: 99%

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Tuberous sclerosis complex diagnosed from oral lesions

et al. 2013

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CONTEXT: Tuberous sclerosis complex (TSC) is a genetic disease in the group known as neurocutaneous syndromes, with dominant autosomal inheritance. It is characterized by skin and adnexal lesions and central and peripheral nervous system tumors, with neurological and psychiatric findings. It may affect the heart, kidneys, eyes, face, bones, lungs, stomach and dentition. CASE REPORT: We present the case of a 66-year-old man with dermatological signs that included hypopigmented maculae, confetti-like lesions, shagreen plaque, angiofibromas on nasolabial folds, neck and back, nail dystrophy and periungual fibromas on fingers and toes. An electroencephalogram produced normal results, but magnetic resonance imaging showed a nodular image measuring 1.2 x 1.0 cm close to the Monro foramen, which was similar to cerebral parenchyma and compatible with a subependymal giant-cell astrocytoma. A conservative approach was taken, through control imaging examinations on the lesion for seven years, with absence of any expansive process or neurological symptoms. Abdominal ultrasonography revealed a solid, heterogenic and echogenic mass with a calcified focus, measuring 4.6 x 3.4 cm, in the right kidney, compatible with angiomyolipoma. The patient was treated by means of complete nephrectomy because of malignant areas seen on histopathological examination and died one month after the procedure. This case report illustrates the importance of oral clinical findings such as dental enamel pits and angiofibromas in making an early diagnosis of TSC, with subsequent screening examinations, treatment and genetic counseling. RESUMO CONTEXTO:O complexo esclerose tuberosa é uma doença genética pertencente ao grupo das facomatoses, de herança autossômica dominante, caracterizada por lesões acometendo pele e anexos, além de tumores do sistema nervoso central e periférico, com presença de achados neurólogicos e psiquiátricos, podendo acometer coração, rins, olhos, dentes, mucosa oral e outros órgãos. RELATO DE CASO: Apresentamos o caso de um paciente do sexo masculino, 66 anos de idade, que apresentava diversos sinais dermatológicos, como máculas hipopigmentadas, lesões em confete, placas tipo shagreen, angiofibromas nas regiões cervical, nasolabial e dorsal, distrofias ungueais e fibromas nos dedos das mãos e pés. Embora o encefalograma não tenha mostrado alterações, a ressonância nuclear magnéti-ca apresentou imagem nodular com aspecto semelhante ao parênquima cerebral, medindo 1.2 x 1.0 cm e próxima ao forame de Monro, compatível com astrocitoma subependimário de células gigantes. Abordagem conservadora foi escolhida por meio de controle imaginológico da lesão por sete anos, com ausência de sintomas neurológicos ou processos expansivos. A ultrassonografia de abdômen total revelou massa medindo 4.6 x 3.4 cm, com áreas ecogênicas e heterogêneas, apresentando focos de calcificação no rim direito, com padrão semelhante ao de um angiomiolipoma. O paciente foi submetido a nefrectomia total, devido à presença de áreas de malignidade ao exam...

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Lipomatous Tumors, Liposarcoma, Benign Adipocyte-Containing Nonneoplastic Lesions, and Focal Fatty Changes of the Liver

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis

Cited by 20 publications

References 21 publications

Spontaneous rupture of renal angiomyolipoma during pregnancy

Spontaneous rupture of renal angiomyolipoma during pregnancy

Tuberous sclerosis complex diagnosed from oral lesions

Lipomatous Tumors, Liposarcoma, Benign Adipocyte-Containing Nonneoplastic Lesions, and Focal Fatty Changes of the Liver

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