Renal angiomyolipoma and lymphangioleiomyomatosis in pregnancy

Iruloh, Chibuike; Keriakos, R.; Smith, Daniel J.; Cleveland, Trevor J.

doi:10.3109/01443615.2013.812622

Cited by 16 publications

(12 citation statements)

References 38 publications

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“…For patients with hemodynamically unstable bleeding of AML, emergency arterial embolization or surgery is required [ 19 ]. If patients are hemodynamically stable, conservative management is preferred [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Shen

Gao

et al. 2021

Orphanet J Rare Dis

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Background Lymphangioleiomyomatosis (LAM) is a rare disease that almost exclusively affects women of reproductive age. Patients are warned of the increased risks if they become pregnant. However, information on pregnancy in patients after the diagnosis of LAM is limited. Methods Patients were collected from the LAM registry study at Peking Union Medical College Hospital, Beijing, China. Patients with a history of pregnancy after the diagnosis of LAM were included. Medical records were reviewed, and baseline information and data during and after pregnancy were collected in May 2018. Results Thirty patients with a total of 34 pregnancies after the diagnosis of LAM were included. Livebirth, spontaneous abortion and induced abortion occurred in 10, 6 and 18 pregnancies, respectively. Sirolimus treatment was common (17/34). A total of 6/10, 5/6, and 6/18 patients with livebirths, spontaneous abortions, and induced abortions respectively, had a history of sirolimus treatment. Ten pregnancies (29.4%) had LAM-associated complications during pregnancy, including the exacerbation of dyspnea in 7 patients, pneumothorax in 3 patients (2 resulting in induced abortion and 1 successful parturition), and spontaneous bleeding of renal angiomyolipomas in 2 patients (both having successful parturition). No chylothorax was found during pregnancy. There were six pregnancies in six patients (17.6%) who had a history of livebirth after sirolimus treatment for LAM (all having successful parturition and healthy infants); two of these patients reported exacerbated dyspnea after parturition compared with before pregnancy. Conclusions Patients with LAM, especially those taking sirolimus before pregnancy, were at a higher risk of spontaneous abortion. Complications such as pneumothorax, bleeding of renal angiomyolipoma, and exacerbated dyspnea during pregnancy were common. In patients without spontaneous abortion, sirolimus discontinuation before or during pregnancy did not lead to increased adverse neonatal outcomes.

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Section: Discussionmentioning

confidence: 99%

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Shen

Gao

et al. 2021

Orphanet J Rare Dis

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“…AMLs during pregnancy are characterized by faster growth, potential invasiveness, and an increased risk of rupture with massive retroperitoneal hemorrhage [28,29]. To reduce the risk of further bleeding or rupture in labor, a cesarean section is recommended before full term [30]. There have been 2 case reports antenatal AML embolization.…”

Section: Discussionmentioning

confidence: 99%

Varying Outcomes among Patients with Large Angiomyolipomas according to the Treatment Method

Park

Kim

et al. 2021

Urol Int

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Purpose: This study aimed to evaluate the outcomes of large angiomyolipoma (AML) treatment by selective arterial embolization (SAE) versus nephron-sparing surgery (NSS) using a robotic surgical system. Materials and Methods: Between January 2011 and June 2018, we retrospectively reviewed 25 patients who underwent robot-assisted partial nephrectomy (RAPN) or SAE for large AMLs. Ten patients underwent RAPN, and 15 underwent SAE. Patient demographics, AML characteristics, and operative and postoperative clinical outcomes were recorded and analyzed. Outcomes were compared between patients who underwent RAPN and patients who underwent SAE. Specifically, changes in renal function and size were evaluated after the treatment. Results: The mean age of the patients was 52.9 years, and 22 of 25 patients were female. The mean maximum AML diameter on computed tomography was 8.9 cm, and 8 patients had multiple masses. Twenty-two of 25 patients had moderate to high RENAL complexity. Patients who underwent SAE had more symptoms (p = 0.018) and higher RENAL complexity scores (p = 0.013) on average. On average, tumor size decreased by 99% among RAPN patients and by 58% among SAE patients (p = 0.001). Although the mean pretreatment estimated glomerular filtration rate (eGFR) was higher among RAPN patients (99.8 vs. 80.0 mL/min/1.73 m2, p = 0.043), there were no significant changes in eGFR in either group after the treatment. One patient in the RAPN group experienced complications, but the postoperative ileus resolved without intervention. Conclusions: Both RAPN and SAE were effective and feasible treatment options for large AMLs. The AML characteristics and the condition of the patient might be important in determining the appropriate treatment method.

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“…One case was reported with uneventful course after vaginal delivery in a patient treated conservatively for RAML rupture ( 69 ). Other challenging special conditions were reported and successfully managed ( 70 – 74 ).…”

Section: Risk Of Bleedingmentioning

confidence: 99%

The Risks of Renal Angiomyolipoma: Reviewing the Evidence

Seyam

Alkhudair

Kattan

et al. 2017

jkcvhl

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Renal angiomyolipoma (RAML), though a rare benign tumor, may impose a significant morbidity or even mortality due to its unique characteristics and the complications subsequent to its treatment. The classic tumor variant is composed of smooth muscular, vascular, and fatty components. The most straightforward diagnosis is when the fat component is abundant and gives a characteristic appearance on different imaging studies. In fat-poor lesions, however, the diagnosis is difficult and presumed a renal cell carcinoma. Yet, some variants of RAML, though rare, express an aggressive behavior leading to metastasis and mortality. The challenge lies in the early detection of benign variants and identifying aggressive lesions for proper management. Another challenge is when the vascular tissue component predominates and poses a risk of hemorrhage that may extend to the retroperitoneum in a massive life-threatening condition. The predicament here is to identify the characteristics of tumors at risk of bleeding and provide a prophylactic treatment. According to the clinical presentation, different treatment modalities, prophylactic or therapeutic, are available that span the spectrum of observation, embolization, or surgery. Renal impairment may result from extensive tumor burden or as a complication of the management itself. Improvement of diagnostic techniques, super-selective embolization, nephron-sparing surgery, and late treatment with the mammalian target of rapamycin inhibitors have provided more effective and safe management strategies. In this review, we examine the evidence pertaining to the risks imposed by RAML to the patients and identify merits and hazards associated with different treatment modalities.

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Renal angiomyolipoma and lymphangioleiomyomatosis in pregnancy

Cited by 16 publications

References 38 publications

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Varying Outcomes among Patients with Large Angiomyolipomas according to the Treatment Method

The Risks of Renal Angiomyolipoma: Reviewing the Evidence

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