This is cotinued from Angiomyolipoma in kidney tumors part 1
Clinical Presentation
The prevalence of this tumor in the general population is low. A series of 8,501 autopsies in patients without TSC showed angiomyolipoma in 2 men and 25 women. A Japanese study of 12 970 men and 4971 women through population-based ultrasonography identified 13 men (0.1%) and 11 women (0.22%) with angiomyolipoma, respectively, as confirmed by CT or tissue diagnosis. Angiomyolipoma can occur sporadically (80%) or in association with TSC (20%). There is a female predominance (4: 1) in the sporadic form, but not in tumors associated with tuberous sclerosis. Tuberous sclerosis is an autosomal dominant with incomplete penetrance. Was first described by Bourneville in 1880 in a child with mental retardation, epilepsy and brain damage characteristics sclera (tubers). Bourneville and Brissaud later in 1900, noted the association of the syndrome with renal tumors. Vogt described the classic triad of seizures, mental retardation and sebaceous adenoma. This triad has been replaced by a constellation of findings that establish the diagnosis.
When patients with tuberous sclerosis are monitored, more than half of them are the development of angiomyolipoma. In a combined analysis of Nelson et al. , Suggest that angiomyolipoma associated with tuberous sclerosis, in contrast to sporadic cases is likely to be found in an earlier age (mean age 30 vs 52 years), with larger tumors (8.9 vs 5.4 cm) frequent multicentricity (97 vs 13%) and bleeding (44 vs 14%).
Steiner et al. found that tuberous sclerosis-associated tumors were more likely to grow (67 vs 21%) and require surgical intervention (50 vs 28%) within 4 years of follow-up. De Luca et al. reported on 51 patients with sporadic angiomyolipoma had immediate surgery, or were under observation. Ninety-two percent of patients observed (mean tumor size of 1.5 cm) showed no radiological angiomyolipoma growth during the 5 year period. Large tumors (> 4 cm) were more likely to grow (46 vs 27%) or require surgical intervention (54 vs 7%) than smaller tumors.
Angiomyolipoma was once considered a rare benign hamartoma. However, with increased use of imaging studies, is now recognized as relatively common injury. Can present with symptoms such as flank pain, a tender palpable mass, and gross hematuria or as an incidental finding in radiological studies. Morbidity secondary to angiomyolipoma are related to retroperitoneal hemorrhage (Wunderlich syndrome) and renal failure secondary to invasion of normal kidney tissue.
The radiologic diagnosis has improved in recent years with the advent of helical CT and cut thin section. Identification of fat in renal injury is the key to the radiological diagnosis. Some of the lesions contain a small amount of fat that can not be detected and may lead to nephrectomy. Fat has been reported in RCC tumors, secondary to the invasion and perirenal fat trap. MRI can help differentiate angiomyolipoma from RCC in selected cases and evaluation during pregnancy.
The incidental diagnosis of angiomyolipoma should also promote a series of analysis for tuberous sclerosis. Historically there was an association between renal cell carcinoma, angiomyolipoma and tuberous sclerosis. Renal cell carcinoma clear cell appear to develop at a higher rate in patients with tuberous sclerosis, in general. Eble et al. He also suggested that some tumors diagnosed historically as renal cell carcinoma in this group of patients may have been epithelioid angiomyolipoma, overestimating the association.
Treatment and Prognosis
Renal angiomyolipomas are usually slow-growing tumors, and its morbidity is secondary to its growth. With the lack of randomized trials, there is considerable controversy regarding the indication for the treatment of asymptomatic angiomyolipoma, and organ preservation with partial nephrectomy is a valid approach in this context. Asymptomatic, benign-looking small can be observed. minimal hematuria usually resolves with hydration and bed rest. In this approach, patients should be advised to avoid contact sports and should be followed closely to assess the growth pattern of the lesion. The main reasons for intervention must be a suspicion of malignancy in a lesion with low-fat, relief of symptoms secondary to spontaneous bleeding and the risk of rupture or other complications. European surveys indicate that 1% of people with tuberous sclerosis is on dialysis, emphasizing the importance of preserving renal function.
Surgical excision or core needle biopsy with immunohistochemical staining should be considered when there is no diagnostic accuracy. Symptomatic angiomyolipoma can be managed by angio-embolization or surgical removal. When clinical risk factors such as childbearing age, large tumor, suspicion or difficulty TSC provided with periodic imaging are present, asymptomatic angiomyolipoma can be managed by observation or intervention, according to individual preferences.
Retrospective data show that patients who have symptoms or bleeding are more likely to have larger tumors. Oesterling et al. proposed a threshold of 4 cm to the risk of symptoms and intervention in asymptomatic patients.
Prospective data of De Luca et al. and others suggest that larger lesions may become symptomatic over time, especially in patients with TSC. These studies have also shown that there is no need to treat all asymptomatic lesions large as it can grow slowly without morbidity. Based on the available evidence suggests an intervention in an asymptomatic patient must be based on the overall evaluation of the clinical situation, including tumor size, tumor growth pattern, the presence of tuberous sclerosis patient comorbidity , renal function, pregnancy plans, and compliance.
Angiographic embolization has become a common modality for surgical management of these tumors. In a review of embolization by Nelson et al., Complications were reported in 10% of cases, including abscess (5%) and pleural effusion (3%). A death from respiratory failure in a patient with preexisting pulmonary disease were reported. recurrent or persistent bleeding symptoms need to repeat embolization and surgery in 14% and 16% of patients, respectively.
Clinical Presentation
The prevalence of this tumor in the general population is low. A series of 8,501 autopsies in patients without TSC showed angiomyolipoma in 2 men and 25 women. A Japanese study of 12 970 men and 4971 women through population-based ultrasonography identified 13 men (0.1%) and 11 women (0.22%) with angiomyolipoma, respectively, as confirmed by CT or tissue diagnosis. Angiomyolipoma can occur sporadically (80%) or in association with TSC (20%). There is a female predominance (4: 1) in the sporadic form, but not in tumors associated with tuberous sclerosis. Tuberous sclerosis is an autosomal dominant with incomplete penetrance. Was first described by Bourneville in 1880 in a child with mental retardation, epilepsy and brain damage characteristics sclera (tubers). Bourneville and Brissaud later in 1900, noted the association of the syndrome with renal tumors. Vogt described the classic triad of seizures, mental retardation and sebaceous adenoma. This triad has been replaced by a constellation of findings that establish the diagnosis.
When patients with tuberous sclerosis are monitored, more than half of them are the development of angiomyolipoma. In a combined analysis of Nelson et al. , Suggest that angiomyolipoma associated with tuberous sclerosis, in contrast to sporadic cases is likely to be found in an earlier age (mean age 30 vs 52 years), with larger tumors (8.9 vs 5.4 cm) frequent multicentricity (97 vs 13%) and bleeding (44 vs 14%).
Steiner et al. found that tuberous sclerosis-associated tumors were more likely to grow (67 vs 21%) and require surgical intervention (50 vs 28%) within 4 years of follow-up. De Luca et al. reported on 51 patients with sporadic angiomyolipoma had immediate surgery, or were under observation. Ninety-two percent of patients observed (mean tumor size of 1.5 cm) showed no radiological angiomyolipoma growth during the 5 year period. Large tumors (> 4 cm) were more likely to grow (46 vs 27%) or require surgical intervention (54 vs 7%) than smaller tumors.
Angiomyolipoma was once considered a rare benign hamartoma. However, with increased use of imaging studies, is now recognized as relatively common injury. Can present with symptoms such as flank pain, a tender palpable mass, and gross hematuria or as an incidental finding in radiological studies. Morbidity secondary to angiomyolipoma are related to retroperitoneal hemorrhage (Wunderlich syndrome) and renal failure secondary to invasion of normal kidney tissue.
The radiologic diagnosis has improved in recent years with the advent of helical CT and cut thin section. Identification of fat in renal injury is the key to the radiological diagnosis. Some of the lesions contain a small amount of fat that can not be detected and may lead to nephrectomy. Fat has been reported in RCC tumors, secondary to the invasion and perirenal fat trap. MRI can help differentiate angiomyolipoma from RCC in selected cases and evaluation during pregnancy.
The incidental diagnosis of angiomyolipoma should also promote a series of analysis for tuberous sclerosis. Historically there was an association between renal cell carcinoma, angiomyolipoma and tuberous sclerosis. Renal cell carcinoma clear cell appear to develop at a higher rate in patients with tuberous sclerosis, in general. Eble et al. He also suggested that some tumors diagnosed historically as renal cell carcinoma in this group of patients may have been epithelioid angiomyolipoma, overestimating the association.
Treatment and Prognosis
Renal angiomyolipomas are usually slow-growing tumors, and its morbidity is secondary to its growth. With the lack of randomized trials, there is considerable controversy regarding the indication for the treatment of asymptomatic angiomyolipoma, and organ preservation with partial nephrectomy is a valid approach in this context. Asymptomatic, benign-looking small can be observed. minimal hematuria usually resolves with hydration and bed rest. In this approach, patients should be advised to avoid contact sports and should be followed closely to assess the growth pattern of the lesion. The main reasons for intervention must be a suspicion of malignancy in a lesion with low-fat, relief of symptoms secondary to spontaneous bleeding and the risk of rupture or other complications. European surveys indicate that 1% of people with tuberous sclerosis is on dialysis, emphasizing the importance of preserving renal function.
Surgical excision or core needle biopsy with immunohistochemical staining should be considered when there is no diagnostic accuracy. Symptomatic angiomyolipoma can be managed by angio-embolization or surgical removal. When clinical risk factors such as childbearing age, large tumor, suspicion or difficulty TSC provided with periodic imaging are present, asymptomatic angiomyolipoma can be managed by observation or intervention, according to individual preferences.
Retrospective data show that patients who have symptoms or bleeding are more likely to have larger tumors. Oesterling et al. proposed a threshold of 4 cm to the risk of symptoms and intervention in asymptomatic patients.
Prospective data of De Luca et al. and others suggest that larger lesions may become symptomatic over time, especially in patients with TSC. These studies have also shown that there is no need to treat all asymptomatic lesions large as it can grow slowly without morbidity. Based on the available evidence suggests an intervention in an asymptomatic patient must be based on the overall evaluation of the clinical situation, including tumor size, tumor growth pattern, the presence of tuberous sclerosis patient comorbidity , renal function, pregnancy plans, and compliance.
Angiographic embolization has become a common modality for surgical management of these tumors. In a review of embolization by Nelson et al., Complications were reported in 10% of cases, including abscess (5%) and pleural effusion (3%). A death from respiratory failure in a patient with preexisting pulmonary disease were reported. recurrent or persistent bleeding symptoms need to repeat embolization and surgery in 14% and 16% of patients, respectively.
The advantages of selective embolization including the preservation of renal function, the recovery time of 2-5 days, and the ability to selectively embolize bleeding vessels without major surgery. The effect of embolization to reduce tumor size appears to be resistant to a median follow up of 23 months. Embolization seems to be appropriate for acute bleeding caused by rupture of the tumor, resulting in the stabilization and the elimination of the need for surgical treatment. elective embolization of the tumor may also be appropriate for symptomatic patients with multiple renal tumors in which the preservation of renal function is essential for the prevention of dialysis, and also for patients with limited reserve renal function or poor performance.
Surgery for angiomyolipoma is usually reserved for patients with symptoms unresponsive to conservative, with injury having renal vein or soft tissue invasion, or suspected malignancy in the images. Nelson et al. reported that 19% of patients underwent enucleation or partial nephrectomy, 35% underwent total nephrectomy, 6% underwent embolization, and 40% observation.
Conservative surgery is the preferred modality for the benign nature of tumor. Fazeli-Matin and Novick reported 27 patients with angiomyolipoma that underwent partial nephrectomy, of whom 21 had a contralateral solitary kidney or impaired.
All kidneys were operated after functional surgery, including seven with tumors larger than 12 cm. None of the patients required dialysis after surgery and none had recurrent symptoms angiomyolipoma in a median follow up of 39 months. total nephrectomy should be reserved for patients with a nonfunctioning kidney secondary to angiomyolipoma replacement, with strong evidence of malignancy on radiological or pathological examination, and in situations where other conservative measures have failed.
Angiomyolipoma with thrombus involving the renal vein, inferior vena cava and right atrium has been reported. This may be asymptomatic or may present with fatal thromboembolic consequences. Because of the risks of stroke, the traditional intervention included total nephrectomy with tumor thrombectomy, although there are several reports that angiomyolipoma has been treated with embolization followed by partial nephrectomy to preserve renal function.
Pregnancy can complicate the treatment of young women with angiomyolipoma. While the incidence of bleeding during pregnancy is low, the consequences can be catastrophic, with damage to the mother and fetus.
Hormonal link also suggest that these tumors can grow faster secondary to the changed environment, leading to rupture of the tumor. Optimal diagnostic methods may be limited by pregnancy, and may be difficult to distinguish tumor rupture or ruptured uterus placenta. This gives further credence to the belief that women with known angiomyolipoma larger than 4 cm, intending to conceive should be treated prophylactically to prevent the risk of rupture
Surgery for angiomyolipoma is usually reserved for patients with symptoms unresponsive to conservative, with injury having renal vein or soft tissue invasion, or suspected malignancy in the images. Nelson et al. reported that 19% of patients underwent enucleation or partial nephrectomy, 35% underwent total nephrectomy, 6% underwent embolization, and 40% observation.
Conservative surgery is the preferred modality for the benign nature of tumor. Fazeli-Matin and Novick reported 27 patients with angiomyolipoma that underwent partial nephrectomy, of whom 21 had a contralateral solitary kidney or impaired.
All kidneys were operated after functional surgery, including seven with tumors larger than 12 cm. None of the patients required dialysis after surgery and none had recurrent symptoms angiomyolipoma in a median follow up of 39 months. total nephrectomy should be reserved for patients with a nonfunctioning kidney secondary to angiomyolipoma replacement, with strong evidence of malignancy on radiological or pathological examination, and in situations where other conservative measures have failed.
Angiomyolipoma with thrombus involving the renal vein, inferior vena cava and right atrium has been reported. This may be asymptomatic or may present with fatal thromboembolic consequences. Because of the risks of stroke, the traditional intervention included total nephrectomy with tumor thrombectomy, although there are several reports that angiomyolipoma has been treated with embolization followed by partial nephrectomy to preserve renal function.
Pregnancy can complicate the treatment of young women with angiomyolipoma. While the incidence of bleeding during pregnancy is low, the consequences can be catastrophic, with damage to the mother and fetus.
Hormonal link also suggest that these tumors can grow faster secondary to the changed environment, leading to rupture of the tumor. Optimal diagnostic methods may be limited by pregnancy, and may be difficult to distinguish tumor rupture or ruptured uterus placenta. This gives further credence to the belief that women with known angiomyolipoma larger than 4 cm, intending to conceive should be treated prophylactically to prevent the risk of rupture