Spontaneous rupture of renal angiomyolipoma with tuberous sclerosis during long-term follow-up: a case report]

We present a case of spontaneous rupture of bilateral renal angiomyolipoma (AML) with tuberous sclerosis. A 46-year-old woman was admitted with sudden onset of severe left flank pain. She had been diagnosed to have bilateral AML with tuberous sclerosis 15 years earlier. Four years after the initial diagnosis, spontaneous rupture of right renal AML occurred and right renal embolization of the right renal artery was performed. The treatment for the left renal AML was not performed. Eleven years later in 2000, spontaneous rupture of contralateral renal AML occurred and left renal embolization of the left renal artery was performed. We evaluated the efficacy of selective arterial embolization in right AML and the change of the tumor size during a 10-year follow up after embolization. The right AML had decreased 86% in 11 years. Selective arterial embolization is an effective and safe treatment for AML. We evaluated the natural history of left AML and calculated the doubling time to be about 1,370 days for the first period of 4 years and about 2,075 days for the second period of 11 years. Although the growth change was very slow, we should observe the tumors carefully on computed tomography or ultrasound to prevent life-threatening hemorrhage.     

Nephron-sparing tumorectomy for a large benign renal mass: A case of massive bilateral renal angiomyolipomas associated with tuberous sclerosis

A case of massive bilateral angiomyolipomas (AML) associated with tuberous sclerosis in a 33-year-old woman is reported. She was hospitalized because she had been experiencing abdominal fullness and epigastralgia. Several imaging studies revealed massive bilateral renal tumors and she was diagnosed as having renal AML associated with tuberous sclerosis. Left nephrectomy was carried out after renal arterial embolization for intratumor hemorrhage. Two years after left nephrectomy, nephron-sparing surgery (tumorectomy) for right AML was done because of an increase in the size of the right renal AML and she hoped for a future pregnancy. The left kidney with AML weighed 5700 g and the right AML weighed 1700 g. Postoperative serous creatinine did not differ from that before operation and an increase in the size of the residual tumor was not observed 8 months after operation. We consider that tumorectomy is an effective therapy in patients with a very large tumor involving a solitary kidney.     

Bilateral massive renal angiomyolipoma concurrent with oncocytoma in tuberous sclerosis complex associated with pulmonary lymphangioleiomyomatosis

We describe a patient with tuberous sclerosis complex and massive bilateral renal angiomyolipomas (AMLs) in association with pulmonary lymphangioleiomatosis, who experienced hemorrhagic shock because of rupture of the left renal AML. She was treated with transarterial embolization, followed by bilateral nephrectomy. The total tumor burden weighed 5800 g, making it the largest AML by weight in published reports. The histopathologic examination revealed concurrent oncocytomas with AMLs in the right kidney, indicating a possible shared mechanism in the pathogenesis of these two disorders. This case also represents a rare, but significant, overlap between renal AML, pulmonary lymphangioleiomatosis, and tuberous sclerosis complex.     

Bilateral Renal Cell Carcinoma in a Patient with Tuberous Sclerosis

A case of pure bilateral renal tell carcinoma (RCC) in a 21-year-old female diagnosed as having tuberous selerosis is reported. She underwent a left nephrectomv because of her loss of appetite possibly caused by the tumor compressing her intestines. The preoperalive CI scan showed the presence of adipose tissue in bilateral renal tumors, which is highly suggestive of angiomvolipoma (AML). Histological examination, however, revealed no area or component ot the tumor with features characteristic of AML.     

Clinical presentation of epithelioid angiomyolipoma

OBJECTIVE: Epithelioid angiomyolipomas (AML) of the kidney are malignant tumors with aggressive clinical behavior. METHODS: We reviewed cases of epithelioid AML recently diagnosed at our institution to highlight the spectrum of clinical presentations. RESULTS: In all cases, renal lesions seen on computed tomography were suspicious for renal cell carcinoma (RCC). Histologically, these tumors can resemble RCC. The diagnosis of epithelioid AML was established by positive staining for melanoma and smooth muscle cell markers, and presence of perivascular epithelioid cells. One patient presented with a renal tumor extending into the inferior vena cava to the level of the hepatic veins. Two patients developed recurrent, metastatic disease following nephrectomy. One patient with tuberous sclerosis and multiple, bilateral AML developed an enhancing renal tumor that did not contain any fat densities. A partial nephrectomy was performed and pathology revealed epithelioid AML adjacent to conventional AML. CONCLUSIONS: These tumors are distinguished from RCC by positive immunostaining for melanoma markers and smooth muscle cell markers. They resemble conventional RCC on imaging. Epithelioid AML may be locally aggressive and metastasize.     

Malignant transformation of renal angiomyolipoma: a case report

We report a case of renal angiomyolipoma (AML) with malignant transformation. A 28-year-old woman developed large bilateral renal masses 5 months before admission to our hospital. She was diagnosed with tuberous sclerosis when she was 4 years old. Total nephrectomy of the left kidney was performed, but she died during the operation. Although the focal region of the resected tumor had the appearance of a classic AML, most of the lesion showed a diffuse proliferation of atypical epithelioid cells resembling that in renal cell carcinoma. The epithelioid cells had extremely pleomorphic and hyperchromatic nuclei with frequent mitotic figures, including atypical forms. Immunohistochemical analysis revealed that the atypical epithelioid cells and the typical AML lesions were both positive for HMB-45 but that the former were negative for epithelial and myogenic markers. The smooth muscle cells and thick-walled vessels were focally positive for muscle-specific actins. Furthermore, the atypical epithelioid cells were immunoreactive for p53, whereas the foci of the typical AML were negative. Examination of the microdissected paraffin-embedded tissues revealed p53 mutations in the malignant epithelioid areas in AML but not in the renal parenchyma or typical AML areas. In this case it is proposed that p53 mutation may play an important role in malignant transformation of renal AML.     

Management of renal angiomyolipoma in complex clinical situations.

Renal angiomyolipoma (AML) is associated with complex clinical situations such as tumour in a solitary kidney, bilateral, large or multicentric tumours or those associated with tuberous sclerosis (TS) or pregnancy. Management in these situations may be challenging. Fifteen patients (20 kidneys) were admitted with symptomatic AML over last 10 years. Eleven patients had one or the other complicating factor. Ten patients had a tumour of >10 cm, 4 had TS, 5 had multiple and bilateral tumours, 1 patient was pregnant and 1 had a solitary functioning kidney. With the newer imaging modalities correct diagnosis was possible in 12 cases and renal cell carcinoma (RCC) was suspected in 3 cases. Selective angioembolization (SAE) was done in 3 patients, which successfully controlled bleeding in all. Nephron-sparing surgery (NSS) was performed in 5 patients. Total nephrectomy was done in 4 cases, in 3 due to suspicion of RCC and in 1 due to extensive involvement of the kidney. Three patients with multiple and bilateral tumours were chosen for conservative treatment and none developed recurrence of bleeding on strict follow-up. In a pregnant patient, bleeding was successfully controlled with angioembolization. However, 1 patient with a solitary functioning kidney with large-sized tumour (20 x 18 cm) underwent NSS. In conclusion, the basis of management of AML is preservation of renal tissue, which can be effectively achieved with SAE or NSS. In a solitary functioning kidney, NSS or SAE is the ideal treatment, if feasible. The patients in the TS group are usually more complicated and require life-long follow-up after initial management with NSS or SAE. Pregnant AML patients can be safely managed with SAE. Conservative treatment without any intervention and regular follow-up may be more helpful in some patients with multiple, bilateral extensive tumours.     

Renal epithelioid angiomyolipoma with malignant behavior

The patient was a 22-year-old woman with a history of tuberous sclerosis who complained of abdominal distention. Computed tomography showed bilateral renal masses with minimal fat tissue that were unlike classical angiomyolipoma (AML), as well as enlargement of the paraaortic lymph nodes, pleural effusion, and ascites, similar to other advanced malignant neoplasms such as renal cell carcinoma and renal sarcoma. We performed a left nephrectomy to relieve the symptoms. A pathological examination revealed that the tumor was composed of polygonal epithelioid cells positive for both αSMA and HMB-45, as well as spindle-shaped cells, while immunoreactivity proved the tumor to be an eAML, a subtype of AML with malignant potential. Although the patient was scheduled for postoperative adjuvant chemotherapy, her condition worsened and she died on postoperative day 24 before receiving chemotherapy. We discuss this case with reference to previous reports.     

Intra-sinusal renal angiomyolipoma. Differential diagnosis from renal artery aneurysm

We report a case of atypical renal angiomyolipoma (AML) because of its clinic presentation like colic renal pain, its uncommon intrasinusal location, its low fatty content, and especially because of the diagnostic doubt with a renal artery aneurysm that could not be resolved before the surgery in spite of making Doppler ultrasound, renal one sided arteriography, computed tomography (CT) and magnetic resonance imaging (MRI). We gave up doing a percutaneous needle biopsy because of the location, the association with hematoma and the possibility of a vascular origin. It has been said that diagnostic images, mainly ultrasound and CT, have a high capacity to get a renal AML diagnosis before the surgery. However when there is an atypical characteristics association like we have talked about, dismissing other pathologies is necessary. We believe AML must be considered one of these because of its difficult differential diagnosis.     

Bench Surgery With Renal Autotransplantation for Angiomyolipoma of Renal Sinus

Background

The treatment of angiomyolipomas (AML) of the renal sinus is challenging and often requires nephrectomy. In this study, we report on 5 patients who underwent extracorporeal AML resection and subsequent renal autotransplantation (RA) for renal sinus AML.

Methods

As of May 2013, 5 patients had undergone extracorporeal AML resection and RA for renal sinus AML at our institute. Separation of the AML in renal sinus is performed by combined sharp and blunt dissection. Tissue between AML and renal sinus is ligated where blood vessels cannot be excluded. When complicated with AML of the renal parenchyma, the AMLs were resected using ultrasound guidance.

Results

Renal nuclear scan showed that kidney grafts were perfused uniformly without evidence of segmental infarction at day 7 posttransplantation in all patients. No significant deterioration in renal function and tumor recurrence was noted over a median follow-up of 3 months (range: 1 to 6 months). All patients presented with normal blood pressure values. No intraoperative complications occurred.

Conclusions

Bench surgery and subsequent RA method, due to its advantage of kidney preservation, is a valuable option for renal sinus AML treatment.     

Percutaneous nephrolithotomy with renal angiomyolipomas: a rare challenge

Renal angiomyolipomas (AML) are vascular tumors associated with a risk of spontaneous bleeding. Renal trauma may also initiate such hemorrhage. We present a case in which we initially avoided direct puncture and the possible risk of bleeding through extensive renal AMLs and then subsequently performed a direct puncture through the tumors. A 21-year-old obese male patient with tuberous sclerosis and mental retardation presented to our institution with left renal colic and was found to have a staghorn calculus. A CT scan revealed extensive bilateral renal AMLs. The patient had previously undergone renal angioinfarction for an enlarging right-sided AML, and nuclear renography demonstrated 70% function from the left side. The patient had a nephrostomy access created on the morning of a scheduled percutaneous nephrolithotomy under three-dimensional CT guidance. There was no clinically significant bleeding. Intraoperatively, a second access site was required in order to render the patient stone free. This was done using standard biplanar fluoroscopic technique and traversed an AML. Both tracts were balloon dilated prior to placement of a 34F Amplatz sheath. Postoperatively, the patient had an uneventful recovery. A CT scan performed 1 day postoperatively revealed no retroperitoneal collection. This case demonstrates that renal access can be achieved with remarkable accuracy using 3D CT imaging. Furthermore, although this approach seems most prudent in the case of extensive renal AMLs, fluoroscopically guided renal access and dilation to 34F was not associated with bleeding in this patient.     

Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma

Renal angiomyolipoma (AML) is typically a solid lesion, composed of varying amounts of adipose, vascular, and muscular tissue, lacking an epithelial component. Although it is known that entrapped renal tubules may be observed in AML, presentation as a cystic mass has not been previously reported. We report the clinicopathologic and immunohistochemical features of four cystic renal AML. The lesions were found in 2 male and 2 female patients, ranging in age from 37 to 76 years, none with a history of hormonal therapy. One of the four patients had known tuberous sclerosis, and this patient and 1 other presented with bilateral cystic renal lesions. Grossly, the lesions measured between 1.3 and 4.5 cm in greatest dimension. Histologically, the lesions were each composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) a compact subepithelial "cambium-like" layer of cellular, müllerian-like AML stroma with prominent admixed chronic inflammation; and 3) muscle-predominant AML with associated dysmorphic blood vessels exterior to the cellular subepithelial stroma. Immunohistochemically, the stromal components of all four lesions labeled with HMB-45 and Melan-A, most intensely in the cellular subepithelial stroma, whereas smooth muscle actin and desmin demonstrated the opposite pattern, with greatest intensity in the muscle-predominant AML areas. Immunoreactivity for estrogen and progesterone receptors (ER, PR) and CD10 was present in all cases, with strongest and most diffuse staining in the subepithelial AML cells. The cyst lining cells were positive for pancytokeratin and soy bean agglutinin (the latter characteristic of distal nephron epithelium) but did not label for ER, PR, or melanocytic markers. Although these distinctive lesions may be confused with mixed epithelial-stromal tumor, their lack of female predominance or association with hormone therapy, their distinctive dysplastic blood vessels and disorganized exterior muscular layer, along with their immunohistochemical profile, support their classification as AML with epithelial cysts. Whereas the cysts appear to arise primarily from entrapped collecting ducts, the peculiar subepithelial condensation of AML stromal cells, with morphologic and immunohistochemical features suggesting differentiation toward endometrial stroma, may represent epithelial-induced müllerian differentiation not previously reported in AML.     

Metastatic breast carcinoma to renal angiomyolipomas in tuberous sclerosis

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease manifesting as hamartomatous involvement of several organs, including brain, kidneys, skin, heart, lungs, and retina. We report the first case of a patient with TSC, with large bilateral renal angiomyolipomas (AML), who developed multiple soft tissue masses within her primarily fatty AMLs, resulting from metastatic breast cancer.     

Nephron-sparing surgery in multiple bilateral angiomyolipomas

We report on a 17-year-old white woman with multiple bilateral renal angiomyolipomas (AMLs) in the absence of tuberous sclerosis. Multiple hyperdense lesions were detected in both kidneys by sonography. A computed tomography (CT) scan confirmed mainly fatty tissue. Sparing as much functional tissue as possible, eight AMLs of the right kidney were resected. The largest removed tumour measured 7 x 4 x 2.4 cm. Renal function was completely preserved. An AML is a benign, generally unilateral renal tumour. Treatment is necessary in cases of flank pain, spontaneous bleeding, obstruction by tumour growth and tumours exceeding 4 cm in diameter. Patients who present are often symptomatic due to pain, retroperitoneal bleeding or haematuria. An AML occurs either sporadically or in association with tuberous sclerosis. Bilateral or unilateral multiple AMLs are rare.     

Conservative management of giant symptomatic angiomyolipomas in patients with the tuberous sclerosis complex

OBJECTIVE: To present two patients with the tuberous sclerosis complex in whom giant angiomyolipomas (AMLs) were managed conservatively for >20 years, as large (>4 cm) symptomatic AMLs are generally treated with embolization or nephrectomy because of the risks of haemorrhage. PATIENTS AND METHODS: The first patient initially presented with bilateral renal AMLs replacing >70% of his parenchymal volume, and a large left renal cyst. Since presentation he was hospitalized three times, but has had no transfusions or procedures to treat his AMLs. His renal function has remained stable during this 21-year interval. The second patient initially presented with bilateral renal AMLs that were not amenable to nephron-sparing surgery. After removing her nonfunctioning left kidney, the 24-cm AML in her right kidney was managed conservatively for >20 years, during which she had 44 transfusions and 11 hospitalizations before uncomplicated right nephrectomy and subsequent need for haemodialysis. CONCLUSION: Conservative management of AMLs can preserve renal function for >20 years in patients with tuberous sclerosis at high risk of end-stage renal disease.     

Solitary fibrous tumor of the adrenal gland with renal cell carcinoma and angiomyolipoma at the same time; a case report

Solitary fibrous tumor (SFT) is a neoplasm of pleura and its occurrence in the retroperitoneal space is rare. We report a case of SFT of the adrenal gland associated with ipsilateral renal cell carcinoma (RCC) and angiomyolipoma (AML). A 48-year-old woman was referred to our hospital for a left renal AML. Computed tomography (CT) in our hospital showed a left adrenal mass (25 x 20 mm). Because the adrenal tumor was nonfunctioning, she was followed at outpatient clinic. Four years later, CT showed an increase in the left adrenal tumor size (42 x 30 mm) and a left RCC. Left adrenectomy and partial nephrectomy for RCC and AML were simultaneously performed. Histological examination revealed adrenal SFT and clear cell carcinoma and AML of the kidney. We present a brief review on histological characteristics of retroperitoneal SFT and its occurrence in the adrenal grand region.     

Ruptured renal angiomyolipoma treated by transcatheter arterial embolization: report of two cases

We report two cases of spontaneous rupture of renal angiomyolipoma (AML). In the first case, a 22-year-old woman was admitted with lower abdominal pain. She was diagnosed with rupture of left renal AML. Transcatheter arterial embolization (TAE) was performed for three times to preserve renal function, and the size of AML decreased to 6.5 cm from 10 cm. In the second case (74-year-old woman), the chief complaint was lower abdominal pain. The clinical diagnosis of this patient was rupture of right renal AML. The size of this AML markedly reduced due to TAE. TAE is an effective therapy for rupture of renal AML.     

A case of angiomyolipoma of the renal sinus

The case of a 63-year-old woman with a relatively rapidly growing angiomyolipoma (AML) originating from the right renal sinus is presented. The tumor had enlarged to more than fivefold in volume (7.4 × 6.7 × 10.1 cm) in the 7 years following its detection (5.6 × 3.4 × 4.6 cm) and had significantly pushed aside the duodenum and the inferior vena cava. The tumor was surgically resected together with the right kidney, and pathological examination revealed AML originating from the renal sinus. An AML involving or originating from the renal sinus is exceedingly rare; only 14 cases have been reported. Rapidly growing AMLs of the renal sinus are difficult to differentiate from liposarcoma because of similar findings in imaging studies. It should be noted that AML of the renal sinus can be a differential diagnosis of retroperitoneal fatty mass. Furthermore, an enlarged AML in the renal sinus may push aside other organs, such as the ileum, pancreas, and major vessels. Clinicians should determine whether these patients should be placed under active surveillance or be treated with invasive measures, such as radical nephrectomy.     

Pulmonary angiomyolipoma recurring 26 years after nephrectomy for angiomyolipoma: benign clinical course

Angiomyolipoma (AML), a benign renal tumor, has various clinical forms, and the nature of AML is not sufficiently understood because of few reports of long-term observation. We report a 57-year-old female without tuberous sclerosis who developed multiple pulmonary AML 26 years after a right nephrectomy for renal AML. A computed tomogram demonstrated multifocal round lesions with lipid-like density throughout both lung fields. An open lung biopsy revealed a histological diagnosis of pulmonary AML. An abdominal computed tomogram was also compatible with splenic AML. She has been in good health for 12 years since the lung biopsy over the 38 years since the nephrectomy. This case suggests that multicentric AML can recur at distant organs even after long-term silence in a patient who has a past history of renal AML.     

Management of renal angiomyolipoma: a report of 53 cases

OBJECTIVE: To evaluate the clinical, therapeutic and prognostic aspects of renal angiomyolipoma (AML) in patients either monitored by clinical and radiological follow-up or treated by surgical therapy. PATIENTS AND METHODS: Fifty-three patients with renal AML were divided in two groups; 33 patients in group 1 were monitored by annual clinical and ultrasonographic follow-up and 20 in group 2 were treated with surgical therapy. Two patients had tuberous sclerosis (TS) with synchronous bilateral and multiple lesions. Apart from the patients with TS, there were 38 lesions in group 1 and 25 in group 2. The mean (range) follow-up of group 1 was 60.2 (12-164) months. RESULTS: In group 1, the diagnosis was most often incidental, after ultrasonography performed for symptoms unrelated to AML. In group 2, the suspicion of a malignant renal lesion, and spontaneous tumour rupture with bleeding and perirenal haematoma, were the main indications for surgical treatment. The mean lesion diameter was significantly greater in group 2 (5.4 cm) and in symptomatic patients (8.1 cm). In group 1, 92% of renal AMLs showed no radiographic changes, serious complications or new renal or extrarenal lesions during the follow-up. Only three lesions grew, after 22, 85 and 164 months, respectively. Of the 20 patients in group 2, 14 underwent conservative surgery. CONCLUSION: Small (<4 cm) isolated AMLs, detected incidentally, showed a low risk of developing during long-term follow-up. Such patients may be followed conservatively by ultrasonography every 2 years. Spontaneous perinephric haemorrhage is related to the size of the lesion. When surgery is indicated (by symptoms or diagnostic doubt), a conservative procedure can be performed in most of cases.