Angiomyolipoma Patients Research Articles

Conservative treatment of renal angiomyolipomas in patients with tuberous sclerosis

Renal angiomyolipoma (AML) associated with tuberous sclerosis (TS) presents a treatment dilemma due to multifocal tumors with a potential for significant growth and subsequent hemorrhage. We reviewed the literature and our experience with AML and TS patients to determine the long-term behavior of these lesions. We reviewed 8 patients (16 renal units) with bilateral renal AMLs and comorbid TS. We evaluated their renal function, renal imaging, and clinical course. Patients were followed for a mean of 11.5 years (range 3.5 - 21 years). The records of 8 patients (7 females, 1 male) with a mean age of 33.1 years (range 21 - 54) were evaluated. The mean serum creatinine of these patients at the time of diagnosis was 0.75 mg/dl (range 0.4 - 1.1). Mean serum creatinine at last follow-up was 0.83 mg/dl (range 0.6 - 1.3). The average size of the largest lesion was 13.9 cm (range: 0.5 - 28). Of the 8 patients, 6 received treatment during the course of their disease, including arterial embolization of 7 renal units in 5 symptomatic patients (2 patients needed 2 embolizations). Partial nephrectomy was performed on 2 renal units in 2 patients, and a total nephrectomy was performed in 1971 on another patient. Currently, all 8 patients have stable renal function; 4 patients are asymptomatic with regards to their lesions, while the other 4 patients report transient flank pain adequately controlled with oral analgesics (2 patients with propoxyphene plus acetaminophen 100/650 mg PO t.i.d. p.r.n., the other 2 patients with ibuprofen 600 mg PO p.r.n.). None of the patients experienced life-threatening hemorrhage or required dialysis. Our study and a review of the literature have not revealed an obvious or quantitative risk of morbidity or mortality from renal hemorrhage directly related to AMLs of any specific size in TS patients. Due to multiple lesions and distortion of anatomy it can be difficult to distinguish individual lesions for preemptive treatment in asymptomatic patients. If size criteria alone are used, multiple treatments will be required over the course of the patient's life. Also, preemptive treatment exposes patients to iatrogenic morbidity. Consideration should be given to medical management of AMLs in TS patients with asymptomatic, slowly enlarging tumors that maintain features of an AML. Embolization, partial nephrectomy, or other ablative treatments (i.e. cryotherapy and RFA) can be reserved for symptomatic patients.

Role of low nuclear grading of renal carcinoma cells in the functional profile of tumor-infiltrating T cells.

Tumor-infiltrating lymphocytes (TILs) from biopsies of 9 selected patients with pT1pN0M0 renal cell carcinoma (RCC) were analyzed at the clonal level for phenotypic distribution, cytokine secretion profile and antitumor cell proliferation and cytotoxicity. T-cell clones generated from RCCs were able to produce higher amounts of interleukin-4 (IL-4) and interferon-gamma (IFN-gamma) than the corresponding clones derived from peripheral blood mononuclear cells, thus suggesting a recruitment into tumors of T cells with peculiar functions. Moreover, CD4+ T-cell clones generated from TILs of nuclear grading 2 (G2)-type RCC patients produced significantly higher amounts of IL-4 and IL-10 and lower amounts of IFN-gamma than the corresponding clones generated from G1-type RCC and 2 renal angiomyolipoma (AML) patients. In addition, T-cell clones generated from lymphocytes infiltrating the peritumoral areas of G2-type, but not those from G1-type, RCC patients produced higher and lower amounts of IL-4 and IFN-gamma, respectively, than the corresponding clones derived from intratumoral T cells of the same patients. The proportion of T-cell clones derived from G2-type tumors and proliferating to autologous tumor cells (ATCs) was significantly lower than that of clones generated from G1-type RCC or AML patients. However, irrespective of their source, they exhibited similar cytokine profiles and produced comparable amounts of IL-4, IL-10 and IFN-gamma. Furthermore, the proportion and the production of both IL-4 and IFN-gamma of G2-type RCC-derived T-cell clones with cytotoxic activity against ATC were significantly lower than those of cytolytic clones generated from AML and G1-type RCCs. The concentrations of IL-4, IL-10 and IFN-gamma produced by the cytolytic clones from G2-type RCC were also lower than those produced by their noncytolytic counterparts obtained from the same patients. These data address the association of the nuclear grading of neoplastic cells with different local tumor-specific T-cell responses in RCC.

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 × 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.