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Active surveillance (AS) of sporadic renal angiomyolipomas (AML) is under-utilised because of an old dogma fearing a life-threatening retroperitoneal hemorrhage when tumour size exceeds 4cm. The objective of this study was to report the outcome of AS in patients with sporadic AML greater than 4cm.The results of AS in 35 patients managed for sporadic renal AML greater than 4cm were analysed. During AS, tumour growth, occurrence of new symptoms and/or complications, discontinuation of AS protocol, reason for discontinuation as well as subsequent treatment options were reported.Within a median follow-up of 36 months, 16 (46 %) patients discontinued AS at the end of the study period (mean follow-up 55±66, median 36 months). Patients who discontinued AS were more symptomatic at diagnosis but had similar age, mean tumour size and sex ratio. Active treatment-free survival was 66 % at 5 years. Retroperitoneal hemorrhage was reported in 3 (8.5 %) patients. None of these bleedings required transfusion or monitoring in an intensive care unit. Other reasons for discontinuation were pain (37 %), patient preference (19), changes in the radiological appearance of the tumour (19 %), and hematuria (6 %).This study showed that AS in AML bearing patients was feasible even in the setting of tumours larger than 4cm. More than 50% of the patients were still on AS at 5 years. Discontinuation of AS was not related to bleeding complications in most cases.3.

Hereditary predispositions to adult kidney tumors involve around 5% of tumors and include a dozen of autosomal dominant syndromes. The most frequent tumors encountered in these setting are clear cell renal cell carcinomas, papillary renal cell carcinomas, chromophobe renal cell carcinomas and angiomyolipomas. Their detection is essential in order to adapt individual care and perform genetic screening of at-risk relatives, especially in the national french network PREDIR, labeled by the National Cancer Institute and dedicated to hereditary predispositions to kidney tumors. Targeted genetic analysis, which was guided in particular by the renal tumor subtype, has recently evolved into genetic analysis using panels of genes. Pathologist contribution's remains however central in the diagnosis of hereditary forms since we currently have immunohistochemical biomarkers that allow us to diagnose two specifically hereditary entities: hereditary leiomyomatosis and renal cell carcinoma associated-renal cell carcinoma, associated with a loss of fumarate hydratase and succinate dehydrogenase-deficient renal cell carcinoma associated with a loss of succinate deshydrogenase B expression. These diagnoses must however be confirmed by the identification of pathogenic germline variation in the corresponding genes. Improvement of kidney tumors characterization has also lead to identify new subtypes, expanding the algorithm of renal tumors associated with hereditary setting. Here we aim to review all subtypes of adult renal tumors encountered in predisposition syndromes.

To describe in a large paediatric cohort the characteristics of hypopigmented and depigmented (hypochromatic and achromic) macules with no clear diagnosis but potentially evocative of tuberous sclerosis (TS).This was a retrospective multicentre study performed between 2010 and 2017 at a reference centre for rare skin diseases; it included all children consulting for hypochromic and achromic macules. A descriptive analysis was made of the characteristics of macules with no clear diagnosis, enabling them to be classified in three secondary groups: TS certain, TS ruled out, TS uncertain.Of the 3300 children seen during this 7-year period 7,265 were consulting for hypochromic or achromic macules, with no clear diagnosis in 18 cases: 7 girls and 11 boys of median age at 7.21 years (range: 4 months to 16 years and 7 months). The lesions were congenital in 7 cases. The number of macules varied, with over 20 in some cases. The majority were in the form of ash-leaf spots, followed by the oval form. Two children were diagnosed at clinical examination, and 16 underwent it is not examinations, resulting in a diagnosis of certain ST in 6 of these cases. No particular characteristics of the macules appeared to guide the clinical examination towards ST or isolated lesions. Café-au-lait spots were more frequent in the group in which ST was ruled out than in the other two groups: 67% vs. 33% and 33%. Neurologic involvement was more common in children with certain or uncertain ST than in children in whom ST was ruled out (83% and 67% vs. 11%).No identified characteristics of stains enabled the clinical examination to confirm or rule out tuberous sclerosis. Screening for acute any signs of ST is essential. Diagnostic efficacy is enhanced by additional exams.

Bourneville's tuberous sclerosis or Tuberous Sclerosis Complex (TSC) is an autosomal dominant hereditary phakomatosis associated with angiomyolipomas (AML) of the kidney. The aim of this study was to identify the prevalence of TSC in patients diagnosed and cared for AML in our department of urology.All the patients with AML were included between March 2009 and June 2016 in a French university hospital. Each patient was reviewed in consultation with a clinical examination and imaging. Specific clinical criteria were used to refer patients to genetic analysis. Patients with a high TSC probability had a genetic analysis to search TSC1 and TSC2 genes mutations.In all, 28 patients were included and 3 (11%) were diagnosed TSC. The median age of the patients was 62 years (36-82 years). The most frequent clinical criteria were facial angiofibromas in 7 patients (25%). Among the 8 patients (29%) with evocative clinical criteria, a mutation of the TSC1 and TSC2 genes was identified in 3 patients (11%) with a diagnosis of TSC made before the AML diagnosis.In this study, 8 patients (29%) presented clinical criteria suggestive of TSC, preferentially dermatological. The diagnosis was confirmed by screening TSC1 and TSC2 genes mutations in 3 patients (11%), nevertheless prevalence of TSC is most probably underestimated by the genetic mosaïcisme of this pathology.

Renal epithelioid angiomyolipoma (E-AML) is a rare mesenchymal tumor of the kidney included in the family of tumor with perivascular epithelioid cell differentiation (PEComas) and is frequently associated with tuberous sclerosis complex. Since its clinical and radiological features are not specific, the diagnosis remained mostly pathological. Microscopically, E-AML demonstrate proliferation of more than 80% of epithelioid cells with atypia, often associated with necrosis, hemorrhage, mitotic activity and vascular invasion. Tumor's cells are immunohistochemically positive for melanocytic and smooth muscle markers. The pathologic prognostic predictors are not well known and the malignancy remained based on the identification of distant metastasis. We herein report a case of E-AML diagnosed in a 58-year-old woman and review analysis in the literature to discuss the prognostic indicators of malignancy useful for risk stratification of E-AML and to manage the patients.

Kidney diseases are frequent, but most of the time, they develop unnoticed. This paucity of symptoms may lead to delayed diagnosis with important consequences on their outcome. Nevertheless, specific systemic signs such as skin lesions, joint pain or electrolytes disturbances may sometimes alert the clinician and direct the diagnosis to an underlying nephropathy. A high awareness of clinicians is warranted to recognize these red flags and diagnose these diseases early, as illustrated by two clinical cases discussed in this article.

L'angiomyolipome épithélioide est une forme rare d'angiomyolipome à potentiel malin, considéré récemment par l'OMS comme une entité à part dans la classification des tumeurs rénales. Cette lésion pose un problème dans le diagnostic différentiel avec les carcinomes à cellules claires. Il n'y a pas de critère spécifique clinique ou radiologique caractérisant cette tumeur. L'immunohistochimie en révélant la positivité des cellules épithélioide au marqueur HMB45 est essentielle au diagnostic. Le traitement doit être discuté en concertation pluridisciplinaire.

Les angiomyolipomes épithélioïdes rénaux (AMLeR) sont des tumeurs rares (identifiées chez moins de 0,1 patients pour 1000 habitants) et représentent 8% des angiomyolipomes (AML) opérés. Il a longtemps été considérécomme un hamartome mais plusieurs articles récents font penser qu'il s'agir d'une tumeur dérivant de cellules épithélioïdespérivasculaires. L'angiomyolipome épithélioïde est une forme rare d'angiomyolipome à potentiel malin, composé decellules épithélioïdes posant des problèmes de diagnostic différentiel avec les carcinomes à cellules rénales. L'immunohistochimie,en révélant la positivité des cellules épithélioïdes au marqueur HMB45 est essentielle au diagnostic. Les auteursrapportent l'aspect tomodensitométrique et histologique d'angiomyolipomes épithélioïdes chez deux patientes.

L'angiomyolipome rénal est une tumeur rénale bénigne ayant une composante graisseuse. À travers une série de 8 cas nous montrons les critères diagnostiques et les particularités de prise en charges de l angiomyolipome rénal. Notre étude porte sur 8 cas d'angiomyolipomes rénaux durant une période de 4 ans, explorés par une échographie et une tomodensitométrie abdominales. le diagnostic a été retenu sur les données de l'imagerie dans tous les cas. Deux patients suivis pour une sclérose tubéreuse de Bourneville depuis l'enfance. L'âge moyenest de 42 ans. L'échographie aretrouvé un aspect hyperéchogène hétérogène dans tous les cas. La tomodensitométrie a permis la détection de la composante graisseuse. Le diagnostic radiologique de l'angiomyolipome rénal repose essentiellement sur la détection de la composante graisseuse. L'association à une sclérose tubéreuse de Bourneville est un argument diagnostique supplémentaire.

INTRODUCTION: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting mainly young women. BACKGROUND: The respiratory manifestations are characterized by a progressive cystic destruction of the lung parenchyma. Extrapulmonary involvement includes benign renal tumours called angiomyolipomas and abdominal lymphatic masses called lymphangioleiomyomas. At the pathological level, the cellular proliferation found in LAM is in part due to the presence of mutations in the tumour suppressor genes TSC1 and TSC2 (Tuberous Sclerosis Complex). These mutations lead to the activation of the mTOR pathway, which is currently the main therapeutic target. mTOR inhibitors such as sirolimus or everolimus have shown a beneficial effect on the decline in pulmonary function and a reduction of angiomyolipoma size, but are necessary in only some patients. PERSPECTIVES: LAM cells have migratory properties mediated by the formation of new lymphatic vessels. They are also able to secrete metalloproteases, which enhance their invasiveness. Moreover, the expression of estrogen and progesterone receptors by LAM cells suggests a possible role for sex hormones in the pathogenesis of the disease. CONCLUSION: A better understanding of mTOR-independent mechanisms would allow the development of novel therapeutic approaches.

The diagnosis of angiomyolipoma with tumor thrombus from the renal vein into the inferior vena cava is rare, especially during pregnancy. We report the case of a 31-year-old pregnant woman who was diagnosed with a 9 cm angiomyolipoma of the right kidney with inferior vena cava thrombosis, managed by active surveillance during pregnancy and delayed surgery after childbirth.

L'angiomyolipome est la tumeur bénigne la plus fréquente des masses dolides du rein, elle représente un cadre de fréquence de 1 à 3% des tumeurs du rein, sa composition histologique est faite de trois contingents: graisseux, fibres musculaires lisses et vasculaires a des proportions variables, elle sévit sur un cadre sporadique et peut s'exprimer dans un cadre congénitale comme manifestation de la sclérose tubéreuse de Bourneville. Nous rapportons l'expérience du service d'Urologie B du CHU IBN Sina de Rabat dans la prise en charge de huit cas d'angiomyolipome sur une période s’étalant sur six ans en précisant les manifestations cliniques, données de l'imagerie, le résultat histologique et la conduite thérapeutique. Les manifestations cliniques ne lui sont pas spécifiques, elle peut se manifester par des lombalgies, crise de colique néphritiques, hématurie, masse palpable dans le flanc. La TDM reste l'examen radiologique le plus sensible en mettant en évidence la présence de graisse au sein de la masse, l'histologie confirme le diagnostic et il n'y a aucun consensus qui régit la prise en charge de ce type de tumeur, on peut admettre une surveillance pour les petites masses de moins de 4 cm et traité les formes symptomatiques ou qui dépasse les 4 cm par embolisation ou chirurgie partielle ou totale. Les nouvelles thérapeutiques focales peuvent révolutionner la prise en charge de ce type de tumeur bénigne, mais les études sont toujours en cours.

We report the case of a 63-year-old woman presenting a 26cm right renal angiomyolipoma with intratumoral arteriovenous fistula responsible for a high-output right heart failure. A radical surgical treatment after preoperative embolization allowed rapid improvement of cardiac symptoms with an uneventful postoperative course.

Renal tumors associated with hereditary diseases are a rare entity characterized by many renal tumors and other manifestations such as pulmonary, neurological and dermatological expressions. The management requires a close collaboration between surgical specialties and geneticists. Precocious, bilateral and/or multifocal tumors call to mind a hereditary disease. Early diagnosis and screening are essential to optimize a strict observation and a most conservative treatment.

Resume But Presenter notre experience de l’embolisation en urgence ou programmee des angiomyolipomes. Patients et methodes Etude retrospective (1999–2009) incluant 20 patients, parmi lesquels cinq avaient une hypothyroidie. Groupe I Embolisation en urgence : 11 patients ages de 61,4 ± 15,6 ans ; AML de 8,2 ± 2,8 cm presentant une hemorragie retroperitoneale par rupture spontanee etaient traites par embolisation. Deux etaient en choc hemorragique. Une transfusion de 3,4 culots/patient a ete faite chez cinq patients. Groupe II, embolisation preventive : neuf patients, âges de 58,3 ± 15,2 ans, tous asymptomatiques, ayant des AML de 5,2 ± 2,2 cm traites par embolisation unilaterale. Resultats Groupe I : l’embolisation a ete efficace chez 100 % des patients. Aucun incident peroperatoire. A un mois, la reduction du volume tumoral etait de 40 %. A huit mois, un patient a subi une nephrectomie a cause d’une nouvelle rupture, et un autre, une deuxieme embolisation a 14 mois. Le resultat technique etait maintenu apres 18 mois dans 83 % des cas. Deux patients ont developpe une HTA controlee par monotherapie et cinq avaient des sequelles renales ischemiques limitees. Groupe II : aucun incident peroperatoire et aucune complication postoperatoire. Un mois apres embolisation, la reduction du volume tumoral etait de 23 %. Apres 24 mois, les patients etaient asymptomatiques. Une seule re-embolisation a ete faite a 20 mois (duplicite arterielle). Des sequelles renales ischemiques limitees ont ete rapportees chez un seul patient, mais aucune insuffisance renale. Conclusions L’embolisation de necessite restait la methode de choix en urgence avec d’excellents resultats et peu de complications a distance. L’embolisation programmee prevenait efficacement le risque hemorragique, sans impact sur la fonction renale, avec un cout economique faible par rapport a une hospitalisation et une prise en charge en urgence. La signification de l’association AML–hypothyroidie observee dans notre serie necessite une confrontation a des cohortes plus importantes.

Angiomyolipoma is the most frequent benign renal solid tumor. Because of the lack of fat component on the CT scan, diagnosis of this tumor is hard and can require percutaneous biopsy of unknown renal tumor. The follow-up of the poor fat CT scan component AML (PFCT AML) is uncertain.Five hundred percutaneous renal biopsy under tomodenstitometry have been realised between 1998 and 2008. There was 41 PFCT AML on the 500 biopsy. By definition, a PFCT AML is an AML where the diagnosis is done on a percutaneous biopsy but where there was no fat component on the first CT scan. We studied and compared clinical, tomodensitometric and histologic parameters of these 41 patients (mean age: 56, 9±11.04; sexe rate M/F: 6/35) where renal AML was diagnosed on percutaneous renal biopsy but without fat component on CT scan. Average size was 26.44±14.68mm. We phone-called 16 patients for the long-term follow-up. Average follow-up was 41±28.3 months. For four patients on 16, initial diagnosis was done in front of local symptoms, for one of the 16 diagnosis was done in front of general symptoms, for one of the diagnosis was done during Bourneville tuberous sclerosis evolution and 10 of the 16 was done fortuitously.After review of the initial CT scan, fat density was found on 24% of them. Ten percent was epithelioid angiomyolipoma. Four renal biopsy on 41 (10%) was epithelioid AML. No epithelioid AML had fat component after the second look of the CT scan. Among the 16 patients who were phone-called, three (19%) underwent a complication. Two had abdominal pain and was treated medically. Initial sizes were 26 and 30mm. Only one patient must be operated by radical nephrectomy for acute hemorrhage. Initial size was 45mm. No neoplasic degeneration was identified for those 16 patients.In our study, the PFCT AML rate was 8.2%. In 25% cases, CT scan read-through shown a fat component and could help for the diagnosis. PFCT AML evolution seems to be the same as a classic AML. Conservative treatment had a good covering because there was no death and no malignant evolution. However, we found 10% of epithelioid angiomyolipoma in which malignant risk is high. PFCT AML diagnosed on renal percutaneous biopsy of unknown renal tumor requires the same management than the classic AML.

To present our experience with emergency or programmed embolization of angiomyolipomas.The retrospective study 1999-2000 included a total of 20 patients with AML, five of whom had hypothyroidism. Group I emergency embolization: 11 patients age being 61.4 ± 15.6 years and the size of AML 8.2 ± 2.8 cm presented retroperitoneal hemorrhage from spontaneous rupture. Two had a hemorrhagic shock. A transfusion of 3.4 blood units per patient was performed for five patients. A clinical and radiological follow-up was done by scanning during the first week and in one month. Group II preventive embolization: nine patients, with age between 58.3 ± 15.2 years and tumor size 5.2 ± 2.2 cm, all asymptomatic. All successfully received a unilateral preventive embolization. A scan was performed one month later.Group I: the embolization was effective in 100% of patients. No intraoperative incident was reported. After one month, the reduction in tumor volume was 40%. At eight months, a patient underwent nephrectomy because of a new fracture, and another a second embolization after 14 months. The technical result was maintained in 83% of cases after 18 months. Two patients developed HTA after embolization controlled by a single treatment, and five had limited renal ischemic sequels. Group II: no intraoperative incidents and no postoperatively complications have been reported. One month after embolization, the reduction in tumor volume was 23%. After 24 months, patients remained completely asymptomatic, no spontaneous bleeding has been reported, no surgery has been performed, and no HTA has been described. Only one re-embolization was done at 20 months (artery duplicity). Limited renal ischemic sequels were reported for one patient but no renal failure.The required embolization became the method of choice in emergency with excellent results and few complications at distance. Programmed embolization effectively prevented the risk of bleeding, without impact on the renal function, with a low economic cost compared to hospitalization and emergency care. The significance of the observed AML--hypothyroidism association in our series requires a confrontation with more important cohorts.

Renal hybrid tumors (HT) are characterized by the association of both oncocytes- and chromophobe-cells within the same tumor. They have been reported in patients with Birt-Hogg-Dube (BHD) syndrome. The aim of this report was to describe two cases of HT and summarize recent literature.Case study was summarized from the patient's medical chart. Review of literature was performed using the National Center for Biotechnology Information (NCBI) database.Two patients were diagnosed with multiple but small tumors of the kidney, and were treated with partial nephrectomy. Pathological analysis of these tumors showed oncocytoma-like and chromophobe-like cells intermixed in the same stroma.HT may constitute a spectrum of tumors between renal oncocytoma and chromophobe renal cell carcinoma. From a pragmatic management perspective, it would be appropriate to consider such tumors as chromophobe carcinoma. In case of HT, a genetic study for BHD syndrome can be proposed to family relatives.

The purpose of this study was to present the role of embolization in the treatment of kidney angiomyolipomas (AML), for preventive care or in case of bleeding.From March 1995 to March 2007, 22 AML in 21 patients (16 women and five men, mean age 38 years) were embolized: five AML were treated for hemorrhagic shock emergency and 16 were preventive embolization. Eight AML were discovered incidentally and nine patients had pain or hematuria. The average size of AML was 70 mm (20 to 130 mm). The embolization was performed with coils or embospheres.Three patients have not been embolized because of renal failure catheterization. The embolization was effective in 100% of patients treated in emergency. The six patients cared for gross hematuria and pain were asymptomatic after embolization. The average tumor size reduction was 32% with a mean time of 53.2 months (five to 101 months). Three patients (14%) have benefited from surgery remotely.Embolization is the best technique for treating bleeding secondary to AML. In preventive treatment or symptomatic, AML embolization is a treatment to offer before considering surgery.

Angiomyolipoma is generally a benign and noninvasive tumor. We report a case of angiomyolipoma with tumor thrombus from the renal vein into the inferior vena cava suggesting a malignant disease.

Renal epithelioid angiomyolipomas (ReAML) are rare tumors (identified in less than 0,1 per thousand in general population) and represent 8% of operated angiomolipomas (AML). The diagnostic is histological, with an epithelioid cell component among the typical AML cells. ReAML are tumors derived from perivascular epithelioid cells (PEComa). There are benign PEComas, potentially aggressive PEComas and malignant PEComas. Most malignant PEComas are ReAML. There are two ReAML clinical entities, sporadic or associated to Tuberous Sclerosis Complex (TSC). ReAML are unique, localized and sporadic solid tumors of the kidney of variable size that can be revealed as classical AML with local symptoms or a complication (hemorrhage). Revelation mode is mostly radiologic. ReAML are fat-poor on CT-scan. They can be misdiagnosed with renal cell carcinoma (RCC). (One third of ReAML are malignant with a locoregional, nodal or metastatic evolution that can lead to death. ReAML treatments are multimodal depending of histology, clinical-radiological entity, evolution and the patient. Partial nephrectomy or follow-up are the benign entity treatment. Radical nephrectomy eventually followed by doxorubicine or rapamycine treatments are recommended for potentially aggressive and malignant entities.

To explain the high incidence of misdiagnosis of angiomyolipoma (AML) prior to surgery.Between 1989 and 2007, 2,657 patients were operated for a renal tumor at Dupuytren hospital in Limoges and at Cochin hospital in Paris. In 85 cases (3.2%), tumors were AMLs on pathology. The group of patients in which the diagnosis was done preoperatively was compared to the one in which the diagnosis was missed.Mean age of patients was 57-years-old and the sex-ratio was five women for one man. The mean size of AMLs was 5.4 cm. The patients were symptomatic in 46% of cases (39/85). The diagnosis of AML was ignored preoperatively in 62 patients (73%). In multivariate analysis, the small size of the AML, low proportion of fat and male sex were significantly associated with misdiagnosis of AML (p0.001, p0.018 and p0.008, respectively).The incidence of misdiagnosis of AML preoperatively is high. The diagnosis seems particularly difficult when the tumor is small or contains a small proportion of fat. In addition, this study highlights that the diagnosis of AML is frequently ignored in men. The increased resolution of CTscan and the use of preoperative biopsies for tumors less than 4 cm could be helpful to decrease the incidence of useless surgery of AMLs.

Angiomyolipoma is a rare benign mesenchymal tumor. It often arises in the kidney and in association with tuberous sclerosis or lymphangioléiomyomatosis. Extrarenal locations have been described, especially in the liver. Only a few cases have been described in the head and neck region and these are usually not associated with tuberous sclerosis or lymphangioléiomyomatosis.We report a case of angiomyolipoma located in the parotid gland.A 43-year-old man consulted for treatment with a slow-growing nodule located in his right parotid gland. Ultrasound examination and magnetic resonance imaging revealed a well-limited lesion. Fine needle aspiration cytology was not suspicious. Partial parotidectomy was performed and the tumor showed the characteristic appearance of angiomyolipoma, with an admixture of fat smooth cells and tortuous thick-walled blood vessels. Genetic analysis showed anomalies on chromosomes 7 and 18.This article discusses the presentation and management associated with this exceptional tumor.

Renal tumours, in tuberous sclerosis, are mainly benign and correspond to angiomyolipomas, multiple and bilateral, sometimes enormous with an hemorrhagic risk, frequently associated with cysts of variable size and number and seldom with high metastatic carcinomas. Chronic renal failure is rare and related to focal glomerulosclerosis secondary to hyperfiltration due to nephronic reduction owing to surgery or to tumour invasion, in particular cysts. Data about 65 patients with tuberous sclerosis and chronic renal failure were collected thanks to a national survey. They were females in 63.1% of cases, with a mean age of 29, 2 years at the time of the diagnosis of tuberous sclerosis, revealed in near half of cases by renal involvement. There were angiomyolipomas in 23.1% of cases, cysts in 18.5%, both in 53.8% and malignancies in 12.3%. All but one of the 48 patients with end stage renal failure were treated by dialysis; 20 were transplanted, with good results.

The purpose of this study is to present the role of embolization in the treatment of renal angiomyolipoma (AML) in cases of hemorrhage and to prevent bleeding.Over a period of 10 years, 35 AMLs in 34 patients, recruited in two medical centers, were treated with embolization: 16/35 AML were treated urgently to stop bleeding, and 19/35 AML had preventive embolization. Six patients were completely asymptomatic and 13 had a history of previous hematoma or flank pain. Catheterization was highly selective in all cases (coaxial microcatheter in 19 cases), and for embolization we used nonresorbable microparticles, coils, and alcohol.When patients presented with acute bleeding, embolization was efficient in 80% of cases; another embolization was necessary in two cases, and surgery in two others. In six of these cases, surgery was planned and done at a later date. When treatment was preventive, one embolization was necessary in 17 cases, and two embolizations per case were necessary in the other two. Over a period of 18 months of follow-up, we observed a 28% decrease in tumor volume; four patients were treated by surgery at a later date.Embolization is the technique of choice to treat a bleeding AML urgently. When preventive treatment is considered, in symptomatic or asymptomatic AML, embolization can be an alternative for surgery, but more data is needed to specify its proper place in the management of these tumors.

Tuberous sclerosis is a rare inherited disease which can involve several organs. Renal involvement is one of the most severe manifestations of the disease. We analysed in this study renal involvement of tuberous sclerosis.We studied retrospectively nine cases of tuberous sclerosis with renal involvement diagnosed between 1994 and 2005. The diagnosis was based on the presence of typical extra-renal manifestations and familial history of tuberous sclerosis.There were eight females and one male. Their mean age was 40.8+/-16.4 years (range: 15-62). At first presentation, we noted hypertension in three cases, proteinuria in seven cases, hematuria in eight cases and renal failure in five cases (end stage renal failure in four cases). By ultrasonography, we found angiomyolipoma in one case, cysts in four cases and angiomyolipoma associated to cysts in four cases. Nephrectomy was performed in two patients, in one case for hydronephrosis secondary to urolithiasis and in the other for hemorrhagic complication. In the later, pathological examination revealed renal carcinoma. At last evaluation, two patients were lost to follow-up, one had normal serum creatinine, five were on renal replacement therapy and the latter died from sepsis following kidney removal.In our patients, renal disease was diagnosed late at chronic renal failure stage. Patients with end-stage renal failure require dialysis and renal transplantation, but we recommend binephrectomy after starting dialysis and before transplantation due to the risk of cancer and bleeding related to angiomyolipomas.

A 40 year-old 2nd gesta pregnant woman (34.5 weeks of amenorhea) was admitted to hospital for abdominal pain and arterial hypotension which were rapidly related to a retroperitoneal haematoma due to left kidney bleeding. Emergency cesarean delivery under general anaesthesia was undertaken because of foetal distress. Exploration of the retroperitonal space after foetal extraction confirmed the presence of a large haematoma and abnormal left renal morphology. The retroperitoneal space was drained without any further intervention. Subsequently, abdominal and thoracic computerised tomographic examination showed bilateral dysplasia of the kidneys and pulmonary cysts consistent with the diagnosis of renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. The case report is of interest because of the circumstances of discovery of the disease and because nephrectomy was not necessary to control the bleeding of the left kidney. Six months after the incident the patient and the child are in good condition.

Since 1998 new entities have surfaced in renal tumor classification and have been included in the WHO 2004 classification. In this article, we will discuss the following entities: multilocular clear cell renal carcinoma, Xp11 translocation carcinoma, low grade mucinous tubular carcinoma, epithelioid angiomyolipoma, benign mixed epithelial and stromal tumor. We will investigate new concepts of hybrid oncocytoma and chromophobe renal cell carcinoma and the syndrome of Birt-Hogg-Dube which is associated to kidney tumors. At least, we will touch on new elements in the Bellini carcinoma definition.

The aim is to perform a literature search on the role of pregnancy in the rupture of renal angiomyolipoma. Articles published from 1952 to 2004 in the Medline database were searched using the keywords renal angiomylipoma and pregnancy. Pathologies associated with angiomylipoma (lymphangioleiomyomatosis and Bourneville tuberous sclerosis) were taken into account. Seventy-two cases of association of renal angiomyolipoma and pregnancy were found, out of which 58 presented a haemorrhage. In only 26% of the cases, renal angiomyolipoma had been documented prior to pregnancy. Clinical presentation was similar to cases occurring among non pregnant women: abdominal pains (88%), hypotension or shock (33%) and hematuria (24%). Average size of the rupture was 11,7 cm. Rupture does not occur solely with the first pregnancy but occurred equally during the first, second and third pregnancy. Average gestation age upon occurrence of haemorrhage was 27 weeks with a minimum at ten weeks. Therapeutic strategies at the time of shock or hypotension were total nephrectomy in 79% of the cases, 7% polar nephrectomy, 7% embolisation followed by nephrectomy and 7% abstention. A causal role of pregnancy in the atraumatic rupture of angiomyolipoma is not clearly defined. Nevertheless, many arguments (whether it be abdominal mechanical pressure, hormonal or histological ones) suggest that a pregnancy could increase the risk of renal angiomyolipoma rupture. These patients should have a medical follow-up period at closer intervals during their pregnancy and the postpartum phase.

Frequently found in kidney, angiomyolipoma is a rare mesenchymal tumor when diagnosed in the liver and usually benign composed of proliferative blood vessels, fatty tissue and smooth muscle. We report the case of a 67-year-old woman who underwent a left hepatectomy for a 4th segment tumor unidentified after imaging and fine needle biopsy. Final anatomopathologic examination revealed an epithelioïd hepatic angiomyolipoma with signs of malignant behaviour as vascular and lymphatics embolus and invaded left portal vein thrombosis. During the subsequent 24-month follow-up, no recurrence was observed. A review of the literature found only two cases of malignant hepatic angiomyolipoma with fatal issue, however, their incidence must be underrated because of their scarcity and the difficulty of their diagnosis, which needs immunohistochemical confirmation with HMB 45 in particularly. Advances in imaging and anatomopathology in particular with the concept of PEComa (Perivascular-Epithelioïd Cell) as the unifying feature should lead to the recognition of the various variant patterns and cell types. The latter which are important for a correct diagnosis, in order to obtain reliable data about frequency, possible malignant behaviour and therefore consensus management for hepatic angiomyolipoma.