Malignant transformation of renal angiomyolipoma

In the present paper, two cases of malignant transformation of renal angiomyolipoma without tuberous sclerosis are reported. Pathological examination revealed that, in both cases, in addition to the areas affected by typical angiomyolipoma, there were areas that contained elevated numbers of perivascular epithelioid cells with prominent nuclear pleomorphism. Immunohistochemical examination revealed that both cases were negative for keratin and epithelial membrane antigen, but were positive for the melanogenesis-related marker HMB-45. Metastatic diseases appeared 40 months after radical nephrectomy in the first case and 18 months in the second case.     

Therapeutic embolization of renal angiomyolipoma: a case report

A case of bilateral renal angiomyolipoma without tuberous sclerosis is reported. A 49-year-old woman was admitted to the general practitioner with a sudden onset of severe left flank pain. An excretory urogram and ultrasonogram revealed an enlargement of the left kidney. She was subsequently referred to our clinic for further investigation and treatment. Computed tomographic scan and magnetic resonance imaging using Tl-weighted image showed several tumors with a fatty, dense area in the bilateral kidney. An arteriogram demonstrated a hypervascular renal mass with aneurysms in her left kidney. Diagnosis of bilateral renal angiomyolipoma was confirmed by percutaneous needle biopsy. Superselective embolization of the tumor was successfully performed, preserving normal renal tissue. Gelatin sponges containing Carboquone (CQ sponge) were used as embolic material. Angiomyolipoma has become relatively easy to diagnose by CT, ultrasound, MRI and so on. However, there are some cases of angiomyolipoma which are indistinguishable from renal cell carcinoma using these modes of testing. Therefore, in selecting a conservative management, we indicated that percutaneous biopsy or open biopsy should be done to confirm the results of the above procedures. Moreover, therapeutic embolization for angiomyolipoma was concluded to be very useful.     

Spontaneous rupture of renal angiomyolipoma: a case report

A 55-year-old woman presented with sudden right lower abdominal pain. Computed tomography demonstrated retroperitoneal hematoma associated with minus-density area, diagnosed as spontaneous rupture of angiomyolipoma. Super-selective transarterial embolization was performed, but anemia and right abdominal pain became worse in spite of conservative therapy including transfusion, indicating re-rupture of the renal tumor. Although partial nephrectomy was planned, right nephrectomy was finally performed because of massive intraoperative bleeding. Accurate diagnosis and prompt treatment are required when life-threatening rupture of renal tumor is suspected.     

Malignant hepatic angiomyolipoma: report of a case and review of literature

Hepatic angiomyolipoma, a constituent of the group of tumors showing differentiation resembling perivascular epithelioid cells, is primarily appreciated in its benign form. Regardless of their location, this family of tumors is characterized by the presence of mature adipocytes, blood vessels, and spindle-epithelioid cells. These tumors also possess similar immunohistochemical profiles, including positivity for melanocytic (HMB-45) and smooth muscle (smooth muscle actin) markers. Here, we present a case of malignant hepatic perivascular epithelioid cells that was initially confined to the liver, treated by resection, and subsequently recurred within and metastasized beyond the liver. We take this opportunity to report the fourth case of malignant hepatic angiomyolipoma (HAML) and review the literature. We will discuss the features that aid in distinguishing between benign and malignant HAML, and their similarities. In summary, the common features of both benign and malignant HAML include the following: the 3 basic histologic components of AML, expression of melanocytic and smooth muscle markers, invasion into adjacent normal parenchyma, and cytologic atypia. The unique features of malignant HAML are as follows: clinical evidence of aggressive behavior such as metastasis or death owing to disease, coagulative necrosis, and loss of CD 117 expression.     

Non-ischemic tumor enucleation for renal angiomyolipoma: a case report

A case of renal angiomyolipoma, successfully treated with non-ischemic tumor enucleation, is reported. A 16-year-old Japanese female visited another hospital with a chief complaint of general fatigue. She was diagnosed with angiomyolipoma of the right kidney, 7 cm in the long axis, which developed exteriorly at the lower pole. A nephrectomy was recommended. The patient visited us for a second opinion. We judged that nephron-sparing surgery was applicable to this case. The patient underwent non-ischemic tumor enucleation using a microwave tissue coagulator via retroperitoneal approach. The patient was discharged from our hospital 9 days after the surgery. Since a preoperative diagnosis with renal angiomyolipoma can be obtained relatively easily, maximum efforts for nephron-sparing surgery should be made.     

Malignant transformation of angiomyolipoma.

Angiomyolipomas are well recognized but uncommon tumors that can involve the kidney. To our knowledge a well documented case of malignant change of angiomyolipoma has never been reported. We report a case of renal angiomyolipoma and leiomyosarcoma with a clearly identified transition of benign to malignant elements seen in the resected tissue.     

Exophytic renal angiomyolipoma. A case report

A case of a large exophytic renal angiomyolipoma is presented. Despite the features on excretory urography suggestive of an extrarenal mass, computed tomography provided information concerning the origin and pathological nature of the lesion.     

Bilateral renal angiomyolipoma associated with tuberous sclerosis: report of a case

The authors report a case of bilateral renal angiomyolipoma, associated with tuberous sclerosis revealed by abdominal pains, in a 30-year-old man. The diagnosis, suspected by the clinic and ultrasonography, is affirmed by CT scan. In light of this case, the authors review the features of this disease, the ambiguous character of angiomyolipoma, the prognosis and choice of management. Several elements incited us to adopt therapeutic abstention with regular monitoring of the patient for this begin tumor with slow evolution.     

Epithelioid angiomyolipoma : a case report

A 63-year-old woman with a right renal tumor diagnosed by ultrasound, consulted our hospital in October 2008. The findings of her physical examination were unremarkable. The results of urinalysis and other routine blood tests were normal. The urinary cytology was negative for malignant cells. Dynamic computed tomography showed a right renal mass (diameter, 7.5 cm), which was enhanced in the early phase and washed out in the late phase. We initially thought that the patient had renal cell carcinoma. Therefore, laparoscopic right nephrectomy was performed in October 2008. The tumor section was found to be encapsulated, and focal hemorrhage and necrosis were observed. Histological examination of the tumor by hematoxylin-eosin staining revealed that it contained polygonal cells, eosinophilic cytoplasm and large nuclei. Immunohistochemical staining of anticytokeratin antibodies AE1/AE3 and CAM5.2 (markers for renal cell carcinoma) was negative. However, immunohistochemical staining of HMB-45, a marker for melanoma, was positive. The patient was finally diagnosed with epithelioid angiomyolipoma. She did not show any evidence of tumor recurrence for 25 months after the surgery.     

Spontaneous rupture of a renal angiomyolipoma in pregnancy: a case report]

A 32-year-old woman in the 36th week of pregnancy was admitted to obstetrics with a complaint of left flank pain. Magnetic resonance imaging demonstrated a left perinephric hematoma before the cesarean section. Spontaneous rupture of renal angiomyolipoma was suspected by computed tomographic scan and renal angiography after the cesarean section. We attempted partial nephrectomy, but performed nephrectomy because of perinephric adhesion. This is the 11th case of spontaneous rupture of renal angiomyolipoma during pregnancy in the Japanese literature.     

Giant renal angiomyolipoma with an uncommon growth pattern: a case report

A case of a giant renal angiomyolipoma with uncommon growth pattern in a 66-year-old female is reported. The tumor originated from the upper pole of the left kidney and simultaneously grew posteriorly in a sheet-like fashion while a spheroid mass projected upwards. With magnetic resonance imaging (MRI), the relationship between the tumor and adjacent organs was clear. Tumorectomy employing cavitron ultrasonic surgical aspirator (CUSA) was performed, and proved to be a safe and simple procedure. There has been no recurrence of the growth 2 years post-operatively.     

转移性恶性肾血管平滑肌脂肪瘤三例报告

目的 探讨转移性恶性肾血管平滑肌脂肪瘤(AML)的诊断和治疗. 方法转移性恶性AML患者3例.男1例,女2例.年龄分别为36、55和37岁.主诉为持续性患侧腰痛1～6个月.2例B超、CT检查发现患肾实性占位.1例B超见肾肿块内不均匀高回声光团,CT测得脂肪负值信号.2例行根治性肾切除术,1例行肾切除术.3例均未放化疗. 结果 肿块切面灰红、灰黄,质软.病理镜下观察:2例瘤细胞呈上皮样,核有异型,伴坏死;1例大多区域为梭形平滑肌,脂肪细胞和血管混杂生长,部分区域细胞呈上皮样.免疫组化结果 均为VM(+),HMIM5(+),Melan-A(+),S-100(-),CK(-).3例病理诊断均为恶性肾AML,2例为上皮样型,1例为经典型、部分上皮样型.1例术后7年出现肺转移,行肺叶切除加清扫,随访12年无瘤生存.1例术后6年复发,行肿块切除术;术后lO年发现肝、后腹膜转移,再行肿块部分切除,随访12年带瘤生存.1例术后4个月出现肝、后腹膜转移,行肿块部分切除,3个月后失访. 结论 恶性肾AML罕见,诊断依靠病理和免疫组化检查.治疗方法无特殊.     

Renal angiomyolipoma with liposarcomatous transformation: a case report and review of the literature

Renal angiomyolipoma is generally benign, although an uncommon subtype (epithelioid angiomyolipoma) may behave more aggressively. Sarcomatous transformation of the disease is exceedingly rare. We report the first case of a high grade round cell liposarcoma arising in a renal angiomyolipoma.     

Malignant transformation of a multiple cartilaginous exostosis – a case report

Summary. A patient is reported who had multiple cartilaginous exostoses with malignant transformation of a pelvic osteochondroma.

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Résumé. On décrit le cas d’un patient atteint d’exostoses ostéocartilagineuses multiples avec dégénérescence maligne d’un ostéochondrome pelvien en chondrosarcome. On passe ensuite en revue 94 autres cas décrits par d’autres auteurs. L’incidence de la dégénérescence de la maladie ostéogénique exostosante est de 15%, l’age moyen des patients étant de 31 ans. Les hommes sont deux fois plus touchés que les femmes. Plus de 85% des tumeurs étaient situées sur la partie proximale de l’humérus, sur le tronc ou sur la partie proximale du fémur. Le laps de temps entre le premier sympt?me et l’établissement du diagnostic était en moyenne de deux ans et quatre mois. Une augmentation de taille était le sympt?me majeur. Dans 9 cas sur 10, l’histologie révélait un chondrosarcome. Malgré les traitements à visées curatives, un patient sur deux faisait deux récidives en moyenne. Chez 10% des patients des métastases étaient détectées. Un patient sur quatre avait une survie postopératoire de plus de 5 ans. Suit une discussion sur les déductions diagnostiques et thérapeutiques.

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Accepted: 23 September 1996     

Extrarenal retroperitoneal epithelioid angiomyolipoma: a case report

We present a very rare case of retroperitoneal extrarenal epithelioid angio-myolipoma (AML). A 64-year-old man without tuberous sclerosis was admitted to our hospital for further examination of an abdominal mass lesion. Imaging analyses revealed a heterodensity and heterointensity retroperitoneal mass covering the left renal surface by ultrasonography, computed tomography and magnetic resonance imaging. Surgical exploration was done, and the tumor was resected with the left kidney radically. Histopathologically high cellular atypia was remarkable, and diagnosis was extrarenal epithelioid AML because of consistent immunostaining positive for HMB-45.