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Classical renal angiomyolipoma (AML) is a common tumor that, in most cases, follows a benign course and has clearly defined radiologic and histological characteristics. However, rare cases of clinically aggressive or malignant AML, the epithelioid variant of AML (EAML), have been reported. Here, we review the five cases of EAML diagnosed since 2000 at our institution to highlight the spectrum of clinical, radiological and histological characteristics. In all cases, renal lesions seen on computed tomography (CT) were considered as suspicious for renal cell carcinoma (RCC) without tumor extension or metastasis. One of the two patients with definitive tuberous sclerosis complex had a small conventional AML with fat content in the other kidney. Histologically, these tumors can resemble sarcomatoid RCC or high grade RCC. The final diagnosis is established by the presence of perivascular epithelioid cells and immunohistochemistry markers. There was no evidence of local recurrence or metastatic disease found by sonography, CT and magnetic resonance imaging during the follow-up period. EAMLs are capable of aggressive or malignant clinical behavior. Approximately one third of the reported EAMLs show advanced disease; therefore, we recommend that it is important to treat and closely follow-up such cases, similar to that for RCC, because of its malignant potential.
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PMID:Epithelioid angiomyolipoma of the kidney mimicking renal cell carcinoma: a clinicopathologic analysis of cases and literature review. 1941 18

The vast majority of focal liver lesions are hyperintense on T2-weighted magnetic resonance (MR) images. Rarely, however, hepatic nodules may appear totally or partially hypointense on those images. Causes for this uncommon appearance include deposition of iron, calcium, or copper and are related to the presence of blood degradation products, macromolecules, coagulative necrosis, and other conditions. Although rare, low signal intensity relative to surrounding liver on T2-weighted images may be seen in a wide spectrum of lesions. Examples include cases of focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma, metastases, leiomyoma, siderotic or dysplastic nodules, nodules in Wilson disease, granuloma, and hydatid cyst. On fat-suppressed T2-weighted images, nodules with a lipomatous component, such as lipoma, angiomyolipoma, hepatocellular adenoma, and hepatocellular carcinoma may also appear partially or totally hypointense. The conjunction of other MR imaging findings and their integration in the clinical setting may allow a correct diagnosis in a considerable proportion of cases. The cause for T2-weighted hypointensity may not be, however, always recognized, and only pathologic correlation may provide the answer. The aims of this work are to discuss the causes and mechanisms of hypointensity of liver lesions on T2-weighted images and proposing an algorithm for classification that may be useful as a quick reminder for the interested reader.
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PMID:The hypointense liver lesion on T2-weighted MR images and what it means. 1990 Oct 85

As epithelioid cellular morphology can be seen in clinically benign usual angiomyolipomas (AMLs), we divide epithelioid AMLs into those without and with atypia, the latter category associated in the literature with malignant potential. We herein report the histologic spectrum and biologic behavior of 40 consecutive cases of epithelioid AML with atypia and assess whether cases can be stratified prognostically based on clinical and pathologic features. Atypical epithelioid cells were defined as atypical polygonal cells with abundant cytoplasm, vesicular nuclei, prominent nucleoli, and nuclear size that exceeds x2 the size of adjacent nuclei. The degree of atypia was divided to moderate and severe. Cases with bland epithelioid cells with minimal variation in nuclear size were not included. Mean age was 50.5 years (range 17 to 81), and the female to male ratio was 1.6:1. Average tumor size was 7.2 cm (range 1.0 to 17.7). The percentage of epithelioid component ranged from 5%-100% (mean 68%). Of the epithelioid component, the percentage of cells exhibiting nuclear atypia ranged in individual cases from 5% to 100% (mean of 58.4% atypical cells); 26/40 (65%) cases showed severe nuclear atypia. Cells displaying severe nuclear atypia were typically of large size with abundant cytoplasm, compared with those with moderate atypia being of small to intermediate in size with scant to moderate amount of cytoplasm. Neoplastic multinucleated giant cells and necrosis was present in 22 cases (55%) and 15 cases (37.5%), respectively. Mitoses were identified in 72.5% (29/40) of cases and ranged from 1 to 6 per 10 hpf with 7 cases showing atypical mitotic figures. Lymphovascular invasion or renal vein invasion was present in 3 cases each. Hilar and perinephric fat involvement was present in 5 and 6 cases, respectively. Clinical follow-up was available in 34 out of the 40 cases. Of the 34 cases, 9 (26%) were malignant and showed local recurrence or distant metastases. Of the 9 patients with malignant tumors, 4 died of the disease at 6, 12, 15, and 34 months after the original diagnosis was rendered, and 4 were alive with disease (mean follow-up period of 52 mo, range 24 to 72 mo). Twenty-four patients showed no evidence of recurrence and/or metastases with a mean follow-up period of 34 months (range 1 to 156 mo). We compared the 21 cases of atypical epithelioid AMLs that exhibited a benign clinical course with a minimum follow-up period of 6 months postsurgery to the 9 cases with malignant behavior. All of these were more frequently observed in clinically malignant cases: older age, larger tumor size, higher percentage of epithelioid component, severe atypia, higher percentage of atypical cells, higher mitotic count, atypical mitotic figures, necrosis, lymphovascular invasion, and renal vein invasion. Using these features, we developed a predictive model of 4 atypical features that included: (1) > or =70% atypical epithelioid cells, (2) > or =2 mitotic figures per 10 hpf, (3) atypical mitotic figures, and (4) necrosis; the presence of 3 or all of the features was highly predictive of malignant behavior. This model accurately categorized 78% of clinically malignant and 100% of the clinically benign epithelioid AMLs with atypia.
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PMID:Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. 2041 Aug 12

Focal incidental renal lesions are commonly encountered on positron emission tomography (PET)/computed tomography (CT) imaging. The vast majority of these lesions are benign. However, the interpretation of renal lesions can be problematic if the imaging criteria of simple cysts are not met. Limited literature exists on the characterisation of renal masses with metabolic imaging. The purpose of this article is to focus on the imaging features of benign and malignant renal masses with PET/CT. The lesions discussed include renal cyst, angiomyolipoma, oncocytoma, renal cell carcinoma, renal metastases and other infiltrating neoplastic processes affecting the kidney. Both the anatomical and metabolic features which characterise these benign and malignant entities are described. We emphasise the importance of viewing the CT component to identify the typical morphological features and discuss how to best use hybrid imaging for management of renal lesions. Metabolic imaging has a promising role in the imaging of renal lesions and can help prevent unnecessary biopsies and ensure optimal management of suspicious lesions.
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PMID:Role of FDG PET/CT in imaging of renal lesions. 2071 15

Epithelioid angiomyolipoma (eAMLoma) is an uncommon renal mesenchymal tumor with malignant potential. It is composed of tumor cells arranged in an epithelioid manner. Differential diagnosis from renal cell carcinoma is often challenging because of its epithelioid morphology. Herein is reported a case of eAMLoma, involving a 49-year-old man with eAMLoma. The patient had undergone radical nephrectomy via retroperitoneal laparoscope successfully. He had an uneventful postoperative recovery. The tumor was positive for Desmin, Hmb45, and Sma. We recommend surgical treatment and a follow-up regimen similar to that for renal carcinoma. There was no recurrence and metastases after 1-year follow-up.
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PMID:Renal epithelioid angiomyolipoma without obvious local progress in 10 years: a case report and literature review. 2104 79

Epithelioid angiomyolipomas (perivascular epithelioid cell tumors) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to classic angiomyolipoma. Although approximately 120 cases are published, mostly as case reports with variably used diagnostic criteria, the pathologic prognostic predictors of outcome are unknown. We analyzed the clinicopathologic parameters in a large series of 41 cases of pure epithelioid angiomyolipomas of the kidney, which we designate as pure (monotypic) epithelioid PEComas to contrast them from classic angiomyolipomas that are regarded by some as PEComas. We use the terminology "pure" to separate these cases from those that may have variable epithelioid components. The mean age of the patients was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of patients were symptomatic at presentation with metastatic disease at onset in 12 cases. Follow-up and/or disease progression information were available for 33 of 41 cases (mean, 44.5 mo and median, 24.5 mo; range, 4 to 240); 9 patients had a history of associated tuberous sclerosis. Recurrence and metastasis were seen in 17% and 49% of patients; 33% of patients died of disease. Lymph node involvement was seen in 24% of patients; the liver (63%), lung (25%), and mesentery (18.8%) were the most common metastatic sites. Clinicopathologic parameters associated with disease progression (recurrence, metastasis, or death due to disease) in univariate analysis included associated tuberous sclerosis complex or concurrent angiomyolipoma (any metastasis, P=0.046), necrosis (metastasis at diagnosis, P=0.012), tumor size >7 cm (progression, P=0.021), extrarenal extension and/or renal vein involvement (progression, P=0.023), and carcinoma-like growth pattern (progression, P=0.040) (the 5 adverse prognostic parameters for pure epithelioid PEComas). Tumors with <2 adverse prognostic parameters (13 cases) were considered to be low risk for progression tumor, with 15% having disease progression. Tumors with 2 to 3 adverse prognostic parameters (14 cases) were considered to be "intermediate risk," with 64% having disease progression. Tumors with more than 4 or more adverse prognostic parameters (6 cases) were considered to be high risk, with all patients having disease progression. Of tumors with 3 or more adverse prognostic parameters, 80% had disease progression. An exact logistic regression analytic model showed that only carcinoma-like growth pattern and extrarenal extension and/or renal vein involvement were significant predictors of outcome (P=0.009 and 0.033, respectively). Our data of a large series with uniform definitional criteria confirm the malignant potential for pure epithelioid PEComas and provide adverse prognostic parameters for risk stratification in these patients.
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PMID:Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. 2126 37

Malignant pigmented clear cell epithelioid cell tumor of the kidney is a rare variant of perivascular epithelioid cells tumors (PEComa) or epithelioid angiomyolipoma (AML). PEComa is characteristically composed purely of epitheloid cells. The fat cells and the blood vessels that are typical of classic AML are absent. Most epithelioid AML cases are benign; however, malignant epithelioid AML of the kidney has been occasionally reported in adults in association with tuberous sclerosis. We report the radiological-pathological features of a malignant pigmented clear cell epithelioid renal tumor in a 15-year-old boy presenting with extensive metastases but without clinical evidence of tuberous sclerosis.
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PMID:Malignant pigmented clear cell epithelioid cell tumor (PEComa) in an adolescent boy with widespread metastases: a rare entity in this age group. 2159 5

A 47-year-old man presented with dull pain in the left upper abdomen for 1 year. Computed tomograph (CT) examination revealed a very large heterogeneously enhancing mass in the left kidney, measuring up to 28 cm. The mass was accompanied by several enlarged lymph nodes in the peri-aortic region. Radical nephrectomy was performed and pathologic evaluation revealed sheets of epithelioid cells and piecemeal necrosis consistent with malignant epithelioid angiomyolipoma (EAML) with regional lymph node metastases. The tumor cells were strongly positive for human melanosome-associated protein (HMB-45) on immunohistochemical staining. There was neither metastasis nor recurrence 2 years postoperatively. EAML is a rare tumor of mesenchymal tissue with potential for aggressive behavior. If this neoplasm is suspected based on CT features, EAML should be confirmed by pathology and immunohistochemistry.
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PMID:A massive renal epithelioid angiomyolipoma with multiple metastatic lymph nodes. 2172 28

This Review documents examination techniques, sonographic features and clinical considerations in ultrasound assessment of gynecological tumors. The methodology of gynecological cancer staging, including assessment of local tumor extent, lymph nodes and distant metastases, is described. With increased technical quality, sonography has become an accurate staging method for early and advanced gynecological tumors. Other complementary imaging techniques, such as computed tomography and magnetic resonance imaging, can be used as an adjunct to ultrasound in specific cases, but are not essential to tumor staging if sonography is performed by a specialist in gynecological oncology. Ultrasound is established as the method of choice for evaluating local extent of endometrial cancer and is the most important imaging method for the differential diagnosis of benign and malignant ovarian tumors. Ultrasound can be used to detect early as well as locally advanced cancers that extend from the vagina, cervix or other locations to the paracolpium, parametria, rectum and sigmoid colon, urinary bladder and other adjacent organs or structures. In cases of ureteric involvement, ultrasound is also helpful in locating the site of obstruction. Furthermore, it is specific for the detection of extrapelvic tumor spread to the abdominal cavity in the form of parietal or visceral carcinomatosis, omental and/or mesenteric infiltration. Ultrasound can be used to assess changes in infiltrated lymph nodes, including demonstration of characteristic sonomorphologic and vascular patterns. Vascular patterns are particularly well visualized in peripheral nodes using high resolution linear array probes or in the pelvis using high-frequency probes. The presence of peripheral or mixed vascularity or displacement of vessels seems to be the sole criterion in the diagnosis of metastatic or lymphomatous nodes. In the investigation of distant metastases, if a normal visceral organ or characteristic diffuse or focal lesions (such as a simple cyst, hepatic hemangioma, renal angiomyolipoma, fatty liver (steatosis)) are identified on ultrasound, additional examinations using complementary imaging methods are not required. If, however, less characteristic findings are encountered, especially when the examination result radically affects subsequent therapeutic management, an additional examination using a complementary imaging method (e.g. contrast-enhanced ultrasound, computed tomography, magnetic resonance imaging, positron emission tomography) is indicated.
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PMID:Ultrasound scanning of the pelvis and abdomen for staging of gynecological tumors: a review. 2189 32

Perivascular epithelioid cell neoplasms, also known as PEComas, are unique mesenchymal tumors exhibiting perivascular epithelioid cell differentiation, characterized by a mixed myogenic and melanocytic phenotype. PEComas arising in visceral organs outside of the kidney, liver, and lung are rare, and often pose problems in diagnosis. Examples of this neoplasm originating in the adrenal gland are limited. The present report details the clinical and pathologic features of an unusual case of a pure epithelioid PEComa (epithelioid angiomyolipoma) of the adrenal gland exhibiting clinically malignant behavior in the form of pulmonary metastases, a feature not previously described in tumors of this site. The diagnosis was supported by immunohistochemical studies demonstrating expression of myoid and melanocytic antigens. The present case serves to emphasize the potential of PEComa for clinically aggressive behavior and the importance of distinguishing this tumor from other epithelioid neoplasms that are more commonly encountered in the adrenal gland.
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PMID:Malignant PEComa of the adrenal gland. 2215 7


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