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MR imaging is useful in differentiating and characterizing renal masses. A careful evaluation of the signal characteristics and morphology of a renal mass combined with the ancillary imaging findings and patient history should assist the radiologist in making the proper diagnosis or recommending the appropriate treatment in most cases. This pictorial essay demonstrates the typical MR imaging features of common renal masses including renal cell carcinoma (RCC), oncocytoma, angiomyolipoma, metastases, transitional cell carcinoma (TCC), lymphoma, and arteriovenous malformation (AVM), and highlights several potential diagnostic pitfalls in making the proper diagnosis.
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PMID:Masses and pseudomasses of the kidney: imaging spectrum on MR. 1548 30

Epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, which is characterized by the presence of polygonal cells with densely eosinophilic cytoplasm and varying degrees of nuclear atypia. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported in the literature. We report a case of epithelioid angiomyolipoma of the kidney that occurred in a 38-year-old woman. The tumor was composed of diffuse sheets of epithelioid cells, adipocytes and only scattered thick-walled blood vessels. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm. Hemorrhage, necrotic foci and clusters of foamy macrophages were present. HMB-45, CD117 (c-kit) and CD68 were detected in the epithelioid cells. There was no expression of cytokeratin, epithelial membrane antigen or desmin. The patient showed no evidence of recurrence or metastatic disease 9 months after nephrectomy.
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PMID:Epithelioid angiomyolipoma of the kidney. 1573 17

Fat-containing tumors of the liver are a heterogeneous group of tumors with characteristic histologic features, variable biologic profiles, and variable imaging findings. Benign liver lesions that contain fat include focal or geographic fatty change (steatosis), pseudolesions due to postoperative packing material (omentum), adenoma, focal nodular hyperplasia, lipoma, angiomyolipoma, cystic teratoma, hepatic adrenal rest tumor, pseudolipoma of the Glisson capsule, and xanthomatous lesions in Langerhans cell histiocytosis. Malignant liver lesions that can contain fat include hepatocellular carcinoma, primary and metastatic liposarcoma, and hepatic metastases. Identification of fat within a liver lesion can be critical in characterization of the lesion. The imaging characteristics of a lesion coupled with the pattern of intratumoral fatty change are helpful in narrowing the differential diagnosis. Although the presence of fat can be demonstrated with computed tomography or ultrasound, magnetic resonance imaging is the most specific imaging technique for demonstration of both microscopic and macroscopic fat.
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PMID:Fat-containing lesions of the liver: radiologic-pathologic correlation. 1579 52

The purpose of this study was to evaluate the short- and long-term results of 104 consecutive laparoscopic adrenalectomies performed during a period of 10 years in two specialist centers. One hundred four patients underwent laparoscopic adrenalectomy in two specialist centers in Italy between 1994 and 2003. Indications to laparoscopic adrenalectomy were aldosterone-secreting adenoma (20%), pheochromocytoma (24%), cortisol-secreting adenoma (11.5%), incidentaloma (26.9%), multiple endocrine neoplasia (MEN) type 2A (2.8%), adrenal metastases from lung cancer (3.8%), adrenal cyst (6.7%), and angiomyolipoma (3.8%). Transperitoneal anterior and lateral approaches were adopted in 17 and 84 patients, respectively. Retroperitoneal approach was adopted in three patients. Mean operative time was 108 +/- 39.1 minutes (range, 40-300 minutes). There was no correlation between adrenal tumor diameter and operative time. Mean intraoperative blood loss was 106 mL (range, 40-600 mL). Intraoperative complication rate and conversion rate were 4.8 per cent (5 cases). Laparoscopic adrenalectomy is a safe procedure. After a relatively short learning curve, it can be performed successfully by any surgeon with low operative morbidity and mortality. The size of the adrenal tumor should not be considered a contraindication to this procedure.
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PMID:Ten years of laparoscopic adrenalectomy: lesson learned from 104 procedures. 1594 6

PEComas, occasionally associated with the tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers. This family of tumors includes angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations. Because non-AML/non-LAM PEComas are extremely rare and their natural history and prognostic features undefined, we present our experience with 26 PEComas of soft tissue and the gynecologic tract, the largest series to date. We also performed a detailed review of the literature, with special attention to features predictive of clinical behavior. All PEComas exclusive of AML and LAM were retrieved from our consultation files. Immunohistochemistry for pan-cytokeratin (CK), S-100 protein, smooth muscle actins (SMA), desmin, vimentin, HMB45, Melan-A, microphthalmia transcription factor (MiTF), TFE3, CD117, and CD34 was performed. Clinical follow-up information was obtained. Fisher's exact test was performed. The median patient age was 46 years (range, 15-97 years); there was a marked female predominance (22 females, 4 males). Sites of involvement included the omentum or mesentery (6 cases), uterus (4 cases), pelvic soft tissues (3 cases), abdominal wall (2 cases), uterine cervix (2 cases), and vagina, retroperitoneum, thigh, falciform ligament, scalp, broad ligament, forearm, shoulder, and neck (1 case each). The tumors ranged from 1.6 to 29 cm in size (median, 7.8 cm). Tumors were epithelioid (N = 9), spindled (N = 7), or mixed (N = 10). Multinucleated giant cells were present in 18 cases. High nuclear grade was noted in 10 cases, high cellularity in 7 cases, necrosis in 8 cases, and vascular invasion in 3 cases. Mitotic activity was 0 to 50 mitotic figures (MF)/50 high power fields (HPF) (median, 0 MF/50 HPF) with atypical MF in 6 cases. IHC results were: SMA (20/25), desmin (8/22), HMB45 (22/24), Melan-A (13/18), MITF (9/18), S-100 protein (8/24), CK (3/23), vimentin (12/14), TFE3 (5/17), c-kit (1/20), and CD34 (0/7). Clinical follow-up (24 of 26 patients, 92%; median, 30 months; range, 10-84 months) showed 3 local recurrences and 5 distant metastases. At last available clinical follow-up, 2 patients (8%) were dead of disease, 4 patients (17%) were alive with metastatic or unresectable local disease, and 18 patients (75%) were alive with no evidence of disease. No patient in our series had a history of tuberous sclerosis complex. Recurrence and/or metastasis was strongly associated tumor size > median size (8 cm), mitotic activity greater than 1/50 HPF, and necrosis. We conclude that PEComas of soft tissue and gynecologic origin may be classified as "benign," "of uncertain malignant potential," or "malignant." Small PEComas without any worrisome histologic features are most likely benign. PEComas with nuclear pleomorphism alone ("symplastic") and large PEComas without other worrisome features have uncertain malignant potential. PEComas with two or more worrisome histologic features should be considered malignant. Occasional PEComas express unusual markers, such as S-100 protein, desmin, and rarely CK. The role of TFE3 in PEComas should be further studied.
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PMID:Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. 1632 28

Epithelioid angiomyolipoma (AML) is the prototype of a heterogeneous group of lesions characterized by the presence of HMB-45 positive cells with clear cytoplasm, perivascular distribution, and combined myomelanocytic features, so-called perivascular epithelioid cells (PECs). These lesions are being increasingly referred to as PEComas. PEComas have been reported at diverse anatomic sites, but mainly in the abdominopelvic cavity and rarely in parenchymatous organs, skin, and soft tissues. Gastrointestinal (GI) PEComas are exceptionally rare, with less than 10 cases documented so far. Rare examples of PEComas with pleomorphic histology could have been misinterpreted as unusual variants of carcinoma or sarcoma. To make a contribution to the differential diagnosis of difficult-to-classify pleomorphic GI sarcomas, we report on a malignant pleomorphic neoplasm with features of PEComa involving the terminal ileum in a 63-year-old woman. Fourteen months after resection of the primary tumor, a huge abdominopelvic recurrence was successfully resected, but no distant metastases were detected. The differential diagnosis and malignancy criteria of GI PEComas will be discussed.
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PMID:Perivascular epithelioid cell sarcoma (malignant PEComa) of the ileum. 1634

Unusual radiologic manifestations of a renal cell carcinoma (RCC) can present a diagnostic challenge. These manifestations include bilateral synchronous multifocal tumors, a small RCC with synchronous adrenal metastasis, and RCC associated with bulky abdominal lymphoma. Less common manifestations include multiseptated cystic carcinoma simulating a moderately complex renal cyst at ultrasonography (US), paraaortic metastatic adenopathy as the only sign of an undetectable primary renal neoplasm, RCC causing a large arteriovenous fistula, RCC simulating angiomyolipoma, and a nonfunctioning kidney due to transparenchymal renal propagation of cancer associated with a tumor thrombus occluding the renal vein. Radiologists should be aware of the possibility of tumor multifocality or of adrenal metastases from a high-grade small renal tumor, as well as of the association of RCC with lymphoma. They should also be aware of the importance of following up a multiseptated cystic mass found at US or a Bosniak category IIF renal cyst, since these lesions can serve as early indicators of cystic carcinoma. Because the clinical implications of and therapeutic strategies for RCC vary depending on imaging characterization of the nature and extent of the disease, familiarity with its more unusual radiologic manifestations facilitates accurate diagnosis and management.
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PMID:Renal cell carcinoma: unusual imaging manifestations. 1641 54

The purpose of this article is to provide an update on imaging techniques useful for detection and characterization of fat in the liver. Imaging findings of liver steatosis, both diffuse steatosis and focal fatty change, as well as focal fatty sparing, are presented. In addition, we will review computed tomography (CT) and magnetic resonance (MR) findings of focal liver lesions with fatty metamorphosis, including hepatocellular carcinoma, hepatocellular adenoma, focal nodular hyperplasia, angiomyolipoma, lipoma, and metastases.
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PMID:Fat in the liver: diagnosis and characterization. 1647 2

Neoplasms of perivascular epithelioid cells (PEComas) have in common the coexpression of muscle and melanocytic immunohistochemical markers. Although this group includes entities with distinct clinical features, such as angiomyolipoma, clear cell sugar tumor of the lung, and lymphangioleiomyomatosis, similar tumors have been documented in an increasing diversity of locations. The term PEComa is now generally used in reference to these lesions that are not angiomyolipomas, clear cell sugar tumors, or lymphangioleiomyomatoses. While most reported PEComas have behaved in a benign fashion, malignant PEComas have occasionally been documented. We present a case of hepatic PEComa with benign histologic features, which nonetheless presented with metastases to multiple sites nearly 9 years later. This case represents the second documented malignant PEComa of the liver, as well as the longest follow-up of a surviving patient with a malignant PEComa, emphasizing both the need for criteria that more accurately predict the behavior of PEComas and the necessity of long-term follow-up of patients with PEComas.
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PMID:Malignant neoplasm of perivascular epithelioid cells of the liver. 1687 28

Perivascular epithelioid cell tumor (PEComa) is rare entity and has been described only recently. By immunohistochemistry and genetics it belongs to the family of tumours which comprises angiomyolipoma, clear cell "sugar" tumor of lung, lymphangioleiomyomatosis and clear cell myomelanotic tumor of ligamentum falciforme/teres hepatis. We describe an unusual case of hepatic PEComa arising in a 55-year-old woman with previous history of glioblastoma. Histologically the tumor grew in expansive way, and was composed of clear and eosinophilic epithelioid cels, without vascular or lipomatous component characteristic of angiomyolipoma. There was mild nuclear pleomorphism, sporadic mitotic activity and haemorrhage without necrosis. On immunohistochemistry, the tumor was HMB-45+50, Melan-A and smooth muscle actin positive. Tyrosinase, S-100 protein, cytokeratin coctail, EMA, vimentin, muscle specific actin, CD10, TTF-1, hepatocyte, desmin and cyclin D1 were negative. Sporadic nuclear p53 positivity was seen. The main differential diagnosis of hepatic PEComa includes clear cell variant of liver cell adenoma and hepatocellular carcinoma, metastases of various clear cell carcinomas and metastasis of malignant melanoma. In respect of uncertain biologic potential of PEComa, long term follow up is indicated.
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PMID:[Perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature]. 1737 Apr 72


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