1/32. Malignant pigmented clear cell epithelioid tumor of the kidney: clear cell ("sugar") tumor versus malignant melanoma.A 73-year-old woman presented with an hemorrhagic kidney tumor initially interpreted as a renal cell carcinoma (RCC). A retroperitoneal recurrence infiltrating the duodenal wall was made up of clear cells, some of which contained Fontana-Masson positive pigment, immunopositive for HMB-45, S-100 protein, actin, and vimentin. The same immunohistochemical profile was retrospectively reproduced in the kidney tumor, where melanosomes were also found ultrastructurally. Lipomatous differentiation was not observed. There was no history of malignant melanoma (MM), or stigmata of tuberous sclerosis. The patient died of disease 5 years after the initial diagnosis. This neoplasm can be considered as a malignant, pigmented, clear-cell epithelioid variant of angiomyolipoma, or "sugar" tumor of the kidney, with the peculiarity of having a previously unreported component of pigmented cells visible on light microscopy. This finding raises the possibility that the exceptional cases of MM reported in renal parenchyma may be pigmented variants of epithelioid angiomyolipoma rather than true MM.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/32. Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions.angiomyolipoma (AML) is a benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex (TSC) and shows frequent allelic losses at chromosome arm 16p. It has been suggested recently that the melanogenesis marker-positive perivascular epithelioid cell (PEC) has been found consistently in AML. The authors report a 50-year-old woman without evidence of TSC affected by classic renal AML containing an area composed of atypical epithelioid cells with the same morphoimmunophenotypic characters of PEC. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lung and abdominal metastases that showed morphologic and immunohistochemical features overlapping those of the epithelioid area of the previously removed AML. Genetic analysis showed that the classic AML and its epithelioid area as well as the pulmonary and abdominal metastases shared the same allelic loss on chromosome arm 16p. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic AML with morphologic, immunophenotypic, and genetic demonstration of its clonal origin.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/32. Epithelioid angiomyolipoma: appearance on fine-needle aspiration report of a case.Epithelioid angiomyolipoma is a recently recognized clinicopathologic entity first described by Martignoni et al. in 1995. Since then, several articles have further clarified its histogenesis and histologic features. Due to the presence of polygonal cells with voluminous cytoplasms, this neoplasm is often mistaken for renal-cell carcinoma. In this case presentation, we describe the cytologic features of an epithelioid angiomyolipoma obtained by fine-needle aspiration. The histogenesis and how it relates to diagnosis is briefly discussed. The importance of ancillary techniques in the differential diagnosis of epithelioid cells obtained in a renal aspirate is reviewed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/32. association of cystic nephroma with angiomyolipoma in the same kidney.The synchronous occurrence of two different renal tumors in a patient is a rare event. Here we report an incidentally discovered coexistence of cystic nephroma with angiomyolipoma in the right kidney. The patient was a 68-year-old woman without the tuberous sclerosis complex. To the best of our knowledge, this combination of neoplasms has not yet been described before. This rare situation emphasizes the need for careful diagnosis and the correct management of coincident renal neoplasms.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
5/32. Lymph nodal involvement by renal angiomyolipoma.angiomyolipoma of the kidney is a clonal neoplasm, apparently part of a family of neoplasms derived from perivascular epithelial cells. A 40-year-old woman presented with right flank pain and an otherwise non-significant medical history. An abdominal computed tomography scan revealed an 18 cm solid mass in the mid-portion of the right kidney and multiple perihilar lymph nodes. Presumptive diagnosis was renal cell carcinoma. Right radical nephrectomy and a perihilar lymph node dissection was performed through a Chevron incision for the anticipated diagnosis of renal adenocarcinoma. The renal tumor was diagnosed as angiomyolipoma and a component was identified pathologically in a dissected lymph node. There was no evidence of tumor recurrence in the follow-up period of eight years. The consensus from other studies suggests that this phenomenon is a manifestation of the multicentric nature of angiomyolipoma, rather than due to metastasis. Genetic studies may resolve this question in the future.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
6/32. Laparoscopic wedge resection of a renal mass in a solitary kidney.Laparoscopic radical nephrectomy has gained acceptance as a viable means of managing renal neoplasms. Partial nephrectomy has traditionally been performed through a flank or transabdominal incision, with its attendant morbidity and required hospitalization. Laparoscopic renal-sparing surgery may be more technically demanding, but remains beneficial to the patient if performed with adherence to oncological principles. We present a case of laparoscopic renal-sparing surgery in a patient with a renal mass in a solitary kidney. We discuss the technical and oncological challenges of this approach.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/32. Renal angiomyolipoma extending into the right atrium.Renal angiomyolipomas (AMLs) are benign renal tumors that may possess the features of a malignant neoplasm, such as local and vascular invasion. We describe the diagnosis and management of a rare case of AML associated with tumor thrombus extending into the right atrium.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/32. Concurrent angiomyolipomas and renal cell carcinoma harboring metastatic foci of mammary carcinoma in the same kidney: an incidental autopsy finding in a patient with a follow-up of thirty years.The synchronous occurrence of three different types of renal tumor in a patient is rare. We report a case of conventional (clear cell) renal cell carcinoma harboring metastatic foci of mammary carcinoma associated with two angiomyolipomas in the left kidney incidentally discovered at the autopsy. The patient was a 75-year-old woman, without the tuberous sclerosis complex, who had undergone left radical mastectomy and radiotherapy for an infiltrating duct carcinoma of breast 30 years before. This tumor was widely disseminated at autopsy, but the nontumorous renal parenchyma was free of metastases. To the best of our knowledge this combination of neoplasms has not been described before. This case shows the important role played by autopsy in the accurate investigation of interrelations among coexisting tumors.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/32. Epithelioid angiomyolipoma of the ovary: a case report and literature review.angiomyolipoma (AML) is a benign mesenchymal neoplasm that mainly occurs in the kidney either sporadically or in patients with tuberous sclerosis complex (TSC). Extrarenal AML is uncommon. We describe a 39-year-old female with a history of TSC and bilateral multicentric renal AML who presented with a persistent cystic ovarian mass that fluctuated in size during 2 years of ultrasonographic observation before its removal by salpingo-oophorectomy. The 4.5-cm mass was solid and cystic and tan-yellow. Microscopic examination showed an admixture of epithelioid cells, smooth muscle bundles, large thick-walled blood vessels, and mature adipose tissue. The epithelioid cells had abundant eosinophilic cytoplasm and many had bizarre atypical nuclei including multinucleated forms. Mitoses were rare. Typical smooth muscle cells and the epithelioid cells were strongly immunoreactive for HMB-45. To our knowledge, this represents the first report of an AML arising in the ovary. The differential with other oxyphilic tumors of the ovary is discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/32. Renal disease in adults with TSC2/PKD1 contiguous gene syndrome.The most common renal lesions of tuberous sclerosis complex, an autosomal-dominant syndrome resulting from losses of TSC1 (9q34) or TSC2 (16p13.3), are renal cysts and angiomyolipomas. Epithelial neoplasms are less common. The TSC2 gene lies adjacent to PKD1, the major gene responsible for autosomal-dominant polycystic kidney disease. Recently, a deletion mutation disrupting both TSC2 and PKD1 has been described in young children with tuberous sclerosis complex with severe renal cystic disease. This disease has been termed the TSC2/PKD1 contiguous gene syndrome. We describe the lesions in the resected kidneys of two adults with TSC2/PDK1 contiguous gene syndrome, at the time of the nephrectomies: a 31-year-old man and his 44-year-old mother. The four kidneys were enlarged reniform masses composed of cysts lined by flattened, cuboidal, or, infrequently, large deeply eosinophilic epithelial cells. The kidneys also contained numerous classic angiomyolipomas and rare intraglomerular microlesions. In the son the largest tumor was a monotypic epithelioid angiomyolipoma. In the wall of his left renal pelvis there was a plaque-shaped, HMB-45-positive localized lesion of lymphangioleiomyomatosis. This is the first description of the renal lesions in adults with genetically confirmed TSC2/PDK1 contiguous gene syndrome. The pathologic findings highlight the importance of thorough sampling for histology in polycystic kidney diseases and indicate that the observation of an angiomyolipoma in biopsy material from patients with enlarged cystic kidneys should suggest the diagnosis of TSC2/PKD1 contiguous gene syndrome, even in cases without ultrasonographic and macroscopic evidence of angiomyolipoma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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