scholarly journals Gastrointestinal: Epithelioid angiomyolipoma of the pancreas

2022 ◽  
Author(s):  
X Wang ◽  
H Xue ◽  
X Chang ◽  
Z Jin
Keyword(s):  
Epithelioid Angiomyolipoma

Renal malignant epithelioid angiomyolipoma with pelvic and paraaortic lymph node metastasis

2018 ◽  
Author(s):  
Gözde Kir ◽  
Tuce Soylemez ◽  
Rabia Burcin Girgin ◽  
Oguz Devrim Yardimci
Keyword(s):  
Lymph Node ◽  
Lymph Node Metastasis ◽  
Paraaortic Lymph Node ◽  
Epithelioid Angiomyolipoma ◽  
Node Metastasis ◽  
Paraaortic Lymph Node Metastasis

scholarly journals Cathepsin K: A Novel Diagnostic and Predictive Biomarker for Renal Tumors

Cancers ◽  
2021 ◽  
Vol 13 (10) ◽  
pp. 2441
Author(s):  
Anna Caliò ◽  
Matteo Brunelli ◽  
Stefano Gobbo ◽  
Pedram Argani ◽  
Enrico Munari ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Therapy Response ◽  
Cathepsin K ◽  
Mtor Inhibitors ◽  
Predictive Marker ◽  
Predictive Biomarker ◽  
Renal Tumors ◽  
Epithelioid Angiomyolipoma

Cathepsin K is a papain-like cysteine protease with high matrix-degrading activity. Among several cathepsins, cathepsin K is the most potent mammalian collagenase, mainly expressed by osteoclasts. This review summarizes most of the recent findings of cathepsin K expression, highlighting its role in renal tumors for diagnostic purposes and as a potential molecular target. Indeed, cathepsin K is a recognized diagnostic tool for the identification of TFE3/TFEB-rearranged renal cell carcinoma, TFEB-amplified renal cell carcinoma, and pure epithelioid PEComa/epithelioid angiomyolipoma. More recently, its expression has been observed in a subgroup of eosinophilic renal neoplasms molecularly characterized by TSC/mTOR gene mutations. Interestingly, both TSC mutations or TFE3 rearrangement have been reported in pure epithelioid PEComa/epithelioid angiomyolipoma. Therefore, cathepsin K seems to be a downstream marker of TFE3/TFEB rearrangement, TFEB amplification, and mTOR pathway activation. Given the established role of mTOR inhibitors as a pharmacological option in renal cancers, cathepsin K could be of use as a predictive marker of therapy response and as a potential target. In the future, uropathologists may implement the use of cathepsin K to establish a diagnosis among renal tumors with clear cells, papillary architecture, and oncocytic features.

scholarly journals More than Pus – Primary Hepatic Epithelioid Angiomyolipoma Masquerading as Liver Abscess

2021 ◽  
pp. 1-8
Author(s):  
Wen Hao Justin Leong ◽  
Xia Huang Andrew Tan ◽  
Ennaliza Salazar
Keyword(s):  
Liver Abscess ◽  
Epigastric Pain ◽  
Epithelioid Angiomyolipoma ◽  
Rare Type ◽  
Function Test ◽  
History Of ◽  
Cluster Of Differentiation ◽  
Epithelioid Variant ◽  
Minimal Fluid ◽  
Mesenchymal Tumours

Hepatic angiomyolipomas (AML) are rare mesenchymal tumours of which the epithelioid type is a rare type with malignant potential. We report a case of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess. A 46-year-old man presented with a 5-day history of fever with epigastric pain and nausea. On the night of admission, his temperature spiked to 39°C, his blood pressure was 135/79, his heart rate 98, his liver function test revealed albumin 37 g/L, bilirubin 25 μmol/L, ALP 298 U/L, ALT 247 U/L, and AST 344 U/L. The clinical suspicion was hepatobiliary sepsis and intravenous ceftriaxone was commenced. CT of the abdomen showed an ill-defined hypodense focus in segment 4A/8 (4.5 × 3.5 cm) with a minimal fluid component implying a developing abscess or phlegmon. The images were reviewed by a radiologist and showed minimal fluid for percutaneous drainage. MRI of the liver was performed to further characterize the lesion and revealed a solid mass with nodular areas of arterial enhancement and washout, suspicious of neoplasm. Ultrasound-guided liver biopsy of the lesion was performed. Histology showed a histiocyte-rich epithelioid neoplasm consistent with the epithelioid variant of AML. Immunohistochemical staining was positive for human melanoma black 45, melan-A and cluster of differentiation 68. He successfully underwent liver resection of segment 4A/8 after 6 weeks of antibiotics. To our knowledge, this is the first reported case in the literature of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess.

scholarly journals Renal epithelioid angiomyolipoma with epithelial cysts: demonstration of Melan A and HMB45 positivity in the cystic epithelial lining

2012 ◽  
Vol 16 (5) ◽  
pp. 397-401 ◽  
Author(s):  
José do Egypto Pereira Filho ◽  
Daniele Meneses de Amorim ◽  
Gloria Maria Maranhão Sweet ◽  
Luiz Antonio Rodrigues de Freitas ◽  
Paulo Roberto Fontes Athanazio ◽  
...  
Keyword(s):  
Epithelial Lining ◽  
Epithelioid Angiomyolipoma ◽  
Epithelial Cysts

Baseline and contrast-enhanced ultrasound features of hepatic epithelioid angiomyolipoma

2021 ◽  
pp. 1-15
Author(s):  
Pei-Li Fan ◽  
Zheng-Biao Ji ◽  
Jia-Ying Cao ◽  
Chen Xu ◽  
Yi Dong ◽  
...  
Keyword(s):  
Resistive Index ◽  
Malignant Potential ◽  
Arterial Phase ◽  
Hepatic Tumors ◽  
Contrast Enhanced Ultrasound ◽  
Uncertain Malignant Potential ◽  
Epithelioid Angiomyolipoma ◽  
Tumor Vessels ◽  
Contrast Enhanced ◽  
Ultrasound Features

BACKGROUND: Recurrence or metastasis after surgery had been reported in hepatic epithelioid angiomylipoma (epi-AML). Most hepatic epi-AMLs were misdiagnosed with hepatocellular carcinoma or other hepatic tumors before surgery. OBJECTIVE: To describe the baseline and contrast-enhanced ultrasound (CEUS) features of hepatic epi-AMLs and to explore the potential ultrasonic features for prognosis. METHODS: The retrospective study enrolled 67 patients (56 females, 11 males) with 67 pathologically confirmed hepatic epi-AML lesions. All the lesions were examined by baseline ultrasound and 42 lesions were examined using CEUS with SonoVue (Bracco, Milan, Italy) before surgery. RESULTS: Baseline ultrasound features of hepatic epi-AMLs included heterogeneous echo (86.6%), well-defined border (68.7%), hypoecho (64.2%), regular morphology (62.7%), peripheral-tumor arc-shaped or ring-like vessels (53.7%), and low value of resistive index (0.51±0.08). CEUS features of hepatic epi-AMLs included arterial phase hyper-enhancement with smooth and well-defined margin (100%), peripheral-tumor ring-like vessels (57.1%), and intra-tumor vessels (52.4%). Some CEUS features, including arterial phase heterogeneously tortuous filling, intra-tumor vessels and peripheral-tumor ring-like vessels were more commonly found in hepatic epi-AMLs of uncertain malignant potential/malignant than in benign hepatic epi-AMLs (p <  0.05). CONCLUSIONS: Baseline ultrasound and CEUS features may be useful in diagnosis of hepatic epi-AML, and some CEUS features may be indicative of its malignant potential.

scholarly journals Epithelioid angiomyolipoma with involvement of inferior vena cava as a tumor thrombus: A case report

2011 ◽  
Vol 27 (2) ◽  
pp. 72-75 ◽  
Author(s):  
Deyi Luo ◽  
Jiang Gou ◽  
Le Yang ◽  
Yong Xu ◽  
Qiang Dong ◽  
...  
Keyword(s):  
Case Report ◽  
Inferior Vena Cava ◽  
Tumor Thrombus ◽  
Inferior Vena ◽  
Vena Cava ◽  
Epithelioid Angiomyolipoma

scholarly journals Epithelioid Angiomyolipoma

2020 ◽  
Author(s):  
Keyword(s):  
Epithelioid Angiomyolipoma

Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series

2021 ◽  
Author(s):  
Ragheed Saoud ◽  
Tanya W. Kristof ◽  
Clark Judge ◽  
Vaibhav Chumbalkar ◽  
Tatjana Antic ◽  
...  
Keyword(s):  
Pathological Features ◽  
Single Institution ◽  
Epithelioid Angiomyolipoma ◽  
Clinical And Pathological Features

Epithelioid Angiomyolipoma: A Morphologically Distinct Variant That Mimics a Variety of Intra-abdominal Neoplasms

2011 ◽  
Vol 135 (5) ◽  
pp. 665-670
Author(s):  
Ozgur Mete ◽  
Theodorus H. van der Kwast
Keyword(s):  
Correct Diagnosis ◽  
Smooth Muscle Tumor ◽  
Epithelioid Cell ◽  
Cell Phenotype ◽  
Renal Oncocytoma ◽  
Nerve Sheath Tumor ◽  
Epithelioid Angiomyolipoma ◽  
Perivascular Epithelioid Cell ◽  
Show Evidence ◽  
Clear Cytoplasm

Abstract This review examines the histopathologic, immunohistochemical, ultrastructural, and molecular biologic features of epithelioid angiomyolipoma (EAML), with an emphasis on the differential diagnosis of intra-abdominal EAML. Epithelioid angiomyolipoma is an uncommon mesenchymal tumor with malignant potential, frequently associated with tuberous sclerosis complex. Histologically, EAML is characterized by sheets or nests of large polygonal epithelioid cells with abundant eosinophilic or occasionally clear cytoplasm, often with prominent nucleoli, and EAML may include multinucleated and markedly pleomorphic forms. As these tumors share a distinctive perivascular epithelioid cell phenotype, they belong to the PEComa tumor family. Nearly all EAMLs show immunoreactivity for both melanocytic and myoid markers. Ultrastructurally, EAMLs show evidence of melanogenesis by the presence of premelanosomes. Epithelioid angiomyolipoma can pose significant diagnostic challenges as it mimics morphologically a variety of neoplasms including renal cell carcinoma, renal oncocytoma, adrenal cortical neoplasm, epithelioid smooth muscle tumor, epithelioid peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, epithelioid melanoma, hepatoblastoma, and hepatocellular carcinoma. The variation in immunophenotype in these tumors requires a prudent use of immunohistochemistry, which may occasionally need complementation by electron microscopy to establish the correct diagnosis.

scholarly journals MiT Family Translocation Renal Cell Carcinoma: from the Early Descriptions to the Current Knowledge

Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1110 ◽  
Author(s):  
Anna Caliò ◽  
Diego Segala ◽  
Enrico Munari ◽  
Matteo Brunelli ◽  
Guido Martignoni
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clinical Course ◽  
Current Knowledge ◽  
Wide Spectrum ◽  
Cathepsin K ◽  
World Health ◽  
Epithelioid Angiomyolipoma ◽  
Immunohistochemical Markers

The new category of MiT family translocation renal cell carcinoma has been included into the World Health Organization (WHO) classification in 2016. The MiT family translocation renal cell carcinoma comprises Xp11 translocation renal cell carcinoma harboring TFE3 gene fusions and t(6;11) renal cell carcinoma harboring TFEB gene fusion. At the beginning, they were recognized in childhood; nevertheless, it has been demonstrated that these neoplasms can occur in adults as well. In the nineties, among Xp11 renal cell carcinoma, ASPL, PRCC, and SFPQ (PSF) were the first genes recognized as partners in TFE3 rearrangement. Recently, many other genes have been identified, and a wide spectrum of morphologies has been described. For this reason, the diagnosis may be challenging based on the histology, and the differential diagnosis includes the most common renal cell neoplasms and pure epithelioid PEComa/epithelioid angiomyolipoma of the kidney. During the last decades, many efforts have been made to identify immunohistochemical markers to reach the right diagnosis. To date, staining for PAX8, cathepsin K, and melanogenesis markers are the most useful identifiers. However, the diagnosis requires the demonstration of the chromosomal rearrangement, and fluorescent in situ hybridization (FISH) is considered the gold standard. The outcome of Xp11 translocation renal cell carcinoma is highly variable, with some patients surviving decades with indolent disease and others dying rapidly of progressive disease. Despite most instances of t(6;11) renal cell carcinoma having an indolent clinical course, a few published cases demonstrate aggressive behavior. Recently, renal cell carcinomas with TFEB amplification have been described in connection with t(6;11) renal cell carcinoma. Those tumors appear to be associated with a more aggressive clinical course. For the aggressive cases of MiT family translocation carcinoma, the optimal therapy remains to be determined; however, new target therapies seem to be promising, and the search for predictive markers is mandatory.

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