My journey started the summer of 2014. Little is known about the biological behavior of Xp11.2 translocation renal cell carcinomas (RCCs) as few clinical studies have been performed using a large sample size. Cytogenetics and immunohistochemical analysis identified 4 (1.6%) and 3 (1.5%) cases of Xp11 translocation RCC, respectively. The median age of the 7 patients was 41 years (range, 15-59 years); 15% of RCC patients (4 of 26) who were younger than age 45 years had this type of RCC. Xp11 translocation renal cell carcinoma (RCC) is a distinctive subtype of RCC that affects more children and young adults. Both neoplasms have gene fusions between X (TFE3) and chromosome 17 (ASPL). Five patients had received prior systemic therapy. Immunostaining of the secondary tumor was consistent with Xp11 translocation morphology. The t(6;11) RCCs harbor a specific Alpha-TFEB gene fusion. 1–4 It was initially described in 2006 in conjunction with end-stage renal disease. Choueiri, T. K. et al. Renal cell carcinoma (RCC) associated with Xp11.2 translocation (TFE3-RCC) has been recently defined as a distinct subset of RCC classified by characteristic morphology and clinical presentation. to Xp11.2 translocation renal cell carcinoma. Xp11 translocation carcinoma is a rare sub- We report a patient with metastatic Xp11 type of renal cell carcinoma (RCC) commonly translocation RCC who was responsive to treat- ment with the multikinase inhibitor sorafenib. In the present study, we review the clinical course of Xp11.2 translocation renal cell carcinoma in our institution.MethodsWe identified eight cases with Xp11.2 translocation RCC between 2007 and 2010 from the pathological archives of the Taipei Veterans General Hospital. Five patients had undergone fluorescent in situ hybridization analysis and all demonstrated a translocation involving chromosome Xp11.2. We painfully waited for the path report. To investigate the clinical characteristics, treatments and prognosis of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 tRCC), the epidemiological features and treatment results of 34 cases of Xp11.2 tRCC, which were diagnosed by immunohistochemistry staining of TFE3 and fluorescence in situ hybridization at our center, were … 4606 Background: Adult translocation renal cell carcinoma (RCC), bearing TFE3 gene fusions, is a recently recognized entity for which prognosis and therapy remain poorly understood. Xp11 Translocation Renal Cell Carcinoma: Delayed but Massive and Lethal Metastases of a Chemotherapy-Associated Secondary Malignancy Alan Caroe A recently described subtype of renal cell carcinoma (RCC) bearing chromosome translocations involving a breakpoint at Xp11 and resulting in gene fusions involving the TFE3 transcription factor gene often presents in the pediatric population. Xp11 translocation renal cell carcinoma in adults: a clinicopathological and comparative genomic hybridization study. Xp11.2 translocation renal cell carcinoma (RCC)involves fusion between the transcription factor binding to IGHMenhancer 3 (TFE3) in chromosome Xp11.2 and various partners, andwas classified as a separate subset of RCCs by the World HealthOrganization in 2004 (1). Christine Farrell-LePage is a long-term survivor of stage 3 translocation Xp11.2 Renal Cell Carcinoma, a rare subtype of kidney cancer. Renal cell carcinomas associated with Xp11.2 translocations (TFE3 gene fusions) are rare tumors predominantly reported in children.We studied 5 cases of translocation carcinoma in adult patients, 18 years or older (mean age, 32.6 years). Despite most instances of t(6;11) renal cell carcinoma having an indolent clinical course, a few published cases demonstrate aggressive behavior. Renal cell carcinoma is historically known as the ‘great masquerader’ with 40% of patients experiencing a paraneoplastic syndrome. The effect of vascular-endothelial growth factor (VEGF)-targeted therapy in this histologic subtype is characterized. Xp11.2 translocation renal cell carcinoma (tRCC) represents a rare subtype of renal cell carcinoma (RCC) and was included as a separate entity since 2004. Translocation carcinoma represents one-third of renal cancer in paediatric patients but less than 3% of renal cancers in patients aged 18–45 years where the clinical course is often rapidly terminal. Microphthalmia Transcription Factor (MITF)family translocation renal cell carcinoma (tRCC) is a rare RCC subtype harboring TFE3/TFEB translocations. When treated with VEGF-targeted therapy, 3 patients achieved a partial response, 7 patients had stable disease, and 5 patients developed progressive disease. They expected this to be a recurrence of her original cancer however pathology proved it to be xp 11.2 translocation renal cell carcinoma. Zou H, Kang X, Pang LJ, et al. August 12, 2020 As an aggressive tumor, adult Xp11.2 translocation renal cell carcinoma (RCC) tends to occur in local invasion and lymphatic metastasis even at an early stage, which makes it differ from conventional RCC subtypes, such as clear cell RCC, papillary RCC, and chromophobe RCC. and molecular characterization of renal cell carcinoma in children and young adults. Patients usually do not experience symptoms. Living Resources. Fluorescence in situ hybridization analysis revealed a break-apart of the transcription factor E3 (TFE3) genes in the left tumor. We reported a rare case of cutaneous metastases of renal cell carcinoma (RCC) with an Xp11.2 translocation in a 15‐year‐old female. Renal cell carcinoma (RCC) accounts for 3% of malignant tumors and is the seventh leading cause of cancer deaths in the USA. This review provides insight into the clinicopathologic features, prognosis, treatment, and classification of Xp11 translocation RCC and its mesenchymal counterparts, emphasizing the impact of individual fusion variants on specific clinicopathologic features of Xp11 translocation RCC and the relationships among these Xp11 translocation-associated neoplasms. the small number of TFE gene fusion-related renal tumors described in the literature, the exact biologic behavior and impact of current treatment modalities remain to be uncertain. (Top) A progression‐free survival of 7.1 months was reported for 15 patients with advanced Xp11 translocation renal cell carcinoma who were treated with vascular endothelial growth factor (VEGF)‐targeted therapy. Key words: Xp11.2 translocation renal cell carcinoma – sunitinib – transcription factor E3 – nephrotic syndrome INTRODUCTION About 1–3% of renal cell carcinomas (RCCs) with Xp11.2 translocation that result in gene fusions involving the tran-scription factor E3 (TFE3) gene have been classified as clear-or papillary-type RCCs (1,2). Interested in receiving weekly updates about the Renal Cell Carcinoma Center of Excellence? We determined the incidence of Xp11 translocation renal cell carcinoma (RCC) in adult patients using cytogenetics and immunostaining for TFE3. ASPS is a rare tumor mostly arising in the extremities or the head and neck regions of adolescents or young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC. cell kidney cancer. Abstract: Renal cell carcinoma (RCC) is uncommon in adolescents and young adults, although rare variants of the disease, including Xp11.2 translocation carcinoma, collecting duct carcinoma, and renal medullary carcinoma, occur with a higher preponderance in young patients. Clinicopathological features of Xp11.2 translocation renal cell carcinoma Bumjin Lim , Dalsan You , In Gab Jeong , Taekmin Kwon , Sungwoo Hong 1 , Cheryn Song , Yong Mee Cho 2 , Bumsik Hong , Jun Hyuk Hong , Hanjong Ahn , Choung Soo Kim Tumor Boards. This is her story. Although RT-PCR and DNA sequencing are the final diagnoses of the molecular identity of Xp11.2 translocation RCC, experienced pathologists could confirm the histologic diagnosis based on the distinctive morphologic features with positive TFE3 immunochemical nuclear stain.
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