ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2020
ALK rearranged renal cell carcinoma (ALK-RCC): a multi-institutional study of twelve cases with identification of novel partner genes CLIP1, KIF5B and KIAA1217.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers, 3 of them syntheses that pooled it.
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42 citing papers in PubMed, 3 syntheses or guidelines pooled it, 99 citations in OpenAlex.
- Activity of ALK Inhibitors in Renal Cancer with ALK Alterations: A Systematic Review.International journal of molecular sciences · 2022Pooled it
- New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Guideline
- Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Guideline
- Renal cell carcinoma with a novel RAB1A::ALK fusion: expanding the molecular spectrum of ALK-rearranged RCC.Virchows Archiv : an international journal of pathology · 2026Article
- Immunohistochemical Surrogates for Molecularly Defined Renal Tumors.Diagnostics (Basel, Switzerland) · 2026Review
- SDH-deficient renal cell carcinoma with intracytoplasmic mucinous material: a case report and literature review.Journal of pathology and translational medicine · 2026Article
- The evolution of renal cell carcinoma classification.Journal of the Chinese Medical Association : JCMA · 2026Review
- Case Report: imaging features of anaplastic lymphoma kinase-rearranged renal cell carcinoma with a novelFrontiers in oncology · 2026Article
- Case Report: KIF5B-ALK-rearranged renal cell carcinoma.Frontiers in oncology · 2026Article
- Recent developments in eosinophilic renal neoplasms: what's new, true and important?Histopathology · 2026Review
- Molecularly defined renal cell carcinomas.Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation Oncology · 2026Review
- ALK-Rearranged renal neoplasms: update on an institutional cohort.Virchows Archiv : an international journal of pathology · 2025Article
- Article
- Molecular and therapeutic landscape of non-clear cell renal carcinoma.Nature reviews. Urology · 2025Review
- Advances in renal cancer: diagnosis, treatment, and emerging technologies.La Radiologia medica · 2025Review
- Malignant epithelioid tumors with EWSR1::CREB fusion involving the kidney: a report of two cases.Virchows Archiv : an international journal of pathology · 2025Article
- Current Evidences and Future Perspectives in the Management of Metastatic Non-Clear Cell Renal Cell Carcinoma.International journal of urology : official journal of the Japanese Urological Association · 2025Review
- Molecular Insights into the Diagnosis of Anaplastic Large Cell Lymphoma: Beyond Morphology and Immunophenotype.International journal of molecular sciences · 2025Review
- [Undifferentiated and dedifferentiated renal cell carcinomas : Morphomolecular aspects and differential diagnosis in light of recent developments].Pathologie (Heidelberg, Germany) · 2025Article
- Paediatric renal tumours: an update on challenges and recent developments.Virchows Archiv : an international journal of pathology · 2025Review
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Authors and funding
21 authors at 14 institutions in 8 countries.
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Abstract
ALK rearranged renal cell carcinoma (ALK-RCC) has recently been included in 2016 WHO classification as a provisional entity. In this study, we describe 12 ALK-RCCs from 8 institutions, with detailed clinical, pathological, immunohistochemical (IHC), fluorescence in situ hybridization (FISH), and next generation sequencing (NGS) analyses. Patients' age ranged from 25 to 68 years (mean, 46.3 years). Seven patients were females and five were males (M:F = 1:1.4). Tumor size ranged from 17 to 70 mm (mean 31.5, median 25 mm). The pTNM stage included: pT1a (n = 7), pT1b (n = 1), and pT3a (n = 4). Follow-up was available for 9/12 patients (range: 2 to 153 months; mean 37.6 months); 8 patients were alive without disease and one was alive with distant metastases. The tumors demonstrated heterogeneous, 'difficult to classify' morphology in 10/12 cases, typically showing diverse architectural and cellular morphologies, including papillary, tubular, tubulocystic, solid, sarcomatoid (spindle cell), rhabdoid, signet-ring cell, and intracytoplasmic vacuoles, often set in a mucinous background. Of the remaining two tumors, one showed morphology resembling mucinous tubular and spindle cell renal cell carcinoma (MTSC RCC-like) and one was indistinguishable from metanephric adenoma. One additional case also showed a focal metanephric adenoma-like area, in an otherwise heterogeneous tumor. By IHC, all tumors were diffusely positive for ALK and PAX8. In both cases with metanephric adenoma-like features, WT1 and ALK were coexpressed. ALK rearrangement was identified in 9/11 tumors by FISH. ALK fusion partners were identified by NGS in all 12 cases, including the previously reported: STRN (n = 3), TPM3 (n = 3), EML4 (n = 2), and PLEKHA7 (n = 1), and also three novel fusion partners: CLIP1 (n = 1), KIF5B (n = 1), and KIAA1217 (n = 1). ALK-RCC represents a genetically distinct entity showing a heterogeneous histomorphology, expanded herein to include unreported metanephric adenoma-like and MTSC RCC-like variants. We advocate a routine ALK IHC screening for "unclassifiable RCCs" with heterogeneous features.
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