scholarly journals Succinate Dehydrogenase–Deficient Renal Cell Carcinoma: A Short Review

2018 ◽  
Vol 142 (10) ◽  
pp. 1284-1288 ◽  
Author(s):  
Gang Wang ◽  
Priya Rao
Keyword(s):  
Succinate Dehydrogenase ◽  
Renal Cell ◽  
Germ Line ◽  
Short Review ◽  
Renal Cell Carcinomas ◽  
Characteristic Appearance ◽  
Hereditary Tumor Syndrome ◽  
Eosinophilic Cytoplasm ◽  
Genes Encoding

Succinate dehydrogenase (SDH) is a mitochondrial enzyme complex composed of 4 protein subunits (SDHA, SDHB, SDHC, and SDHD). Germ line mutations of the genes encoding these SDH subunits result in hereditary syndromes harboring pheochromocytomas/paragangliomas, gastrointestinal stromal tumors, renal cell carcinomas, and pituitary adenomas. SDH-deficient renal cell carcinomas are rare, with a mean age of 38 to 40 years. Histologically, these tumors show a characteristic appearance that includes a solid, nested, or tubular architecture with variable cysts. Cells are typically cuboidal, have indistinct cell borders and eosinophilic cytoplasm, and show flocculent intracytoplasmic inclusions. Loss of immunohistochemical staining for SDHB is the hallmark of these tumors. Although most SDH-deficient renal cell carcinomas are clinically indolent, some tumors may behave aggressively, particularly those with a high nuclear grade, tumor necrosis, or sarcomatoid differentiation. Accurate classification of these tumors is important for clinical follow-up, screening, and genetic evaluation of the patients and other family members for this hereditary tumor syndrome.

scholarly journals Succinate Dehydrogenase–Deficient Renal Cell Carcinoma

2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Succinate Dehydrogenase ◽  
Renal Cell ◽  
World Health ◽  
Low Grade ◽  
Long Term Follow Up ◽  
Health Organization

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.

Percutaneous and laparoscopic-assisted cryoablation of small renal cell carcinomas.

2012 ◽  
Vol 30 (5_suppl) ◽  
pp. 462-462
Author(s):  
Tommy Kjaergaard Nielsen ◽  
Oyvind Ostraat ◽  
Michael Borre ◽  
Truls E Bjerklund Johansen
Keyword(s):  
Complication Rate ◽  
Renal Cell ◽  
Residual Tumor ◽  
Observation Time ◽  
Treatment Modalities ◽  
Minor Bleeding ◽  
Renal Cell Carcinomas ◽  
Cancer Specific Survival ◽  
Co Morbidity

462 Background: To evaluate the complication rate and short term oncological outcome of small renal cell carcinomas treated with cryoablation. Methods: 91 biopsy verified renal cell carcinomas were cryoablated between 2006-11. Patients treated had primarily T1a tumors, but exceptions were made. Patients with tumors not directly accessible percutaneously were selected for laparoscopy. To ensure complete coverage of the tumor, cryoneedle placement and freezing procedure was peroperatively monitored by ultrasound in both modalities. Presice machine with Ice-Seet and Ice-Rod needles from Galil Medical was used. Treatment was considered successful when tumors gradually shrunk and showed no sign of contrast enhancement, assessed by CT or MRI. Results: Mean patient age and tumor size was 65 yr [17 - 83] and 26 mm [10 - 62], respectively [min-max]. Treatment modalities consisted of percutaneous route; n=34 (47%) and laparoscopic guided; n=39 (53%). The mean follow-up time was 19 months [1 - 56]. 18 patients were excluded from follow-up analysis (8 pt. with short observation time, 5 pt. treated despite severe co-morbidity with no planned follow-up, 3 pt. lost to follow-up, 1 pt. emigrated, 1 pt. died as a result of bleeding from perforation of iliac artery by troicar). Residual tumor was diagnosed by imaging in 10 patients (perc n=6 and lap n=4, p>0,05) after a mean follow-up time of 13 months [1 - 31]. Diagnosis was confirmed by biopsy in all but one case, in which the diagnosis was necrosis. Of the 10 patients with residual tumor, 8 patients were reablated and 2 patients were referred to oncological treatment. Cancer specific survival was 100%. Overall survival was 91%. Complications: 8 pt. had minor bleeding in relation to cryoneedle removal, requiring Tachosil. 1 pt. had subcutaneous bleeding, 2 pt. was readmitted due to pain and discharged the following day. No patients required blood transfusion. Conclusions: Although percutaneous and laparoscopic guided cryoablation are minimally invasive procedures and the overall complication rate is low, serious complications may occur. Close follow-up and continuous evaluation of criteria for patient selection and relapse is necessary. An international database is recommended.

Incidence of Multiple Sporadic Renal Cell Carcinomas in Patients Referred for Renal Radiofrequency Ablation: Implications for Imaging Follow-Up

2011 ◽  
Vol 197 (3) ◽  
pp. 671-675 ◽  
Author(s):  
Michael D. Beland ◽  
Farrah J. Wolf ◽  
David J. Grand ◽  
Damian E. Dupuy ◽  
William W. Mayo-Smith
Keyword(s):  
Radiofrequency Ablation ◽  
Renal Cell ◽  
Renal Cell Carcinomas

scholarly journals Repeat Percutaneous Radiofrequency Ablation of T1 Renal Cell Carcinomas is Safe in Patients with Von Hippel–Lindau Disease

2021 ◽  
Author(s):  
Joel Wessendorf ◽  
Alexander König ◽  
Hendrik Heers ◽  
Andreas H. Mahnken
Keyword(s):  
Radiofrequency Ablation ◽  
Renal Cell ◽  
Case Series ◽  
Progression Free Survival ◽  
Renal Cell Carcinomas ◽  
Ct Guided ◽  
Free Survival ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

Abstract Purpose Patients with Von Hippel-Lindau disease often develop multifocal, metachronous renal cell carcinomas which require therapy. The purpose of this retrospective single-center study is to evaluate the outcomes of radiofrequency ablation (RFA) in the treatment of renal cell carcinomas in patients with Von Hippel-Lindau disease. Materials and Methods 9 patients (4 male, 5 female, 47.9 ± 10.7 y/o) with Von Hippel-Lindau disease underwent 18 CT-guided percutaneous RFA procedures for the treatment 21 renal cell carcinomas (largest diameter: 32.9 ± 8.6 mm, cT1a: 16, cT1b: 5). Seven patients were previously treated either by partial or radical nephrectomy. Technical success, effectiveness, safety, progression-free survival, overall survival and tumor characteristics were analyzed. Results All RFA procedures were technically successful without major complications. There were 5 minor complications. No residual or recurrent tumor was seen in the ablation zone during a follow-up of 34.0 ± 18.1 months (0–58 months). No patient required dialysis during follow-up. One patient died after 63 months after the first treatment due to complications from a cerebellar hemangioblastoma. No endpoint was reached for overall or progression-free survival. Conclusions The results from this limited case series suggest that RFA of RCCs in patients with VHL is a safe and effective therapy, which can preserve sufficient renal function even after renal surgery.

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