JavaScript is NOT enabled !

* Show help

Workplace: Aquesta Pathology Toowong QLD Australia

1 hits in Medvik Filters

Online article

Expanding the clinicopathological spectrum of succinate dehydrogenase-deficient renal cell carcinoma with a focus on variant morphologies: a study of 62 new tumors in 59 patients

Fuchs, Talia L
Author Fuchs, Talia L ORCID Sydney Medical School, The University of Sydney, Sydney, Australia Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, St Leonards, NSW, Australia NSW Health Pathology, Department of Anatomical Pathology, Royal North Shore Hospital, NSW Health Pathology, St Leonards, NSW, Australia
Maclean, Fiona
Author Maclean, Fiona Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, St Leonards, NSW, Australia Anatomical Pathology, Douglass Hanly Moir Pathology, Sonic Healthcare, Macquarie Park, NSW, Australia Discipline of Pathology, Macquarie Medical School, Macquarie University, Macquarie Park, NSW, Australia
Turchini, John
Author Turchini, John Sydney Medical School, The University of Sydney, Sydney, Australia Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, St Leonards, NSW, Australia Anatomical Pathology, Douglass Hanly Moir Pathology, Sonic Healthcare, Macquarie Park, NSW, Australia Discipline of Pathology, Macquarie Medical School, Macquarie University, Macquarie Park, NSW, Australia
Vargas, A Cristina
Author Vargas, A Cristina ORCID Sydney Medical School, The University of Sydney, Sydney, Australia Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, St Leonards, NSW, Australia Anatomical Pathology, Douglass Hanly Moir Pathology, Sonic Healthcare, Macquarie Park, NSW, Australia Discipline of Pathology, Macquarie Medical School, Macquarie University, Macquarie Park, NSW, Australia
Bhattarai, Selina
Author Bhattarai, Selina Leeds Teaching Hospital, Leeds, UK
Agaimy, Abbas
Author Agaimy, Abbas ORCID Institute of Pathology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, Erlangen, Germany
Hartmann, Arndt
Author Hartmann, Arndt Institute of Pathology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, Erlangen, Germany
Kao, Chia-Sui
Author Kao, Chia-Sui Stanford University Medical Center, Stanford, CA, USA
Ellis, Carla
Author Ellis, Carla Northwestern University, Chicago, IL, USA
Bonert, Michael
Author Bonert, Michael McMaster University, Hamilton, Ontario, Canada

Modern pathology. 2022 ; 35 (6) : 836-849. [pub] 20211223

Mod Pathol
ISSN 1530-0285
Medvik
Source

Most succinate dehydrogenase (SDH)-deficient renal cell carcinomas (RCCs) demonstrate stereotypical morphology characterized by bland eosinophilic cells with frequent intracytoplasmic inclusions. However, variant morphologic features have been increasingly recognized. We therefore sought to investigate the incidence and characteristics of SDH-deficient RCC with variant morphologies. We studied a multi-institutional cohort of 62 new SDH-deficient RCCs from 59 patients. The median age at presentation was 39 years (range 19-80), with a slight male predominance (M:F = 1.6:1). A relevant family history was reported in 9 patients (15%). Multifocal or bilateral tumors were identified radiologically in 5 patients (8%). Typical morphology was present at least focally in 59 tumors (95%). Variant morphologies were seen in 13 (21%) and included high-grade nuclear features and various combinations of papillary, solid, and tubular architecture. Necrosis was present in 13 tumors, 7 of which showed variant morphology. All 62 tumors demonstrated loss of SDHB expression by immunohistochemistry. None showed loss of SDHA expression. Germline SDH mutations were reported in all 18 patients for whom the results of testing were known. Among patients for whom follow-up data was available, metastatic disease was reported in 9 cases, 8 of whom had necrosis and/or variant morphology in their primary tumor. Three patients died of disease. In conclusion, variant morphologies and high-grade nuclear features occur in a subset of SDH-deficient RCCs and are associated with more aggressive behavior. We therefore recommend grading all SDH-deficient RCCs and emphasize the need for a low threshold for performing SDHB immunohistochemistry in any difficult to classify renal tumor, particularly if occurring at a younger age.

MeSH
Adult MeSH
Hyperplasia MeSH
Immunohistochemistry MeSH
Carcinoma, Renal Cell * genetics pathology MeSH
Middle Aged MeSH
Humans MeSH
Young Adult MeSH
Kidney Neoplasms * genetics pathology MeSH
Necrosis MeSH
Aged, 80 and over MeSH
Aged MeSH
Succinate Dehydrogenase genetics MeSH
Check Tag
Adult MeSH
Middle Aged MeSH
Humans MeSH
Young Adult MeSH
Male MeSH
Aged, 80 and over MeSH
Aged MeSH
Female MeSH
Publication type
Journal Article MeSH

Collections

Published

Filters

* Show help

* Show help

Refine by MeSH