Literature DB >> 28710192

Organ-sparing surgery for large cell calcifying Sertoli cell tumour in a patient with Carney complex.

Maria José Freire1, Pedro Nunes1, Luà S Sousa1, Arnaldo Figueiredo1,2.   

Abstract

Carney complex is a rare genetic disease characterised by a complex of myxomas, spotty pigmentation and endocrine overactivity. At diagnosis, about one-third of male patients presents with testicular tumours, namely large cell calcifying Sertoli cell tumours, which are often multicentric and/or bilateral and have a low malignant potential. Although radical orchiectomy is the gold standard for the treatment of testicular neoplasms, a conservative approach with partial orchiectomy or tumourectomy may be the best treatment option for these patients, allowing the preservation of endocrine function, fertility and body image. We present a case of a 19-year-old man with a known history of Carney complex with early identification of a small testicular tumour treated with organ-sparing surgery. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Entities:  

Keywords:  surgical oncology; urinary and genital tract disorders; urological cancer; urological surgery

Mesh:

Year:  2017        PMID: 28710192      PMCID: PMC5534819          DOI: 10.1136/bcr-2017-219557

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  16 in total

Review 1.  Carney complex: the first 20 years.

Authors:  Sosipatros A Boikos; Constantine A Stratakis
Journal:  Curr Opin Oncol       Date:  2007-01       Impact factor: 3.645

2.  Haploinsufficiency at the protein kinase A RI alpha gene locus leads to fertility defects in male mice and men.

Authors:  Kimberly A Burton; Deborah A McDermott; David Wilkes; Melissa N Poulsen; Michael A Nolan; Marc Goldstein; Craig T Basson; G Stanley McKnight
Journal:  Mol Endocrinol       Date:  2006-05-25

Review 3.  Large cell calcifying Sertoli cell tumor of testis.

Authors:  Shams K Halat; Lee E Ponsky; Gregory T MacLennan
Journal:  J Urol       Date:  2007-06       Impact factor: 7.450

Review 4.  Organ-sparing surgery for adult testicular tumours: a systematic review of the literature.

Authors:  Gianluca Giannarini; Klaus-Peter Dieckmann; Peter Albers; Axel Heidenreich; Giorgio Pizzocaro
Journal:  Eur Urol       Date:  2010-01-20       Impact factor: 20.096

5.  Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex.

Authors:  L S Kirschner; J A Carney; S D Pack; S E Taymans; C Giatzakis; Y S Cho; Y S Cho-Chung; C A Stratakis
Journal:  Nat Genet       Date:  2000-09       Impact factor: 38.330

6.  Genetic heterogeneity and spectrum of mutations of the PRKAR1A gene in patients with the carney complex.

Authors:  L S Kirschner; F Sandrini; J Monbo; J P Lin; J A Carney; C A Stratakis
Journal:  Hum Mol Genet       Date:  2000-12-12       Impact factor: 6.150

Review 7.  Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation.

Authors:  C A Stratakis; L S Kirschner; J A Carney
Journal:  J Clin Endocrinol Metab       Date:  2001-09       Impact factor: 5.958

8.  Testicular tumors in Carney's complex.

Authors:  Robert Washecka; Martin I Dresner; Stacey A A Honda
Journal:  J Urol       Date:  2002-03       Impact factor: 7.450

9.  Mutations in regulatory subunit type 1A of cyclic adenosine 5'-monophosphate-dependent protein kinase (PRKAR1A): phenotype analysis in 353 patients and 80 different genotypes.

Authors:  Jérôme Bertherat; Anélia Horvath; Lionel Groussin; Sophie Grabar; Sosipatros Boikos; Laure Cazabat; Rosella Libe; Fernande René-Corail; Sotirios Stergiopoulos; Isabelle Bourdeau; Thalia Bei; Eric Clauser; Alain Calender; Lawrence S Kirschner; Xavier Bertagna; J Aidan Carney; Constantine A Stratakis
Journal:  J Clin Endocrinol Metab       Date:  2009-03-17       Impact factor: 5.958

10.  Frozen section analysis-guided organ-sparing approach in testicular tumors: technique, feasibility, and long-term results.

Authors:  Hannes Steiner; Lorenz Höltl; Christoph Maneschg; Andreas P Berger; Hermann Rogatsch; Georg Bartsch; Alfred Hobisch
Journal:  Urology       Date:  2003-09       Impact factor: 2.649

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