RSS-Feed abonnieren
PDF herunterladen
DOI: 10.5935/2526-8732.20220343
Incidental finding of sex cord tumor with annular tubules in ovary associated with Peutz-Jeghers syndrome
Achado incidental de tumor do cordão sexual com túbulos anulares em ovário associado à síndrome de Peutz-JeghersFinancial support: None to declare.
ABSTRACT
Sex cord tumor with annular tubules (SCTAT) is a rare ovarian pathology. It is usually an incidental finding in asymptomatic patients. In about 30% of cases, this tumor is associated with the Peutz-Jeghers syndrome (PJS). This case report describes an incidental finding of SCTAT during an excisional surgery of a giant mucinous cystadenoma in the contralateral ovary. In addition to the finding of a rare tumor in association with a rare syndrome, a literature review on the diseases, their association, and management was performed.
RESUMO
O tumor do cordão sexual com túbulos anulares (TCSTA) é uma patologia ovariana rara. Geralmente é um achado incidental em pacientes assintomáticos. Em cerca de 30% dos casos, esse tumor está associado à síndrome de Peutz-Jeghers (SPJ). Este relato de caso descreve um achado incidental de TCSTA durante uma cirurgia excisional de um cistoadenoma mucinoso gigante no ovário contralateral. Além do achado de um tumor raro associado a uma síndrome rara, foi realizada uma revisão da literatura sobre as doenças, sua associação e manejo.
Keywords:
Peutz-Jeghers syndrome - Sex cord-gonadal stromal tumors - Ovarian sex cord-stromal tumours - Mucinous cystadenoma - Mucinous ovarian tumorsPalavras-chave:
Síndrome de Peutz-Jeghers - Tumores do estroma cordão-gonadal sexual - Tumores do cordão sexual ovariano - Cistadenoma mucinoso - Tumores mucinosos do ovárioPublikationsverlauf
Eingereicht: 04. April 2022
Angenommen: 18. Mai 2022
Artikel online veröffentlicht:
22. Juni 2022
© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)
Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil
Nathalia Beck Corrêa, Laura Paggiarin Skonieski, Francisco Costa Beber Lemanski, Rafael Baldissera. Incidental finding of sex cord tumor with annular tubules in ovary associated with Peutz-Jeghers syndrome. Brazilian Journal of Oncology 2022; 18: e-20220343.
DOI: 10.5935/2526-8732.20220343
-
REFERENCES
- 1
Chen X,
Crapanzano JP,
Gonzalez AA,
Hamele-Bena D.
Cytologic features of needle aspiration of ovarian sex cord tumor with annular tubules:
Report of two cases and literature review. Diagn Cytopathol 2018; Jul; 46 (07) 627-631
http://doi.wiley.com/10.1002/dc.23908
MissingFormLabel
- 2
Mayerson BD,
Cuello FM,
Brañes YJ,
Leiva CV,
Castiblanco GA.
Tumor de los cordones sexuales con túbulos anulares del ovario no asociado a síndrome
de Peutz-Jeghers: reporte de un caso. Rev Chil Obstet Ginecol [Internet] 2006; 71
(01) 57-62 Available from: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0717-75262006000100010&lng=en&nrm=iso&tlng=en
MissingFormLabel
- 3
Nevozinskaya Z,
Korsunskaya I,
Sakaniya L,
Perlamutrov Y,
Sobolev V.
Peutz-Jeghers syndrome in dermatology. Acta Dermatovenerol Alp Pannonica Adriat [Internet]
2019; Sep; 28 (03) 1357 Available from: http://www.ncbi.nlm.nih.gov/pubmed/31545393
MissingFormLabel
- 4
Maity P,
Das N,
Chatterjee U,
Basak D.
Sex-cord tumor with annular tubules with unusual morphology in an infant with Peutz-Jeghers
syndrome. Fetal Pediatr Pathol [Internet] 2020; Apr; 41 (02) 306-311 Available from:
http://www.ncbi.nlm.nih.gov/pubmed/32609034
MissingFormLabel
- 5
Senn D,
Videira H,
Haagsma B,
El-Bahrawy M,
Madhuri TK.
Sex cord tumour with annular tubules-an unusual case of abdominal pain. J Obstet Gynaecol
Canada [Internet] 2021; Mar; 43 (03) 361-364 Available from: https://linkinghub.elsevier.com/retrieve/pii/S170121632030582X
MissingFormLabel
- 6
Schultz KA,
Harris AK,
Schneider DT,
Young RH,
Brown J,
Gershenson DM.
et al.
Ovarian sex cordstromal tumors. J Oncol Pract 2016; 12 (10) 940-946 https://doi.org/10.1200/JOP.2016.016261
MissingFormLabel
- 7
Hemminki A,
Markie D,
Tomlinson I,
Avizienyte E,
Roth S,
Loukola A.
et al.
A serine/threonine kinase gene defective in Peutz-Jeghers syndrome. Nature [Internet]
1998; Jan; 391 (6663) 184-187 Available from: http://www.nature.com/articles/34432
MissingFormLabel
- 8
Jenne DE,
Reomann H,
Nezu J,
Friedel W,
Loff S,
Jeschke R.
et al.
Peutz-Jeghers syndrome is caused by mutations in a novel serine threoninekinase. Nat
Genet [Internet] 1998; Jan; 18 (01) 38-43 Available from: http://www.nature.com/articles/ng0198-38
MissingFormLabel
- 9
Utsunomiya J,
Gocho H,
Miyanaga T,
Hamaguchi E,
Kashimure A.
Peutz-Jeghers syndrome: its natural course and management. Johns Hopkins Med J [Internet]
1975; Feb; 136 (02) 71-82 Available from: http://www.ncbi.nlm.nih.gov/pubmed/1117595
MissingFormLabel
- 10
Swanger RS,
Brudnicki A.
Ultrasound of ovarian sexcord tumor with annular tubules. Pediatr Radiol [Internet]
2007; Dec; 37 (12) 1270-1271 Available from: http://www.ncbi.nlm.nih.gov/pubmed/17764009
MissingFormLabel
- 11
Qian Q,
You Y,
Yang J,
Cao D,
Zhu Z,
Wu M.
et al.
Management and prognosis of patients with ovarian sex cord tumor with annular tubules:
a retrospective study. BMC Cancer [Internet] 2015; Dec; 15 (01) 270 Available from:
http://bmccancer.biomedcentral.com/articles/10.1186/s12885-015-1277-y
MissingFormLabel