PMID- 36413217
OWN - NLM
STAT- MEDLINE
DCOM- 20230518
LR  - 20230530
IS  - 1668-3501 (Electronic)
IS  - 0325-0075 (Linking)
VI  - 121
IP  - 3
DP  - 2023 Jun 1
TI  - Persistent spontaneous pneumothorax as a primary manifestation of primary 
      synovial sarcoma of the lung: a case report.
PG  - e202202714
LID - 10.5546/aap.2022-02714.eng [doi]
AB  - Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, 
      uncommon in pediatrics (prevalence: 0.1-0.5%) that predominantly affects 
      adolescents and young adults. Overall survival has been reported to be close to 
      30% at 5 years. Here we report the case of a previously healthy 12-year-old male 
      patient who presented with cough, chest pain, and dyspnea of sudden onset as 
      initial manifestation of left pneumothorax, which persisted after 4 days and 
      required surgical resection of pulmonary bullous lesion. A histological diagnosis 
      of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, 
      which showed chromosomal translocation between chromosomes X and 18: t(X;18) 
      (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or 
      recurrent pneumothorax, it is important to rule out secondary causes, including 
      pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for 
      early diagnosis and aggressive treatment.
CI  - Sociedad Argentina de Pediatria.
FAU - Oneglia, M Macarena
AU  - Oneglia MM
AUID- ORCID: 0000-0002-0575-689X
AD  - Department of Pulmonology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
FAU - Fernandez Sarda, M Sol
AU  - Fernandez Sarda MS
AUID- ORCID: 0000-0001-7642-6399
AD  - Department of Pulmonology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
FAU - Felizzia, Guido
AU  - Felizzia G
AUID- ORCID: 0000-0002-0240-4311
AD  - Department of Hematology and Oncology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. 
      Juan P. Garrahan, City of Buenos Aires, Argentina.
FAU - Alvarez, Mariana
AU  - Alvarez M
AUID- ORCID: 0000-0002-0708-6949
AD  - Department of Anatomic Pathology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan 
      P. Garrahan, City of Buenos Aires, Argentina.
FAU - Barrenechea, Marcelo
AU  - Barrenechea M
AUID- ORCID: 0000-0002-9538-9527
AD  - Department of Surgery; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
FAU - Giubergia, Veronica
AU  - Giubergia V
AUID- ORCID: 0000-0003-0237-8392
AD  - Department of Pulmonology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
FAU - Dicembrino, Manuela
AU  - Dicembrino M
AUID- ORCID: 0000-0002-2652-2881
AD  - Department of Pulmonology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
FAU - Castanos, Claudio
AU  - Castanos C
AUID- ORCID: 0000-0002-3662-5067
AD  - Department of Pulmonology; Hospital de Pediatria S.A.M.I.C. Prof. Dr. Juan P. 
      Garrahan, City of Buenos Aires, Argentina.
LA  - eng
LA  - spa
PT  - Case Reports
PT  - Journal Article
TT  - Neumotorax espontaneo persistente como manifestacion primaria de sarcoma sinovial 
      pleuropulmonar primario: a proposito de un caso.
DEP - 20221124
PL  - Argentina
TA  - Arch Argent Pediatr
JT  - Archivos argentinos de pediatria
JID - 0372460
SB  - IM
MH  - Male
MH  - Adolescent
MH  - Young Adult
MH  - Humans
MH  - Child
MH  - *Pneumothorax/etiology/complications
MH  - *Sarcoma, Synovial/complications/diagnosis/pathology
MH  - *Lung Neoplasms/complications/diagnosis
MH  - Cough
MH  - Lung/pathology
OTO - NOTNLM
OT  - bullae
OT  - lung malignancies
OT  - pediatrics
OT  - pneumothorax
OT  - synovial sarcoma
EDAT- 2022/11/23 06:00
MHDA- 2023/05/18 19:12
CRDT- 2022/11/22 10:56
PHST- 2023/05/18 19:12 [medline]
PHST- 2022/11/22 10:56 [entrez]
PHST- 2022/11/23 06:00 [pubmed]
AID - 10.5546/aap.2022-02714.eng [doi]
PST - ppublish
SO  - Arch Argent Pediatr. 2023 Jun 1;121(3):e202202714. doi: 
      10.5546/aap.2022-02714.eng. Epub 2022 Nov 24.