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Sökning: WFRF:(Johan Lundberg) > Örebro universitet > Lodefalk Maria 1968 > Granulosa cell tumo...

  • Kiss, EszterDepartment of Pediatrics, Örebro University Hospital, Örebro, Sweden (författare)

Granulosa cell tumors in girls : Preliminary results of a meta-analysis of new and published cases

  • Artikel/kapitelEngelska2023

Förlag, utgivningsår, omfång ...

  • S. Karger,2023
  • printrdacarrier

Nummerbeteckningar

  • LIBRIS-ID:oai:DiVA.org:oru-110892
  • https://urn.kb.se/resolve?urn=urn:nbn:se:oru:diva-110892URI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:vet swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • Background: Granulosa cell tumors (GCT) originate from sex cord/stromal tissue in the gonad. They are typically located in an ovary, but extra-gonadal localisation exists. These tumors are extremely rare in children and no systematic review has been published. The objective of this systematic review is to examine the following questions: What is the clinical picture of girls with a GCT? How are these patients treated and what is their prognosis?Methods: To be included in the review, the article had to present a new case with GCT fulfilling the following criteria: female human fetus or a girl aged < 19 years with clinical information included a tumor containing granulosa cells.The databases MEDLINE, Embase, Web of Science, and CINAHL were searched in November 2021. To find new cases, we asked pediatric endocrinologists in Sweden to report patients after informed consent had been secured. We also collected data from a Swedish paediatric reference pathology laboratory.Results: The search identified 1,894 published references of which 35 were duplicates. We have screened 1,859 abstracts. We are in the process of reading 824 selected articles in full text to check for eligibility. Individual participant data has been extractedfrom 20 of the published reports for preliminary results. Nineteen new Swedish cases with a GCT were identified.The preliminary analysis of 39 patients’ data shows an average age of 7.3 years at the time of diagnosis (range: antenatal diagnosis up to 18 years of age). Symptoms at presentation were: prepubertal breast enlargement, vaginal discharge/bleeding, abdominal distension or pain, pubic hair growth, fever, constipation, swelling of vulva or cliteromegaly, hyperpigmentation of the skin, primary/secondary amenorrhea, headache, hirsutism and advanced linear growth.The histopathological diagnosis was juvenile GCT in 76.9%, adult GCT in 12.8%, a mixed type of juvenile and adult GCT in 7.7% and another type of tumor containing granulosa cell component in 2.6% of the cases.All patients received surgical treatment except one with a post-mortem GCT diagnoses. Adjuvant chemotherapy was administered in two cases.Three patients (7.7%) died, two of them due to late discovery of the primary tumor and one secondary to local recurrence of the tumor with metastases 4 years after the primary diagnosis.Conclusion: GCT can present in all pediatric ages and often, but far from always, with endocrine symptoms such as peripheral precautious puberty. Data from this systematic review will hopefully promote early recognition of this malignant disease.

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Wessmann, SandraDepartment of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Cancer diagnostics, Karolinska University Hospital, Stockholm, Sweden (författare)
  • Carlson, Joseph W.Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Laboratory Medicine, Keck School of Medicine, University of Southern California, Los Angeles, USA (författare)
  • Lundberg, ElenaInstitute of Clinical Science/Pediatrics, Umeå University, Umeå, Sweden (författare)
  • Stenmarker, MargarethaDepartment of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; Futurum/Department of Paediatrics, Region Jönköping County, Jönköping, Sweden; Department of Paediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden (författare)
  • Bobeck, JohanDepartement of Pediatrics, Region Kalmar, Kalmar, Sweden (författare)
  • Lodefalk, Maria,1968-Örebro universitet,Institutionen för medicinska vetenskaper,Region Örebro län,Department of Pediatrics; University Health Care Research Center(Swepub:oru)malk (författare)
  • Department of Pediatrics, Örebro University Hospital, Örebro, SwedenDepartment of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Cancer diagnostics, Karolinska University Hospital, Stockholm, Sweden (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:Hormone Research in Paediatrics: S. Karger96:Suppl. 4, s. 126-1271663-28181663-2826

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