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Paeds Caseshaematology-oncology-and-transfusion

Paeds Cases · haematology-oncology-and-transfusion

Germ-cell tumours: Case

Clinical long case of a fifteen-year-old girl presenting with abdominal pain and a large pelvic mass from a stage three ovarian yolk sac tumour with a markedly raised alpha-fetoprotein, covering the serum marker and the imaging pathway, the fertility-sparing surgery, the platinum-based PEB chemotherapy, the marker response and the late-effects and the fertility surveillance.

paediatric oncology long case4 min readVerification in progress

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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A fifteen-year-old girl is brought to the emergency department with two weeks of progressive lower abdominal pain and a palpable pelvic mass. A pregnancy test is negative. The serum alpha-fetoprotein is markedly elevated at six thousand nanograms per millilitre, and the beta-human chorionic gonadotropin is normal. The pelvic ultrasound and the magnetic resonance imaging show a large complex solid and cystic left ovarian mass measuring twelve centimetres with no evidence of the distant metastases on the computed tomography of the chest. The examiner asks how you frame the problem, how you confirm and stage the diagnosis, how you manage the tumour, and how you build the survivorship and the fertility plan.

Framing the case

This fifteen-year-old girl has the classic presentation of an ovarian germ cell tumour with the markedly raised alpha-fetoprotein that points to the yolk sac histology. The abdominal pain and the large pelvic mass, the negative pregnancy test, and the alpha-fetoprotein at six thousand nanograms per millilitre with the normal beta-human chorionic gonadotropin, together make the malignant nongerminomatous ovarian germ cell tumour the working diagnosis, and the first decision is to confirm and to stage the tumour and to plan the surgery and the chemotherapy. The framework that organises the case is the histology, the yolk sac tumour that secretes the alpha-fetoprotein, and the stage, which together drive the risk-adapted therapy. [3][10]

Immediate assessment and the diagnostic pathway

The assessment begins with the airway, the breathing and the circulation, because the adolescent with the large abdominal mass may have the torsion or the rupture that produces the acute surgical abdomen, and the haemodynamic stability is confirmed first. The serum alpha-fetoprotein, the beta-human chorionic gonadotropin and the lactate dehydrogenase are sent, and the markedly raised alpha-fetoprotein with the normal beta-human chorionic gonadotropin makes the yolk sac tumour the leading diagnosis. The pelvic ultrasound and the magnetic resonance imaging define the complex solid and cystic mass, and the computed tomography of the chest stages the metastatic disease. The girl is over one year of age, so the alpha-fetoprotein is not confounded by the physiological neonatal elevation, and the raised value is genuinely significant. [3][10]

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References5ShowHide
  1. [4]Frazier AL, Stoneham S, Rodriguez-Galindo C Comparison of carboplatin versus cisplatin in the treatment of paediatric extracranial malignant germ cell tumours: A report of the Malignant Germ Cell International Consortium Eur J Cancer, 2018.PMID 29859339
  2. [5]Lawrence NJ, Chan H, Toner G Protocol for the P3BEP trial (ANZUP 1302): an international randomised phase 3 trial of accelerated versus standard BEP chemotherapy for adult and paediatric male and female patients with intermediate and poor-risk metastatic germ cell tumours BMC Cancer, 2018.PMID 30157803
  3. [3]O'Neill AF, Xia C, Krailo MD alpha-Fetoprotein as a predictor of outcome for children with germ cell tumors: A report from the Malignant Germ Cell International Consortium Cancer, 2019.PMID 31355926
  4. [10]Dicken BJ, Billmire DF, Krailo M Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study Pediatr Blood Cancer, 2018.PMID 29286555
  5. [12]Williams LA, Frazier AL, Poynter JN Survival differences by race/ethnicity among children and adolescents diagnosed with germ cell tumors Int J Cancer, 2020.PMID 31304572
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