Germ-cell tumours: Case

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]

The surgery and the staging

The surgery is the fertility-sparing unilateral salpingo-oophorectomy, with the preservation of the uterus and the contralateral ovary, because the unilateral ovarian germ cell tumour is curable with the ovarian preservation and the future fertility is protected. The contralateral ovary is inspected, and the peritoneal washings and the omental biopsy are taken for the staging. The histology confirms the yolk sac tumour, and the stage three designation, with the local extension or the nodal involvement, is established. The postoperative alpha-fetoprotein is followed serially, because the marker fall tracks the response to the chemotherapy and the marker rise signals the residual or the recurrent disease. [10]

The named diagnosis and the definitive management

The diagnosis is a stage three ovarian yolk sac tumour. The definitive management is the fertility-sparing surgery, already performed, followed by the platinum-based PEB chemotherapy of the cisplatin, the etoposide and the bleomycin, given in three to four cycles. The alpha-fetoprotein falls with each cycle, and the marker response confirms the treatment effect. The contemporary survival of the localised and the regional malignant ovarian germ cell tumour is excellent, in the range of over ninety percent for the localised disease and seventy to eighty percent for the higher-stage disease, and the gonadal dysgenesis is sought and counselled because it is associated with the worse outcome. [4][3]

The girl is enrolled on the national protocol, and the multidisciplinary team is assembled: the paediatric oncologist, the paediatric or the gynaecological surgeon, the fertility specialist, the clinical nurse specialist, the social worker and the psychologist. The family is taught the recurrence signs, the late effects, the central line care and the school support. The fertility and the reproductive counselling are begun early, because the platinum chemotherapy carries the gonadotoxicity and the fertility preservation is considered before the gonadotoxic exposure wherever the staging allows. [5][10]

The chemotherapy toxicity and the surveillance

The examiner asks the candidate to outline the toxicity and the surveillance. The bleomycin produces the pulmonary fibrosis, the dose-dependent injury that is monitored with the pulmonary function tests, and the cisplatin produces the sensorineural hearing loss and the nephrotoxicity, monitored with the audiology and the renal function. The myelosuppression and the infection risk are the acute toxicities of each cycle, and the growth factor support is used as the protocol dictates. The alpha-fetoprotein is monitored serially for the recurrence, and the magnetic resonance imaging is performed at the intervals, because the recurrence most often declares within the first two years. [4][5]

Communication and the family

The family is counselled honestly and hopefully. The candidate names the diagnosis, explains that the ovarian yolk sac tumour secretes the alpha-fetoprotein that the treatment tracks, and that the contemporary survival is excellent with the fertility-sparing surgery and the platinum-based chemotherapy. The parents and the girl are introduced to the multidisciplinary team, given a written plan, taught the recurrence and the late-effects surveillance, and supported by the social work and the psychology. The fertility and the reproductive counselling are addressed with the sensitivity that the age and the gender demand, and the survivorship plan is begun from the day of diagnosis. [12]