Germ cell Tumours Fiji 1 December 2016 - PowerPoint Presentation

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Germ cell Tumours

Fiji 1 December 2016Dr Jane Skeen- for the NZ NCCN Pacific working group

Germ cell tumoursRare and highly varied group of tumoursOccurs at several anatomical sitesHistological spectrum from mature to immature teratoma to 4 different malignant subtypesPrior to platinum based chemotherapy -survival poorBEP :bleomycin etoposide and cisplatin – nephro and ototoxic

JEB : bleomycin etoposide and carboplatin

Risk groups

LOWINTERMEDIATEHIGHGonadal Stage 1 (regardless of AFP)Testis <5yrs, any AFP, Stages 2-4

All Stage 4 tumours except testis <5yrs and germinoma and seminoma

Testis ≥5yrs, AFP <10000, Stage 2+3

AFP ≥10000 except all

Stage 1 tumours and testis <5yrs Stages 2-4

All

other sites

AFP <10000, Stage 2+3, except thoracic tumours

All thoracic

tumours Stages 2-4

Pure germinoma, seminoma,

any site, Stages 2-4

Pure

HCG secreting tumours, any HCG, Stage 2+3

Histological classification Germ cell tumoursGerminomaTeratoma -Mature -Immature

-Malignant teratomaEmbryonal carcinomaYolk sac tumourChoriocarcinomaGonadoblastomaMixed malignant germ cell tumour

PI GC 1 Protocol6* cycles of identical treatment for all stages and histologiesCarboplatin (JM8), Etoposide, Bleomycin (JEB)Each cycle 21

days(* Number of cycles (x+2) where x is the number required to achieve CR)CR assessment will be assisted by ability to measure and follow appropriate tumour markers  Total therapy duration usually no more than 6 months

Initial EvaluationComplete history including family history.Complete physical examination

Chest X-ray and abdominal ultrasound.CT scan of affected area plus chest and abdomen .Full blood and platelet count.Urea, creatinine, electrolytes, calcium, liver function testsTumour Markers- alpha fetoprotein / βHCG / Ca 125

Eligibility/TreatmentAll patients with biopsy proven Germ cell Tumours (with the exception of Stage 1 Testicular tumours)Stage 1 testicular tumours that post-

orchidectomy show decreasing AFP at expected rate, will receive no further treatment unless there is arise in the AFP or clinical recurrence. The initial cycle of therapy should be given as soon as practically possible after appropriate supportive care has been given. This will include correction of anaemia (if applicable), treatment of infection and any

co-morbidities.

TreatmentThe half-life of alpha fetoprotein is 4-7 days AFP levels should reduce by at least 50% each week. If AFP fails to normalise or having fallen starts to rise again, reimaging indicated, with a view to second look surgery.

9 yr old - femaleJanuary 2007 presented with a painful distended abdomen History of becoming unwell whilst on extended holiday

Transferred to Starship Imaging : an extensive non-calcified abdomino-pelvic mass, ascites, peritoneal and subdiaphragmatic deposits AFP (alpha-fetoprotein) > 60,000 unitsPresumptive diagnosis : Germ cell tumour

12.1.07 Laparotomy and biopsy of mass large intra-operative bleed tumour appeared necrotic omentum was studded with nodulespresumed prior tumour rupture

urinary output compromised due to the raised intra-abdominal pressure related to the ascites

Chemotherapy commenced carboplatin/etoposide (JE)Evening of 18.1.07 collapsed Resuscitated then admitted to PICU for ventilation

Abdomen decompressed after insertion of pigtail catheter under ultrasound guidance 3 litres of ascitic fluid was drained and the catheter remained in situ

Histology : tumour was haemorrhagic and necrotic with a typical endodermal sinus pattern Features of yolk sac tumour with positive markers for alpha-fetoprotein, other tumour markers negative

No ovarian tissue seen in the biopsies Presumed extra gonadal advanced stage III yolk sac tumour Presumed that tumour ruptured pre-biopsy with subsequent peritoneal seeding (family later stated abdominal massage) With chemotherapy- good chance of cure

α

-FP decline

Received 6 cycles chemotherapy ( JE/JEB x5) (carboplatin/Etoposide/bleomycin)Post cycle 6 : AFP 46.4 (N< 10)Second look surgery 21 May: right ovary normal left in situ- some residual tissue resected

28 May AFP 11.4Histology- no viable tumour5 June AFP normal 4Remains well AFP normalLast reviewed September 2016Wants to join the Navy