Introduction/Background*Elevated Alpha-Feto Protein (AFP) in a young female with ovarian mass is virtually diagnostic of Malignant Germ Cell Tumours1. We describe a case with outstanding clinical dilemma where the cause of raised AFP remains unsubstantiated.
Methodology A 13 year old girl presented with lower abdominal discomfort. Ultrasound evaluation suggested large left adnexal dermoid cyst. AFP was elevated at 728ng/dL2. CT scan showed left adnexal mass and a suspicious small lesion in liver without any other abdominal lesion. She was overweight with grade-2 fatty liver, mildly raised alkaline-phosphatase, hepatomegaly with family history of liver malignancy.
A torted left-adnexal smooth mass was removed during surgery. Peritoneal washing, opposite ovary and systematic peritoneal cavity examination were unremarkable. HPE was inconclusive as the tumour was necrotic. After a gap she attended for follow up and on 4th postoperative-month AFP level was 534.84ng/dL.
Further CT and MRI did not reveal any liver lesions. Right ovary had features of polycystic ovary (PCO). On 5th postoperative-month PET/CT revealed FDG avid 3.5 cm solid-cystic lesion in right adnexa with SUV Max of 5.6, suspicious of malignancy. Patient and family underwent thorough counselling between extent of surgeries vs chemotherapy.
Result(s)*On second surgical evaluation the right ovary appeared normal and wedge biopsy was benign. Soon after surgery she attained menarche. At 19th month post index surgery, AFP remained elevated; steady at mid-500 ng/dL level without radiological abnormalities. She is on pathway for weight reduction and regular follow up.
Conclusion*This case report enriches the limited literature on rare reasons for non-hepatic and non-germ cell tumour AFP elevation. Moderate metabolic avidity on PET/CT may signify intense hormonal activities in premenstrual ovary3. Causes like Hereditary Persistence of AFP (HPAFP), persistent elevated AFP due to non-hereditary mutations in enhancer and silencer regions of AFP transcription4, dietary inflammatory agents and autoimmune neuroinflammation5 are some of issues which need further research. It is important to recognise these conditions to avoid inappropriate clinical decisions and minimise anxiety level of all concerned4. There is need for worldwide registry and in-depth research with genome and exome sequencing to explore raised AFP with unaccommodating classical pathologies.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.