Presentation
5 year history of an enlarging vulval mass.
Patient Data
Multiloculated cystic mass tracking from the right inguinal region into, and expanding, the right labia. Well encapsulated. No fat density areas are seen on T1. Some enhancement of the wall and peripheral septations post contrast. No evidence of invasion/involvement of adjacent structures.
Heterogeneous, lobulated pleural based mass seen at the posterior aspect of the right upper lobe and posterior superior right lower lobe. The lesion is largely comprised of fat and soft tissue density. No bony destruction.
There is a similar lobulated lesion arising from the anterolateral right pleural at the 5th and 6th rib interspace.
No pulmonary lesions.
Markedly enlarged well encapsulated heterogeneous abdominal mass, appearing centred at the retroperitoneum/base of the small bowel mesentery. The mass displaces small bowel loops to the right and inferiorly, as well as displacing the left kidney superolaterally. Vessels are also displaced to the right.
Case Discussion
This is an unusual case of an exceptionally rare tumour; lipoblastoma-like tumour of the vulva. This patient presented with a locally recurrent large vulval mass. The patient subsequently developed large fat containing pleural and abdominal lesions consistent with metastasis. The pleural lesions were treated with a wedge resection and chemoradiation, but with subsequent development of new lesions elsewhere.
The primary vulval lesion histology was of a well circumscribed myxoid adipocytic lesion. Supplementary FISH testing demonstrated rearrangement of PLAG1, which is unusual for lipoblastoma. The location, gender and age of the patient were also unusual for lipoblastoma. Consensus multi-centre expert pathologist opinion was of a lipoblastoma-like tumour of the vulva. This is a very rare tumour which has histological morphology similar to that of lipoblastoma, but has a larger spindle cell component, lobulated adipocytes and multivacuolated lipoblasts - which were seen in this case. The pleural lesion histology was of a similar tumour type to the vulval lesion.
There are only a small handful of reported case studies of lipoblastoma-like tumour of the vulva in the literature.
Primary differentials include lipoblastoma and liposarcoma.
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