Treatment

Nicola has completed a course of treatment for a rare ovarian tumour.

She presented at the age of 23 years in labour with her first baby. Unfortunately the labour became obstructed and she had a caesarian section. At the caesarian section she was noted to have extensive masses within the pelvis and large masses within the liver. Prior to labour she had been perfectly well and no abnormalities had been noted on routine antenatal scans, physical examination or routine blood tests. She was entirely asymptomatic.

The histology was reviewed by Joya Parwadi who sought the opinion of others and undertook extensive immunocytochemical staining and cytogenetic studies. The consensus following the studies was that she had a desmoplastic small round cell tumour of the ovary. Histology and immunochemistry was compatible with this together with a characteristic t(11 ;22) translocation.

As you will know, desmoplastic small round cell tumours are rare entities and are most commonly seen in adolescent boys and young men. However, appraisal of the literature revealed something in the region of 100 cases reported in ovarian cancer - none occurring during pregnancy. Appraisal of the literature revealed rather depressing results with a very high mortality rate even when the tumour appeared localised at operation. Although the outlook is almost uniformly poor, there are anecdotal case reports of good responses to aggressive multi-agent chemotherapy and surgery.

Given the age of the patient and her situation and determination to do everything possible to overcome her tumour, we decided to embark on chemotherapy with two alternating non-cross resistant chemotherapy regimes. She received Cisplatin 100 mg IV on day one together with Doxorubicin 25 mg per metre squared IV on days one, two and three in the first regime. This was alternated with the TIP (Paclitaxel 175 mg per metre squared IV day one, Cisplatin 20 mg per metre squared day one to five, Ifosfamide 1 gram per metre squared IV days one to five together with Mesna 1 gram per metre squared IV days one to five). She received 3 cycles of each of these regimes to a total of 6 cycles in all.

The intention was to give 2 cycles of this chemotherapy and if she was obtaining a major response to take stem cells with a view of high dose alkylating agent therapy with peripheral stem cell support at the end of her initial chemotherapy.

However, scans during the course of her 6 cycles of chemotherapy showed that although she was responding she was only doing so modestly and was clearly not going to obtain a complete remission. We felt that it would be important for her to attain at least a complete remission if we were going to consider high dose chemotherapy.

CT scan at the end of her chemotherapy revealed reduction in the size of the pelvic masses and some reduction in the liver metastases but there is still clearly extensive disease. She tolerated the chemotherapy very well, despite having multiple admissions with neutropenic fever. She is a very determined person who has come to terms largely with the diagnosis, but clearly is anxious to seek any way to try and overcome the tumour. One of the research procedures in this area has been to consider using Gleevec in patients who are c-KIT positive. Unfortunately immunohistochemistry showed that she did not stain for c-KIT.

We talked about the possibility of surgical resection. The gynaecologists feel that this is unlikely to be helpful - on the basis of the extent of residual disease within the pelvis and of course on the major involvement of the liver.

1st October 2003

1st October 2004

Nicola has more chemotherapy planned see Latest News for details.