Malignant struma ovarii harboring a unique NRAS mutation: case report and review of the literature.

Struma ovarii (SO), a rare tumor containing at least 50% of thyroid tissue, represents approximately 5% of all ovarian teratomas; its malignant transformation rate is reported to occur in up to 10% of cases and metastases occur in about 5-6% of them. We describe a 36-year old woman who underwent laparoscopic left annessectomy two years earlier because of an ovarian cyst. Follow-up imaging revealed a right adnexal mass, ascitis and peritoneal nodes that were diagnosed as comprising a malignant SO with peritoneal secondary localizations at histopathology performed after intervention. Restaging with 18F-FDG-PET/CT scan, abdominal CT and ultrasonography showed abnormalities in the perihepatic region and presacral space and left hypochondrium localizations. The patient underwent thyroidectomy, hepatic nodulectomy and cytoreductive peritonectomy: histopathological examination did not show any malignant disease in the thyroid and confirmed the presence of peritoneal localizations due to malignant SO; molecular analysis detected NRAS Q61K mutation in exon 3, whereas no mutations were identified on the BRAF gene. The patient underwent radioiodine treatment: serum Tg was decreased at first follow-up after three months of 131I-therapy. We believe that our case raises some interesting considerations. First, pathologists should be aware of this entity and should check for the presence of point mutations suggesting an aggressive disease behavior, which could be beneficial for an optimal therapeutic approach. Second, although most of the knowledge in this field comes from case reports, efforts should be made to standardize the management of patients affected by malignant SO, including use of practice guidelines.

Is the standardized uptake value of FDG-PET/CT predictive of pathological complete response in locally advanced rectal cancer treated with capecitabine-based neoadjuvant chemoradiation?

OBJECTIVES: Our aim was to assess FDG-PET/CT as a surrogate biomarker of the pathological complete response in locally advanced rectal cancer treated with neoadjuvant chemoradiation. METHODS: T3-4 and/or N+ rectal cancer patients were treated prospectively with capecitabine-based chemoradiation and total mesorectal excision 7-8 weeks later. FDG-PET/CT uptake was obtained at baseline, after 2 weeks, and 6 weeks following treatment completion, calculating the maximum standardized uptake value (SUV) and percentage difference to identify the early and late metabolic 'response index'. RESULTS: Thirty-one patients were treated from January 2009 to January 2012 at the Istituto Nazionale dei Tumori of Milan. One patient was excluded due to surgery refusal. The pathological complete response rate was 30%. Early FDG-PET/CT was performed in 24 consenting patients and failed to show predictive utility. On the contrary, significant differences in late SUV value and response index were observed between complete and noncomplete pathological responders (p = 0.0006 and 0.03). In multivariate analysis including most relevant SUV parameters, none of them was independently associated with a pathological complete response. With receiver operating characteristic curve analysis, a late SUV threshold <5.4 had 81% sensitivity and 100% specificity, with 90% overall accuracy. CONCLUSIONS: We evidenced a possible predictive role of late FDG-PET/CT for the assessment of pathological response in locally advanced rectal cancer following neoadjuvant chemoradiation.