The largest reported papillary thyroid carcinoma arising in struma ovarii and metastasis to opposite ovary: case report and review of literature

BACKGROUND: Malignant struma ovarii (MSO) is a very rare, germ cell tumor of the ovary, histologically identical to differentiated thyroid cancers. Struma ovarii (SO) is difficult to diagnose on clinical basis or imaging and is mostly discovered incidentally, with few published cases in the literature. CASE PRESENTATION: A 42-year old primiparous woman presented with abdominal pain and midline pelvic palpable firm mass arising from the pelvis. Imaging showed pelvic solid cystic mass. Total abdominal hysterectomy, bilateral salpingo-oopherectomy (TAH BSO) and infracolic omentectomy were performed. Histopathology revealed left ovary papillary thyroid carcinoma (PTC) arising in SO (11 cm) and metastatic papillary thyroid carcinoma in the right ovary. Thyroid functions tests were all normal, ultrasound thyroid showed two complex nodules in the left thyroid lobe. Total thyroidectomy was decided, but the patient refused further surgical management and was lost to follow up as she left the country. We undertook a comprehensive literature search, and MSO and thyroid management data from 23 additional publications were analyzed and tabulated. This PTC MSO is probably the largest reported in the literature. CONCLUSIONS: Among the different surgeries for MSO, TAH + BSO appears to have the best clinical outcome. However, unilateral salpingo-oopherectomy/ unilateral oophorectomy and bilateral salpingo-oopherectomy also seem effective. Ovarian cystectomy alone seems associated with higher recurrence. There remains no consensus on the associations between MSO tumor size and potential extent of metastasis, and about the management of thyroid gland. However, surveillance and thyroid gland work up to detect concurrent thyroid cancer are recommended.