Malignant Clinical Course of “Proliferative” Ovarian Struma: Diagnostic Challenges and Treatment Pitfalls

Struma ovarii (SO) is a monodermal teratoma predominantly composed of thyroid tissue (TT) showing benign, “proliferative”, or malignant histology. By imaging, a 38-year-old patient with lower backache revealed a 6.2-cm vertebral lesion (L5). Core biopsy showed well-differentiated TT without features of papillary carcinoma. A 3.5-cm left ovarian mature teratoma (lacking TT) and peritoneal nodules (showing well-differentiated TT) were also identified and surgically removed. Thyroid ultrasound and cytological examination resulted negative. Four years before, left ovarian cystectomy was performed for a histologically “proliferative” SO. According to the malignant clinical course and WHO classification, this case was overall reassessed as a recurring well-differentiated follicular carcinoma arising in SO (WD-FC-SO), despite lacking malignant histological features in any specimens. Immunophenotype: TTF-1+/PAX-8+/thyroglobulin+/CK7+/chromogranin-/synaptophysin-/inhibin-/calretinin-/HNF1B-; Ki-67 index < 5%. Polymerase chain reaction analysis resulted negative for BRAF(V)(600E) mutation. The patient refused further treatments, without recurrence after 17 months. The clinical behavior of SO may be unpredictable. Histologically benign or proliferative strumas extraordinarily metastasize, while SO with malignant features may not recur. The exceptional evidence of peritoneal implants of well-differentiated TT (peritoneal strumosis) in patients with histologically benign SO represents a metastasis of WD-FC-SO (like in our case). A multidisciplinary approach including clinical, laboratory, radiologic, and histopathological data is required.