Incidence, clinical characteristics, and survival outcomes of ovarian strumal diseases: a retrospective cohort study.

BACKGROUND: Struma ovarii (SO) is a rare tumor and may transform into ovarian strumal carcinoid (OSC) and/or malignant struma ovarii (MSO), but the incidence, clinical characteristics, and survival outcomes have not been well defined. METHODS: We conducted a retrospective study of patients with ovarian strumal diseases treated in the our hospital between 1980 and 2022. Subgroup analyses of SO, OSC, and MSO were subsequently performed. RESULTS: A total of 275 cases (2.14%) were identified in a cohort of 12,864 patients with ovarian teratomas, where SO, OSC, and MSO accounted for 83.3%, 12.0%, and 4.7% of cases, respectively. There were no significant differences in age, tumor sizes, elevated tumor markers, and ascites among the three subgroups. At initial treatment, all patients with SO or OSC had FIGO stage I disease except one SO patient presenting metastatic disease, ten patients had MSO confined to the ovary, whereas other three patients had metastatic diseases. Two patients with SO respectively relapsed at peritoneum and anterior mesorectum, while none of the OSC patients presented tumor recurrence or death despite different surgical procedures employed. The 5-year recurrence-free survival rate was 88.9%, and only one death occurred at 9.5 years after diagnosis in patients with MSO. Radioiodine therapy showed satisfactory therapeutic efficacy, but these patients showed poor responses to the chemotherapy. CONCLUSION: 2.14% of ovarian teratoma could be classified as SO, of which 12.0% and 4.7% of SO may transform into OSC and MSO, repsectively. The survival outcomes were excellent even after SO transformed into OSC or MSO. SYNOPSIS: SO occupied 2.14% of ovarian teratoma, where 12.0% and 4.7% of SO may transform into OSC and MSO, respectively, and had excellent survival outcomes.

Metastatic malignant struma ovarii: a case report and review of the literature on the management of malignant struma ovarii.

OBJECTIVES: To present a case of metastatic struma ovarii, to review the literature on malignant struma ovarii and to discuss the management in locoregional and metastatic disease. METHODS: We present a case of an 82-year-old patient with a malignant struma ovarii and liver metastasis. The patient was treated with pelvic surgery, total thyroidectomy, radioactive iodine therapy and TSH suppression therapy with levothyroxine. We performed a PubMed search for case reports of metastatic struma ovarii. RESULTS: 43 cases of metastatic struma ovarii were identified. 53.5% of patients presented with metastatic disease at diagnosis. Mean time to development of metastasis was 6.9 years in the group with initial locoregional disease. First-line treatment was pelvic surgery in all patients. Thyroidectomy was performed in 83.7% of patients, subsequent radioactive iodine therapy in 79.1%, followed by TSH suppression therapy in 46.5% of patients. Mean time of follow-up after diagnosis of metastases was 3.6 years, ranging from 0.5 to 24 years. At the end of the follow-up, 51.1% of patients were free of disease, 34.9% were alive with disease, 7.0% died of disease and 7.0% were lost to follow-up. CONCLUSION: The majority of patients with metastatic struma ovarii were treated with pelvic surgery, total thyroidectomy and radioactive iodine therapy. Suppression of TSH with levothyroxine was given in less than half of the patients. In non-metastatic setting, the same approach could be considered depending on the patient profile.

Malignant Struma Ovarii Τreated With Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC).

BACKGROUND/AIM: Malignant struma ovarii is an extremely rare tumor entity among ovarian tumors. In the presence of ascites and peritoneal metastases, the preoperative appearance may resemble the most common epithelial ovarian carcinoma (EOC) and accordingly, the surgical therapy may be identical if a preoperative histology diagnosis is not possible. The objective of this case report is to present a patient with histopathologically confirmed malignant struma ovarii who underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC) with the aim of complete tumor resection. CASE REPORT: This study reports on a patient with preoperatively proven peritoneal metastasis of an 18 cm ovarian tumor with large struma ovarii and papillary thyroid carcinoma within the struma, who was treated with CRS and HIPEC after neoadjuvant chemotherapy. CONCLUSION: This disease has a significantly better prognosis than EOC, however, HIPEC could provide an additional effect in examining the presence of peritoneal metastasis.

Clinical characteristics and survival outcomes of malignant struma ovarii confined to the ovary.

BACKGROUND: Malignant struma ovarii (MSO) is a unique type of ovarian malignancy that data on the survival outcome is limited and management strategy remains controversial due to its extreme rarity. METHODS: To investigate the clinical characteristics and treatment options in patients with MSO confined to the ovary, while also evaluating the recurrent-free survival (RFS) and overall survival (OS) rate in this population, a retrospective study was conducted. One hundred twenty-five cases of MSO confined to the ovary were enrolled and their clinical characteristics, treatment strategies, and results of follow-up were analyzed. OS and RFS were assessed by Kaplan-Meier analyses and Cox regression models. RESULTS: The most common pathological subtype in this cohort was papillary carcinoma (44.8%). Other reported subtypes, in order of prevalence, were follicular variant of papillary carcinoma, follicular carcinoma, and mixed follicular-papillary carcinoma. Surgical treatment options varied in this cohort that 8.0% of the patients received ovarian cystectomy, 33.6% underwent unilateral salpingo-oophorectomy (USO), 5.6% received bilateral salpingo-oophorectomy (BSO), 21.6% received total abdominal hysterectomy with BSO (TAH/BSO), and 17.6% were treated with debulking surgery; 20.0% of them received radioiodine therapy (RAI). Twenty-seven patients experienced recurrence with a median RFS of 14.0 years (95% confidence interval [CI], 9.5-18.5). The 5-year and 10-year recurrent rate were 27.1, 35.2%, respectively. Eight patients died during follow-up, with five attributed to MSO; the 5-year, 10-year, and 20-year OS rate was 95.3, 88.7 and 88.7%, respectively. However, the univariate and multivariate Cox regression showed no potential risk factor for RFS and OS. CONCLUSION: Patients with MSO confined to the ovary had an excellent survival outcome, despite varied treatment strategies, and the recurrent rate was relatively high. We recommend USO as the preferred surgical option in this population since more aggressive surgery does not improve outcomes and the benefits of RAI are uncertain.

Metabolic Response by 18F-FDG PET/CT in Metastatic Malignant Struma Ovarii Treated With Targeted Therapies.

Malignant struma ovarii (MSO) is a rare malignant ovarian tumor, histologically identical to differentiated thyroid cancers. Given the rarity of this disease, there are no treatment guidelines, and the place of imaging for response assessment remains controversial. We report a metabolic response assessed by F-FDG PET/CT in a 71-year-old woman with radioiodine-refractory metastatic MSO treated by targeted therapies (first line with lenvatinib and second line with pazopanib). This case of exceptional response also highlights the usefulness of F-FDG PET/CT for therapeutic assessment of targeted drugs in such a rare clinical entity of malignant MSO.

Metastatic Bilateral Strumal Carcinoid: A Case Report and Review of the Literature.

Primary ovarian carcinoids are very rare tumors that belong to the germ cell family of ovarian malignancies. They account for less than 1% of all carcinoid tumors and for less than 0.1% of all ovarian neoplasms. Recurrences are even rarer, with only few cases reported in the literature. Strumal carcinoid has recently been recognized as an extremely rare distinct entity. We report on a patient with bilateral mature cystic teratoma with millimetric foci of ovarian strumal carcinoid who developed lymph node para aortic metastasis after 30 years from primary diagnosis. Our case is thus far the second report of a metastatic strumal carcinoid and the first one in which strumal carcinoid occurred bilaterally and was also metastatic.

Extensive peritoneal implant metastases of malignant struma ovarii treated by thyroidectomy and 131I therapy: A case report.

RATIONALE: Malignant struma ovarii is extremely rare in the clinic. The diagnosis and modalities of treatment are still controversial. Here we describe a case of extensive peritoneal implant metastasis originating from malignant struma ovarii discovered 14 years after ovariectomy and chemotherapy. PATIENT CONCERNS: A 48-year-old female was admitted to our clinic due to hematochezia with a past history of left malignant struma ovarii. Enhanced computed tomography (CT) examination suggested multiple metastasis nodules in the abdomen and pelvic cavity. DIAGNOSES: Laparoscopy biopsy results of intraperitoneal nodules showed a metastasis of papillary thyroid carcinoma. While pathological examination after total thyroidectomy showed no definite malignant tumor component in the thyroid tissue. Finally, combined with the patient's past history of malignant struma ovarii, peritoneal implantation metastasis derived from the malignant struma ovarii was diagnosed. INTERVENTIONS: The patient was treated by total thyroidectomy and iodine 131 (I) therapy. Post-therapy iodine scan and the single-photon emission computed tomography/computed tomography (SPECT/CT) fusion image showed iodine uptake in the distal descending colon, sigmoid colon, rectal lesions, and a larger lesion in the liver. OUTCOME: After treatment, although the thyroid globulin remained at a high level 3 months after treatment, the patient's hematochezia was relieved. LESSONS: Therefore, thyroidectomy followed by adjuvant I treatment should be recommended in patients with malignant struma ovarii as metastatic risk is difficult to predict based on histopathologic examination.

Transplantation of cryopreserved ovarian tissue in a patient affected by metastatic struma ovarii and endometriosis.

In this case report, the outcomes of cryopreserved ovarian tissue transplantation performed in a patient affected by struma-ovarii associated with mature cystic teratoma, recurrent endometriotic cysts and diffuse peritoneal malignant struma-ovarii implants were described. Before cryopreservation, the patient underwent two left ovarian surgeries for enucleation cysts 8 years after righ salpingo-oophorectomy for struma-ovarii. Ovarian biopsy was collected in another hospital and transported to our laboratory for cryopreservation. The patient was submitted to radioiodine-therapy for metastases from malignant struma-ovarii. After treatment she experienced premature ovarian failure. Ten years after cryopreservation, a first orthotopic transplantation was performed in the left ovary and in a peritoneal pocket. Before transplantation, ovarian samples were analyzed to assess neoplastic contamination and tissue quality. Three years later, a second transplantation was heterotopically performed in abdominal subcutaneous sites. The analysis on thawed ovarian tissue did not reveal micrometastasis and they showed follicle and stroma damages. After transplantation few small follicles were observed at ultrasound examination and hormonal levels remained at menopausal values. To date no ovarian function recovery has been observed. The report highlights that ovarian tissue cryopreservation after multiple ovarian surgery may have some limitations. An accurate counseling should be offered to patients who wish to preserve fertility.

Malignant Struma Ovarii With a Predominant Component of Anaplastic Carcinoma.

Struma ovarii exhibiting malignant histology are uncommon, and aggressive clinical courses with initial extraovarian spread are even more rare. This report describes a case of malignant struma ovarii with a predominant anaplastic carcinoma component. A 65-yr-old, gravida 2, para 2, female presented with lower abdominal discomfort and pain. She had a 12×10×7.5 cm tumor in the right ovary. Intraoperative diagnosis was high-grade spindle cell tumor. Right salpingo-oophorectomy and hysterectomy were performed. Macroscopically, the tumor invading the right tube was a yellow-white solid mass with focal microcysts containing greenish liquid and focal calcification. The tumor was histologically characterized by a spindle cell and pleomorphic sarcomatous component, and a minor component of benign-looking thyroid tissue with ossification. Immunohistochemically, the sarcomatous component was focally positive for CAM 5.2, EMA, thyroid transcription factor-1, and thyroglobulin, indicating anaplastic carcinoma. The patient was treated with chemotherapy and is alive, yet with tumor, 25 mo after surgery. This is the first case of malignant struma ovarii with a predominant component of anaplastic carcinoma. This type of malignant struma ovarii may lead to diagnostic problems, and sampling and differential diagnosis among sarcomatous ovarian tumors are important for making the correct diagnoses.

Treatment for malignant struma ovarii in the eyes of thyroid surgeons: a case report and study of Chinese cases reported in the literature.

Malignant struma ovarii (MSO) is a rare malignant ovarian germ cell tumor that has been scarcely reported by thyroid surgeons focusing on treatment. There are no golden standards for its treatment. There has not been any Chinese case included in the English language literatures. This is the first study by collecting all Chinese cases with clinical information. We emphasize on using I therapy after operation.Presented is a case of struma ovarii with malignant histologic features who underwent definitive initial surgery of reproductive system tumors and a total thyroidectomy combined with thyroid-stimulating hormone (TSH)-suppressive therapy following treatment with I. Furthermore, a Chinese full-text database literature search for cases of MSO was performed, and advisable clinical data were collected following our treatment advice.Clinical data from 34 additional cases were compiled. As Chinese genetic background and environment are different from those of Western countries, our clinical data closely mirror theirs in some aspects. In addition, we provide a rare gene mutation type of MSO by the case from our department.Integrating literatures with the experience of thyroid surgeons, we recommend "multidisciplinary joint treatment" for MSO, namely traditional radical initial surgery of ovarian cancer and a total thyroidectomy combined with TSH-suppressive therapy following treatment with I for those who do not desire preservation of fertility.

131I therapy using rhTSH stimulation and dosimetry in metastatic malignant struma ovarii.

A 38-year-old woman with metastatic malignant struma ovarii, including massive liver metastases and retroperitoneal lymphadenopathy, underwent ovarian resection and retroperitoneal lymph nodes excision, partial hepatectomy, and radiofrequency ablation for liver metastases. She underwent thyroidectomy and received three I treatments using recombinant human thyrotropin stimulation and radioiodine dosimetry. posttherapy I imaging, anatomic images, and thyroglobulin levels showed significant diminution in the tumor burdens and remarkable decline in thyroglobulin levels. This case provided valuable information on recombinant human thyrotropin-assisted I ablation in conjunction with dosimetry in an unusual presentation of iodine-avid malignant struma ovarii with bulky metastases.

Malignant struma ovarii: the west of Scotland experience and review of literature with focus on postoperative management.

INTRODUCTION: Malignant struma ovarii is an extremely rare ovarian tumour containing malignant thyroid carcinoma within differentiated thyroid tissue, as the predominant tissue type. Surgery for suspected ovarian tumour and incidental pathological diagnosis is the most common presentation. Evidence supporting any particular approach to the clinical management of this condition is limited, mainly consisting of case reports, small series or pathological case series. There is no randomised evidence for postoperative management in view of the rarity of this condition. The opinion is divided between conservative management versus total thyroidectomy and radio-iodine ablation. METHODS: We carried out a retrospective review of our series with focus on postoperative management of this rare condition. A review of existing literature was also carried out. RESULTS: Six patients with a median age of 52 years presented with various symptoms of abdominal pain, pressure or menstrual problems. After the initial gynaecological resection and specialised pathology review, they were subsequently treated with total thyroidectomy and administration of radioactive iodine. All of these six patients are in remission at a median follow up of 60 months. CONCLUSION: We favour aggressive postoperative management with total thyroidectomy and radioactive iodine, and long-term follow up of these patients.

Histologically bland "extremely well differentiated" thyroid carcinomas arising in struma ovarii can recur and metastasize.

Struma ovarii (SO) infrequently harbor carcinomas that are histologically similar to those arising in the eutopic thyroid. We identified 10 such cases in our files. Eight patients presented with pelvic-related symptoms whereas 2 were incidentally discovered during pregnancy, all with disease confined to the ovary. There were 8 papillary thyroid carcinomas (PTCs) (2 classic and 6 follicular variant) and 2 poorly differentiated thyroid carcinomas. Two of the 10 thyroid carcinomas relapsed after an initial diagnosis of "benign" struma. Both occurred in young women with ovarian cysts discovered during pregnancy. The cystectomy from 1 patient showed thyroid follicles with nuclear features of the follicular variant of PTC whereas the cyst from the second patient showed thyroid follicles with subtle nuclear features, suggestive but not diagnostic of PTC. Both patients presented with disseminated PTC 3 and 4 years after the initial diagnosis, involving the pelvis in both cases and also the liver parenchyma in 1 case. The 2 patients received radioactive iodine therapy after thyroidectomy and are both alive with disease 6 years after diagnosis. The criteria separating hyperplastic nodules from well-differentiated follicular variant of PTC in the thyroid gland seem to be applicable to thyroid-type carcinomas arising in SO. The propensity for adverse clinical behavior does not seem to be related to the grade or histologic type of carcinoma in this small series. The hormonal milieu during pregnancy may lead to progression of malignant SO and such patients should be closely followed, particularly if their treatment consists of cystectomy alone.

Metastatic struma ovarii: the burden of truth.

A 58-year-old woman was found to have metastatic thyroid cancer in her liver. This was identified when she was investigated for upper abdominal pain and underwent biopsy of hepatic lesions. She had no palpable thyroid nodule and had a normal ultrasound of the thyroid. Previously, both ovaries had been removed because of tumors. The pathologic findings in one of the ovaries could not be recovered because the procedure was more than 40 years ago, when the patient was a teenager. By a process of elimination, a diagnosis of metastatic struma ovarii was established. Treatment of metastatic thyroid cancer from struma ovarii, including removal of the normal thyroid and administration of I-131, is presented.

[Malignant struma ovarii: a case report]. / Goitre ovarien malin: une observation.

Malignant transformation of struma ovarii is exceptional (less than 1%). The histolological diagnosis of malignancy is difficult especially in the well differentiated forms. Immunohistochemistry is highly contributive in the anaplastic forms. The prognosis is relatively favorable except for the metastatic and undifferentiated forms. We report a case of malignant struma ovarii with metastasis observed in a 65-year-old woman who died rapidly from her disease. In light of this observation, we discuss the diagnositic, management and outcome features of these particular tumors.

Malignant struma ovarii: an unusual presentation.

Malignant struma ovarii is a rare disease; only a few cases are well documented in the literature. Thus, the overall prognosis and modalities of treatment are still somewhat controversial. In this article, the authors report a case of malignant struma ovarii discovered 4 years after ovariectomy after metastasis to the lungs and bones. Review of the pathology of the ovarian struma did not reveal the classic criteria of malignancy, there were, however, many features considered to be atypical and thus suspicious. The patient was treated by total thyroidectomy followed by repetitive doses of 131I. However, because of difficulties in increasing the level of endogenous thyrotropin (TSH) because of functional thyroid metastases in such an advanced disease, recombinant human thyrotropin (rhTSH; Thyrogen, thyrotropin alpha, Genzyme Corporation, Cambridge, MA) was used before administration of radioiodine. With this therapeutic protocol, the patient is still clinically stable 2 years after diagnosis.

Diagnosis and treatment of disseminated struma ovarii with malignant transformation.

Struma ovarii are specialized teratomas consisting of thyroid tissue. They may demonstrate all pathologic features seen in the thyroid gland. Malignant transformation of thyroid tissue in struma ovarii is uncommon and is rarely recurrent or metastatic. We report the diagnosis and treatment of a recurrent struma ovarii with malignant transformation, and intraperitoneal, retroperitoneal, and hepatic metastases.

[Struma ovarii: an ovarian neoplasm]. / Struma ovarii--nowotwór jajnika.

Authors shows three cases of very rarely occurring ovarian struma. First case--was discovered in a patient with normal physiological hormonal activity. Second case--was discovered in a postmenopausal patient with hyperthyroidism and with endometrial carcinoma. Third case--was discovered in a postmenopausal patient, without any complaints, where an ovarian tumor was found during periodical gynaecological examination. Histopathological evaluation of the material was decisive for the final diagnosis.

Paraplejia secundaria a metástasis de un carcinoma folicular de Strumma ovarii / Secundary paraplegia a to metastasis of follicular carcinoma of ovarian struma

Se presenta el caso clínico de una paciente de 47 años de edad a la que dos años antes se les practicó histerectomía con salpingo-oforectomía bilateral por tumor ovárico reportado como un tumor de la granulosa. Acude nuevamente con cuadro de paraplejía secundaria a comprensión medular por fractura patológica de vértebra dorsal (T7-8). Se le practica laminectomía de la lesión vertebral osteolítica, la que se reporta como correspondiente a un carcinoma folicular de tiroides metastásico. Posteriormente es sometida a una tiroidectomía total, la pieza de esta ocasión se reportó como "glándula tiroidea normal". En busca de la lesión primaria se reexamina la pieza y laminillas de histerectomía, diagnosticándose un carcinoma folicular y struma ovarii. Es tratada con Iodo 131, recupera la motilidad y cinco años después se encuentra en perfectas condiciones, haciendo vida normal. Se hacen comentarios sobre dicha entidad