HISTOPATHOLOGY INDIA.COM Myxoid Tumours of Soft Tissue

 

                  

Struma ovarii is an extremely rare tumour that occasionally undergoes      malignant transformation.

Struma ovarii, a teratoma in which thyroid tissue is the  predominant or           sole component account for 2.7% of all ovarian tumours.

Because struma ovarii is composed of thyroid tissue, it is conceivable         that the pathogenetic events involved in thyroid follicular transformation               may take place also in struma ovarii.

Pathogenesis is unclear. In addition to symptoms and signs caused by                the presence of a mass, struma may be associated with a number of         unusual clinical manifestations.

Struma ovarii is rare ovarian tumour that is characterized by the             presence of at least 50% thyroid tissue on histologic examination.

The presence of thyroid tissue in the ovary implies either metastatic              thyroid carcinoma, or a monophyletic teratoma such as  "struma ovarii".

Always look out for other teratomatous elements, especially carcinoid        tumour which is specifically associated with thyroid tissue in the ovary.

The thyroid tissue here can be affected by the full range of disease           processes which would otherwise affect the native thyroid gland.

Interpretation of malignant transformation in the tissue is difficult, but for papillary carcinoma, the criteria are the same as for the thyroid gland.

If the patient develops autoimmune thyroiditis, the "struma ovarii" shows     similar changes to the native gland.

                         

Malignant Struma Ovarii - A Case Report and Review of the Literature. Gynecol Obstet Invest.2007 Sep 21;65(2):104-107.

Background: Struma ovarii is a rare monodermal ovarian teratoma composed predominantly of mature thyroid tissue. Of these cases, 5-8% are clinically hyperthyroid and 5-10% of these tumors are malignant. Case Report: A 53-year-old female presented with a 19 x 5 x 5 cm pelvic mass that was treated with bilateral salpingo-oophorectomy, pelvic and para-aortic lymph node sampling, omentectomy and appendectomy and staging for an ovarian tumor. There was no evidence of distant metastases or lymph node invasion. Re-evaluation of the patient after surgery revealed that she was clinically euthyroid and there was no thyroid malignancy. Histopathology revealed papillary thyroid carcinoma arising in struma ovarii (malignant struma ovarii). Conclusion: Malignant struma ovarii is a very rare malignant ovarian teratoma. In young patients unilateral oophorectomy and complete surgical staging should be considered when the tumor is confined to the one ovary (stage Ia). Long-term follow-up for the detection of metastases or tumor recurrence by serial serum thyroglobulin and (131)I scan or positron emission tomography/computed tomography may be required in selected patients with this rare tumor.

Malignant struma ovarii with a focus of papillary thyroid cancer: a case report. Arch Gynecol Obstet.2007 Sep 20;

Struma ovarii is a rare form of ovarian neoplasm and its malignant transformation is even rarer. Because of its rarity, there is no consensus about its diagnosis and management in the literature. A 53-year-old woman with left adnexial mass underwent total abdominal hysterectomy with bilateral salpingo-oopherectomy. Postoperatively, she was diagnosed with a malignant struma ovarii. Postoperative thyroid ultrasonography revealed a 0.5-cm solid nodule in the thyroid gland and total thyroidectomy was done. Pathology report was nodular hyperplasia of benign character. She is currently disease-free for 1 year but long-term follow-up with thyroglobulin levels is necessary due to reports of increasing recurrence rates.

BRAF in papillary thyroid carcinoma of ovary (struma ovarii).Am J Surg Pathol. 2007 Sep;31(9):1337-43.

BACKGROUND: Malignant struma ovarii (MSO) are rare tumors that arise from ectopic thyroid tissue in the ovary, benign struma ovarii (BSO). Most MSO are histologically classified as papillary thyroid carcinomas (PTC). Oncogenic activation of BRAF (35% to 69%), RAS (10%), or RET (5% to 30%) is common in PTC, and the mutations correlate with tumor subtype, patient age, and clinical behavior. In this study, we explored the possible role of these genes in the development of BSO and MSO. DESIGN: Six paraffin-embedded cases of MSO with histopathologic features of PTC (4 follicular variants, 1 classic, and 1 metastasis of a classic) and 9 BSO were identified. BRAF, NRAS, and KRAS mutations were evaluated using a combination of polymerase chain reaction, denaturing high performance liquid chromatography, and automated sequencing. RET alterations were screened by fluorescence in situ hybridization with multicolor probes. Corresponding benign tissues were evaluated when available. RESULTS: BRAF mutations were present in 4 of 6 MSO and none of 9 BSO. The BRAF mutations included V600E (2 cases), K601E, and a novel deletion/substitution TV599-600M. Neither MSO nor BSO contained alterations in NRAS, KRAS, or RET. CONCLUSIONS: The development of MSOs with PTC features is associated with BRAF mutations of the type commonly observed in PTC, suggesting a common pathogenesis for all PTCs regardless of location. In contrast, mutations in the RET/RAS/RAF pathway are not found in BSO. The prognostic significance of BRAF mutation status in MSO remains to be determined.

A case of concomitant occurrence of struma ovarii and malignant transformation of cystic teratoma. Int J Surg Pathol. 2007 Jul;15(3):318-20.

A 77-year-old woman received a total abdominal hysterectomy and bilateral salpingo-oophorectomy because of a tumor in the left ovary. The surgical specimen measured 8.5x4.5x4.0 cm, and the solid lesion measured 4.0x3.5x3.5 cm. The solid lesion was diagnosed as struma ovarii. The cyst wall partially comprised squamous epithelium-like and ciliated columnar epithelium-like cells. The tumorous lesion of the cyst wall revealed a poorly differentiated adenocarcinoma. Immunohistochemically, the tumor cells were positive for cytokeratin7, and were negative for cytokeratin20 and thyroid transcription factor-1. The authors diagnosed that struma ovarii and other parats coexisted as a poorly differentiated adenocarcinoma that had arisen from a mature ovarian cystic teratoma. As for the identification of the origin of adenocarcinomas arising from mature ovarian cystic teratomas, more cases need to be identified and investigated.

Malignant struma ovarii: a case report and review of the literature. J Endocrinol Invest.2007 Jun;30(6):517-20.

Malignant struma ovarii is a very rare disease. Only few cases are reported in literature. Because of its rarity, there are various approaches to its treatment. We describe a case of malignant struma ovarii, in a 37 year-old female who presented for non cyclic, chronic pelvic pain and the presence of a right ovarian cyst with mean diameter of 7 cm. The patient was treated with laparoscopic right ovariectomy and with multiple biopsies of omental, left ovary and utero-sacral ligament. The patient underwent subsequently total thyroidectomy and radioiodine (131I) ablation. A Medline literature search was performed; we found 48 cases of malignant struma ovarii. The therapeutic management of the disease is very different in the described case; particularly after surgical removing of the ovarian mass, the treatment is still controversial. We think that the management of malignant struma ovarii could be the same than carcinoma of the thyroid, so after surgical removing of ovarian neoplasm, we recommend thyroidectomy, radiotherapy with 131I and levothyroxine suppressive therapy.

Benign and malignant struma ovarii: report of three cases and review of the literature.J BUON. 2002 Jan-Mar;7(1):67-70.

Struma ovarii belongs to the group of monodermal and highly specialized teratomas and comprises less than 5% of mature teratomas. Thyroid tissue is present exclusively or predominantly. Malignant transformation in struma ovarii is uncommon, and when present, it exhibits a follicular pattern most of the times. Three patients with the diagnosis of struma ovarii are presented herein. Two of them had a unilateral adnexal mass with ascites in one case. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy were carried out in both patients. In both patients thyroid hormonal status was normal pre and postoperatively. The third patient had a histology compatible with follicular carcinoma of the thyroid tissue of struma ovarii; strumal carcinoid was also present. Postoperatively monochemotherapy with etoposide was administered for 6 cycles every 3 weeks. Thyroid hormones and thyroid ultrasonography (US) were also normal in this patient. Surgical management is the treatment of choice for struma ovarii which also represents a preventive measure for possible future malignant transformation.

The enigma of struma ovarii.Pathology.2007 Feb;39(1):139-46.

Since its first description in the early part of the twentieth century, struma ovarii has elicited considerable interest because of its many unique features; however, at present a number of aspects remain enigmatic. Although the typical presentation is that of a pelvic mass, unusual clinical manifestations such as hyperthyroidism, ascites, and Meigs' syndrome have been recognised. Uncommon macroscopic and especially histological patterns in struma can cause difficulties in diagnosis. Cystic strumas are challenging to diagnose both macroscopically and histologically. Proliferative changes within struma can be misdiagnosed as cancer. In regard to the occurrence of thyroid-type carcinoma in struma ovarii, precise terminology should be utilised, and the term 'malignant struma ovarii' should be avoided because it has been used for several different pathological entities. Papillary carcinoma is the most commonly occurring thyroid-type carcinoma in ovarian struma; however, cases of follicular carcinoma are not infrequent. Histological malignancy in struma does not necessarily equate with biological malignancy, and the majority of thyroid-type carcinomas do not spread beyond the ovary. Strumal carcinoid, a neoplasm apparently unique to the ovary containing elements of both struma and carcinoid, has been misdiagnosed as 'malignant struma ovarii' in the past. The differential diagnosis of extra-ovarian spread of struma includes the usual types of thyroid cancer, minimal deviation follicular carcinoma, and peritoneal strumosis. This review emphasises articles both recent and past that have significantly advanced our knowledge of struma ovarii and related neoplasms.

Malignant struma ovarii with thyrotoxicosis. Gynecol Oncol. 2001Sep;82(3): 575-7.

BACKGROUND: Malignant struma ovarii is seldom diagnosed preoperatively due to the rarity of the disease itself and the even rarer complications of thyrotoxicosis. CASE: A 48-year-old woman presented with symptoms of hyperthyroidism and a pelvic tumor raising the possibility of ovarian malignancy. Hormonal findings revealed increased thyroid function, but the thyroid gland was normal in size and texture. Thus, she was diagnosed preoperatively as having a hormone-producing malignant struma ovarii. At surgery, a FIGO stage Ia ovarian papillary adenocarcinoma of the thyroid was found. An immunohistochemical tumor stain for thyroglobulin was positive and the ovarian venous thyroglobulin level was extremely high. Findings of hyperthyroidism disappeared over several weeks. CONCLUSION: Malignant struma ovarii can be diagnosed preoperatively. Complications of thyrotoxicosis should be kept in mind when evaluating an ovarian tumor.


October 2007

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