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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 578-580
Unilateral malignant struma ovarii in a case of bilateral ovarian teratoma with raised CA-125 level: A rare case with treatment dilemmas


1 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India
2 Department of Gynaecologic Oncology, Tata Memorial Hospital, Parel, Mumbai, India

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Date of Web Publication20-Sep-2011
 

   Abstract 

Struma ovarii are specialized form of mature ovarian teratoma comprised predominantly of thyroid tissue (>50%). Most of the struma ovarii are benign; rarely can they undergo malignant transformation. Elevated CA-125 levels with benign struma ovarii have been seen in only 5 cases in literature. The association of malignant struma ovarii and high CA-125 levels with pseudo-Meig syndrome has been reported in only 2 cases in English literature. We describe a case of a 46-year-old multigravida who presented with an abdominal mass and raised CA-125 levels. Radiological investigations revealed bilateral cystic adnexal masses with ossified elements on left side suggesting a teratoma. Intraoperative frozen section and final pathology revealed bilateral teratoma with follicular variant of papillary thyroid carcinoma arising in the left ovary. To the best of our knowledge, this is the first case of malignant struma ovarii in combination with bilateral teratoma. The dilemmas related to preoperative diagnoses with elevated CA-125 levels, mimicking an epithelial ovarian neoplasm; intraoperative frozen section consultation; management and follow-up issues in this rare malignancy are discussed.

Keywords: Bilateral teratoma, CA125, malignant struma ovarii, papillary thyroid carcinoma

How to cite this article:
Menon S, Deodhar K, Rekhi B, John A, Maheshwari A. Unilateral malignant struma ovarii in a case of bilateral ovarian teratoma with raised CA-125 level: A rare case with treatment dilemmas. Indian J Pathol Microbiol 2011;54:578-80

How to cite this URL:
Menon S, Deodhar K, Rekhi B, John A, Maheshwari A. Unilateral malignant struma ovarii in a case of bilateral ovarian teratoma with raised CA-125 level: A rare case with treatment dilemmas. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Nov 30];54:578-80. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/578/85098



   Introduction Top


Mature ovarian teratomas can harbor small foci of thyroid tissue in approximately 5% to 15% of cases. Struma ovarii is a rare monodermal teratoma which predominantly shows mature thyroid tissue. [1],[2],[3],[4] Malignant struma ovarii is even rarer and constitutes about 0.1% to 0.3% of all ovarian teratomas and about 5% of all cases of struma ovarii. [1] The biochemical association of an elevated CA-125 in cases of either benign or malignant struma ovarii is extremely uncommon and is seen in combination with pseudo-Meig syndrome. [2],[3] Absence of defined diagnostic criteria and the rarity of malignant struma ovarii contribute to both diagnostic difficulties and management problems for the pathologist and clinician, respectively. We describe an unusual case of a follicular variant of papillary carcinoma arising in a struma ovarii in a middle-aged woman with bilateral ovarian teratoma, with high CA-125 levels and absence of ascites and hydrothorax.


   Case Report Top


A 46-year-old multigravida presented with history of abdominal pain to the Department of Gynecologic Oncology of our institute. She did not have any medical comorbidity. There were no complaints of altered bowel and bladder habits. Per vaginum bimanual examination revealed bilateral adnexal masses. On pelvic ultrasonography, bilateral predominantly cystic masses with solid components were demonstrated. The patient was further investigated with a computed tomography scan of abdomen and pelvis, which showed a 14 Χ 10-cm left adnexal mass with ossified elements in ovary and a 6 Χ 5.5-cm right adnexal mass. Radiological impression favored a diagnosis of teratoma of left ovary with possible malignancy of right ovary. There was no free fluid in the abdomen. The serum CA-125 level was found to be 1371 IU/mL (normal, 0-35 IU/mL). A tentative preoperative diagnosis of teratoma with possible malignant transformation was entertained, and the patient was counseled to undergo an exploratory laparotomy with total abdominal hysterectomy, bilateral salpingo-oophorectomy. At laparotomy, the left ovarian mass appeared to be mostly cystic with palpable solid areas. The capsule was intact, and there were no significant pelvic or para-aortic lymph nodes. The omentum was grossly unremarkable. A frozen section was requested for primary diagnosis. At frozen section, cutting open the left ovary revealed it to be predominantly cystic with focus of solid Rokitansky's protuberance [Figure 1], which on further sectioning revealed tooth structures. The cystic areas had brownish color, and wall thickness ranged from 0.3 to 0.8 cm. Microscopically, a section from another solid area of muscle-like consistency was found to be constituted by thyroid tissue with follicular architecture [Figure 2]. On higher magnification, the nuclear features of papillary carcinoma of thyroid in the form of overlapping, nuclear grooves and intranuclear inclusions were recognized [Figure 3]. A diagnosis of follicular variant of papillary carcinoma of thyroid (FVPCT) arising in struma ovarii was suggested at frozen section. On final histopathology, more sections were examined from left ovary, and a diagnosis of malignant struma ovarii arising in a teratoma was established. The tumor showed immunoreactivity to thyroglobulin, TTF-1 (thyroid transcription factor-1), CK19 and HBME-1. The right ovary measured 4.5 Χ 3.5 Χ 2 cm, and the gross revealed a firm yellow-white area. Microscopically, sections from right ovary showed skin tissue, salivary gland elements and adipose tissue. Omentum was free of tumor. On follow-up at 3 months, the thyroid gland was unremarkable on clinical and sonographic assessments, and the serum CA-125 levels were normalized to 27.0 IU/mL.
Figure 1: Gross photograph of the cut-open left ovary showing solid areas on left half and cystic area on right half. The arrow shows the Rokitansky's protuberance with tooth structure

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Figure 2: Follicular variant of papillary carcinoma showing predominantly follicular pattern admixed with scanty foci having papillae-like configuration (H and E, x100)

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Figure 3: Characteristic nuclear features of papillary thyroid carcinoma are noted with nuclear overlapping and abundant nuclear grooves (H and E, x200)

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   Discussion Top


Struma ovarii consists of thyroid tissue derived from germ cells of a mature teratoma. As malignant transformation is very rare, the histopathological diagnosis of malignancy in a struma ovarii is controversial due to lack of uniform diagnostic criteria. [5] All the known thyroid-type carcinomas have been described in struma ovarii, some of which may cause diagnostic difficulties with ovarian tumors like granulosa cell tumor or carcinoid. [6],[7]

Geist and Smith [4] proposed diagnostic criteria based on cellular atypia, invasion and metastases. The criteria for diagnosis of malignancy were refined by Devaney et al., and the presence of typical nuclear features as in primary thyroid carcinoma, viz., ground-glass overlapping nuclei, nuclear grooves and inclusions, was accepted as the most useful criterion for papillary carcinoma. Presence of papillary architecture only or closely packed follicular pattern only in the absence of typical nuclear characteristics was designated as a category of proliferative struma. [5]

In our patient, a raised CA-125 level and ossified elements on radiology suggested a possibility of malignant transformation in a teratoma. Bilateral pure struma ovarii with raised CA-125 levels are known to occur.[8] The association of malignant struma ovarii with elevated CA-125 levels is even rarer and has been described in only 2 cases.[1] However, in spite of exhaustive search of the existing literature, we were unable to find any documented case of bilateral teratoma with malignant struma ovarii and associated elevated CA-125 level. In the present case, raised CA-125 levels were recorded preoperatively, and typical morphological features of papillary thyroid carcinoma were present on histology, even at frozen section consultation. The serum CA-125 levels were normalized after surgery.

The biological behavior of these tumors is unpredictable. Metastases from malignant struma ovarii have been documented in the literature but are rare occurrences (5% to 6% of malignant struma ovarii cases). [4] A universal guideline on the surgical management and postoperative treatment of patients with thyroid-type carcinoma arising in struma ovarii has not been devised to date. Surgical treatment ranges from total abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy to conservative surgery like unilateral oophorectomy or cystectomy for fertility preservation. Adjuvant therapy includes external radiotherapy, chemotherapy, thyroidectomy and radioactive iodine ablation. [7] Thyroidectomy has a dual role in management. Firstly, it excludes a primary thyroid carcinoma with metastasis to the ovary; and secondly, it potentiates any subsequent I131 therapy by removing all normally functioning thyroid. [9] Our patient had normal thyroid gland on clinical, biochemical and radiological evaluation.

In a recent study, features that suggested an adverse outcome in malignant struma ovarii [10] were presence of adhesions, peritoneal fluid (≥1 L) or ovarian serosal rent. Also this study found that the size of the strumal component rather than the overall size of the ovarian teratoma also had some predictive value for adverse outcome. In the present case, none of these parameters for adverse outcome were present. The patient had no evidence of disease at 6 months and has been counseled to be on a regular follow-up, with abdominal ultrasound at half-yearly intervals.

In conclusion, we have reported a hitherto undescribed case of malignant struma ovarii arising in a case of bilateral ovarian teratoma with raised CA-125 levels. The present case highlights that CA-125 may be raised in unusual tumors of the ovary and there is a need for close communication between the surgeon and the pathologist, especially during intraoperative frozen consultations, for arriving at a correct diagnosis. Treatment in malignant struma ovarii needs to be individualized, as presently no consensus on guidelines exists. Long-term follow-up is required in most cases as the biologic behavior is unpredictable in such cases.

 
   References Top

1.Amalaseelan JV, Perera KA. An unusual variant of malignant struma ovarii. Ceylon Med J 2008;53:55-6.  Back to cited text no. 1
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2.Hatami M, Breining D, Owers RL, Del Priore G, Goldberg GL. Malignant struma ovarii-a Case Report and review of the literature. Gynecol Obstet Invest 2008;65:104-7.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Mitrou S, Manek S, Kehoe S. Cystic struma ovarii presenting as pseudo-Meigs' syndrome with elevated CA125 levels. A Case Report and review of the literature. Int J Gynecol Cancer 2008;18:372-5.  Back to cited text no. 3
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4.Dardik RB, Dardik M, Westra W, Montz FJ. Malignant struma ovarii: Two Case Reports and a review of the literature. Gynecol Oncol 1999;73:447-51.  Back to cited text no. 4
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5.Devaney K, Snyder R, Norris HJ, Tavassoli FA. Proliferative and histologically malignant struma ovarii: A clinicopathologic study of 54 cases. Int J Gynecol Pathol 1993;12:333-43.  Back to cited text no. 5
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6.Szyfelbein WM, Young RH, Scully RE. Struma ovarii simulating ovarian tumors of other types. A report of 30 cases. Am J Surg Pathol 1995;19:21-9.  Back to cited text no. 6
[PUBMED]    
7.Zhang X, Axiotis C. Thyroid- type carcinoma of struma ovarii. Arch Pathol Lab Med 2010;134:786-91.  Back to cited text no. 7
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8.Rana V, Srinivas V, Bandyopadhyay S, Ghosh SK, Singh Y. Bilateral benign non functional struma ovarii with Pseudo-Meigs' syndrome. Indian J Pathol Microbiol 2009;52:94-6.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.DeSimone CP, Lele SM, Modesitt SC. Malignant struma ovarii: A Case Report and analysis of cases reported in the literature with focus on survival and I131 therapy. Gynecol Oncol 2003;89:543-8.  Back to cited text no. 9
[PUBMED]  [FULLTEXT]  
10.Robboy SJ, Shaco-Levy R, Peng RY, Snyder MJ, Donahue J, Bentley RC, et al. Malignant struma ovarii: An analysis of 88 cases, including 27 with extraovarian spread. Int J Gynecol Pathol 2009;28:405-22.  Back to cited text no. 10
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Santosh Menon
Department of Pathology, 8th Floor, Annexe Building, Tata Memorial Hospital, Dr. Ernest Borges Marg, Parel, Mumbai - 400 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.85098

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