Massive ovarian edema: A case report presenting as a diagnostic dilemma.

Massive ovarian edema is a rare clinical entity, posing a significant clinical challenge as it can be easily mistaken for neoplasm. Our case was a 20-year-old young woman who presented with a self-limiting episode of abdominal pain along with large solid pelvis mass. On physical examination, she had abdominal tenderness with guarding. Ultrasound examination revealed large solid ovarian mass with moderate ascites. With the diagnosis of ovarian neoplasm, laparotomy was performed, and intraoperative frozen section excluded malignancy with differentials suggesting of fibromatosis/massive ovarian edema. The patient underwent unilateral salpingo-oophorectomy. Histopathological examination confirmed the diagnosis of massive ovarian edema. Massive ovarian edema should be suspected in women at the fertility age range with solid enlargement of the ovary so that these young patients can be treated conservatively where fertility preservation is mandatory.

Introduction

Massive ovarian edema is a rare tumor-like condition affecting young women, first described by Kalstone in 1969.[1] Massive ovarian edemas can involve one or both ovaries[2] and it has been observed during pregnancy.[3] The etiology of this entity is not clear. It has been suggested that massive enlargement of the ovary without neoplastic change results from interference with the venous and lymphatic flow due to partial and complete torsion of the mesovarium, but not the arterial blood flow. As a result, there is stromal cell luteinization in the edematous ovary, occurring as a response to torsion and subsequent ischemia.[2] It is often important to recognize this condition as it is usually misdiagnosed as malignancy and hence results in overtreatment of younger patients with resultant loss of hormonal function and fertility.[4] In the present case report, we emphasize the need for a strong clinical suspicion preoperatively so that the patient can be treated conservatively. After an extensive literature search, we found that very few cases have been published from Indian population.

Case Report

Our patient was a 20-year-old female who presented with pelvic mass along with a history of self-limiting episodes of abdominal pain for 4 months. Her menstrual cycles were regular and had uneventful past medical history. On physical examination, she had lower abdominal tenderness with tender mobile adnexal mass felt during bimanual examination. Ultrasound examination showed a right ovarian mass measuring 10 cm × 6.5 cm with hypoechogenic foci at the periphery with moderate ascites. The serum level of cancer antigen-125 (CA-125) (14.4 U/ml), alpha-fetoprotein (0.82 IU/ml), and β-human chorionic gonadotropin (β-HCG) (0.100 mIU/ml) were within normal limits. Computed tomography abdomen study revealed relatively defined lobulated multicystic mass lesion in the right pelvic region with the markedly dilated vascular channels at the pedicle of the lesion concluding the strong possibility of mucinous ovarian neoplasm. Laparotomy was performed which revealed grossly enlarged right ovary with twisted pedicle, with intact smooth capsule, with normal left adnexae. Intraoperative frozen section was carried out, which ruled out malignancy, suggested massive ovarian edema and fibromatosis. Right salpingo-oophorectomy with peritoneal cytology was performed. Postoperative period was uneventful. Gross examination revealed ovarian mass with attached fallopian tube measuring 11 cm × 8.5 cm × 8.5 cm. External surface appeared smooth and glistening. On cut section, ovary was yellow, solid with few cyst filled with clear serous fluid and blood clot [Figure 1]. Histopathological examination revealed markedly edematous ovarian stroma with few dilated and normal ovarian follicles surrounded by luteinized cells at places and proliferating blood vessels. One section revealed ovarian follicle surrounded by small foci of fibromatosis. Peripheral rim of normal ovarian tissue was seen. No evidence of any tumor was seen on multiple sections [Figures 2–4]. Routine microscopy and cytology of peritoneal fluid were within normal limits. The final histopathological diagnosis was massive ovarian edema.

Figure 1

Gross photograph showing enlarged solid ovary with cystic change

Figure 2

Low power view showing markedly edematous ovarian stroma with peripheral normal ovarian tissue (H and E ×100)

Figure 4

Scanner view showing ovarian follicle with edematous ovarian stroma (H and E ×40)

Scanner view showing ovarian follicle surrounded by fibroblastic proliferation (H and E ×40)

Discussion

Massive edema of the ovary is a rare tumor-like condition occurring in young women.[5] Menstrual irregularities, abdominal distention, and infertility are found in the majority of cases.[6] Masculinization is a common presentation in many adult cases, precocious puberty in prepubertal girls, and some cases present with masculinization associated with low serum level of gonadotropins indicating autonomous ovarian hormone production. This hormone production is due to stromal luteinization as suggested by Chervenak et al.[4] Kalstone suggested that luteinization might be caused by mechanical stimulus of stretching the stroma by edema fluid.[1] Another explanation for the edema and abnormal hormonal production is a derangement of a local paracrine factor, such as insulin-like growth factor, epidermal growth factor, or cytokines.[6]

Morphological examination of the ovarian mass appeared gray-white, soft, and exuded watery fluid after cutting with a knife due to the pressure of the edema. On microscopic examination, hypocellular, edematous stroma is seen with a thin rim of compressed cortical stroma at the periphery of the mass. Clusters of luteinized stromal cells are present in a minority of cases. Necrosis and hemorrhage are unusual. In some of the studied cases, foci of fibromatosis can be seen in massive ovarian edema. A study by Young and Scully in 1984 of 25 patients with ovarian enlargement showed that 14 cases had fibromatosis as predominant histology, six of which also had massive ovarian edema; 11 cases showed massive ovarian edema as predominant pathology, eight of which contained foci of fibromatosis; and seven of 25 cases showed evidence of ovarian torsion.[7] The similar age range and clinical manifestations of these two processes and the overlap in their histological features suggest that they are closely related. Radiological imaging in most of the situations can be ambiguous, however with the addition of tumor markers such as β-HCG, lactic dehydrogenase, CA-125, and alpha-fetoprotein; the differential diagnosis can be scaled down, differentiating condition from dysgerminomatous and mixed germ cell tumors. An intraoperative frozen section is always valuable at the time of surgery and can assist in performing fertility sparing surgery. After an extensive review of literature regarding the management of massive ovarian edema reveals that majority of patients were overtreated with salpingo-oophorectomy, as the lesions were mistaken for primary ovarian neoplasm which was similar to our patient who underwent unilateral salpingo-oophorectomy.[5] Geist et al. stated that this entity should be suspected in women who presents with painful abdomen in a reproductive age group with solid enlargement of the ovary, normal biochemical markers and definitive surgical treatment should be undertaken only after confirmed pathological diagnosis.[58] However, when the condition of ovarian edema is suspected at surgery, the appropriate treatment is wedge resection, removing 30% or more of the ovary to exclude the secondary causes of the condition. Cheng et al. reported that with de-torsion, wedge resection, and plication of the ovary, the patient was successfully relieved of abdominal pain and experienced no recurrence during the follow-up period.[9]

Conclusion

Massive ovarian edema is a rare cause of ovarian mass in women of a reproductive age group. For the clinicians and pathologist, it is important to know the benign nature of this disease, as it is easily mistaken for neoplasm and these young patients should be treated more conservatively to preserve their hormonal functions and fertility.

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Conflicts of interest

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