Massive ovarian oedema is a tumor-mimicking condition occurring in young women [
1]. It is often considered to be the result of complete torsion of the ovary to the extent that it interferes with venous and lymphatic drainage but is insufficient to cause necrosis [
2,
4]. In addition to this mechanism, there is the pathophysiological mechanism of the partial ovarian torsion. This is based to the histopathological evidence of a hematoma [
4]. There are many authors suggesting that partial torsion is a likely explanation for this perplexing disorder [
4] and it considered to be a variant of polycystic ovary syndrome [
5]. As a result of the enlargement of the ovary, the intrabdominal pressure rises, causing a pressure phenomenon in the nearby area and the patient usually presents with adnexal mass. The character of the symptomatology, acute pain or profound diffuse pain depends on the character of the torsion. In case of an acute torsion, the abdominal pain with the clinical presentation of an acute abdomen is the main symptom. In most of the cases described in the literature there are menstrual irregularities, infertility and abdominal distension [
6]. Massive ovarian oedema is considered to be the result of ovarian lymphatic dysfunction. The question that rises is; how could the lymphatic dysfunction cause this kind of symptomatology? The answer is hidden in the unique capacity of the ovary to remodel its tissue structure and vascular network continuously and under a strictly controlled process.
The lymphatic vessels have a special morphology without a structural basement membrane and an overlapping layer of vascular endothelial cells. The functionality of these cells is controlled from the Vascular Endothelial Growth Factor (VEGF) and its receptor (VEGFR2) [
7]. There are scientific studies showing that the inhibition of VEGFR2 prohibits the normal luteinization process [
8,
9]. The ovarian lymph contains among others; hormones including progesterone, estradiol and inhibin that are transferred back to the ovarian arteries via retrograde transfer and then they promote the feedback in the hormonal regulation of the ovary [
10,
11]. Given the mechanism mentioned above, the lymphatic dysfunction prohibits the normal luteinization of the ovary and causes hormonal problems. This is the mechanism that is thought to be responsible for the formation of primary ovarian oedema [
12]. On the other hand massive ovarian oedema has been correlated in the literature with retroperitoneal lymphoma, metastatic carcinoma, polycystic ovary syndrome, metastatic-cervical carcinoma [
12]. In those cases, massive ovarian oedema is characterized “secondary massive ovarian oedema” as a result of lymphatic vascular blockage. In patients under hormonal therapy and especially in therapy with clomiphene citrate the increase of LH and FSH cause changes in the lymphatic vasculature [
13]. The menstrual irregularities can be a result of low serum levels of gonadotropins, because of an autonomous ovarian hormone production [
14,
15]. This hormone production is a result of stromal luteinization according to Chervenak et al. [
14]. On the other hand, Kalstone et al. suggested that the luteinization might be caused because of the mechanical stimulus of increasing quantity by oedema fluid which is stretching the stroma [
1,
15]. Another theory for the formation of the oedema and the abnormal hormone production is the impact of insulin-like growth factor, epidermal growth factor or cytokines in the ovarian stroma cells [
4]. There are very few literature references on massive ovarian oedema as a permeation of the ovarian lymphatics by metastatic carcinoma [
16,
17]. The mechanisms mentioned above explain why masculanization and precocious puberty are common features among women suffering from massive ovarian oedema. The most interesting part of this clinical entity is the histopathological findings, which set the diagnosis. The ovarian stromal cells which are separated by copious oedema fluid with presence of atretic follicles without any involvement of the tunica albuginea and the superficial cortical zone are characteristically uninvolved [
2]. A thin rim of compressed cortical stroma is recognized at the periphery of the mass. Necrosis and hemorrhage are unusual. Additionally, the presence of focal stream luteinization has also been described. The oedema of the stroma is thought to provoke the activation of fibroblasts and myofibroblasts in the stroma as a reaction to the oedema [
18]. The therapeutic approach varies. The great majority of cases are unilateral and the most common treatment is unilateral salpingo-oopherectomy [
19]. Frozen section is an option for preventing unnecessary catastrophic reproductive outcomes, on the other hand we have always to keep in mind the risk of recurrence [
20]. Another option is wedge resection, which involves the removal of a minimum 30 % of the ovarian volume. This is performed in order to exclude secondary massive ovarian oedema. The possibility of postoperative adhesions is an argument to the complete removal of the ovary, because it provokes fertility issues [
6,
21]. Laparoscopy can also be a therapeutic option for massive ovarian oedema as it combines diagnostics and therapy [
22,
23]. Massive ovarian oedema is a result of the symptomatology and the intraoperative findings, as these lesions are often mistaken for primary ovarian neoplasms at laparotomy. Taking under consideration the age of the patients presenting with this entity, the preservation of fertility should be our first thought and conservative treatment must be the rule [
19].