Mitotically active fibrothecoma in association with sclerous peritonitis

Mitotically active fi brothecoma in association with sclerous peritonitis Bdioui Ahlem1*, Ben Khlifa Sarra1, Kaabia Ons2, Missaoui Nabiha3, Hmissa Sihem4 and Mokni Moncef1 1Departement of pathology, Fathat Hached Hospital of Sousse, Tunisia 2Departement of obstetric gynecology, Fathat Hached Hospital of Sousse, Tunisia 3Research Unit 03/UR/08‐13, Cancer Epidemiology and Cytopathology in Tunisian Centre, Medicine Faculty, Sousse, Tunisia 4Departement of pathology Sahloul Hospital of Sousse, Tunisia Received: 24 February, 2020 Accepted: 20 April, 2020 Published: 21 April, 2020


Introduction
Mitotically active fi brothecoma is recently described as a category of ovarian fi bromatous tumors, defi ned by mitotic fi gures more than 4 per 10 high power fi elds, without moderate to severe atypia. Association with sclerosing peritonitis is an enigmatic and rare condition, fi rst described in 1994 by Clement et al. with less than 50 pathologically characterized cases [1][2][3]. The right ovary, fallopian tubes, and uterus were Abstract Background: Mitotically active fi brothecoma associated with sclerosing peritonitis is an enigmatic and rare condition, described by Clement et al. in 1994, with fewer than 50 pathologically characterized cases. Association of these two lesions is extremely rare and the etiology currently remains unclear; therefore, association with antiepileptic drugs was suggested.
Case presentation: We report the case of young female, with history of epilepsy, presented with acute severe abdomenal pain. Ultrasound revealed a 20 cm mass, possibly arised from ovary, which was associated with septate ascites. The microscopic examination of the mass showed a morphology fi brothecoma with areas of oedema and high mitotique index but without severe atypia.

Conclusion:
Mitotically active fi brothecoma associated to sclerosing peritonitis is relatively rare condition. Although its disturbing presentation, the behavior of this rare condition is benign; therefore this entity should be well known by surgeon and pathologist to ovoid misdiagnosis and overtreatment of patient.

Discussion
Mitotically active fi brothecoma is a recently described category of ovarian stromal tumors, representing less than 10% of fi brothecoma .This entity is defi ned by mitotic fi gures more than 4 per 10 high power fi elds, without moderate or severe atypia [4,5].
The median age of patient is 41 year and most frequent symptoms are abdominal mass, acute pain, ascites as in our case with dysfunctional uterine bleeding [6].
Biologically, two cases are reported in the literature with a high level of CA125 [6].
In gross, the tumor appears as a large ovarian mass, has well-defi ned capsule and the cut-surface showed typical yellow bosselated appearance.
The average size has been reported to be 9.4 cm [6].
Microscopically, the tumor is hypercellular, usually with area of edema, occasionally imparting a microcystic appearance.
Most of the tumor cells are spindle but a minority was round with pale cytoplasm, representing weakly luteinized cells.
The median mitosis was found to be 6, with a maximum of 19 mitosis under 10 high power fi elds. Historically, since 1981, as described by Pratt and Scully, the 4 mitotic activities of the cells was given equal importance as nuclear atypia in predicting the aggressiveness of an ovarian fi bromatous tumour and classifying as fi brosarcoma. The tumour which exhibit a mitotic activity of ≤ 3/10 HPF is labelled fi bromas, while those with ≥ 4/10 HPF mitotic fi gure were considered malignant in nature [4,[7][8][9].
As time elapsed and more cases were reported, tumours that had high mitotic counts of ≥ 4/10HPF but minimal nuclear atypia were found to be less aggressive and followed a more Immunohistochemical studies have shown that the tumor cells to be reactive with calretinin, Cluster of differentiation 56(CD56), Pan Cytokeratin AE1/ AE3, smooth muscle actin, desmin and variably with alpha inhibin, epithelial membrane antigen, beta-catenin, CD34, and transforming growth factorbeta with focal nuclear positivity for estrogen and progesterone receptors [3].
In rare cases, as the case of our patient, fi brothecoma may be associated with sclerosing peritonitis. The etiology and pathogenesis of this association is not clear; but a relation with anti-epileptic drug has been discussed. Our case consolidate this hypothisis, since our patient, as the case reported by Pati et al, was under anti epilepsy drug [3,10].
On the other hand, some author proposed fi brosing soluble cytokines secreted by tumor cells to be responsible for the peritoneal manifestations [3].
Microscopically, the process of peritonitis consists of fi broblastic and myofi broblastic cell proliferation separated by collagen and fi brin, mesothelial cell proliferation, and occasionally mononuclear infl ammatory cells [3].
Currently, the concept is that the tumor is benign in behavior although complications due to sclerosing peritonitis bring down the quality of life [3].
No standard treatment for mitotically fi brothecoma currently exists, although overtreatment should be avoided in women requiring preservation of fecundity. Zong et al. suggested that other risk factors, such as tumor size, growth rate and the evaluation of Ki-67, should be considered [6,11].

Conclusion
Mitotically active fi brothecoma associated to sclerosing peritonitis is relatively rare condition. Although its disturbing presentation with a large tumor size, higher mitotic index and peritoneal involvment, the behavior of this rare condition is benign; therefore this entity should be well known by surgeon and pathologist to ovoid misdiagnosis and overtreatment of patient. Standard guideline of treatment is yet to be formulated for this entity.
The authors declare that there are no confl icts of interest associated with this manuscript.