JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
adjacent to the tumor showed stromal thecal luteinization. The ovary showed focal cystic follicles with the tumor occupying the interior of the ovary. The ovarian surface was ruptured but the fallopian tube was uninvolved. An omental biopsy showed focal gliomatosis peritonei fibrovascular adhesion but with no immature neuroectodermal elements were noted. A cul-de-sac nodular implant was positive for tumor involvement. Ascitic fluid cytology was negative for malignant involvement but did have mesothelial cells and macrophages in a background of proteinaceous fluid-pT2bpNx International Federation of Gynecology and Obstetrics (FIGO) stage IIB. The subject tolerated the surgery well. She began to experience abdominal swelling again on post-operative day (POD) three, and went into spontaneous labor on POD five. Within one hour of first complaining of abdominal cramping on that day, she was delivered vaginally of a male infant at 24 weeks and two days EGA. The baby was intubated and taken to the neonatal intensive care unit, where he died of extreme prematurity and respiratory failure on day two of life. The mother experienced an uncomplicated postpartum course, other than continued abdominal distension. Computerized tomography (CT) scanning revealed a large amount of ascites (but no other abnormalities) was present in the abdomen. The subject underwent extensive counseling regarding fetal demise, as well as prognosis and subsequent therapy for her cancer. She agreed to receive chemotherapy, including a combined regimen of Bleomycin, Etoposide and Cisplatin (BEP), given intravenously once every four weeks for three total cycles. After the first administration of BEP, the ascites resolved, and the subject reported that she was feeling much better. She tolerated the treatment well, and a CT scan performed after the third (final) cycle showed no evidence of disease.
stain for pankeratin, as well as EMA (weakly) and glypican-3. Medulloepithelioma has been reported to be reactive for glypican-3. The medulloepithelioma component had a very high nuclear proliferation index with the background neuroglial tissue having only moderate reactivity. In general, ovarian malignant neuroectodermal tumors are highly aggressive and the prognosis is poor especially in the presence of extra-ovarian spread. The gliomatosis peritonei (GP) in this patient is not necessarily an indicator of poor prognosis as its behavior is benign, since mature glial cells are not aggressive and remain stable for long periods of time. 7 However, on rare occasions, GP can induce florid vascular proliferation that may result in peritoneal hemorrhage and shock or transform into a secondary malignant glial tumor. 8 The patient’s presentation with massive ascites makes her prognosis somewhat more concerning. The rarity of this tumor precludes a randomized clinical study and the management in the setting of pregnancy further complicates this case. Fertility-preserving surgery followed by chemotherapy for early stage primitive neuroectodermal tumors PNET of the ovary with successful pregnancies has been reported 9 but the outcome in more advanced cases is uncertain. The optimum chemotherapy regimen for the treatment of PNET transformed from germ cell tumors of the ovary remains unknown. 2 Management of these cases is based on case reports and largely extrapolated fromour knowledge of the transformed male germ cell testicular tumors. For PNETs that are transformed from germ cell tumors, some authors advocate for the use of platinum based chemotherapy. Many other authors however have felt that despite arising as germ cell tumors, PNETs are usually resistant to cisplatin-based treatment 10 and therefore advocate chemotherapy regimens comprising of doxorubicin, ifosphamideandcyclophosphamide, directedat the transformed PNET component. 11,12 In one of the largest series by Ehrlich and colleagues CAV/IE(cyclophosphamide/doxorubicin/vincristine alternating with Ifosphamide and etoposide), was the treatment of choice for PNETs transformed from testicular teratoma. 12 Yet other authors had used integrated chemotherapy regimen that targets both PNET and germ cell tumor. 13 Although published evidence is limited, a few cases of PNET have been reported in pregnancy, suggesting a possible effect of maternal hormones.
DISCUSSION
The incidence of ovarian cysts during pregnancy varies from 1-in-100 to 1-in-2000 pregnancies andmalignant ovarian tumors during pregnancy are uncommon. 2 Further, ovarian germ cell tumors, while rare overall, usually consist of mature teratomas and most often occur in postmenopausal women, Hinshaw et al., 2012. 6 Primitive neuroectodermal tumors are presumed to arise from the cells of the primitive neuroectoderm located in the neural crest. 3 Pathologists usually restrict the term teratoma to tumors differentiating tissues from all three germ layers, 7 and many ovarian teratomas do in fact contain tissue from all three layers. However, monodermal ovarian teratomas with one-sided tissue differentiation (monophyletic teratomas), such as Struma Ovaarii are frequently reported. Interestingly in this case, immunostaining revealed the neuropil/neuroglial tissue was reactive for synaptophysin, NSE, S-100 protein, neurofilament and GFAP. The primitive neural tube/medulloepithelioma patterns were not reactive but did
CONCLUSION
PNET of the ovary during pregnancy is rare. The management involves a combination of surgery and chemotherapy. Disease stage appears to be the most important prognostic factor for the mother, and EGA at diagnosis is the most important consideration for the infant. Survival at 24 weeks EGA (as in this case) is uncommon and frequently complicated by multiple developmental defects. The mother/patient should be carefully counseled regarding all these factors, preferably in a multidisciplinary setting which includes an obstetrician, oncologist and neonatologist.
J La State Med Soc VOL 170 MARCH/APRIL 2018 41
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