Patients with disorders of sex development (DSD), especially those with gonadal dysgenesis and hypovirilization, are at risk of developing the so-called type II germ cell tumors (GCTs). Both carcinoma in situ and gonadoblastoma (GB) can be the precursor lesion, resulting in a seminomatous or non-seminomatous invasive cancer. SRY mutations residing in the HMG domain are found in 10–15% of 46,XY gonadal dysgenesis cases. This domain contains two nuclear localization signals (NLSs). In this study, we report a unique case of a phenotypical normal woman, diagnosed as a patient with 46,XY gonadal dysgenesis, with an NLS missense mutation, on the basis of the histological diagnosis of a unilateral GB. The normal role of SRY in gonadal development is the upregulation of SOX9 expression. The premalignant lesion of the initially removed gonad was positive for OCT3/4, TSPY and stem cell factor in germ cells, and for FOXL2 in the stromal component (ie, granulosa cells), but not for SOX9. On the basis of these findings, prophylactical gonadectomy of the other gonad was performed, also showing a GB lesion positive for both FOXL2 (ovary) and SOX9 (testis). The identified W70L mutation in the SRY gene resulted in a 50% reduction in the nuclear accumulation of the mutant protein compared with wild type. This likely explains the diminished SOX9 expression, and therefore the lack of proper Sertoli cell differentiation during development. This case shows the value of the proper diagnosis of human GCTs in identification of patients with DSD, which allows subsequent early diagnosis and prevention of the development of an invasive cancer, likely to be treated by chemotherapy at young age.

Monodermal teratomas of the ovary can take the form of carcinoid tumors of which there are several types, mucinous carcinoid being the least common. Very few cases of primary mucinous carcinoid of the ovary have been reported in the literature and the behavior of these tumors over the long term is unclear. We describe a case of primary mucinous carcinoid of the ovary in a 39-year-old woman treated with unilateral salpingo-oophorectomy, where a metastasis occurred in the contralateral ovary ten years later. This case demonstrates that mucinous carcinoid of the ovary can metastasize even after a long interval, and careful follow-up of patients, particularly those treated conservatively, is appropriate.

Background

Malignant struma ovarii is a rare malignant germ cell tumor of the ovary. Due to the rarity of this disease, treatment has not been uniform throughout the published literature.

Cases

We present three cases of malignant struma ovarii. Following primary surgery, all were subsequently treated with thyroidectomy and 131I ablation therapy, two patients as first line management, one following the occurrence of metastatic disease.

Conclusion

Histological diagnosis of malignant struma ovarii is similar to that of well differentiated thyroid carcinoma (WDTC). In line with the latest advice on treatment of WDTC, we believe that the best option for patients with malignant struma ovarii is surgical removal of the ovarian lesion followed by total thyroidectomy which allows the exclusion of primary thyroid carcinoma, and in addition, allows radioiodine (131I) ablation therapy for (micro) metastasis. After thyroidectomy, thyroglobulin can be used as a tumor marker for follow-up. Moreover, nuclear medicine imaging using radioiodine (123I) can be performed to demonstrate metastatic carcinoma. A multidisciplinary approach is essential.

Background

The median laparotomy is frequently used by abdominal surgeons to gain rapid and wide access to the abdominal cavity with minimal damage to nerves, vascular structures and muscles of the abdominal wall. However, incisional hernia remains the most common complication after median laparotomy, with reported incidences varying between 2-20%. Recent clinical and experimental data showed a continuous suture technique with many small tissue bites in the aponeurosis only, is possibly more effective in the prevention of incisional hernia when compared to the common used large bite technique or mass closure.

Methods/Design

The STITCH trial is a double-blinded multicenter randomized controlled trial designed to compare a standardized large bite technique with a standardized small bites technique. The main objective is to compare both suture techniques for incidence of incisional hernia after one year. Secondary outcomes will include postoperative complications, direct costs, indirect costs and quality of life.

A total of 576 patients will be randomized between a standardized small bites or large bites technique. At least 10 departments of general surgery and two departments of oncological gynaecology will participate in this trial. Both techniques have a standardized amount of stitches per cm wound length and suture length wound length ratio's are calculated in each patient. Follow up will be at 1 month for wound infection and 1 year for incisional hernia. Ultrasound examinations will be performed at both time points to measure the distance between the rectus muscles (at 3 points) and to objectify presence or absence of incisional hernia. Patients, investigators and radiologists will be blinded during follow up, although the surgeon can not be blinded during the surgical procedure.

Conclusion

The STITCH trial will provide level 1b evidence to support the preference for either a continuous suture technique with many small tissue bites in the aponeurosis only or for the commonly used large bites technique.

Trial registration

Clinicaltrials.gov NCT01132209