Jean Bouquet de Joliniere

Small cell carcinoma of the ovary (SCC) is a very rare (less than 1 % of ovarien neoplasia), highly undifferentiated, aggressive malignancy affecting young women and linked to a poor prognosis. Overall survive rate is very low (about 16 %). SCCOHT has recently been shown to be associated with SMARCA4 gene mutations and shows some genetic similarities to malignant rhabdoid tumors (MRT). After a reminder of the clinical, histological description of the SCCOHT and concensus about the medical management, we describe the rare case of a 22 years old patient with complete remission after diagnosis of un undifferentiated SCCOHT stage IV treated by conservative surgery and high-doses chemotherapy, 30 months after diagnosis. Thus far, no standard therapy exists for SCCOHT. Treatment modalities are surgery, chemotherapy, radiotherapy and autologous stem cell transplant after high-dose chemotherapy. Research for new treatments includes target therapy. Autologous stem cell transplant after high-dose adjuvant chemotherapy seems to lead to the best survival rates. Invasiveness of the treatment depends on the stage of the disease, age of the patient and her fertility-sparing desire. An international collaboration will be needed to standardise practices due of the small number of patients.

Endometriosis is a common, hormone-dependent gynecologic disease. Undiagnosed in large proportion of women, managing therapies depend on the impact of quality of life and includes hormonal treatment and pelvic surgery. Less likely endometriosis can occur in post-menopausal women. Malignant transformation of endometriosis is a rare but well-described process, most of time occurring in the ovary, and justifies the practitioner not to underestimate this pathology. We present a case of a 61 year old woman with a symptomatic endometriotic pelvic mass, status post hysterectomy, with no history of endometriosis diagnosed beforehand.

The presence of tumor cells can be identified in the lymph node when metastasis has occurred from the primary cancer site into the lymph node (1) If the sentinel lymph node ganglion is negative for the presence of tumor cells at the time of histological examination, the other lymph nodes are also negative in 99% of cases. If no tumor cells are identified in the sentinel lymph node ganglion by histological examination, the other lymph nodes are also negative for the presence of tumor cells in 99% of cases. The sentinel lymph node advantageously replaces axillary dissection as a staging method in breast cancer T1 and T2 (2). Approximately 40% of breast cancers metastasize to axillary lymph nodes and metastatic extension depends on disease stage. Sentinel lymph nodes are affected in the following stages: T1a (4.3%), T1b (19.5%), T1c (23.8%), T2 (48.9%), T3 (66.7%).

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The authors report a case of pseudomyxoma peritonei with gelatinous peritoneum in a 47-year-old-woman. The main symptom for discovery was a chronic pelvic abdominal pain. This disease is particularly rare. The gelatinous substance is often associated with a malignant ovarian tumor or appendicitis perforated. Currently, on the whole, an exploratory laparoscopy allows diagnosis, biopsies, and appendectomy. The treatment is essentially surgical. The prognosis depends on grade (1/3) and response to chemotherapy. This case was presented to the tumor board.

Paget's disease of the breast is a rare cancer. This typical clinical case illustrates the different epidemiological, clinical, histological, therapeutic and evolving aspects of the disease. We report a case of Paget’s disease in a 43-years-old woman who presented eczema of the nipple. Mammography and ultrasounds did reveal a lesion in situ. The patient was scheduled for mastectomy and sentinel node biopsy. She had chosen a radical bilateral surgery. The histological diagnosis was Paget's disease of the breast with a carcinoma in situ. There was no metastasis in either of the sentinel nodes. Paget's disease must be considered with the presence of eczematous involvement of the persistent nipple, which does not respond to local treatment. Ultrasounds, mammography and MRI can allow searching an underlying cancer and guiding the surgical management. There is no evidence at this time that one of the 2 surgical techniques (conservative or mastectomy) would improve survival. The prognosis depends on the presence of a palpable mass and the invasiveness of the cancer

Diagnosis of adnexial torsion is difficult during pregancy (1). The time of decision and laparoscopy is that of the risk of necrosis of the adnexa and, therefore, of the ovarian prognosis. The loss of an ovary can compromise the following fertility. Even if concerns related to laparoscopy in pregnant patients include a limited surgical field, with a risk of uterine injury and negative fetal effects of CO₂ insufflation. Evidence base suggests that minimally invasive surgery can be safe and better than laparotomy for management of adnexal masses during late pregnancy with good postoperative and obstetric outcomes. If for most authors laparoscopy appears to become the best approach for ovarian torsion during pregnancy (2), nonetheless, the ideal surgical laparoscopic approach of adnexa in late pregnancy remains controversial. Since there is no technical gold standard to overcome surgical difficulties which could make laparoscopic procedures as real challenge in patients in second and third trimester (3); at least, in case of radical and non-conservative treatment, the risk for a first trimester of pregnancy is to remove the corpus luteum (1).

Minimally invasive surgery (MIS) can be considered as the greatest surgical innovation over the past thirty years. It revolutionized surgical practice with well-proven advantages over traditional open surgery: reduced surgical trauma and incision-related complications, such as surgical-site infections, postoperative pain and hernia, reduced hospital stay, and improved cosmetic outcome. Nonetheless, proficiency in MIS can be technically challenging as conventional laparoscopy is associated with several limitations as the two-dimensional (2D) monitor reduction in-depth perception, camera instability, limited range of motion and steep learning curves. The surgeon has a low force feedback which allows simple gestures, respect for tissues and more effective treatment of complications. Since 1980s several computer sciences and robotics projects have been set up to overcome the difficulties encountered with conventional laparoscopy, to augment the surgeon's skills, achieve accuracy and high precision during complex surgery and facilitate widespread of MIS. Surgical instruments are guided by haptic interfaces that replicate and filter hand movements. Robotically assisted technology offers advantages that include improved three- dimensional stereoscopic vision, wristed instruments that improve dexterity, and tremor canceling software that improves surgical precision.

Verrucous carcinoma of the vulva is a rare lesion (1). Affecting essentially postmenopausal women, this lesion is a distinct and particular entity in the vulva carcinoma classification and its scalability is uncertain and unpredictable. We present a case concerning a 48-year-old patient, without following after a condyloma acuminate of the vulva (large left lip). The origin of this case will be discussed in this article. The treatment decided was only surgical. A review of literature shows the scarcity of this lesion of the female genital tract.

We report a case of woman with a palpable lump on her left breast. On mammography a huge mass located between the inner and outer inferior breast quadrants of the left breast was found. The ultrasound examination realized later revealed a heterogeneous mass with smooth and lobulated borders. A MRI was also performed, showing an oval mass with heterogeneous areas of enhancement. Finally a core biopsy under sonographic guidance revealed a pseudo angiomatous stromal hyperplasia of the breast (PASH).