Intravenous leiomyomatosis: Case series and review of the literature.

INTRODUCTION: Intravenous leiomyomatosis (ILV) is a rare pathology, part of leiomyoma beyond the uterus (LBU), characterized by benign smooth muscle cell tumor outside of the uterus and mainly affecting premenopausal woman with a medical history of leiomyoma or gynecologic surgical treatment. The treatment depends on the localization of the tumor, age of the patient, initial size, symptoms and the suitability for surgery but should always aims in toto surgical resection. CASE PRESENTATION: Retrospective case series and review of literature. CLINICAL DISCUSSION: Symptoms presented by the patient were aspecific and only localized in the pelvic area. All cases were fortuitous histopathological diagnosis. No relapse was. Two out of 5 patients have pulmonary nodules, only one was biopsied and diagnosed with PBML (pulmonary benign metastasizing leiomyoma). CONCLUSION: IVL and BML are rare disease that can co-exist. Because of tumoral hormonal receptors, hormonotherapy could be an optional treatment but to date no clear efficacy is demonstrated. In case of high recurrence risk such as voluminous initial mass, impairment of broad ligament, failure of total surgical resection, adjuvant hormonotherapy could be useful. Recurrence rate is about 16.6-30% and can occur even dozen years later and even after radical surgery, justifying a regular follow up.

Rare case of remission of a patient with small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) stage IV: Case report.

INTRODUCTION: 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). PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: 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.