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Resume Introduction Les hyperplasies thymiques se presentent sous la forme de masses mediastinales anterieures et posent de reels problemes diagnostiques et therapeutiques. Deux types d’hyperplasies sont individualises : les hyperplasies vraies et les hyperplasies folliculaires. Les donnees de la litterature concernant ces hyperplasies demeurent lacunaires. Notre objectif etait de decrire les caracteristiques cliniques et microscopiques des hyperplasies thymiques a travers une experience uni-institutionnelle sur une periode de 11 ans. Methodes Les hyperplasies thymiques diagnostiquees entre 2009 et 2020 ont ete incluses. Resultats Au total, 46 hyperplasies thymiques ont ete diagnostiquees. Les 46 patients etaient repartis en 33 femmes et 13 hommes avec une moyenne d’âge de 30 ans. Le diagnostic d’hyperplasie thymique vraie a pose un probleme de diagnostic differentiel sur des prelevements biopsiques, avec un thymus involutif dans un cas et un thymolipome dans un second cas. Conclusion Le diagnostic d’hyperplasie thymique est anatomopathologique. Dans les cas litigieux, la confrontation aux donnees anatomo-cliniques et une confrontation avec les mensurations du thymus en fonction de l’âge permettent d’asseoir le diagnostic.

Resume Introduction Les tumeurs carcinoides (TC) bronchiques, scindees en carcinoide typique (CT) et carcinoide atypique (CA), sont des tumeurs rares dont la prise en charge therapeutique reste non codifiee. Methodes Etude retrospective colligeant les cas de TC bronchiques operees dans le service de chirurgie thoracique de l’hopital Abderrahmane-Mami de l’Ariana et recrutees dans les services de pneumologie du Nord de la Tunisie, durant 12 ans. Resultats Quatre-vingt-dix patients ont ete colliges (74 CT et 16 CA). L’âge moyen etait de 45 ans et le sex-ratio H/F = 0,5. La radiographie du thorax etait normale dans 11 cas, ainsi que la fibroscopie bronchique dans sept cas. La tumeur etait classee : stade IA (10 cas), IIA (28 cas), IIB (31 cas), IIIA (15 cas), IIIB (6 cas). La chirurgie a permis une exerese carcinologique reglee chez 78 patients, une exerese elargie chez six patients et une resection conservatrice chez six patients. Une chimiotherapie adjuvante a ete indiquee chez 10 patients. La survie etait de 84 % a cinq ans et de 42 % a 10 ans. Conclusion Le pronostic des TC depend etroitement de leur sous-type histologique. Il est excellent pour les CT apres resection complete, contrairement aux CA, qui sont assimilables aux carcinomes bronchiques bien differencies.

Vascular lesions in Von Recklinghausen’s disease also known as Neurofibromatosis type 1 (NF1), are rare but have a fatal and potentially life threatening complications such as spontaneous hemothorax. An emergent thoracotomy is indicated when there is an active bleeding associated unstable hemodynamic status. Despites surgery is laborious and unpredictable but it have a merit to stop hemorrhage. A conservative management with endovascular embolization or non-operative approach have also been reported in case of hemodynamic stability. We report two case report of spontaneous hemothorax in patient with Recklinghausen disease. A chest tube was immediately inserted for two patients. Due to continuous bleeding and hemodynamic instability (Patient 1), and the increase of pleural effusion volume (Patients 1 & 2), emergent surgery of thorax was done with favourable post-operative follow up.

Resume La localisation thoracique preferentielle du schwannome est mediastinale posterieure. Sa localisation pleurale est tres rare. A notre connaissance, moins de 20 cas ont ete denombre dans la litterature. Nous rapportons deux cas operes de schwannome pleural benin primitif. Le premier est survenu chez une femme âgee de 52 ans qui se presentait pour une douleur thoracique droite et une toux chronique ; l’aspect radiologique etait en faveur d’un kyste hydatique probable du lobe inferieur droit. La seconde observation concerne un homme âge de 37 ans, sans antecedents, asymptomatique, qui presentait une masse mediastinale posterieure gauche de decouverte fortuite a l’imagerie. Via une thoracotomie postero-laterale, l’exploration peroperatoire a note respectivement deux masses pleurales endothoraciques a base d’implantation la plevre parietale, le geste chirurgical pour chacun des cas etait une resection-exerese chirurgicale complete de la tumeur. L’etude anatomopathologique definitive a conclu a deux schwannomes benins primitifs de la plevre.

Introduction: Hydatid disease remains a real public health problem in many traditional pastoral farming countries. Its prevention is the most effective solution. Aim: Our study aimed to assess the knowledge of patients managed for a lung hydatid cyst concerning their pathology. Methods: Between November 2018 and July 2019, we carried out a descriptive cross-sectional study, in a thoracic surgery department, about the knowledge and information that patients with hydatid cyst had about the disease. Results: Thirty-four patients responded to our survey. The mean age was 37.18 ± 18.43 years old. Most of our patients came from rural areas. Among the 30 patients under the age of 60, 12 (40%) were housewives. Three patients (8.8%) had already been operated on for a cyst. Thirty patients (88.3%) reported having contact with dogs and sheep. If a cyst was suspected in the sheep’s offal, 20.6% (7 cases) threw them in the trash and 14.8% gave them to the dog (5 women). Among the respondents, 50% (17 cases) did not know the modes of human contamination. Knowledge of clinical signs suggestive of the disease and modes of contamination were correlated with the level of education. Fourteen patients (41.2%) raised the possibility of disease recurrence. Among the means of prevention, thorough washing of fruits and vegetables was cited in 3 cases (21.4%) and incineration of soiled offal in 1 case (7.2%). Conclusion: Assessment of patients9 knowledge about hydatid disease must be systematic in countries with high endemicity of hydatidosis, in order to identify false practices and correct them, to break the parasitic cycle. Educational messages should target the rural population and be adapted to their level of education.

IntroductionEctopic breast tissue is present in 2–6% of women. Ectopic breast cancer represents an uncommon disease accounting for about 0.3% of all breast neoplasms, limiting the available evidence. Thus, we aim to report long-term outcomes in five cases treated at our institution.Case seriesOur Tunisian patients’ median age was 48 years (33–60 years), and the median follow-up was 8 years (4–10 years). The ectopic breast tissue was located four times in the right axilla. The median tumor size was 25 mm (15–55 mm). Four of the patients underwent a wide local excision and axillary lymph node dissection. Three of those women had positive lymph nodes; thus, they received adjuvant chemotherapy, radiation therapy, and hormone therapy. The patient with a negative lymph node (case 5) had adjuvant radiation therapy and hormonal therapy. One of the patients (case 1) had a positive supraclavicular lymph node and received radiation therapy, chemotherapy, and hormonal therapy. The latter developed a locoregional relapse after 4 years and was treated with mastectomy and chemotherapy. One patient (case 4) had a distant metastasis after 2 years of follow-up and received chemotherapy. The three other patients were free of relapse during their follow-up period.ConclusionPrimary axillary breast carcinoma is a rare entity. Despite the paucity of literature, our findings and authors’ recommendations suggest that local excision can be performed safely with promising outcomes in this subset of patients.

Synovial sarcoma is a soft tissue sarcoma. Its occurrence as a primary mediastinal neoplasm is very rare. We report a case of mediastinal synovial sarcoma in a 14‐year‐old girl with exertive dyspnoea at presentation. On physical examination, there was a superior vena cava syndrome and café‐au‐lait spots. Chest X‐ray revealed a white left hemithorax with mediastinal deviation to the opposite side. Thoraco‐abdomino‐pelvic scanner showed a voluminous posterior cervico‐mediastinal tissue mass. Computed tomography (CT)‐guided biopsy of the mediastino‐pleural mass was performed. Histological examination reported fusocellular malignant mesenchymal proliferation. A complementary immunohistological study with a broad range of antibodies was performed with a high‐grade single‐phase spindle cell synovial sarcoma of the mediastinum. She presented a respiratory distress, did not respond to resuscitation, and died. Mediastinal synovial sarcoma is a rare tumour that is difficult to diagnose. Its slow progression and delay in diagnosis may lead to a fatal evolution., Synovial sarcoma is a soft tissue sarcoma. Its occurrence as a primary mediastinal neoplasm is very rare. We report a fatal case of mediastinal synovial sarcoma in a 14‐year‐old girl with exertive dyspnoea at presentation. We highlight the role of imaging, the means of diagnosis, and the importance of the speed of therapeutic management. Mediastinal synovial sarcoma is a rare tumour that is difficult to diagnose. Its slow progression and delay in diagnosis may lead to a fatal evolution.

Background Neurogenic tumors of the mediastinum are rare tumors located mainly in the posterior mediastinum. The aim of this study was to report a single-institution experience of these tumors. Methods This study was conducted between 2005 and 2017. Bivariate analysis was performed to assess correlations between the cellularity, mitotic index, presence of a capsule, and potential for malignancy of peripheral nerve sheath tumors. Results There were 86 patients enrolled in the study. The mean age was 42.51 years. Nineteen patients were asymptomatic and their tumors were discovered incidentally. Microscopic diagnosis confirmed a schwannoma in 45 cases, neurofibroma in 12, malignant peripheral nerve sheath tumor in 9, ganglioneuroma in 14, ganglioneuroblastoma in 4, and neuroblastoma in 2. Bivariate analysis showed a significant correlation between the absence of a capsule, the degree of atypia, a high mitotic index, and high cellularity with the potential for malignancy ( p Conclusion Even if radiologic means are helpful in suggesting the diagnosis, a positive diagnosis of neurogenic tumors is based on microscopic features. High mitotic activity and cellularity, and severe cytonuclear atypia are the features most suggestive of malignancy.

Objectives:Primary cardiac tumors are rare and account for 0.001 to 0.03% of cardiac lesions. The authors aim to describe the clinical, the microscopic and the therapeutic characteristics of these tumors through a 13-year-experience in order to highlight the diagnostic challenges faced.Methods:We report 10 primary cardiac tumors diagnosed in the Departments of Pathology and Thoracic Surgery of the same hospital through a 13-year-period.Results:Our study was conducted on 7 women and 3 men. The mean age of the patients was 54.22 years (average, 12 to 79 years). Dyspnea represented the most frequent symptom. Physical examination was normal in all patients. Trans-thoracic ultra-sound examination was performed in all patients. Cardiac MRI allowed localizing the tumors in 2 patients. They were located into the left auricle (6 cases), the right auricle (1 case) and the pericardium (3 cases). The microscopic examination was concluded to myxoma (7 cases), haemangioma (2 cases) and hemangioendothelioma (1 case). Surgical resection was possible in 9 patients. It was impossible in the case of hemangioendothelioma because of the adherence. One death was recorded secondary to postoperative arrhythmia. The other patients presented no complications after a follow-up period ranging from 2 months to 5 years.Conclusion:Cardiac tumors are challenging in their diagnosis and management. A positive diagnosis is based on microscopic examination. Surgical treatment plays a key role and is possible in the majority of benign tumors. The prognosis of malignant tumors remains poor even if a complete surgical resection is possible.

Pulmonary adenoid cystic carcinoma (PACC) is an exceedingly rare tumor of low-grade malignancy. Diagnosis is often late, so the tumoral process may be huge at presentation. Surgical resection could be challenging, and the recurrence rate would be greater. We report, hereby, a case of proximal PACC with involvement of the carina in a young male adult, without respiratory distress. Surgical resection was performed through a left pneumonectomy followed by a complex trachea-bronchoplastic procedure. During the operative time, the assisted ventilatory mode was carefully chosen. No adjuvant treatment was needed. Our patient is still under clinicoradiological surveillance and remains disease-free.

Lung carcinoid tumours are rare neoplasms with a favourable prognosis. Bronchoplasty can be a conservative treatment for typical carcinoid tumours and can be applied for patients with limited respiratory function. We report the case of a 34‐year‐old woman, with a polypoid tumour located at the distal right main bronchus. Bronchial biopsy showed a typical carcinoid tumour. After resection of the tumour in the right main bronchus, bronchoplasty was performed by end‐to‐end anastomosis of the remaining right main bronchus, right upper lobar bronchus, and the upper bronchus intermedius. Bronchoscopy showed a good quality anastomosis with slightly reduced endoluminal calibre only. The post‐operative period was uneventful and the patient was discharged at the seventh day. One year later, no complications occurred and the patient is still being followed up regularly., In this article, we try to demonstrate the possibility of creating a "neo‐spur" without lobectomy to treat an intra‐bronchial typical carcinoid tumor.

Introduction: Primary tracheal tumors are rare. They represent less than 1% of primary tumors of the chest. However, they are diagnostic and therapeutic challenges. Our aim was to study the characteristics and management of primary tracheal tumors Methods: We retrospectively reviewed the clinical data from patients who were operated for primary tracheal tumors within 17 years at the thoracic surgery department of Abderrahmen Mami Hospital Ariana – Tunisia Results: There were 5 women and 4 men.The main symptom was dyspnea, accompanied with dysphonia in 1 case and dysphagia in another case. Bronchoscopy showed the lesion within the lumen in all cases. The median size of the tumor was 32.5 mm.The tumor was located in the upper third of the trachea (2 cases), the middle third (4 cases) and the lower third (3 cases). A tissue diagnosis was obtained in all patients by direct biopsy at bronchoscopy. Pathological study revealed : adenoid cystic carcinoma (4 cases), mucoepidermoid carcinoma (1 case), carcinoma with lymphoid stroma (1 case), schwannoma (1 case), glomus tumor (1 case) and cylindroma (1 case). All patients were managed by resection with reconstruction by primary anastomosis. Neck-collar incision was used in 4 cases, median sternotomy in 3 cases and posterolateral thoracotomy in 2 cases. Negative margin resection was achieved in 6 cases. The postoperative course was complicated in 3 cases. Two weeks postoperatively, bronchoscopy showed satisfactory lumen (6 cases), a lesion in the carina (1 case) and a reduced lumen (2 cases) Conclusion: Primary tracheal tumors are the least common neoplasic lesions of the airways. Non specific symptoms delay diagnosis. Resection with primary reconstruction should be preferred in initial treatment

Background: Hydatid cyst is an endemic disease that is common in the Mediterranean countries .Extra pulmonary intrathoracic localization of the cysts such as thoracic wall involving ribs, muscles, and vertebrae is very rare Methods: We retrospectively reviewed the records of 26 patients managed between 2000 and 2020 in the thoracic and cardiovascular surgery department of Abderrahmen Mami Hospital Ariana – Tunisia. Results: There were 9 woman and 17 men, with a mean age of 41 years (18-76).The most common signs were chest pain (12 cases), neurological deficit (5 cases) ,cough and dyspnea (3 cases). Parietal swelling was reported in 10 cases and 1 patient was asymptomatic. Chest X-ray showed an homogeneous opacity (6 cases), bone destruction (4 cases) and was normal in 5 cases. The cysts mostly were located at ribs (16 cases) and ribs with vertebrae (13 cases). Other parts of chest wall were involved like muscles and sub-cutaneous tissue in 1 case in the sternum in 2 cases. CT scan and MRI often revealed the diagnosis .The cyst was mimicking tumor in 1 case.15 patients underwent costal resection; 7 underwent a costovertebral resection. Other surgical procedures were partial resection of the sternum (two cases) and cystectomy with resection of parietal soft tissue (2 cases).the postoperative course was uneventful in 22 cases .reported complications were paraplegia in 1 case , ARDS and death in 1 case and severe sepsis in 2 cases. Albendazole was given for most cases. Conclusion: Current imaging investigations make diagnosis of chest wall hydatidosis easier. Radical surgery combined with antihelminthic therapy is the best treatment. Prevention is necessary.

Introduction: Cystic echinococcosis, which is commonly contracted during childhood or adolescence, is still a serious health problem in many countries. Pulmonary hydatid location is more frequent than hepatic, in children. Material and Methods: The records of 75 children managed for LHC between 2010 and 2019 in a thoracic surgery department were retrospectively reviewed. Results: There were 47 boys and 28 girls, with a mean age of 11.31 years (2–15 years). Parasitic exposure was found in 80% of the cases. The most common signs were: cough (62%), chest pain (49%), hemoptysis (42%) and fever (36%). Imaging tools showed a single LHC in 74 cases and bilateral in one case. It was right seated in 33 cases (45.3%) and left in 42 (56%). Mean size of LHC was 7 cm. Associated hydatic lesions were seen in the liver in 11 cases and in the spleen in one case. All the patients were operated. The most used approach was a video-assisted mini-thoracotomy. LHC was complicated in 34 cases and non-complicated in 41 cases. For the treatment of the residual cavity, parenchyma-saving procedures were performed most of the time: cystectomy in 64 cases (85.3%) and pericystectomy in 6 cases (8%). Anatomical resection was performed in 5 cases (6.6%): 4 complicated cysts and one simple cyst. The postoperative course was uneventful in 97% of the cases. Complications were seen in 2 patients: fever (1 case) and hemothorax (1 case). Secondary pleural hydatidosis was reported in one case during follow-up. conclusion: LHC are often asymptomatic and diagnosed at a later stage in children. Intact cysts should be operated rapidly to avoid rupture and parasitic dissemination. Parenchyma-saving procedures should be performed whenever possible, even in infected cysts.

Partial Anomalous Pulmonary Venous Connection (PAPVC) is a rare congenital abnormality characterized by drainage of one or more pulmonary veins to the right atrium or to one of the systemic veins. This pathology has low prevalence, although it is probably underestimated and is rarely diagnosed in adults. We reported a rare case of hemoptysis in adult associated to isolated partial anomalous pulmonary venous return. He is a patient of 49 years-old man who had a partial anomalous pulmonary venous connection (PAPVC) from the right upper lobe to the superior vena cava without other congenital heart disease. A right bilobectomy (superior and middle lobe) was performed for the treatment for hemoptysis and PAPVC. The patient has an uneventful postoperative recovery and remained well at follow-up 8 months after surgery.

Resume Introduction Le mesotheliome pleural malin (MPM) est une tumeur rare souvent associee a l’exposition a l’amiante. Le temps de latence entre l’exposition et l’apparition du cancer peut atteindre 40 ans. Ce delai est a l’origine du pic d’incidence recent observe dans de nombreux pays notamment la Tunisie. Les donnees cliniques font suspecter le diagnostic qui ne peut etre retenu qu’au terme de l’examen anatomopathologique. Notre objectif etait de decrire les caracteristiques cliniques et anatomopathologiques du MPM en nous basant sur une experience uni-institutionnelle. Patients et methodes Nous avons collige une etude retrospective a propos de 30 MPM diagnostiques sur une periode de 20 ans (1995–2015). N’ont ete inclus que les patients pour lesquels tous les renseignements cliniques, radiologiques et anatomopathologiques etaient disponibles. Tous les diagnostics etablis ont ete relus par 2 pathologistes. Une moyenne de 12 lames/cas a ete relue. La classification des differents types de mesotheliomes a ete faite selon la classification de l’OMS 2015. Resultats L’âge moyen des patients etait de 61 ans [22–80 ans]. Le sex-ratio H/F etait de 6,5. La notion d’exposition a l’amiante a ete mentionnee dans 21 cas. Le signe clinique le plus frequent etait la douleur thoracique qui etait rapportee dans 25 cas. L’examen physique a objective un syndrome pleuretique dans 18 cas (60 %). Les moyens d’imagerie ont objective une tumeur unilaterale dans tous les cas realisant un epaississement pleural diffus dans 17 cas. Au terme du bilan d’extension, 12 patients etaient classes en stade I, 3 en stade II, 14 en stade III et 1 patient en stade IV. Une biopsie pleurale a ete realisee a l’aiguille dans 18 cas, sous thoracoscopie dans 16 cas, sous thoracotomie dans 3 cas et a permis de confirmer le diagnostic dans respectivement 7 cas/18, 16 cas/16 et 3 cas/3. Une biopsie ganglionnaire par mediastinoscopie a ete realisee dans un cas et a permis de poser le diagnostic. Le diagnostic de MPM a ete fait sur une piece operatoire chez 2 patients qui ont eu respectivement une resection tumorale de proprete avec lobectomie superieure droite et une bullectomie. L’examen microscopique a conclu a 17 ME, 4 mesotheliomes sarcomatoides (MS) et 9 mesotheliomes biphasiques (MB). En immunohistochimie, la pan-cytokeratine a ete utilisee dans tous les cas en association a 2 anticorps a valeur diagnostique positive et 2 anticorps a valeur diagnostique negative. Les etudes immunohistochimiques ont ete repetees dans 15 cas et les anticorps le plus souvent redemandes etaient la calretinine et le TTF1. Ceci etait justifie par la mauvaise fixation des prelevements dans un cas et afin de satisfaire un critere qualite a savoir l’obtention d’un marquage intense et diffus dans les autres cas. Une resection chirurgicale a ete realisee chez 2 patients. 15 malades ont ete perdus de vue apres un suivi moyen de 3 mois. Treize malades sont decedes avant ou pendant le traitement. Conclusion Ce travail a permis de mettre en evidence une experience Tunisienne dans le diagnostic et la prise en charge du MPM en Tunisie. Parmi les principaux ecueils rencontres, on peut citer le faible effectif de notre serie et les donnees incompletes dans les dossiers medicaux qui ont conduit a l’exclusion de nombreux cas. Le diagnostic positif anatomopathologique necessite un certain degre d’expertise et chaque laboratoire doit determiner les valeurs diagnostiques des differents anticorps disponibles afin d’optimiser leur utilisation et diminuer les delais diagnostiques.

Cyriax syndrome is a rare entity of the chest wall, which mainly affects young people. It can manifest as abdominal pain in the right or left hypochondriac region, which may be very intense, and often causes problems in the differential diagnosis. We report the case of a 36-year-old man who presented with intense left hypochondrial pain, worsening on exercise. After multiple specialized consultations and several unnecessary and expensive investigations, a diagnosis of Cyriax syndrome was obtained. A thorough physical examination might have been sufficient to evoke the diagnosis.

Tumors of the thymus are rare. Neuroendocrine tumors are extremely rare. We report a case of a 39-year-old man, referred to our hospital for a paraneoplastic Cushing syndrome with a thymic tumor. Despite bilateral adrenalectomy, the patient still had signs of hypercorticism. A CT scan revealed a 25-mm nodule in the thymic compartment. A resection was indicated and surgery was successful. No adjuvant therapy was administrated. After 10 months, the patient was in good health and his clinical symptoms resolved, with no signs of recurrence. Lung and thymic neuroendocrine carcinomas with Cushing syndrome are aggressive with a high risk of recurrence even after a complete resection. A close follow-up is recommended.

Introduction/Background Non-epithelial ovarian tumors represent an heterogeneous group of rare ovarian cancer. The aim of this study was to describe the clinico-pathological features and treatment of those malignancies in a Tunisian single center. Methodology Medical records of 134 patients with non-epithelial ovarian tumors treated between 1980 and 2010 were reviewed. Data collected included clinical features, treatment modalities and prognostic indicators. Results Overall, 94 were germ cell tumors (70%) and 40 sex cord-stromal tumors (30%). Thirty-six (27%) had immature teratoma, 28 (21%) had dysgerminoma, 13 (10%) had mixed germ cell tumors, 12 (9%) had yolk sac tumor, 5 (4%) had embryonal carcinoma, 37 (27%) had adult granulose cell tumors, 2 (1.5%) had juvenile granulose cell tumors and one (0.5%) had leydig and sertoli cells tumor. Median age at diagnosis was 28 years (range 7–95). Mean age was 27 for immature teratoma, 18 for dysgerminoma, 21 for mixed germ cell tumors, 23 for yolk sac tumor, 13 for embryonal carcinoma, 52 for adult granulose cell tumors and 24 for juvenile granulose cell tumors. Ninety-four (70%) presented with abdominal mass and/or pain. Surgery was the first treatment for 99% (n=133) with fertility sparing management in 79%. FIGO staging was as follows: Stage I in 63% (n=84) (stage IC: 23% of stage I), stage II in 6% (n=8), stage III in 24% (n=32), and stage IV in 7% (n=10). Sixty-four (48%) received adjuvant chemotherapy. Median follow-up period was 45 months. The 5-year odds survival was 78%. Reccurence occurred in 18% (n=24).The 5-years disease free survival was 75%. Conclusion As it is usually unilateral and chemosensitive with high incidence in young women, fertility-sparing surgery with optimal staging should be the standard of care of non-epithelial ovarian cancer. Disclosure Nothing to disclose.

Introduction/Background Melanoma of the vulva (MV) is rare; accounting for 10% of vulvar malignancies. Although there is not a standard management for MV, the surgical resection remains the mainstay of the treatment. Methodology We report a case of MV diagnosed and treated at Salah Azaiz institute in 2019. Results A 76-year-old woman presented with a nodule of the vulva with urinary burning. Clinically, we noticed a hard and movable reddish nodule of 30 mm on the clitoris with irregular borders. This lesion seemed to be invading the urethra and the lower third of the vagina. There were no associated inguinal lymph nodes. A biopsy of this lesion concluded to the diagnosis of MV. Pelvic MRI showed a heterogeneous mass of 29*16 mm of the clitoris extended to the labia minora. It invaded the terminal part of the urethra and reached the lower edge of the anterior vaginal wall without extension into it. It also demonstrated millimetric bilateral inguinal lymph nodes. No secondary lesions were found in the CT scan.The patient underwent anterior pelvic exenteration and radical vulvectomy with bilateral inguinal lymph node biopsy. Histological examinations showed free margins and no lymph node metastasis. The patient is alive, with no recurrence, after three months of follow up. Conclusion The MV represents an uncommon tumor; occurring mostly in older women. Because of its poor prognosis, early diagnosis and staging of this cancer is mandatory. Disclosure Nothing to disclose

Introduction/Background Sebaceous carcinoma (SC) is an extremely rare malignant epithelial tumor of the breast it is a breast carcinoma with prominent sebaceous differentiation. Only thirteen examples have been reported. Thus, the aim of this case is to highlight this rare disease and report its clinical, histological and therapeutic features. Methodology We report a case and we reviewed publications using Sciencedirect and PubMed. Results A 36-year-old woman presented with a palpable mass in the left breast. She had a family history of breast cancer. Clinically, we noticed a hard and movable mass of 10 mm in the lower outer quadrant of the left breast. There were no skin lesions or axillary lymphadenopathies. The imaging described two ill-circumscribed lesions of 11 and 9 mm in the lower outer quadrant of the left breast and classified as BI-RADS 4c. Ultrasound-guided microbiopsies demonstrated invasive ductal carinomas grade 3 SBR. She conducted a left lympectomy and sentinel axillary lymph node. The frozen section showed no lymph node metastasis and a bifocal invasive carcinoma wich were 15 mm apart ; so left mammectomy was performed. On microscopic examination, the first focus of 10 mm was a SC SBR grade III. The second one of 6 mm was an invasive ductal carcinoma. Immunohistochemichal analysis has revealed that tumor cells were positive for AE1/AE3, EMA, HER2, PR, ER and AR. Ki67 was of 70%. Then, she will receive chemotherapy and radiotherapy after a fertilty preservation consultation. Conclusion SC of the breast with positive staining for AR is more aggressive. Thus, a careful follow up is necessary. More cases are warranted to clarify the particularities of this lesion, especially its prognosis. Disclosure Nothing to disclose.

Introduction/Background Clear cell carcinoma of the breast is a rare histological subtype characterized by carcinoma cells containing an optically clear cytoplasm. It accounts for 1–3% of all breast cancer cases. Methodology We report on the prognosis of two female patients treated in our institute in 2007 and 2013. We reviewed publications since 1981 to date, using PubMed and Sciencedirect. Results A 36 year-old women presented with a mass in the right breast. Examination showed irregular lump in the upper outer quadrant of her right breast, measuring 15*10 mm. No locoregional nodes were palpable. Breast imaging showed a high density nodule of 20 mm with irregular margins classified as BIRADS 4. A Tru-cut needle biopsy was performed. The microscopic examination showed an invasive ductual carcinoma containing clear cytoplasm. Breast-conserving surgery with axillary sentinel lymph node biopsy was initially performed. Surgical margins were positive. A complementary mastectomy was done.The patient was submitted for chemotherapy. Four months after the surgery,examination revealed a permeation nodule. The patient resumed the chemotherapy and radiotherapy. Five months later a CT-scan showed lung and mediastinal node metastases. The patient died of respiratory failure. A 67 year-old women, presented with a mass in the right breast. Examination revealed a hard and irregular mass of 35 mm in the upper outer quadrant of the right breast. Enlarged lymph nodes were palpable in the right axillary. Breast imaging showed a round, high density mass with partially irregular margins measuring 35 mm classified as BIRADS 5. Breast-conserving surgery with lymph node dissection was performed. The microscopic examination showed a poorly differentiated carcinoma with abundant clear cytoplasm.Two months after surgery, examination showed a permeation nodule. The patient was sent to chemotherapy. After two cycles of chemotherapy, she developed a rhino-orbito-cerebral mucormycosis and died after a month. Conclusion Clear cell carcinoma of the breast is an aggressive subtype with bad prognosis. Disclosure Nothing to disclose.

Introduction/Background Non-epithelial ovarian tumors are uncomm malignancies which can be extremely challenging to treat. The aim of the study was to evaluate the effectiveness of chemotherapy in women with early stage disease. Methodology Medical records of 84 patients diagnosed with stage I non epithelial ovarian cancer between 1985–2015 were reviewed. Tumor characteristics and treatment receipt were described. Results Median age was 28 years [9–95]. Sixty-five had stage Ia disease at diagnosis while 19 presented with stage Ic disease. Fourteen had dysgerminoma (16.7%), 30 had immature teratoma (35.7%), 5 had yolk sac tumor (6%), 3 had embryonal carcinoma (3.6%), 3 had mixed germ cell tumor (3.6%), 28 had adult granulose cell tumor (33.3%) and one had juvenile granulose cell tumor (2.4%). Most of them (n=55, 65%) underwent fertility sparing surgery. Thirty-one (37%) received adjuvant platinum based chemotherapy, among them 17/65 were stage Ia disease and 14/19 were stage Ic disease. Fifty-two (62%) had surgery followed by active surveillance. Fertility sparing surgery was followed by adjuvant chemotherapy in 25% (n=21). After a median follow up of 45 months, 14 relapsed. Among them 6 received adjuvant chemotherapy and 8 underwent surgery followed by surveillance. The 5-year-disease-free-survival (DFS) and 5 year overall survival (OS) were respectively 79% and 46% in the chemotherapy arm and 80% and 51% in the surveillance arm with no statistically significant difference observed in DFS (p=0.4) and OS (p=0.3). Conclusion Fertility sparing surgery with active surveillance is an option which should be discussed within a multi disciplinary team especially in young patients with early stage disease desirous of pregnancy. Disclosure Nothing to disclose.