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Introduction: Squamous cell cancer of the anal canal is a rare tumor with loco-regional evolution often slow, its management is multidisciplinary. Methods: This is a retrospective study conducted at the Oncology Centre in Casablanca, collects 42 cases of patients treated for anal canal cancer during the period from January 2005 to December 2008. The aim of our study was to evaluate our results in terms of management of squamous cell carcinoma of the anal canal. Results: The average age of our patients was 64 years with extremes ranging from 38 to 85 years. The average period of consultation was 10 months. All cases were confirmed by biopsy: it was squamous cell carcinoma in all cases. Patients were classified, with reference to the TNM classification - T1 in 2 cases, T2 in 20 cases, T3 in 10 cases and T4 in 10 cases, lymph nodes were found in 20 patients at initial examination (N2 in 12 cases and 8 cases in N3). The treatment consisted of radiotherapy alone in three patients, chemotherapy based platamine and 5-fluorouracile (for 3 to 4 cycles), followed by a combination radio-chemotherapy with weekly platamine in 22 patients, a combination radio-chemotherapy immediately in 15 patients and radiotherapy followed by brachytherapy in two patients. After a mean of 24 months, 24 patients in complete remission, 15 recurrences, 2 lost seen and 1 death. Patients who developed recurrence were treated by surgery in eight cases and second-line chemotherapy based cisplatine/5FU or capecitabine in seven cases, with stable disease in five patients and treatment failure in 10 patients. [ABSTRACT FROM AUTHOR]

Objective of the Study: The aim of our study was to provide epidemiological and evolutionary profile of nasopharyngeal carcinoma in children under 15 years, as well as showing the experience of our service. Patients and Methods: Retrospective study on nine children with nasopharyngeal carcinoma, treated with radiotherapy-oncology at the University Hospital Ibn Rushd Casablanca, between 2004 and 2009. Results: The average age of patients was 11 years, the sex ratio was 1.6, the average consultation time was 4.3 months. Apart from a case of embryonal rhabdomyosarcoma, all tumors were undifferentiated nasopharyngeal carcinoma-type (UCNT). According to the classification of the American Joint Committee on Cancer had: T4 in 45% and T3 in 55%. N0 11%, 22% N1, 11% N2 and N3 in 56%. One child was diagnosed with pulmonary metastases at diagnosis. Treatment consisted of induction chemotherapy based on adriamycin and cisplatin combination followed by concomitant chemo radiotherapy 66-70 GY conventional fractionation. We had two deaths during treatment, and the evolution was marked by a complete remission in 55%, an increase in 11%, only one local recurrence and metastasis in 11%. The evaluation of late complications of radiotherapy revealed xerostomia in all patients with sequelae hearing loss and nasal synechia in a single patient. Conclusion: Nasopharyngeal carcinoma in children is a very special form characterized by an aggressive character. Early diagnosis and appropriate technology can improve prognosis and reduce complications. [ABSTRACT FROM AUTHOR]

Introduction: Second to urothelial carcinoma, squamous cell carcinoma (SCC) is the most prevalent epithelial neoplasm of the bladder, accounting for an approximate 3-5% of bladder tumors in Western countries. We systematically reviewed our experience with squamous cell carcinoma of bladder within a period of 5 years in order to study epidemiological characteristics, treatment modalities and patient outcome. Materials and Methods: We performed a retrospective study of seven patients with SCC of the bladder treated at our Radiotherapy and Oncology Department from 2008 to 2012. Results: In our collective, a male predominance with a ratio of 2/1 was observed. The average age at diagnosis was 58 years. The average consultation time was 1-year. The reason for consultation was hematuria in all patients, associated in most cases with irritative signs as pollakiuries. All patients underwent cystoscopy with biopsy. All pure SCCs were muscle invasive carcinoma with a staging of at least T3. Radical cystectomy was treatment of choice in tree cases. Adjuvant treatment was conducted in the tree patients administering either postoperative local irradiation or chemotherapy (methotrexate/cisplatin, gemcitabine/ cisplatine). Two patients inoperable had received a combined radio-chemotherapy exclusive with three cycles of Cisplatin and pelvic irradiation of 60 Gy. The other two patients were very thirsty and had received palliative care. The median overall survival for patients with pure SCC was 7 months. Conclusion: Our data underline the individuality and poor prognosis of squamous cell cancerous lesions of the urinary tract. The histogenesis of pure SCC of the bladder is not conclusively defined. Further studies are needed in fundamental, histopathological and molecular research while clinicians should undertake combined efforts in order to optimize the treatment of patients with rare urological tumor entities. [ABSTRACT FROM AUTHOR]

Introduction: Trastuzumab is a recombinant humanized monoclonal antibody, can improve disease-free survival after chemotherapy for cancer localized HER2 positive breast cancer. However, there is a risk of cardiotoxicity; hence the need for cardiac assessment before and during treatment with trastuzumab. Cardiotoxicity is defined as a decrease in left ventricular ejection fraction (LVEF) ≥10% and a reduction to <50%. The purpose of this study was to retrospectively evaluate the incidence of cardiac dysfunction and characterize the natural history in the Moroccan population. Patients and Methods: Retrospective observational study of 115 patients, treated with Trastuzumab, between January 2010 and January 2011. Radiotherapy Department Oncology, University Hospital Ibn Rushd, Casablanca in Morocco. Results: The study included 115 patients with a mean age of 48 years. Mean LVEF prior trastuzumab was 65% and at the end of treatment 60%. Cardiotoxicity was recorded in 17% of patients, 53% completed treatment and permanent discontinuation of trastuzumab was decided in the remainder (8 patients) following an unresolved decreased LVEF. The analysis of risk factors showed that history of cardiovascular comorbidity in the group who experienced cardiotoxicity was statistically higher than the rest of our sample. Conclusion: Cardiotoxicity is a serious complication of treatment with trastuzumab, which requires careful patient selection and close monitoring. [ABSTRACT FROM AUTHOR]

Introduction: Locally advanced breast cancer forms required neoadjuvant chemotherapy to facilitate breast conserving surgery. The purpose of our study was to determine the impact of both chemotherapy protocols on the prognosis. Patients and Methods: This is a descriptive and analytical retrospective study from 2008 to 2010 in "Centre Mohammed VI pour le traitement des cancers" of University Hospital of Casablanca., We included patients with locally advanced breast cancer who received neoadjuvant chemotherapy. Other histological types were excluded. The capture and analysis of data was done using SPSS 20 and the calculation of survival rates was done by the Kaplan-Meier method and the log-rank test. Results: Included were 102 cases of breast carcinoma. The average age was 46.5 years (24-84 years). The sex ratio women/men was 5.4. Infiltrative ductal carcinoma represented 95.3%, 36.3% of tumors were classified SBRIII and 98.1 % for stage IIIb. The chemotherapy regimens were administered AT50 (53.9%) and AC60 (46.1%). Analysis of surgical specimens has noted that 48% of tumors were classified Grade 1 Knight or MT Sataloff, 13.8% Grade 2 or TB and 7.8% Grade 3 or TC. Depending on the status of N Sataloff, 50% of patients were classified NA. Overall survival was 96.4% with an average of 32.36 months followed. Disease-free survival was 94%. A difference in survival in favor of AT50 protocol (97.8%) compared to the AC60 (94.7%) protocol was noted (P = 0.8). Conclusion: Neoadjuvant chemotherapy was not higly used in our center. The results showed a good response of breast cancers to the chemotherapy without superiority between AT50 and AC60. [ABSTRACT FROM AUTHOR]

Introduction: This study was to describe epidemiology, clinical profile and treatment outcome of patients who received conservative treatment in breast cancer. Patients and Methods: This is a retrospective study about 319 cases of conservative treatment of breast cancer who were treated from January 2008 to December 2010 and followed until November 2013. Analysis was performed using SPSS 20 and the calculation of survival by Kaplan-Meier method. Results: Median age was 48 years. In 92.8% of cases, the sign was a palpable nodule. According the TNM 2009, cancers were classified after surgery, pT1 in 40.4% of cases, pT2 in 56, 4%, pT3 in 2.5%. In 48.1% lymph node involvement (pN+) has been reported. According to the histological type, invasive ductal carcinoma was noted in 89.3% of cases. Grades II and III of SBR were predominant (78.8%). Hormonal receptors were positive in 72.7% of cases and the HER2 positive in 26%. Lumpectomy followed by quadrantectomy were the main types of surgery with 80% and 11% respectively. About 90, 5% of patients received chemotherapy. The most commonly used protocols were AC60 and AT50 respectively 47% and 12.5%. All patients received external beam radiotherapy (46 Gy) followed by boost (15-20 Gy) to the tumor bed or brachytherapy (79.5%), electrons (11.4%) or photon (9.1%). Aesthetic results were judged by 75% of cases. Hormone therapy with tamoxifen or aromatase- inhibitors was prescribed in 57.7% of patient. Average follow-up time was 54 months in our study. The overall survival was 92.5% and the disease-free survival was 82.8%. Conclusion: Conservative treatment remains the most desired in the treatment of breast cancer treatment option. Oncologic and aesthetic results are encouraging in our series. [ABSTRACT FROM AUTHOR]

The coastal and marine environments and resources of the Red Sea andGulf of Aden are globally significant and generally in a healthy state. Current regional issues include localized destruction of coral reefs, seagrass and mangroves; declines in some fisheries; exploitationof some endangered species; pollution from the development and transport of petroleum; and disposal of industrial and municipal wastes. The underlying causes of these issues are the natural vulnerability ofthe Red Sea due to its semi-enclosed nature; economic reliance on the petroleum industry; significant navigation risks; a rapidly increasing coastal population and associated developments; lack of fisheriesinformation, surveillance and management; poor coastal zone planning; and limited technical expertise. Strategic actions addressing theseissues will need to be implemented regionally and focus on coastal zone management that integrates environmental planning, environmental assessment and review; training and institutional development; publicawareness and participation; information gathering especially fisheries statistics; reducing navigation risks; and the development of resource management and conservation strategies. [ABSTRACT FROM AUTHOR]

The leiomyosarcoma of the vagina is extremely rare. This paper reports two observations treated at the Ibn Rochd oncology center of Casablanca. The first patient was 32 years old, and presented a tumor of 15 cm to the detriment of the posterior wall of the vagina. The treatment consisted of a large exeresis of the tumor followed by postoperative radiotherapy. After 50 months, she is still alive. The second patient who was 42 years old, had a 10 cm tumor of the posterior wall of the vagina. A tumorectomy was realized and then the patient was not heard of. The evolution was marked by the appearance of pulmonary metastasis and a palliative chemotherapy was prescribed. This patient is deceased after 31 months.Seventy-five cases only are described in the literature. The surgery is the basic treatment.whereas radiotherapy is still discussed. The prognosis of these tumors remains linked especially to the histological rank. [ABSTRACT FROM AUTHOR]

Abstract: Neuroendocrine carcinoma with large cells is a slightly different tumor from the high rank of malignity. We report a case of breast localization in a 28-year-old patient. It is a locally advanced classified T4dN1M0 tumor that required neoadjuvant chemotherapy. The clinical answer was 75% of the level of the tumor. A standard surgery mastectomy with axillary lymph node dissection was realized, followed by external radiotherapy. The anatomopathologic and the immuno-histochimical study of the operational part confirmed the diagnosis of neuroendocrine carcinoma with large cells expressing the progesterone receptor. The patient is subjected to adjuvant hormonal treatment. After a 12 months retreat, a complete remission is maintained. Considering the scarcity of neuroendocrine carcinoma with large cells of the breast, the therapeutic standard is not yet available and the forecast remains difficult to determine. [Copyright &y& Elsevier]

Objectif de l’étude Le carcinome neuroendocrine à petites cellules de la vessie est une tumeur rare et agressive, il représente moins de 1 % de toutes les tumeurs de la vessie. L’objectif de notre travail était d’étayer les caractéristiques diagnostiques, thérapeutiques et pronostiques de cette entité rare. Matériel et méthode Il s’agit d’une étude rétrospective descriptive portant sur cinq cas de carcinome neuroendocrine de la vessie colligés au centre Mohamed-VI pour le traitement des cancers de Casablanca entre janvier 2008 et décembre 2012. Résultats La médiane d’âge des patients était de 67 ans (41–82), tous étaient de sexe masculin. Le délai moyen de consultation était de 8 mois. Un tabagisme chronique a été retrouvé chez deux patients. Tous les patients ont eu une cystoscopie avec biopsie. Le diagnostic de carcinome neuroendocrine à petites cellules a été retenu après étude immuno-histochimique. La chromogranine A était exprimée chez tous les patients et la synaptophysine chez quatre. Le bilan d’extension a révélé des métastases d’emblée chez un seul patient (pulmonaires et osseuse). Tous les cancers étaient localement évolués. Une chimiothérapie de première intention a été préconisée chez trois. Un seul cancer avait bien répondu à la chimiothérapie néoadjuvante et le patient a été opéré après quatre cycles. Les deux autres patients ont reçu une radiothérapie. Le protocole de chimiothérapie utilisé était l’étoposide et carboplatine, vu que tous les patients avaient une fonction rénale altérée contre-indiquant l’utilisation du cisplatine. L’évolution a été marquée par le décès des cinq patients de leur cancer. Conclusion Le carcinome neuroendocrine à petites cellules de la vessie est une tumeur rare et agressive. Compte tenu de sa rareté et de l’absence d’essais randomisés, la prise en charge diagnostique et thérapeutique de ces tumeurs reste difficile, et malgré un traitement multimodal, le pronostic reste défavorable. [ABSTRACT FROM AUTHOR]

Objectif de l’étude Les tumeurs phyllodes du sein sont des tumeurs rares à potentiel de rechute locale et/ou métastatique élevé. La chirurgie est, jusqu’à nos jours, le traitement de choix, néanmoins, La radiothérapie adjuvante doit être discutée en cas de traitement conservateur ou en présence de facteurs prédictifs de rechute locale. Cette étude avait pour objectif de mettre en exergue la place de l’irradiation locorégionale adjuvante dans le traitement des tumeurs phyllodes. Matériel et méthode Ce travail est une étude rétrospective analytique portant sur des patientes suivies pour une tumeur phyllode entre 2004 et 2012 au sein du CHU Ibn Rochd de Casablanca. Nous avons calculé les probabilités de survie par la méthode Kaplan–Meier avec une analyse unifactorielle en utilisant plusieurs variables. Résultats Treize cas ont été collectés. L’âge médian était de 33,9 ans. Une masse mammaire augmentant rapidement de volume était le signe révélateur le plus fréquent. Le sarcome phyllode était prédominant dans 60 % des cas. Le bilan primaire a conclu à la présence de métastases pulmonaires dans 11 % des cas. Une chirurgie conservatrice a été effectuée chez 18 % des patientes et une mastectomie chez 82 %, avec obtention de marges chirurgicales saines dans 33 % des cas. Une chimiothérapie néoadjuvante a été instaurée chez 47 % des patientes atteintes de tumeur localement évoluée et 58 % des patientes ont reçu une radiothérapie postopératoire devant des marges étroites ou atteintes. Les probabilités de survie globale et de survie sans rechute à 5 ans étaient, respectivement, de 57 et 48 %. En analyse unifactorielle, le taux de contrôle locale à 5 ans a été significativement différent entre chirurgie suivie de radiothérapie et chirurgie seule ( p = 0,02), aucune différence significative en termes de survie globale entre les deux bras n’a été noté ( p = 0,6). Conclusion Cette étude montre l’avantage, qu’apporte l’irradiation des tumeurs phyllodes, dont le profil évolutif reste imprévisible, d’où la nécessité d’une prise en charge optimale selon une approche multidisciplinaire. [ABSTRACT FROM AUTHOR]

Objectif de l’étude Le Maroc est un pays à risque intermédiaire du cancer du cavum avec une incidence de 5 nouveaux cas par 100000 habitants par an. Le diagnostic est souvent tardif dans notre contexte. En effet, les formes localement évoluées et métastatiques représentent 66,5 % des cas selon le registe des cancers du grand Casablanca de 2007. La tomographie par émission de positrons (TEP-scanographie) est aujourd’hui l’examen de choix dans leur bilan d’extension. L’objectif de l’étude était d’évaluer la concordance entre la TEP-scanographie et l’imagerie morphologique (IRM et/ou scanographie ; scanographie thoraco-abdominale) dans la détermination du statut ganglionnaire et métastatique et son impact sur la stratégie thérapeutique. Matériel et méthodes Nous avons mené une étude prospective étalée de janvier 2013 à décembre 2015 chez 19 patients suivis pour cancer du cavum de stade T3-T4 avec atteinte ganglionnaire et ayant bénéficié d’une TEP-scanographie, dont dix lors du bilan initial et neuf au moment de la récidive locale. Résultats Il y avait chez 70 % des patients ayant bénéficié d’une TEP-scanographie lors du bilan initial un envahissement ganglionnaire (dont 10 % de stade N1, 50 % N2 et 10 % N3) contre 60 % sur l’imagerie morphologique (dont 20 % de stade N1, 30 % de N2, et 10 % de N3), 10 % des cancers étaient métastatiques sur la TEP-scanographie (métastases pulmonaires) contre 0 % sur l’imagerie morphologique. Parmi les neuf patients qui ont bénéficié d’une TEP-scanographie lors de la récidive locale, 56 % étaient aussi atteints d’une récidive ganglionnaire cervicale (contre 22 % sur l’IRM) et 22 % d’une métastase à distance. Aucun patient n‘a bénéficié de scanographie thoraco-abdominale lors de la récidive. Conclusion La TEP-scanographie a ainsi permis le changement de la stratégie thérapeutique vis-à-vis du statut ganglionnaire dans 37 % (14 % au moment du bilan initial et 59,5 % à celui dela récidive locale). Elle doit être demandée systématiquement devant chaque récidive du cancer du cavum et devant toute tumeur localement évoluée au moment du bilan initial. [ABSTRACT FROM AUTHOR]

Purpose: This study surveyed radiation oncologists in Morocco to explore current practices and perspectives on brachytherapy for cervix cancer., Methods and Materials: A 37-question survey was conducted in April 2023 among 165 Moroccan radiation oncologists using Google Forms., Results: Of the 93 respondents, 39% treated over 20 patients in 2022 using 3D image-guided brachytherapy (BT) through the HDR technique; 2D techniques were not reported in the last five years. Intracavitary BT is uniformly applied with a tandem and ovoid applicator. Only 14% utilized interstitial needles for hybrid BT. Iridium-192 was the primary radioactive source (63%), followed by cobalt (37%). Ultrasound-guided 47% of applicator insertions. All used CT scans for planning, but only 6% used MRI fusion due to limited availability. Guidelines for target volume and dose prescription were mostly based on GEC-ESTRO recommendations (74%), followed by Manchester Point A (30.4%) and ABS (11%). Over 90% delineated CTV-HR and CTV-IR; 30% delineated GTV. All marked the bladder and rectum, while 52% marked the sigmoid, 5% the small bowel, and 3% the recto-vaginal point. For dosimetry, 12% used ICRU 89 points, 54% used dose-volume histograms (DVH), and 36% used both. Most reported EQD2cc for OARs for the rectum and bladder, with nine still using ICRU point doses. The most common fractionation schema was 7 Gy in four fractions (60%) and 7 Gy in three fractions (55%)., Conclusions: Brachytherapy remains essential for treating cervical cancer in Morocco. Key areas for improvement include MRI fusion-guided brachytherapy, access to advanced applicators, expanding interstitial techniques, and professional training and national referential., (Copyright © 2024 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

The aim of this analysis is to assess radiotherapy's role and technical aspects in an array of rare gastrointestinal (GI) cancers for adult patients. Collection data pertaining to radiotherapy and digestive rare cancers were sourced from Medline, EMBASE, and Cochrane Library. Preoperative chemoradiotherapy improved outcomes for patients with esophageal undifferentiated carcinoma compared with esophageal salivary gland types of carcinomas. For rare gastric epithelial carcinoma, perioperative chemotherapy is the common treatment. Adjuvant chemoradiotherapy showed no benefice compared with adjuvant chemotherapy for duodenal adenocarcinoma. Small bowel sarcomas respond well to radiotherapy. By analogy to anal squamous cell carcinoma, exclusive chemoradiotherapy provided better outcomes for patients with rectal squamous cell carcinoma. For anal adenocarcinoma, neoadjuvant chemoradiotherapy, followed by radical surgery, was the most effective regimen. For pancreatic neuroendocrine tumors, chemoradiotherapy can be a suitable option as postoperative or exclusive for unresectable/borderline disease. The stereotactic body radiotherapy (SBRT) is a promising approach for hepatobiliary malignancy. Radiotherapy is a valuable option in gastrointestinal stromal tumors (GIST) for palliative intent, tyrosine kinase inhibitors (TKIs) resistant disease, and unresectable or residual disease. Involved field (IF) radiotherapy for digestive lymphoma provides good results, especially for gastric extranodal marginal zone lymphoma (MALT). In conclusion, radiotherapy is not an uncommon indication in this context. A multidisciplinary approach is needed for better management of digestive rare cancers., (Copyright © 2023 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Background: Ureteral metastasis from gastric cancers are rare and can be a cause of ureteral obstruction. There have been few published case reports in the literature. In this paper, we report an additional case and a review of the literature of all the previous reported cases., Case Presentation: A 67 years old North African women who was treated four years before for a gastric adenocarcinoma, presented with abdominal pain. Imaging and endoscopy showed a mural stenosis of the left ureter, without any other abnormality. Histopathology confirmed the gastric origin of the metastasis. A palliative chemotherapy was foreseen, but due to the deterioration of the general condition of the patient, she received palliative care. We have also reviewed the literature and reported the previously published cases of ureteral metastasis from gastric cancer., Conclusions: It is worth recalling that in a context of neoplasia and with the presence of signs of ureteral obstruction, it is important to keep in mind the possibility of a ureteral metastasis., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Proposal: A distinct epidemiology, etiology, clinical characteristics, and therapeutic outcomes characterize nasopharyngeal carcinoma (NPC) from other head and neck cancers. An actualized analysis of NPC patients' features enables a global view of NPC management. Accordingly, the current study investigated the epidemiological and clinical characteristics of Moroccan patients with NPC, as well as their 4-years survival outcomes and influencing prognostic factors., Methods: We prospectively analyzed data of 142 histologically confirmed Moroccan patients with NPC between October 2016 and February 2019. Kaplan-Meier and Cox regression analyses were used to assess predictive prognostic factors related to NPC. All analyses were conducted using SPSS version 21 statistical software., Results: In the present study, a net male predominance was found, with a mean age of 44±16.3 years old. Advanced stages of NPC were observed in 64.1% of patients, and 32.4% of patients presented with distant metastasis at diagnosis. The 4-years overall survival, locoregional relapse-free survival, distant metastasis-free survival and progression-free survival were 68.0%, 63.0%, 53.9%, and 39.9%, respectively. Age, N category and distant metastasis were identified as the most important independent prognosis factors for NPC in this cohort (p<0.05)., Conclusion: In conclusion, NPC affects young adults and is frequently diagnosed at advanced disease stages, impacting therefore negatively patients survival; which is in line with data from endemic areas for NPC. The current study clearly highlights that a greater attention should be directed to improving the management of this aggressive malignancy.

Objective: Nasopharyngeal carcinoma (NPC) is a severe malignant disease. Despite its low frequency, NPC is very common in North African population. Radiotherapy is the standard therapeutic treatment of NPC. However, radioresistance hampers the success of treatment. At the molecular scale, radioresistance is due to genetic variations involved in DNA repair pathways in NPC patients. Several studies reported that single nucleotide polymorphisms (SNPs) in excision repair cross complementing group 1 (ERCC1) could be associated with radioresistance. In this optic, the present study aimed to evaluate the association between DNA repair gene polymorphisms ERCC1 C8092A and ERCC1 C118T and radiotherapy response of patients with NPC., Methods: A total of 95 patients with confirmed NPC were recruited at the Mohammed VI Center for Cancer Treatment, Casablanca - Morocco between 2016 and 2018. Two single nucleotide polymorphisms in ERCC1 gene were genotyped. Multiple analysis software was used to assess the correlation between these SNPs and radio-therapeutic response., Results: Sequencing of ERCC1 C8092A polymorphism revealed that CC and CA genotypes were found in 51.6% and 45.3% of cases, respectively, whereas the homozygote AA genotype was reported in only 3.1% of cases. For ERCC1 C118T polymorphism, the heterozygote CT genotype was identified in 49.5% of cases. Homozygotes genotypes CC and TT were detected in 17.9% and 32.6% respectively of NPC cases. Of note, no significant association was found between the ERCC1 C8092A polymorphism and response to radiation therapy (p=0.81). Similarly, there was no significant association between the response to radiotherapy and allelic distribution (p=0.56). Likewise, no correlation was observed neither with genotypes (p=0.07) nor with alleles (p=0.09) of ERCC1 C118T polymorphism and response to radiation therapy., Conclusion: Our results clearly showed that ERCC1 C8092A and ERCC1 C118T polymorphisms were not associated with response to radiotherapy in Moroccan NPC patients. Large studies are warranted to confirm the role of these SNPs in therapeutic response of NPC patients.

The current study was designed to investigate the changes in the circulating Epstein−Barr virus DNA load (EBV DNA) at various time points before and after treatment and its clinical significance in nasopharyngeal carcinoma (NPC). A total of 142 patients with NPC were prospectively enrolled in this study. The plasma EBV DNA concentration was measured before and after treatment using qPCR. The prognostic values of the EBV DNA load were analyzed using the Kaplan−Meier and Cox regression tests. Following multivariate analysis, our data showed that high pre-EBV DNA loads were associated with significantly poorer distant metastasis free survival (DMFS) and progression free survival (PFS); detectable end-EBV DNA loads were associated with significantly worse loco-regional recurrence free survival (LRRFS) and PFS, and the detecTable 6 months-post-EBV DNA loads were associated with significantly poorer overall survival (OS), DMFS and PFS (p < 0.05). Additionally, combining the pre-EBV DNA load and the stage of the disease, our results showed that patients at stage III-IVA with a low pre-EBV DNA load had similar survival rates as patients at stage II with a low or high pre-EBV DNA load, but had better survival rates than those at stage III-IVA with a high pre-EBV DNA load. Taken together, we showed that the change of the EBV DNA load measured at several time points was more valuable than at any single time point for predicting patients’ survival for NPC. Furthermore, combining the pre-EBV DNA load and the TNM classification could help to formulate an improved prognostic model for this cancer.

Objective: The present study aimed to assess the prognostic interest of metabolic and anatomic parameters derived from 2-deoxy-2-[ 18 F]fluoro-D-glucose positron emission tomography/computed tomography ([ 18 F]FDG PET/CT) and head and neck magnetic resonance imaging (HN-MRI) for better management of nasopharyngeal carcinoma (NPC)., Methods: In this study, pre-treatment [ 18 F]FDG PET/CT and HN-MRI parameters of NPC patients diagnosed between January 2017 and December 2018, were prospectively investigated. Correlation between those parameters and 4-year patient's survival outcomes was evaluated using Kaplan-Meier and Cox-regression analyses., Results: Our results revealed a significant association between pre-treatment nodal-maximum standardized uptake value (N-SUV max) and N categories (p = 0.01), between pre-treatment node-to-tumor SUV ratio (NTR) and both tumor size (p = 0.01) and N categories (p = 0.009), as well as between metabolic tumor volume (MTV) and both tumor size and NPC overall stage (p < 0.000). In multivariate analyses, pre-treatment N-SUV max, NTR and MTV were significant independent predictors of overall survival, distant metastasis-free survival, and progression-free survival (PFS) (p < 0.05). N-SUV max and MTV were also found to be significant independent predictors of loco-regional recurrence-free survival (p < 0.05), whereas HN-MRI detection of skull-base bone invasion was an independent factor associated with worse PFS in NPC (p = 0.03)., Conclusions: The present study highlights N-SUV max, NTR and MTV derived from [ 18 F]FDG PET/CT, and skull-base bone invasion defined by HN-MRI, as promising metabolic and anatomic prognosis biomarkers for NPC., (© 2022. The Author(s) under exclusive licence to The Japanese Society of Nuclear Medicine.)

The present meta-analysis was conducted to evaluate the prognostic value of pre and post-Epstein Barr Virus (EBV) DNA load testing and to assess the clinical benefit of using this molecular approach in the prognosis for a better nasopharyngeal carcinoma (NPC) management. Relevant studies were searched in different database until May 2020. Patient´s outcomes overall survival (OS), disease free survival (DFS), progression-free survival (PFS), distant-metastasis-free survival (DMFS), and local-regional-failure-free survival (LRFS), hazard ratios (HRs) and 95% confidence intervals (CIs) were extracted from selected studies. The association of pre and post-EBV DNA load and survival outcomes was assessed using review manager and the pooled HRs with 95% CIs were calculated. Twenty-six eligible studies were included in this meta-analysis, with a total of 9966 patients. Pooled HRs showed that EBV DNA levels before and after treatment are significantly associated with survival outcomes, with HR (95% CI) of 2.09 [1.74, 2.51] for OS, 1.77 [1.19, 2.62] for DFS, 2.53 [2.18, 2.92] for DMFS, 1.78 [1.45, 2.19] for LRFS and 2.17 [1.91, 2.47] for PFS in pre-EBV DNA, and an HR (95%) of 4.52 [2.44, 8.36], 4.08 [2.38, 6.99], 5.59 [ 3.58, 8.71] and 8.88 [5.29, 14.90] for OS, DFS and PFS and DMFS in post-EBV DNA, respectively. High pre and post-EBV DNA levels were significantly associated with poor NPC patient´s survival outcomes; which clearly confirm the high interest to introduce viral EBV DNA load as a prognostic biomarker for NPC management., Competing Interests: The authors declare no competing interests., (Copyright: Imane EL Alami et al.)

Background: The identification of effective prognosis biomarkers for nasopharyngeal carcinoma (NPC) is crucial to improve treatment and patient outcomes. In the present study, we have attempted to evaluate the correlation between pre-treatment plasmatic Epstein-Barr virus (EBV) DNA load and the conventional prognostic factors in Moroccan patients with NPC., Methods: The present study was conducted on 121 histologically confirmed NPC patients, recruited from January 2017 to December 2018. Circulating levels of EBV DNA were measured before therapy initiation using real-time quantitative PCR., Results: Overall, undifferentiated non-keratinizingcarcinoma type was the most common histological type (90.1 %), and 61.8 % of patients were diagnosed at an advanced disease stage (IV). Results of pre-treatment plasma EBV load showed that 90.9 % of patients had detectable EBV DNA, with a median plasmatic viral load of 7710 IU/ml. The correlation between pre-treatment EBV DNA load and the conventional prognostic factors showed a significant association with patients' age (p = 0.01), tumor classification (p = 0.01), lymph node status (p = 0.003), metastasis status (p = 0.00) and overall cancer stage (p = 0.01). Unexpectedly, a significant higher level of pre-treatment EBV DNA was also found in plasma of NPC patients with a family history of cancer (p = 0.04). The risk of NPC mortality in patients with high pretreatment EBVDNA levels was significantly higher than that of those with low pre-treatment plasma EBV-DNA levels (p < 0.05). Furthermore, patients with high pre-treatment EBV-DNA levels (≥ 2000, ≥ 4000) had a significant low overall survival (OS) rates (p < 0.05). Interestingly, lymph node involvement, metastasis status and OS were found to be the most important factors influencing the EBV DNA load in NPC patients., Conclusions: The results of the present study clearly showed a high association between pre-treatment EBV DNA load, the crucial classical prognostic factors (T, N, M and disease stage) of NPC and OS, suggesting that pre-treatment EBV DNA can be a useful prognostic biomarker in clinical decision-making and improving NPC treatment in Morocco.

Background: Breast cancer is the most common cancer in women in the world and in Morocco. Anthracyclines and anti-HER2 therapy are major drugs in the therapeutic management of localized breast cancer. The most serious toxicity of these drugs is cardiotoxicity. Our work aims to assess the prevalence of this toxicity in the Moroccan population., Patients and Methods: We conducted a prospective longitudinal observational study between January 2017 and June 2018. All our patients were followed in The Cardio-Oncology Unit, 1st unit of its kind in Morocco, created thanks to the collaboration between the Mohammed VI Cancer Treatment Center and The Cardiology Departement of Ibn Rochd University Hospital in Casablanca. Eligible patients (n=549) had Stage I-III localized breast cancer, verified histologically, and a pre-treatment adequate cardiac function with a LVEF = 50%, measured with echocardiography, and received systemic cardiotoxic treatment (anthracycines, anti-her2 drugs). All patients received regular monitoring of cardiac function mainly by echocardiography. Cardiotoxicity was defined as a decrease in LVEF of 10 points and / or <50%., Results: A decrease in LVEF was observed in 8.4% of our patients, with 4% symptomatic heart failure. The baseline average LVEF in the cardiotoxicity group was 63.5% (50-77) versus 60.5% (60-74) in the group without cardiotoxicity. 97.1% of these patients received anthracyclines, 98% received trastuzumab against 97% and 65% in the group without cardiotoxicity respectively. Cardiotoxicity was reversible in 6.4% of patients, permanent discontinuation of cardiotoxic treatment was observed in 2.2%. A statistically significant relationship was found between cardiotoxicity and arterial hypertension (HTA) (p = 0.002), trastuzumab (p = 0.0001) and radiotherapy for left breast cancer (p = 0.023)., Conclusion: This is one of the first observational studies in Morocco with a large number of patients, which gives us an idea of the cardiotoxicity of systemic treatments in Moroccan localized breast cancer patients. Our results join those of the literature, but are still worrying and invite us, oncologists and cardiologists, to be more vigilant with this toxicity, which influences the oncological and cardiac prognosis of our patients, especially cancer survivors.

Introduction: given its frequency and severity, colorectal cancer is a major public health problem. Diet plays a key role in preventing this type of cancer. The purpose of our study was to determine dietary risk factors for colorectal cancer in our Moroccan context., Methods: we conducted a case-control study including patients with colorectal cancer compared with controls. The statistical analysis of results was carried out using R software., Results: our study included 225 patients treated for cancer at the Mohammed VI Hospital Center and 225 controls. The average age of our study population at the time of diagnosis was 55.49±14.06 years, including 119 men (52.9%) and 106 women (47.1%) with a sex ratio of 1.12. Associations were found between the highest intakes of red meats, cold meats, sausages and the risk of colorectal cancer (p = 0.0001) with F4 (4-7 times / week) vs F1 (never): OR = 4.4 (1.6-11.9); (p = 0.001), OR = 1.7 (0.5-5.7); (p = 0.003), OR = 5.7 (1.2-27.4)). On the other hand, consumption of fish was associated with a reduced risk of colorectal cancer (p = 0.0001; OR = 0.3 (0.11-0.7)), while consumption of poultry and grilled eggs was not associated with colorectal cancer. We also found that consumption of fresh vegetables and cooked vegetables was low in patients compared to controls (p = 0.0001). Furthermore, a high intake of black coffee was associated with a reduced risk of colorectal cancer (p = 0.0001; F4 vs F1: OR = 0.2 (0.1-0.4))., Conclusion: our study highlights that dietary changes can prevent or impede the growth of colorectal cancer. It is essential to promote balanced diet, rich in fish, vegetables, fruits and fibers without exceeding recommended levels of red meat and avoiding cold meats and sausages., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts., (© Fatima Ezzahra Imad et al.)

Introduction: Colorectal cancer is a true scourge and a major public health problem. The main purpose of this study was to identify the impact of socio-economic factors and education level on the onset of colorectal cancer and of diagnosis stage in the Moroccan population., Methods: We conducted a case-control study of patients treated for cancer at the Mohammed VI center from January 2015 to January 2017. We interviewed, on a prospective basis and using a structured and pre-tested questionnaire, 225 patients and 225 eligible and consenting subjects., Results: The average age of patients was 55.49± 14.06 years. In 53% of cases diagnosis was made at early-stage colorectal cancer while in 47% at advanced stage. In addition, a detailed analysis of the studied population according to the socio-economic status (SES), showed a proportion of 25.33% (patients) versus 17.33% (control) in the low SES group, while, equivalent rates (45.33% patients versus 45.33% control) in the middle SES group. In the high SES group, the rate of patients was only 16.89% patients versus 37.34% control (p = 0.0001). Education and SES were strongly correlated with diagnosis stage, with a significant difference. Then 36.44% of illiterate patients were diagnosed in advanced stage versus 5.33% of patients who had completed their secondary education level or university course (p = 0.02). Similarly 20.45% of patients with low SES were diagnosed in late stage versus 5.33% of patients with higher SES (p = 0.03)., Conclusion: Our results highlight that the risk of developing colorectal cancer is strongly dependent on the education and the socio-economic status of patients. A more thorough investigation is needed to clarify the causes of this inequality., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts., (© Fatima Ezzahra Imad et al.)

Introduction: colorectal cancer is a major public health problem. This study aims to analyze the epidemiological, nutritional, clinical and anatomopathological features of patients with colorectal cancer at the University Hospital in Casablanca., Methods: our case-control study focused on patients assigned to treatment for colorectal cancer in 2015 compared with a control group that didn't have cancer., Results: the average age of our patients was 56.65 years, with a standard deviation of 14.64. The most common histological type of cancer in study patients was Lieberkhünien adenocarcinoma with a proportion of 82%. The analysis of the body mass index showed that 50% of study patients were obese vs 20% of patients in the control group and that 19% of study patients had diabetes vs 8% patients in the control group (p< 0.019). Moreover, the analysis of dietary habits in study patients compared to those of patients in the control group showed that the average frequency of weekly consumption of red meat was higher in study patients than in the control group (4.24 vs 3.26; p = 0.009) and , conversely, for the consumption of fish (0.97 vs 1.76; p = 0.0001). On the other hand, the average consumption of vegetables and fruits was lower in study patients than in the control group (5.00 vs 9.50; p = 0.0001). Concerning the toxic habits of our patients, 32% of study patients were smokers vs. 13% of patients in the control group., Conclusion: our results show that awareness about our dietary habits and changes in our living habits could reduce the incidence of colorectal cancer and therefore mortality and morbidity., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Neuroendocrine small cell carcinoma of the bladder is a rare and aggressive tumour, accounting for less than 1% of all bladder tumours. Given its rarity and the absence of randomized trials, the therapeutic management of these tumours remains difficult. By analogy with small cell lung cancer, multimodal treatment is often proposed. Radical cystectomy plus chemotherapy and chemoradiation therapy are associated with better survival compared to monotherapy. We report our experience in the management of these tumours with literature review., (Copyright © 2018. Published by Elsevier Masson SAS.)

Purpose: Primary breast sarcomas are heterogeneous tumours derived from non-epithelial mammary gland structures. Although they represent a rare entity, their incidence may increase in the coming years owing to conservative approach considered in the treatment of breast cancer. The aim of this work was to highlight the effect of postoperative irradiation in the treatment of these tumours., Materials and Methods: This is a retrospective study conducted at the Mohammed-VI centre for cancer treatment between 2004 and 2011. Survival rates were calculated by the Kaplan-Meier method., Results: Fifteen cases were collected. The median age was 41.9years. Phyllode sarcoma accounted for 66% of this series. Surgical treatment was performed in 93% of the patients with negative margins in 33.33% of the cases. Neoadjuvant chemotherapy was indicated in 46% of the patients with locally advanced tumours and 66% of the patients received postoperative radiotherapy for positive or close margins. Five years overall survival and relapse free survival was not significantly different with the use of adjuvant radiotherapy., Conclusion: Due to the rarity of this entity and the absence of randomized trials, evidence based management is still lacking. However, a multidisciplinary approach is to be required including surgical excision followed by radiotherapy, depending on the tumour characteristics., (Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Breast cancer in men is rare, accounting for approximately 1% of all breast cancers and less than 1% of all neoplasias in men. This study aimed to highlight the clinical histological, prognostic and therapeutic features of this rare tumor in order to contribute to improve the management of these patients. We conducted retrospective study of 40 patients whose data were collected at the Mohammed VI Center for Cancers Treatment in Casablanca from January 2000 to December 2012. The average age was 62 years, the mean consultation time was 12 months, self-exam of a peri-areolar nodul was the main reason for consultation in 90% of cases. Infiltrating ductal carcinoma was the predominant histological type in 90% of cases. Multimodal treatment was based on mastectomy followed by adjuvant therapy including chemotherapy, radiation therapy and/or hormonal therapy, depending on tumor stage and its histological features. The mean follow-up time was 38 months, patient's evolution was characterized by complete remission in 16 patients (40%), local recurrence in 3 patients (7.5%) and metastatic recurrence in 5 patients (12.5%). Metastases mainly occurred in the bones (62%), followed by the lungs and the liver. 10 patients (25%) died. Breast cancer in men is similar to breast cancer in women. However it has its own peculiarities, hence the importance of conducting broader prospective randomised studies in order to improve the treatment and the prognosis of this disease with a high psychosocial impact.

Background: Intellectual disability (ID) features in numerous heritable medical conditions that result from ATRX mutations. Alpha-thalassemia mental retardation syndrome (ATR-X syndrome) is the most notable manifestation of ATRX dysfunction. In addition to ID, genitourinary and craniofacial abnormalities are regularly observed with or without alpha-thalassemia., Aims: The study sought to characterize two cases of ATR-X in a Yemeni family clinically and molecularly., Methods: PCR amplification and Sanger sequencing were used to study the ATRX gene in a Yemeni family. Also, methylation-sensitive PCR was used to perform X-inactivation studies. CADD, SNAP2 and PolyPhen-2 helped to predict the functional consequences of the variant., Results: Molecular testing revealed a novel hemizygous missense mutation (c.5666T>G) in the ATRX gene in the two Yemeni brothers. This mutation was found in a heterozygous state in the mother, with the chromosome harboring the mutated allele being under strongly skewed X-inactivation., Conclusions: The mutated gene is predicted to have a disrupted SNF-2 domain at a conserved residue; p.Leu1889Trp, which is deemed functionally damaging. This report offers, for the first time, full clinical and molecular characterization of a novel ATRX variant in an Arab family.

Granulosa cell tumors is classified into juvenile and adult types and comprise less than 5% of ovarian tumors in women and are much rarer in men which only 45 have been previously reported. We report here a 40-year young man with a left testicular adult type granulosa cell tumor. The tumor measured 5.5X5X4cm; Immunohistochemical stains showed the tumor diffusely positive for inhibin and vimentin. Post operative CT scans shows a lomboaortic lymphnodes treated by four cycles of chemotherapy type BEP (bleomycin, etoposide, cisplatin). The thoraco abdominal CT scans post chemotherapy shows the disappearance of the right testicular nodule and the lomboaortic lymphnodes. 2 years after treatment, the patient is alive and well with no signs of recurrence. Our report highlights one more case of this very rare tumor of the testis, which is quite problematic In terms of prognosis and management, and for this reason seems to have attracted the interest of many researchers recently., Competing Interests: The authors declare no competing interests.

The most common primary sites for bone metastases in men are lung, prostate, kidney, thyroid or bladder. Colorectal origin is rare. Few studies have described this type of metastases; the axial skeleton or the pelvis are the most common metastasis locations. Craniofacial location is exceptional. We here report the case of a 38 years old man treated for metastatic rectal cancer metastasized to temporal bone. He initially had undergone surgical procedure for low anterior resection, tumor was classified as pT3N0M0; 24 months after the patient had left exophthalmos revealing a temporal tumoral process. Evolution and context favoured metastasis. In conclusion, this study reporting an exceptional case of craniofacial bone metastasis from multi-metastatic colorectal cancer will enrich the scarce data reported in the literature related to bone metastases from primary colorectal cancer., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêt.

Objective: The aim of this work was to report a case of an Emirati child who presented with developmental delay and multiple congenital abnormalities that are consistent with distal arthrogryposis type 5D., Clinical Presentation and Intervention: The clinical presentation comprised contractures of the shoulders, elbows, and knees in addition to camptodactyly and neck pterygium. The facial dysmorphic features noted include ptosis and microretrognathia. Importantly, left orchidopexy was also observed and corrected surgically. Whole exome sequencing revealed that the patient is homozygous for the novel c.1184+1G>T variant in endothelin-converting enzyme-like 1 (ECEL1)., Conclusion: This is a case of a novel homozygous splice site mutation in the ECEL1 gene in a child with a phenotype consistent with distal arthrogryposis type 5D. The child was born to consanguineous Emirati parents heterozygous for the novel ECEL1 mutation., (© 2017 S. Karger AG, Basel.)

Background/aims: This study aimed to identify, clinically and molecularly, the causality of Rabson-Mendenhall syndrome in an Emirati family. It is one of the monogenic syndromes of abnormal glucose homeostasis, which result from insulin receptor defects., Methods: A novel nonsynonymous variant in the INSR gene was uncovered by whole exome sequencing and confirmed using Sanger sequencing in the patient and his parents. Various in silico tools were utilized to analyze the functional consequences of the variant., Results: Results revealed a previously unreported INSR variant in the family: c.421C>T (p.Arg141Trp). Homozygosity for the variant was found in the patient, while both parents were heterozygous., Conclusion: The nonsynonymous protein change hit a highly conserved arginine residue in the insulin-binding α-subunit of the receptor and was deemed 'functionally damaging' by a myriad of bioinformatics tools. This report is a step forward along the way of achieving a better molecular and clinical characterization of Rabson-Mendenhall syndrome., (© 2016 S. Karger AG, Basel.)

Primary extra-ganglionic non-Hodgkin's lymphoma (NHL) is rare and its primary intramuscular location is exceptional, as it account for less than 0.5% of all patients. It generally affects men, with an average age of 70 years. Standard treatment is based on surgical excision combined with chemotherapy and radiation therapy. We report the case of a 31-year old patient presenting with muscular pseudotumoral syndrome at the level of the right leg. Histological examination showed intramuscular large B-cell non-Hodgkin's lymphoma. The patient underwent exclusive chemotherapy with complete remission., Competing Interests: Les auteurs ne déclarent aucun conflits d’intérêts.

Purpose: Mucinous carcinoma of the breast accounts for 1 to 4% of all breast cancer. There are two histological subtypes: mixed mucinous carcinoma, where the ductal carcinoma is associated with the colloid component, and pure mucinous carcinoma, with a favorable prognosis, where the mucus surrounds the tumour tissue and constitutes a mechanical barrier limiting cell invasion and making this form less aggressive. Our study aimed to determine retrospectively the main epidemiological, clinical, biological, and therapeutic features, as well as the prognosis of this rare form of breast carcinoma., Materials and Methods: The authors report 32 cases of mucinous carcinoma of the breast diagnosed in Mohammed-VI centre for cancer treatment in Casablanca., Results: The average tumour size was 4.5cm (0.5-7cm). We found ten positive lymph node dissections, seven of them were of mixed mucinous carcinoma with a tumour size ranging between 4 and 7cm. Mucinous carcinoma was pure in 16 cases, mixed in 14 and a neuroendocrine differentiation was found in two cases. Most tumours were of an intermediate histological grade (n=19) with positive hormonal receptors (68%). After a mean follow-up of 30 months, complete remission was maintained in 92% of evaluable patients., Conclusion: Mucinous carcinoma is a rare type of breast cancer, with a favourable prognosis for the pure form., (Copyright © 2016 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Primitive large cell neuroendocrine tumor of the breast is an extremely rare entity. Less than ten cases have been reported in the literature. We report a new case of a 61-year old male patient presenting with locally advanced tumor of the right breast, immediately metastasized to the lungs and pleura, staged cT4bN1M1. The patient received eight cycles of Docetaxel-based chemotherapy every 21 days, with a good clinical and radiological response (>50%), followed by adjuvant tamoxifen with disease stabilization for 18 months. Immunohistochemistry is still essential to determine the neuroendocrine nature of this tumor. Treatment is not well codified due to the rarity of this type of cancer., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêt.

Granulosa Cell Tumors (GCT) of the ovary are rare tumors belonging to the group of sex cord stromal tumors. They represent 0.6 - 3% of all ovarian tumors and 5% of all malignant tumors. Two different types of GCT can be distinguished: juvenile granulosa cell tumor (JGT) which is characterized by its aggressive potential and adult granulosa cell tumor (AGT) which is the most common and the least aggressive type. GCT of the ovary usually develops recurrences within 5 years of initial diagnosis but they rarely develop local or peritoneal metastases. Although treatment options including surgery with or without chemotherapy or radiation have been reported in treating GCT relapses, there are no standardized protocols for the treatment of relapses. We here present our therapeutic strategy in the treatment of longterm relapses of GCT peritoneal carcinomatosis occurring in two patients 10 years after the initial diagnosis and a review of the literature., Competing Interests: Conflits d’intérêts Les auteurs ne déclarent aucun conflit d'intérêts.

Phyllodes tumors of the breast are very rare and are exceptional in children and adolescents; their treatment is based on surgery and radiotherapy, with a good prognosis. We report the case of a 12-year old teenage girl presenting with a mass in the left breast. The diagnosis of a phyllodes tumor was confirmed on the basis of clinical, imaging and histological examinations. Treatment consisted of a large tumorectomy without adjuvant therapy, with good evolution during a 2-year follow-up., Competing Interests: Conflits d’intérêts Les auteurs ne déclarent aucun conflit d'intérêts.

Microcephaly is a rare neurological condition, both in isolation and when it occurs as part of a syndrome. One of the syndromic forms of microcephaly is microcephaly, seizures and developmental delay (MCSZ) (OMIM #613402), a rare autosomal recessive neurodevelopmental disorder with a range of phenotypic severity, and known to be caused by mutations in the polynucleotide kinase 3' phosphatase (PNKP) gene. The PNK protein is a key enzyme involved in the repair of single and double stranded DNA breaks, a process which is particularly important in the nervous system. We describe an Emirati patient who presented with microcephaly, short stature, uncontrollable tonic-clonic seizures, facial dysmorphism, and developmental delay, while at the same time showing evidence of brain atrophy and agenesis of the corpus callosum. We used whole exome sequencing to identify homozygosity for a missense c.1385G > C (p.Arg462Pro) mutation in PNKP in the patient and heterozygosity for this mutation in her consanguineous parents. The Arg 462 residue forms a part of the lid subdomain helix of the P-loop Kinase domain. Although our patient's phenotype resembled that of MCSZ, the short stature and evidence of brain atrophy distinguished it from other classic cases of the condition. The report raises the question of whether to consider this case as an atypical variant of MCSZ or as a novel form of microcephalic primordial dwarfism. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Angiosarcoma of the ear is a very rare and locally aggressive malignant tumor. It accounts for 4-5% of cutaneous sarcomas and less than 1% of all sarcomas. Our study aims to describe and discuss diagnostic and therapeutic modalities of this malignancy. We report the clinical case of a 31 year old patient with bulging mass of the pavilion of the left ear. CT scan was in favor of tumor of the pavilion of the ear invading ipsilateral parotid. Histological diagnosis was in favor of angiosarcoma. The treatment consisted of complete surgical resection followed by adjuvant radiotherapy. She is in complete remission with a follow-up period of 1 year., Competing Interests: Conflits d’intérêts Les auteurs ne déclarent aucun conflit d’intérêt.