Balancing efficacy and adverse reactions using everolimus in a patient with metastatic malignant insulinoma: Case report.

INTRODUCTION: Malignant insulinoma is a rare neuroendocrine tumor responsible for excessive insulin secretion and life-threatening hypoglycemia episodes. Computed tomography (CT) of the abdomen can identify a pancreatic tumor corresponding to insulinoma. Loco-regional metastases define the metastatic cases. The first-line therapeutic approach is surgery, while other medical treatments like diazoxide and everolimus play also a role. These treatments have shown efficacy in regulating blood glucose and, to some extent, controlling tumor progression. CASE PRESENTATION: We present the case of a 48-year-old female who was admitted for severe hypoglycemia episodes. She presented neuroglycopenic symptoms without any other clinical features. High levels of C-peptide and insulin during severe hypoglycemia confirmed the presence of endogenous hyperinsulinism. The CT scan of the abdomen confirmed the existence of an insulinoma along with several hepatic metastases. Surgery was proposed as a first-line approach. However, due to the persistent occurrence of severe hypoglycemia episodes, other treatment options were necessary such as diazoxide and everolimus. Diazoxide caused a significant improvement in the patient's blood glucose levels. Nonetheless, glycemic control was unsustainable, obligating the switch to everolimus, which showed better control of blood glucose levels with challenging management due to the appearance of grade 3 stomatitis as a side effect. The patient died 1 year after the diagnosis due to tumor progression. CONCLUSION: Balancing the benefits of enhanced glycemic control with the difficulties posed by side effect management of everolimus underscores the need to carefully consider both efficacy and potential adverse events.

Increased prevalence of the founder BRCA1 c.5309G>T and recurrent BRCA2 c.1310_1313delAAGA mutations in breast cancer families from Northerstern region of Morocco: evidence of geographical specificity and high relevance for genetic counseling.

BACKGROUND: Inherited mutations in the breast cancer susceptibility genes BRCA1 and BRCA2 (BRCA1/2) confer high risks of breast and ovarian cancer. Because the contribution of BRCA1/2 germline mutations to BC in the Northeastern population of Morocco remains largely unknown, we conducted this first study to evaluate the prevalence and the phenotypic spectrum of two BRCA1/2 pathogenic mutations (the founder BRCA1 c.5309G>T and BRCA2 c.1310_1313delAAGA). This choice was also argued by the presence of an apparent specific geographical connection of these mutations and the Northeastern region of Morocco. METHODS: Screening for the germline mutations c.5309G>T and BRCA2 c.1310_1313delAAGA was performed by sequencing on a total of 184 breast cancer (BC) patients originated from the Northeastern region of Morocco. The likelihood of identifying a BRCA mutation is calculated using the Eisinger scoring model. The clinical and pathologic features were compared between the BRCA-positive and BRCA-negative groups of patients. Difference in survival outcomes was compared between mutation carriers and non-carriers. RESULTS: BRCA1 c.5309G>T and BRCA2 c.1310_1313delAAGA are responsible for a significant proportion of all BC cases (12.5%) and at least 20% of familial BC. The screening of BRCA1/2 genes by NGS sequencing confirmed that there are no additional mutations detected among positive patients. The clinicopathological features in positive patients were in accordance with typical characteristics of BRCA pathogenic mutations. The mean features in the carriers were the early onset of the disease, familial history, triple negative status (for BRCA1 c.5309G>T) and worse prognosis in terms of overall surviving. Our study indicates that the Eisinger scoring model could be recommended to identify patients for referral to BRCA1/2 oncogenetic counseling. CONCLUSION: Our findings suggest that BRCA1 c.5309G>T and BRCA2 c.1310_1313delAAGA mutations may have a strong founder and/or recurrent effect on breast cancer among the Northeastern Moroccan population. There contribution to breast cancer incidence is certainly substantial in this subgroup. Therefore, we believe that BRCA1 c.5309G>T and BRCA2 c.1310_1313delAAGA mutations have to be included in the array of tests aimed at revealing cancer syndrome carriers among subjects of Moroccan origin.

Predictive factors of disease-free survival after complete pathological response to neoadjuvant radiotherapy for rectal adenocarcinoma: retrospective case series.

BACKGROUND: Many data suggest that patients with low rectal adenocarcinoma who achieved ypT0N0 status have improved survival and disease-free survival (DFS) compared to all other stages however only few data are available regarding the specific prognosis factors of this subgroup. This study aimed to evaluate predictive factors for disease free survival after complete pathological response (CPR) in cases of low rectal adenocarcinoma. MATERIALS AND METHODS: From January 2005 to December 2013, all patients with low rectal adenocarcinoma who underwent neoadjuvant chemoradiotherapy followed by total mesorectal excision and achieved CPR were included at 7 Moroccan and 1 Algerian centres. Predictive factors for disease-free survival were analysed by uni and multivariate analysis. RESULTS: Eigthy-four (12.1%) patients achieved a CPR (ypT0N0). Multivariate analysis revealed that both poorly differentiated tumors (OR, 9.23; 95 CI 1.35-62.82; P = 0.023) and the occurrence of perineal sepsis (OR, 13.51; 95 CI 1.96-93.12; P = 0.008) were independently associated with impaired DFS. CONCLUSIONS: Patients with low rectal cancer who exhibited a CPR after neoadjuvant therapy have good prognoses; however, the occurrence of perineal sepsis and/or poor initial differentiation may be associated with impaired DFS in these patients. TRIAL REGISTRATION: The study was retrospectively registered the 28th July 2018 in ClinicalTrials.gov register with the reference NCT03601689.

Significant association between ERCC2 and MTHR polymorphisms and breast cancer susceptibility in Moroccan population: genotype and haplotype analysis in a case-control study.

BACKGROUND: Genetic determinants of breast cancer (BC) remained largely unknown in the majority of Moroccan patients. The purpose of this study was to explore the association of ERCC2 and MTHFR polymorphisms with genetic susceptibility to breast cancer in Moroccan population. METHODS: We genotyped ERCC2 polymorphisms (rs1799793 (G934A) and rs13181 (A2251C)) and MTHFR polymorphisms (rs1801133 (C677T) and rs1801131 (A1298C)) using TaqMan SNP Genotyping Assays. Genotypes were compared in 151 BC cases and 156 population-matched controls. Allelic, genotypic and haplotype associations with the risk and clinicopathological features of BC were assessed using logistic regression analyses. RESULTS: ERCC2-rs1799793-AA genotype was associated with high risk of BC compared to wild type genotype (recessive model: OR: 2.90, 95% CI: 1.34-6.26, p = 0.0069) even after Bonferroni correction (p < 0,0125). MTHFR rs1801133-TT genotype was associated with increased risk of BC (recessive model, OR: 2.49, 95% CI: 1.17-5.29, p = 0.017) but the association turned insignificant after Bonferroni correction. For the rest of SNPs, no statistical associations to BC risk were detected. Significant association with clinical features was detected for MTHFR-rs1801133-TC genotype with early age at diagnosis and familial BC. Following Bonferroni correction, only association with familial BC remained significant. MTHFR-rs1801131-CC genotype was associated with sporadic BC. ERCC2-rs1799793-AA genotype correlated with ER+ and PR+ breast cancer. ERCC2-rs13181-CA genotype was significantly associated large tumors (T ≥ 3) in BC patients. None of these associations passed Bonferroni correction. Haplotype analysis showed that ERCC2 A-C haplotype was significantly associated with increased BC risk (OR: 3.71, 95% CI: 1.7-8.12, p = 0.0002 and p = 0.0008 before and after Bonferroni correction, respectively) and positive expression of ER and PR in BC patients. ERCC2 G-C haplotype was correlated with PR negative and larger tumor (T4). We did not find any MTHFR haplotypes associated with BC susceptibility. However, the less common haplotype MTHFR T-C was more frequent in young patients and in familial breast cancer, while MTHFR C-C haplotype was associated with sporadic BC form. CONCLUSIONS: Our findings are a first observation of association between ERCC2 SNPs and breast cancer in Moroccan population. The results suggested that ERCC2 and MTHFR polymorphisms may be reliable for assessing risk and prognosis of BC in Moroccan population.

First report on molecular breast cancer subtypes and their clinico-pathological characteristics in Eastern Morocco: series of 2260 cases.

BACKGROUND: Breast cancer is the most frequent malignancy among women in Eastern Morocco. In this paper, we provide the first report on molecular breast cancer subtypes in this region. This is the largest population-based study on breast cancer among Moroccan women. METHODS: We analyzed 2260 breast cancer cases diagnosed at the Hassan II Regional Oncology Center between October 2005 and December 2012. Clinico-pathological and therapeutic features were studied. Molecular subtypes were determined and their associations with the clinico-pathological characteristics of the tumors were examined. RESULTS: The mean age at diagnosis was 48.7 years ±11.4. Invasive ductal carcinoma was the predominant histological type (77.1%), followed by lobular invasive carcinoma (15.3%). The mean size of breast tumors was 3.5 cm ± 1.96, and 84% of our patients are diagnosed with tumors of more than 2 cm. Histological grade II tumors were the most frequent (70.4%), followed by advanced histological grade (18%). Lymph node positive tumors were observed in 64.8% of cases and 29.3% of patients had distant metastasis. Most tumors were hormone receptor-positive (73%) and 28.6% were HER2 positive. 86.1% of patients with hormone receptor-positive breast cancer were given hormone therapy, while 68.9% of patients with HER2+ breast cancer received targeted therapy with Herceptin. Luminal A was the commonest molecular subtype, followed by Luminal B, Triple Negative and HER2. The highest prevalence of premenopausal patients was observed in Triple Negative subtype (72.2%), followed by HER2 (64.1%), Luminal B (62.2%), and Luminal A (55.1%). Luminal B subtype had a poorer prognosis than Luminal A. Compared with Triple Negative, HER2 subtype tend to spread more aggressively and is associated with poorer prognosis. CONCLUSIONS: Unlike Western countries, breast cancer occurs at an earlier age and is diagnosed at a more advanced stage in Eastern Morocco. In this region, hormone receptor-positive tumors are predominant and so the majority of breast cancer patients should benefit from hormone therapy. HER2 subtype presents an aggressive tendency, suggesting the importance of anti-HER2 therapy. This study will contribute in developing appropriate screening and cancer management strategies in Eastern Morocco.

Perineal pseudocontinent colostomy is safe and efficient technique for perineal reconstruction after abdominoperineal resection for rectal adenocarcinoma.

BACKGROUND: The aim of this study was to evaluate oncologic results and satisfaction rate results of pseudocontinent perineal colostomy (PCPC) using Schmidt's technique in patients undergoing abdominoperineal resection (APR) for managing low rectal adenocarcinoma. METHODS: From January 1993 and December 2007, One hundred and forty six patients underwent successfully PCPC after abdominoperineal resection for lower rectal adenocarcinoma. There were 75 women, with an average age of 47 years old. All patients received neoadjuvant radiotherapy with or without chemotherapy. Long-term oncological results and satisfaction rate were evaluated. RESULTS: After a median follow up of 36 months (range 12-156) months, the five-year overall survival and disease free survival rate were 74.6% and 60.3% respectively. Local and distant recurrences occurred respectively in 10 (6,8%) and 29 (20%) patients. Seventy-seven percent (77%) of the patients were highly satisfied with this technique and only one patient was unsatisfied. However, none of them accepted the conversion to an abdominal colostomy neither would have changed PCPC for an iliac colostomy at first intent. CONCLUSION: This study showed that pseudocontinent perineal colostomy is a safe and reliable pelvic reconstruction technique after abdominoperineal resection for low rectal adenocarcinoma. It provides high degree of patient satisfaction without compromising oncological results. It is a good option in selected patients, especially in Muslim countries.

Hydatid cyst of the psoas: case report and review of literature.

Hydatid disease (HD) is a parasitic pathology that remains endemic in Morocco and poses a public health problem. Its location in the psoas muscle is a rare entity. We report this case highlighting the diagnostic challenges, therapeutic modalities as well as prognosis. Case Presentation: We report the case of a 40-year-old male patient without a notable pathological history, referred to our department for management of a paravertebral hydatid cyst mass with fluid density and scalloping on the verteral body on thoraco-abdomino-pelvic computed tomography scan. After a pretherapeutic assessment, the patient underwent an exploratory laparotomy which objective a deep right laterovertebral mass. The patient underwent a resection of the protruding dome, lavage using hydrogen peroxide and drainage of the residual cavity. The patient recovered well and was discharged 5 days later on albendazole 10 mg/kg/d. The anatomopathological analysis of the specimen confirmed the diagnosis of hydatid cyst. The evolution was satisfactory for the patient with a recall of 6 months. Discussion: Hydatidosis is an anthropozonosis caused by the larval form of Echinococcus granulosus. It can reach the psoas muscle by great circulation. The diagnosis of HD in the psoas is often difficult. Biology and imaging confirm the diagnosis and the standard treatment remains surgery. Conclusion: HD of the psoas is a rare entity. The diagnosis is based on biology and imaging, and radical treatment is surgery.

An unusual presentation of ovarian carcinoma with supraclavicular lymph node and colorectal metastases leading to spontaneous rectovaginal fistula.

INTRODUCTION AND IMPORTANCE: Secondary metastases to the rectum from primary ovarian cancer are a rare entity and their diagnosis and management are challenging. In this report, we discuss the findings of the case of metastatic ovarian cancer to supraclavicular lymph nodes and the rectum complicated with rectovaginal fistula. CASE PRESENTATION: A 68-year-old woman was admitted for abdominal pain with rectal bleeding. Pelvic examination revealed a left latero-uterine mass. Abdominal-pelvic CT scan showed a tumor mass on the left ovary. A cytoreductive surgery and resection of a non-imaged rectal nodule identified during surgery were performed. The tumor specimens including the rectal metastasis were immunohistochemically confirming a metastatic ovarian cancer using CK7, WT1 and CK20. The patient received chemotherapy and had complete remission. However, she had a recto-vaginal fistula confirmed by imaging and had developed right supraclavicular lymphadenopathy from ovarian cancer later. CLINICAL DISCUSSION: The dissemination of ovarian cancer in the digestive tract can be frequently, through direct invasion, abdominal implantation and lymphatic system. Unusually, ovarian cancer cells may spread to supra-clavicular nodes, because of the connection of the two diaphragmatic stages allowing the lymph flows through the lymphatic vessels. Moreover, rectovaginal fistula is an uncommon complication which can be seen spontaneously or due to certain patient's features. CONCLUSION: In advanced ovarian carcinoma, it is required to properly assess the digestive tract during surgery because imaging can miss metastatic lesions such as our case. The use of immunohistochemistry is recommended to differentiate between primary ovarian carcinoma and secondary metastasis.

A giant primary vaginal sarcoma: A report case and literature review.

Primary vaginal sarcoma is a rare disease entity, reported in less than 3% of cases of vaginal cancers. We report the observation of a patient treated at the regional oncology center of Oujda in Morocco who is presented with a non-metastatic primary vaginal leiomyosarcoma of 20 cm. The treatment consisted of neoadjuvant chemotherapy, followed by hemostatic surgery with tumor resection limits, reinforced by radiotherapy and then a surgical resection with a tumor resection taking away the infiltrated part of the anterior face of the lower rectum and the realization of a left iliac colostomy whose resected tumor part limits were healthy. At present, the patient is 4 years of follow-up without locoregional or distant recurrence.

Radiation-induced angiosarcoma of the breast: a case report.

Introduction: Breast angiosarcoma is a rare, aggressive tumour affecting adult women. It can occur in two forms, primary form and secondary forms or radiation-induced breast angiosarcoma affecting patients with history of breast or chest radiotherapy. Case presentation: The authors report a new case of breast angiosarcoma in 52-year-old women, with history of invasive ductal carcinoma, and reporting a discoloration of her breast skin. The patient did undergo a mastectomy of right breast and adjuvant chemotherapy. Conclusion/discussion: Surgery with total excision associated or not to adjuvant chemotherapy remains the treatment of choice in breast angiosarcoma.

A rare association of Vagus Nerve Schwannoma and Pheochromocytoma: A case report.

Vagus nerve schwannoma is a very rare benign nerve tumor. Pheochromocytoma is a rare, mostly benign tumor of the adrenal medulla with a large clinical spectrum. Their association is uncommon. The management of both tumors depends solely on surgery. The surgery of vagal schwannomas is particularly challenging considering the anatomical compositions of the area. Here, we report a case of a 76 year-old patient with cervical vagal schwannoma and benign pheochromocytoma association. We discuss the diagnosis and the surgical management of these tumors.

A Rare Case Report of Trichilemmal Carcinoma.

Trichilemmal carcinoma (TC) is a rare skin malignant tumor with pillar differentiation. TC presents along with other malignant hair follicle tumors and accounts for only 1% of all adnexal carcinomas. TC usually occurs on sun-exposed skin in elderly people, nevertheless, it can occur at any age. We report a case of trichilemmal cyst carcinoma in a 54-year-old woman presenting with an increasing occipital cyst. A histological examination confirmed the diagnosis and a large excision was performed. Despite the absence of a well-defined consensus on the management of TC, surgical excision with adequate margins seems to be safe in the absence of metastatic lesions. However, in the case of second localization, chemotherapy could be initiated, but again, in this case, no consensus on the appropriate protocols exists.

Acute hepatitis with portal and mesenteric vein thrombosis revealing SARS-CoV-2 infection: Case report and literature review.

Novel coronavirus disease 2019 (COVID-19) is a single-stranded RNA virus identified for the first time in Wuhan, China, and it unfurls quickly worldwide. The corona virus 2019 is a systemic disease which develops a prothrombotic environment, and has an extensive spectrum of clinical presentations in the gastrointestinal and hepatobiliary systems. Ischemic hepatitis (hypoxic hepatitis) is one potential mechanism behind lessened perfusion of the liver. The portal and mesenteric vein thrombosis are extremely rare complications and unusual main manifestations of COVID-19. We report the case of a patient presented acute hepatitis with portal and mesenteric vein thrombosis revealing a SARS-CoV-2 infection. In addition, we discuss the most characteristic elements of the Impact of COVID-19 on liver Injury, and the mechanisms of this damage and the formation of thrombus in portal and mesenteric vein.

Giant symptomatic adrenal myelolipoma: A case report.

INTRODUCTION: Adrenal myelolipomas are rare non-functioning benign tumors composed of adipose and hematopoietic tissues. Most AMLs are discovered incidentally and represent the second most common adrenal incidentaloma. CASE PRESENTATION: A 58-years-old female patient, obese with a history of diabetes and blood hypertension presented with complaints of pain in the left flank. Abdominopelvic computed tomography showed a giant well-defined mass of the left adrenal gland with fat density suggesting adrenal myelolipoma. The patient underwent open left adrenalectomy. The pathological study confirmed the diagnosis of adrenal myelolipoma. DISCUSSION: Most AMLs are asymptomatic, remain stable in size, or grow slowly. Mass effect symptoms and spontaneous rupture are observed more in larger AMLs. The most common symptoms observed are abdominal discomfort/pain, hypochondrial pain, and flank pain. Most of the AMLs are discovered incidentally and the radiological features are accurate in diagnosing AML in up to 90% of the cases, CT is more sensitive for detection than other imaging modalities. The open surgery approach is the standard treatment of choice for giant AML (>10cm) while the minimally invasive approach has been used in only a few cases. CONCLUSION: The therapeutic management is discussed on a case-by-case basis. Surgical treatment is indicated for larger, symptomatic, or rapidly growing AMLs. Meanwhile smaller and asymptomatic AMLs are managed conservatively.

Gastrointestinal stromal tumor in the fourth portion of the duodenum does not express the CD117: A case report.

Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract. Presentation of case: 54-year-old woman with a history of phyllodes tumor of the left breast he patient was admitted to our hospital for management of retroperitoneal soft tissue tumor, attached to the fourth portion of the duodenum, opposite the head of the pancreas. The patient underwent a large excision of the tumor, the duodenojejunal flexure, and the third and fourth portions of the duodenum along with the head of the pancreas. The histopathological examination confirmed the presence of a spindlecell mesenchymal proliferation. These cells do not express CD117, but they express DOG1. A PDGFRA mutation was identified later. The final diagnosis was duodenal GIST. Discussion: few cases of GIST in the fourth portion of the duodenum had been reported in the literature. PDGFRA mutation is identified as GISTs tumorigenesis to 15% of cases, and the diagnosis of GISTs is not based solely on the expression of the protein Kit. Conclusion: the molecular biology examinations are very helpful in the direction of the correct diagnosis in case of negative staining for CD117.

Primary adrenal mature teratoma: A rare case report and review of literature.

Introduction: Teratoma is a germ cell tumor, deriving from totipotent cells. Teratomas usually occur in gonads and are rarely extra-gonadal. The adrenal location is exceptional. Only few cases of primary adrenal teratomas have been reported in literature, mainly in young patients. Case report: We report the case of a 56-year-old female patient who presented with pyrosis, dyspepsia and abdominal pain that was evolving for 5 months. The abdominal computed tomography revealed a voluminous mass of the right adrenal gland and the hormonal evaluation was normal. The patient underwent an open transperitoneal adrenalectomy and the histopathological examination of the specimen confirmed the diagnosis of mature teratoma. Discussion: Adrenal teratomas are commonly asymptomatic and their diagnosis depends mostly on radiologic findings. Malignant transformation is very rare. Surgical excision is the mainstay of treatment with a good prognosis. Conclusion: Open surgery should always be considered in large and adhering teratoma tumors of the adrenal gland. Some pathologic features and tumorigenesis of adrenal teratomas are not entirely elucidated, thus the importance of larger studies in order to comprehend this pathological entity.

Audit of laparoscopic surgery for colon cancer in Morocco: A report of the results of a prospective multicentre cohort study.

Background: Little data is available about colon laparoscopic surgery in low mid-income countries. The aim of this study was to audit the status and results of laparoscopic colon cancer surgery in Morocco. Patients and methods: This was a prospective study performed at 4 academic departments in Morocco between January 1, 2018, and March 31, 2020. All adult patients who underwent elective right or left colonic resection for colon adenocarcinoma were included. The main outcomes were the rate of laparoscopic surgery (LS) and the comparison of its short-term outcomes with open surgery (OS). Results: Among 121 patients included, 52 (43%) underwent laparoscopic resection (0-49.3%). Five surgeons (29%) performed at least one laparoscopic resection. There were more left colectomies in the laparoscopic group (71.2% vs. 39.1%. p = 0.0004), and more extended resections (23.1% vs. 40.6%. p = 0.043) and T4 stage (19% vs. 37.5%. p = 0.037) in the open group. There were no differences in 90-days overall and serious complications. OS patients had significantly more harvested lymph nodes (14 vs. 18. P = 0.007) and higher median surgical margins (6 cm vs. 9 cm. P = 0.003) than LS patients. Conclusions: LS for colon cancer in Morocco is performed by few surgeons, who apply strict patient selection for laparoscopic cases. It was associated with lower quality resections compared to open surgery. There are still many challenges requiring more focus on training, certification, centralization and standardisation of care across the nation.

Retroperitoneal Schwannoma: Two Rare Case Reports.

Schwannomas are neuroectodermal tumors that rarely occur in the retroperitoneal space. We report two cases of patients who presented with abdominal pain. Radiological findings revealed a retroperitoneal mass in both cases. Both patients underwent complete surgical excision with an uneventful postoperative course. The histopathological study confirmed the nature of schwannoma. Complete surgical excision remains the gold standard for the management of these tumors. The preoperative diagnosis is usually difficult; however, the definitive diagnosis is made upon histopathological examination.