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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Colostomy orifice complications: a case report of a prolapsed colostomy with necrosis of the eviscerated greater omentum.

Evisceration and necrosis of the greater omentum at the site of a prolapsed colostomy is a rare situation. Considered an early stoma complication, it often occurs during the first month after surgery. We report the observation and our attitude to such a situation in a 56-year-old patient who underwent initial surgery for a locally advanced rectal adenocarcinoma after receiving neoadjuvant radio-chemotherapy. A loop colostomy for decompression was performed due to large bowel obstruction. On the 10th day after surgery, he was readmitted for an oedematous prolapsed colostomy and a necrotic end of the greater omentum, which eviscerated through the colostomic hole, secondary to severe ascites. Emergency re-intervention involving resection of the prolapsed stoma with the necrotic segment of the omentum was performed. The three factors associated with the development of this rare peri-colostomy complication were: emergency surgery, locally advanced rectal tumor, and increased intra-abdominal pressure.

Hepatic epithelioid hemangioendothelioma simulating liver metastasis: A case report.

INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular origin. Liver involvement is often multinodular simulating metastases. Herein, we report a rare case of HEHE mimicking liver metastases in a female patient. CASE PRESENTATION: A 43-years-old female patient, presented with complaints of pain in the right upper quadrant. Laboratory tests were all within the normal range. Abdominal ultrasound revealed multiple heterogeneous hypoechoic liver lesions. A thoracic and abdominopelvic computed tomography showed bilateral pulmonary micronodules with multiple hypodense hepatic nodules involving both lobes. Percutaneous ultrasound-guided biopsy with pathological study and immunohistochemistry staining revealed the diagnosis of hepatic epithelioid hemangioendothelioma. DISCUSSION: HEHE usually involves both liver lobes with three radiological presentations: single nodular, multiple nodular, or diffuse types, the diffuse type reflects an advanced stage. 3/4 of the cases are initially misdiagnosed as liver metastases or primary liver tumors. The pathological study with the immunochemistry stainings confirms the diagnosis. There is no standard treatment for HEHE due to its rarity and lack of prospective randomized studies. CONCLUSION: HEHE is a rare tumor of vascular origin of unknown etiology with malignant potential and unpredictable course. The therapeutic management of this rare condition is not codified and is discussed on a case-by-case basis. Surgical treatment remains the best option with an excellent outcome.

Pancreatic schwannoma- CT and MRI findings: A rare case report and review of literature.

INTRODUCTION: Pancreatic schwannoma (PS) is an extremely rare benign tumor. Here we describe the Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) results of PS in a 59 years old woman, as well as a review of the literature. CASE PRESENTATION: A 59-year-old woman consulted for atypical epigastralgia without fatigue, weight loss or fever. CT scan and MRI showed a 35 mm inhomogeneous lesion with well-defined margins located in the pancreas head. The diagnosis of pancreatic tumor was made. The pathologic examination of the biopsied mass yielded a diagnosis of pancreatic schwannoma. CLINICAL DISCUSSION: On CT scans, almost all benign PS are well-defined cystic or low-density masses. MRI is helpful in characterizing their typical encapsulation. CONCLUSION: The detection of pancreatic schwannoma is extremely rare. Although multiple imaging modalities are currently available, it is challenging to make an accurate diagnosis before operation.

Infrarenal Vena Cava Leiomyosarcoma Treated With Surgical Resection and Vascular Reconstruction.

Leiomyosarcoma of the inferior vena cava is a rare malignant tumor with a poor prognosis. We report a case of a 39-year-old woman admitted for a surgical resection of a retroperitoneal mass revealed by pain localised in the right lumbar fossa. Computed tomography of the abdomen revealed a heterogeneous retroperitoneal mass compressing the inferior vena cava. Surgical resection was performed with the reconstruction of the inferior vena cava using a Dacron prosthesis, the diagnosis of vessel wall leiomyosarcoma was revealed by histopathology. Surgical resection with clear margins remains the only treatment offering the best survival rate. The complex nature of the surgery of those tumors is a major therapeutic challenge for surgeons.

Giant Gallbladder Revealed by Chronic Cholecystitis Gallstone: A Case Report and Review of the Literature.

We report here an extremely rare case of giant gallbladder and discuss diagnostic circumstances and different ethiopathogenic theories. A 53-year-old woman presented acute right hypochondrium pain. Ultrasonography showed a huge cystic mass with gallstones and a CT scan confirmed the diagnosis of giant gallbladder. Cholecystectomy was performed. Outcomes were uneventful and histopathological examination of the specimen confirmed the presence of chronic cholecystitis. Enlargement of the gallbladder is related to biliary retention. This enlargement is favored by the slow evolution of malignant pathologies. However, some benign situations have been reported in the literature, and giant gallbladder can occur in a benign situation even if its ethiopathogeny is not so clear.

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.