[A rare case of recurrent myocarditis revealing pheochromocytoma]. / Un rare cas de myocardite récurrente révélant un phéochromocytome.

Pheochromocytoma is a rare neuroendocrine tumor characterized by overproduction of catecholamines. The overproduction of catecholamines leads to cardiac remodeling which manifests in several forms ranging from Takotsubo to dilated cardiomyopathy. Studies suggest that pheochromocytoma-induced cardiomyopathy can take various forms depending on the duration of catecholamine exposure. Myocarditis is a fairly rare presentation of cardiac manifestations of pheochromocytoma which are mainly dominated by Takotsubo and dilated cardiomyopathies. We report a rare case of recurrent myocarditis in a young 37-year-old patient revealing the diagnosis of adrenal pheochromocytoma. Through this case and through a review of the literature we will take stock of the epidemiology of cardiac involvement in pheochromocytoma, mainly cardiomyopathies, and we will take stock of the value of diagnosis and early management in improving the prognosis of patients.

The Impact of a Multivisceral Resection and Adjuvant Therapy in Locally Advanced Colon Cancer.

BACKGROUND: Multivisceral resection for locally advanced colon cancer is mandatory to achieve complete tumor resection. We aimed to determine if local multivisceral resections (LMR) for pT4 and pT3 tumors impact perioperative and long-term oncological outcomes. METHODS: All stage II or III colon cancer patients who had surgery between 2004 and 2014 were identified. We analyzed patients with non-multivisceral resections (NMR) for pT4 tumors vs. pT4-LMR. In addition, outcomes were compared to both NMR and LMR pT3 patients. RESULTS: LMR was performed in 55 (29.7%) of all patients with pT4 tumors and in 48 (8.9%) of all patients with pT3 tumors. The most commonly involved areas of extension were the abdominal wall and the small intestine. Transverse colon cancer was correlated with LMR. Morbidity rates were comparable between NMR and LMR, with the exception of higher rates of blood transfusion and postoperative ileus. Over one third of all pT4-NMR patients developed recurrent disease, which was higher compared to all other groups. Subsequently, overall and disease-specific survival, as well as disease-free survival (DFS), was worse for pT4-NMR, even after adjustment for pTN-staging, adjuvant therapy, and R0 resection. Furthermore, when analyzing only curative resections, radial margin < 1 cm along with nodal disease was independent predictor for worse DFS. Long-term outcomes were comparable between pT4-LMR and pT3 patients. CONCLUSIONS: Multivisceral resection for locally advanced colon cancer preserves long-term oncological outcomes without increased postoperative morbidity. Moreover, LMR in pT3 tumors does not contribute to postoperative morbidity. Our study underlines the feasibility and importance of performing LMR when locally advanced cancer is suspected.

[An unusual cause of hypertension]. / Une cause inhabituelle d'hypertension artérielle.

Secondary hypertension is relatively rare. Its etiologies are essentially renal or endocrine. The adrenocortical carcinoma, a rare malignant tumor, is one of the most exceptional causes of hypertension. We report the case of a young woman presenting with hypertension and ascites of great abundance. She had hypokalemia, hepatic cytolysis and cholestasis. Thoracic, abdominal and pelvic CT scans showed adrenal mass, liver and lung nodules, osteolytic lesions of the sacrum. The biopsy of liver nodules confirmed the diagnosis of a metastatic malignant adrenocortical carcinoma. The hormonal investigations revealed cortisol and testosterone hypersecretion. The evolution was quickly fatal, the patient passed out a few days after her hospitalization.

[Bilateral pulmonary embolism revealing granulomatosis with polyangiitis]. / Embolie pulmonaire bilatérale révélant une granulomatose avec polyangéite.

Granulomatosis with polyangiitis is a systemic small-vessels vasculitis. It is characterized by a granulomatous inflammatory reaction around the arterial wall and is, in most cases, accompanied by the presence of cytoplasmic - type anti-neutrophil cytoplasmic antibodies (ANCA) with anti-proteinase 3 specificity. The predominant clinical presentation is frequent involvement of the upper and lower respiratory tract and the kidneys. This vasculitis is rarely revealed by pulmonary embolism. We report the observation of a 47-year-old male who presented with acute dyspnea. Chest X-ray disclosed an excavated opacity. Pulmonary angioscan revealed bilateral pulmonary embolism. The absence of clinical improvement after anticoagulant therapy with antibiotics and the presence of extra-respiratory signs led to the suspicion of a systemic inflammatory disease. The etiological investigations concluded with the diagnosis of granulomatosis with polyangiitis.

The negative impact of understaging rectal cancer patients.

BACKGROUND: Neo-adjuvant chemoradiation followed by surgery and adjuvant therapy is standard treatment of clinical node positive rectal cancer. Understaging leads to delay in treatment with possible detrimental results. This study analyses effects of understaging stage III rectal cancer on long-term outcomes. METHODS: A consecutive series of patients, operated on in MGH between 2004 and 2015 was included. Outcomes of non-neoadjuvantly treated clinical stage I patients who turned out to have pathological stage III disease and neoadjuvantly treated clinical stage III patients were retrospectively reviewed. The latter group was subdivided into patients who had persistent nodal disease (ypN+) and patients without positive lymph nodes after neoadjuvant treatment (ypN0). RESULTS: Of the 204 included patients, 30 had unexpected nodal disease on pathology. Clinical stage I-patients had higher rates of local recurrence, and rectal cancer and overall mortality than ypN0-patients. CONCLUSION: Understaging stage III rectal cancer led to poorer oncologic outcomes, when compared to patients without positive lymph nodes on pathology after neoadjuvant. Future research should focus on identifying patients with treatment susceptible lymph node involvement.

Clinical and molecular findings in a Moroccan family with Jervell and Lange-Nielsen syndrome: a case report.

BACKGROUND: Jervell and Lange-Nielsen syndrome (Online Mendelian Inheritance in Man 220400) is a rare autosomal recessive cardioauditory ion channel disorder that affects 1/200,000 to 1/1,000,000 children. It is characterized by congenital profound bilateral sensorineural hearing loss, a long QT interval, ventricular tachyarrhythmias, and episodes of torsade de pointes on an electrocardiogram. Cardiac symptoms arise mostly in early childhood and consist of syncopal episodes during periods of stress, exercise, or fright and are associated with a high risk of sudden cardiac death. Jervell and Lange-Nielsen syndrome is caused by homozygous or compound heterozygous mutations in KCNQ1 on 11p15.5 or KCNE1 on 1q22.1-q22.2. CASE PRESENTATION: We report the case of a 10-year-old Moroccan boy with congenital hearing loss and severely prolonged QT interval who presented with multiple episodes of syncope. His parents are first-degree cousins. We performed Sanger sequencing and identified a homozygous variant in KCNQ1 (c.1343dupC, p.Glu449Argfs*14). CONCLUSIONS: The identification of the genetic substrate in this patient confirmed the clinical diagnosis of Jervell and Lange-Nielsen syndrome and allowed us to provide him with appropriate management and genetic counseling to his family. In addition, this finding contributes to our understanding of genetic disease in the Moroccan population.

The Impact of Pathologic Complete Response in Patients with Neoadjuvantly Treated Locally Advanced Rectal Cancer-a Large Single-Center Experience.

Small cohort studies demonstrated better oncologic outcomes for patients with pathologic complete response (PathCR) after neoadjuvant treatment for locally advanced rectal cancer. This study reviews long-term outcomes of a large cohort of clinically stage II/III rectal cancer patients who received neoadjuvant chemoradiation and surgery. This is a retrospective analysis of a single-center cohort, including all clinical stage II/III rectal cancer patients who received neoadjuvant chemoradiation and surgery between 2004 and 2014 (n = 271). Cox regressions were done to assess the influence of PathCR on recurrence-free survival (RFS) and overall survival (OS), adjusting for postoperative chemotherapy, clinical AJCC staging, comorbidity, and age where appropriate. PathCR patients had significantly lower distant recurrence rates (4 vs. 15.8%; P = 0.028) and lower disease-specific mortality rates (0 vs. 8.1%; P = 0.052), compared to patients with residual disease. PathCR was associated with longer RFS (HR, 5.6 [95% CI 1.3-23.1] P = 0.018) and longer OS (HR, 3.4 [1.31-10.0] P = 0.014) compared to having pathological residual disease. This large single-center study shows that patients with PathCR have significant longer RFS and OS than patients with residual disease on pathology after neoadjuvant chemoradiation.

[Vertebral primary malignant germ cell tumor of thoracic spine: an exceptional observation]. / Tumeur germinale primitive à localisation vertébrale dorsale chez un adulte : une observation exceptionnelle.

Vertebral primary malignant germ cell tumors are rarely located in thoracic spine. We report the case of a 44-year-old female, in which a symptomatology including dorsal rachidial pain, intercostal neuralgia, straight and transit disorder revealed a thoracic vertebral primary malignant germ cell tumor. The sole location of a vertebral primary malignant germ cell tumor in adult, exceptionally reported in the literature, prompted us to relate this observation.

[Subaortic stenosis with muscularization of the anterior mitral valve leaflet: two cases]. / Diaphragme sous-aortique par muscularisation de grande valve mitrale: à propos de deux cas.

Subaortic stenosis was considered for a long time as a congenital anomaly, but it is considered now as an acquired form of obstacle to the left ventricle ejection. It constitutes 8 to 20% of the causes of obstacle left ventricle. Ventricular septal defect and aortic coarctation are the most frequent anomalies associated with the subaortic stenosis. The anomalies of mitral valve and especially muscularization of the anterior mitral valve leaflet remain very rare and underestimated. The diagnosis is made by the echocardiography and must be systematically looked for because its misunderstanding in preoperative can be at the origin of recurrences. We report in this work two cases of muscularization of the anterior mitral valve leaflet associated to subaortic stenosis. Through these cases and through a review of the literature, we are going to put the point on this rare anomaly.

[Vascular complications of infective endocarditis: a retrospective analysis of 18 cases]. / Les complications vasculaires de l'endocardite infectieuse.

OBJECTIVES: Among 82 patients hospitalized for infective endocarditis between June 1995 and June 2001 at the cardiology B unit of the Rabat University Hospital Morocco, 18 (22%) had one or more vascular complications. We present here a retrospective analysis. PATIENTS AND METHODS: The cohort included 12 men and 6 women, mean age 22 years. Infective endocarditis had grafted on a pre-existing cardiopathy among 17 patients: rheumatic heart disease (n=14), mechanical prosthetic valve (n=2), congenital heart disease (n=1). RESULTS: For 12 patients, vascular disease was the only complication, 1 had two complications and 4 three complications. This gave 26 lesions: 11 neurological complications, 10 arterial diseases involving the limbs including 5 mycotic aneurysms, 2 acute myocardial infarcts, 2 splenic infarcts, and 1 recurrent septic pulmonary embolism. Vascular disease was the inaugural manifestation in 9 patients and 54% of the complications occurred before the end of the second week of antibiotic treatment. Blood cultures were positive in 7 patients (40%). Oral streptococcus was isolated in 5 cases, Gram-negative bacillus in 1 case and Staphylococcus aureus in 1. Echography revealed valvular vegetations in the 16 cases of infective endocarditis on native valves: mitral (n=9), aortic (n=5), mitroaortic (n=1), tricuspid (n=1). Short term outcome was marked by 4 deaths including 3 directly related to the vascular complication. DISCUSSION: We emphasize the variable and diverse features of vascular complications of infective endocarditis. Prevention and early diagnosis are essential to institute optimal management of infective endocarditis.