Intracranial meningeal melanocytoma associated with ipsilateral nevus of Ota. Case report

In this report, the authors review the case of a man with a neurocutaneous syndrome. He presented with an intracerebral melanocytoma associated with a blue nevus of the scalp; its location and its appearance during childhood supported the diagnosis of a nevus of Ota. Meningeal melanocytomas are increasingly being diagnosed, but remain rare. Primary meningeal malignant melanoma is the first differential diagnosis to eliminate. Despite their common embryonic origin. the association of a melanocytoma with a nevus of Ota is rare. A nevus of Ota exhibits the same melanocytic proliferation and affects the trigeminal nerve territory. An ocular effect is not always observed. In contrast to an ocular lesion, a nevus of Ota rarely transforms into a malignant melanoma. It is found only among caucasians. During 4 years of follow-up review after surgery, the patient remained asymptomatic. Other than antiepileptic therapy, he received no complementary treatment and cerebral imaging revealed no evidence of recurrence.     

Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone

The mechanisms by which aging induces muscle impairment are not well understood yet. We studied the impact of aging on Ca2+ homeostasis in the slow-twitch soleus and the fast-twitch extensor digitorum longus (EDL) muscles of aged rats by using the fura-2 fluorescent probe. In both muscles aging increases the resting cytosolic calcium concentration ([Ca2+]i). This effect was independent on calcium influx since a reduced resting permeability of sarcolemma to divalent cations was observed in aged muscles likely due to a reduced activity of leak channels. Importantly the effects of aging on resting [Ca2+]i, fiber diameter, mechanical threshold and sarcolemmal resting conductances were less pronounced in the soleus muscle, suggesting that muscle impairment may be less dependent on [Ca2+]i in the slow-twitch muscle. The treatment of aged rats with growth hormone restored the resting [Ca2+]i toward adult values in both muscles. Thus, an increase of resting [Ca2+]i may contribute to muscle weakness associated with aging and may be considered for developing new therapeutic strategies in the elderly.     

Therapeutic Efficacy of Combined Intraoperative Ablation and Resection for Colorectal Liver Metastases: An International,Multi-institutional Analysis

Background  

Only 10–25% of patients presenting with colorectal liver metastases (CRLM) are amenable to hepatic resection. By combining resection and ablation, the number of patients eligible for surgery can be expanded. We sought to determine the efficacy of combined resection and ablation for CRLM.     

When "abegg" is read and ("A, B, E, G, G") is not: a cortical stimulation study of musical score reading

OBJECT: To spare the cortical areas involved both in musical score reading and in language, the authors used a score reading task during direct cortical stimulation mapping in musicians undergoing operations for brain lesions. The organization of the cortical areas involved in language and score reading, respectively, was analyzed in relation with these surgical data. METHODS: Seven patients with brain lesions were tested using three language tasks and a score-reading task. Preoperatively, none of them had exhibited significant language or musical ability deficits, and all had a special interest in music. All were involved in professional or amateur musical activities. Interference in score reading was found in small cortical areas, mainly in the dominant parietal lobe and sometimes in the frontal gyri. During direct stimulation, interference was either language-specific (15 sites), common to language and score-reading tasks (18 sites), or specific to the score-reading task (four sites). Different patterns of score-reading interferences (score-reading arrest, semantic paraphasia) were observed, probably corresponding to different stages of score reading. Postoperatively, some patients showed transitory score-reading difficulties related to the surgical procedure. CONCLUSIONS: The cortical areas involved in score reading can occasionally be distinct from other language areas. This could explain differential word- and score-reading impairments sometimes observed in musicians with brain lesions. Brain mapping for neurosurgical procedures in musicians should ideally be performed using a score-reading task in addition to standard language tasks, especially for mapping in the dominant parietal lobe.     

Cystic fibrosis at the Reunion Island (France): spectrum of mutations and genotype-phenotype for the Y122X mutation.

BACKGROUND: The Reunion Island is a French administrative department located in the Indian Ocean between the islands of Madagascar and Mauritius. Its population is known to be at a high risk of cystic fibrosis (CF). METHODS: Data concerning all CF patients born at the Reunion Island was extracted from the French CF Registry. Twenty-eight DeltaF508/DeltaF508, 17 Y122X/DeltaF508, and 11 Y122X/Y122X were included in a genotype-phenotype study. RESULTS: The detection rate of the CFTR mutations was 83% among the CF patients born at the Reunion Island. Three CFTR mutations accounted for 75% of the detected CF alleles at the Reunion Island (DeltaF508, Y122X, and 3120 + 1G-->A.). The DeltaF508/DeltaF508, DeltaF508/Y122X, and Y122X/Y122X genotypes accounted for 60.2% of the CF patients. Patients carrying at least one Y122X mutation were pancreatic insufficient, had high sweat chloride values and significantly lower anthropometric measures. The mean anthropometric values in all three groups were lower that in the whole CF population followed in "continental" France. This may reflect the poor compliance and even the refusal of treatment noted by the clinicians. CONCLUSIONS: The distribution of CFTR mutations could be explained by the history of the Reunion Island: admixture of French settlers, African and Asian populations, founder effect and isolation followed by genetic drift. The Y122X allele appears to be associated with a severe phenotype.     

Prognostic Value of BRAF V600 Mutations in Melanoma Patients After Resection of Metastatic Lymph Nodes

Purpose

BRAF ^V600 mutations are frequent in melanomas, and BRAF^V600-targeted therapy have dramatic, but often transitory, efficacy in stage IV patients. Prognosis of patients with American Joint Committee on Cancer (AJCC) stage III melanoma is heterogeneous. We aimed to determine the overall survival (OS) of stage III patients with a nodal deposit of ??2?mm according to BRAF ^V600 mutations and other previously reported prognostic criteria.

Methods

This retrospective study included 105 consecutive patients with stage III cutaneous melanomas. Most patients underwent a prospective follow-up. BRAF ^V600 mutations were detected by sequencing and pyrosequencing of DNA in samples containing >60?% melanoma cells.

Results

BRAF mutations (p.V600E and p.V600K in 83 and 14?% of cases, respectively) were detected in 40?% of the patients. For patients with and without BRAF mutations, death occurred in 83.3 and 60.3?%, with a median OS of 1.4 and 2.8?years, respectively. Patient age, primary melanoma ulceration, number of invaded lymph nodes, AJCC staging at study entry, and BRAF status were linked to OS in the univariate analysis. The only characteristics associated with OS in the multivariate analysis were number of invaded lymph nodes (P?=?0.005, hazard ratio 2.2, 95?% confidence interval 1.3?C3.9) and BRAF status (P?=?0.005, hazard ratio 1.9, 95?% confidence interval 1.2?C3.1).

Conclusions

BRAF ^V600 status could be used to stage melanoma patients with nodal deposits. Our results may also help to plan adjuvant trials in these patients, for whom the low tumor load may induce longer efficacy of BRAF-targeted therapies.     

Medial pancreatectomy: a multi-institutional retrospective study of 53 patients by the French Pancreas Club

BACKGROUND: The results of medial pancreatectomy have been previously reported anecdotally. The purpose of the study was to provide short- and long-term results of MP in a large multicenter collective series. METHODS: From 1990 to 1998, 53 patients (mean age +/- SD = 49 +/- 15 years) underwent medial pancreatectomy for primary cystic neoplasms of pancreas (n = 19), endocrine neoplasms (n = 17), intraductal papillary mucinous neoplasms (IPMN) (n = 6), fibrotic stenosis of the Wirsung's duct (n = 4), or other benign (n = 4) or malignant (n = 3) diseases. The proximal (right) pancreatic remnant was sutured (n = 53), and the distal (left) remnant was either anastomosed to a jejunal loop (n = 26), to the stomach (n = 25), or oversewn (n = 2). Medial pancreatectomy was indicated in 3 patients (6%) because of failed enucleation, in 3 (6%) to prevent worsening of preexisting diabetes, or to prevent de novo diabetes in a patient with chronic pancreatitis, and deliberately in the 47 others. RESULTS: The length of the resected pancreas was 5.0+/- 2.2 cm (range, 2-15). One patient (2%) died from a pancreatic fistula and portal thrombosis. Three patients were reoperated on because of complications related to the left pancreas, which was partially or totally resected. Pancreatic fistula developed in 16 patients (30%). Mean delay for the return of oral feeding was related to the presence of a pancreatic fistula. At follow-up (median = 26 months, range, 12-131), 1 pancreatic recurrence and 1 de novo diabetes occurred in patients without IPMN. In patients with IPMN, the rates of pancreatic recurrence and diabetes were 40% (2/5), respectively. CONCLUSIONS: Medial pancreatectomy effectively preserves long-term endocrine function and is associated with a low risk of local recurrence, except in patients with IPMN. However, there is a high risk (30%) of PF after medial pancreatectomy.     

Paraplegia after routine cardiac surgery: a rare complication

Two cases of spinal cord infarction with clinical paraplegia are discussed in patients undergoing elective and uncomplicated cardiac procedures with cardiopulmonary bypass. In both cases, the primary etiology was likely peripheral embolization. Other possible etiologies are reviewed, previously reported cases are discussed, and possible solutions to avoid this devastating problem are addressed.     

Endovascular control of patent internal thoracic artery graft in aortic valve surgery

Aortic valve replacement in patients who underwent previous coronary artery bypass with a patent internal thoracic artery is often a challenge because of the risk of graft injury during dissection or difficulties to obtain optimum myocardial protection. Different approaches to myocardial protection or internal thoracic graft dissection and control have been described. Endovascular control of the internal thoracic graft by an angioplasty balloon catheter positioned in the operating room before the operation can be a safe and simple alternative. We report the case of a patient who underwent this technique for aortic valve replacement.