Diagnostic performance of [(18)F]fluorodeoxyglucose positron emission tomography-computed tomography in cyst infection in patients with autosomal dominant polycystic kidney disease.

Cyst infection is a common complication of autosomal dominant polycystic kidney disease (ADPKD). Diagnosis is challenging with standard imaging techniques. We aimed to evaluate the diagnostic performance of [(18)F]fluorodeoxyglucose positron emission tomography-computed tomography (18-FDG PET-CT) for the diagnosis of cyst infections among ADPKD patients, in comparison with computed tomography (CT) and magnetic resonance imaging (MRI). All APKD patients who underwent 18-FDG PET-CT for suspected cyst infection between 2006 and 2013 in a French teaching hospital were included. Diagnosis of cyst infection was retained a posteriori on an index of clinical suspicion. 18-FDG PET-CT findings were was considered to be positive in cases of cyst wall hypermetabolism. CT or MRI findings were were considered to be positive in cases of cyst wall thickening (and enhancement if contrast medium was injected) and infiltration of the adjacent fat. A control group of ADPKD patients with 18-FDG PET-CT performed for other reasons was included. Thirty-two 18-FDG PET-CT scans were performed in 24 ADPKD patients with suspected cyst infection. A diagnosis of cyst infection was retained in 18 of 32 cases: 14 with positive 18-FDG PET-CT findings, and four false negatives. There were no false positives and no hypermetabolism of cyst walls in nine ADPKD control patients. 18-FDG PET-CT had a sensitivity of 77%, a specificity of 100%, and a negative predictive value of 77%. 18-FDG PET-CT allowed a differential diagnosis in three patients. In contrast, CT had a sensitivity of 7% and a negative predictive value of 35% (p <0.001 vs. 18-FDG PET-CT). Only eight MRI scans were performed. The diagnostic performance of 18-FDG PET-CT is superior to that of CT in cyst infections, for comparable radiation doses and with no injection of nephrotoxic contrast medium, in ADPKD patients.

Prognostic factors for the recurrence of myxoid liposarcoma: 20 cases with up to 8 years follow-up.

INTRODUCTION: Myxoid liposarcomas (MLS) are the second most common type of liposarcoma. Although some MRI findings are distinctively characteristics of MLS, the diagnosis can be tricky in tumors with a large portion of round cells (RC). Known predictors of an unfavorable outcome include age, tumor size, high RC content and positive resection margins. The goal of this retrospective study was to define prognostic factors for recurrence, with special emphasis on the percentage of RCs and medical care provided in a non-specialized center. PATIENTS AND METHODS: Twenty patients (11 women, 9 men) with a mean age of 44.3 years (18-73) were reviewed after a mean of 55.9 months. Six of these patients had been operated at a non-specialized center. The diagnostic MRI was read by a specialized radiologist and the resection procedures performed by two specialized surgeons. Tumors were labeled as either "pure myxoid liposarcoma" or "myxoid/round-cell liposarcoma". The local recurrence-free survival rate and mortality rate were calculated. RESULTS: Fifteen patients had undergone an MRI during the initial assessment. The typical MRI findings of MLS were present in four of them. The MRI suggested a non-specific lesion in the other 11 patients. After correlation with pathology findings, these tumors contained more than 5% round cells. The fourteen patients treated at our facility had undergone a biopsy, while none of the ones treated outside did. Five patients had R0 resection margins and 15 had R1 margins. Prognostic factors for recurrence consisted of age, tumor size >10 cm, R1 resection margins, FNCLCC grade 2+R1 margins, medical care at a non-specialized center, and >5% round cells. There were eight local recurrences and three metastases (15%). Two patients died (90% overall survival rate). DISCUSSION: The risk of local recurrence was 3.86 times greater in this study when the tumor contained more than 5% RCs, which is consistent with published data. The MLS diagnosis was made only four times based on the initial MRI because misleading nature of high RC tumors. R1 resection margins are a risk factor for local recurrence. However, cases with R1 margins have a recurrence rate that is similar to R0 cases when the surgery is performed at a specialized cancer center. Treatment of MLS in a non-specialized center is a key negative prognostic factor. The reported rate of metastasis varies. Atypical extrapulmonary localizations are common, and often multifocal. MRI has been shown to be superior at detecting secondary lesions and some have suggested that a full-body MRI should be performed. CONCLUSION: Prognostic factors for the recurrence of myxoid liposarcomas have been identified. MRI analysis is not definitive and must be supplemented by a biopsy.

[Giant renal angiomyolipoma with right heart failure]. / Angiomyolipome rénal géant compliqué d'insuffisance cardiaque droite.

We report the case of a 63-year-old woman presenting a 26cm right renal angiomyolipoma with intratumoral arteriovenous fistula responsible for a high-output right heart failure. A radical surgical treatment after preoperative embolization allowed rapid improvement of cardiac symptoms with an uneventful postoperative course.

[Pathological features of radiofrequency ablation (RFA) renal scar CT-imaging in a swine model]. / Cicatrice de radiofréquence rénale : corrélations anatomopathologiques-tomodensitométriques chez le porc. Applications pratiques pour le suivi en imagerie.

PURPOSE: To analyze the changes in vicinal kidney parenchyma after percutaneous RFA. MATERIALS AND METHODS: [corrected] Twenty-four CT-guided RFA procedures were performed on six pigs using 2 cm LeVeen coaxial needles. We studied volume, morphology, cavitation and enhancement of the ablation zones (AZ) before and after the procedure on contrast-injected CT-scans. The kidneys were removed four weeks later and studied in the path lab. RESULTS: All the procedures were successfully completed. Four weeks later, the CT-scans showed AZ that were either clearly circumscribed or with unclear borders, heterogenous areas associating necrosis and infarct tissue and mesenchyma showing a process of apoptosis around the edges. A treatment considered as incomplete on the CT-scan (presenting as an enhancement) was always associated with necrosis on the histology slides, although the necrotic areas behaved in various different ways on the CT-scan after injection of contrast medium: an enhancement of more than 10 HU did not mean that no necrotic tissue was present. CONCLUSION: RFA causes heterogenous tissue changes, associating necrotic and ischemic zones and an apoptotic reaction. The mechanisms of these changes and their therapeutic significance should be studied. CT-scans performed immediately after RFA procedure and one month later are not predictive of the efficacy of the treatment because an enhancement of the AZ does not mean that it is not necrotic. The value of a CT-scan performed one month after the procedure is debatable, because the tissue remodeling that occurs in the kidneys is not definitive at this time-point.

Desmoplastic malignant melanoma: a study of ten cases and status of BRAF mutation.

BACKGROUND: Desmoplastic malignant melanoma (DM) is a rare variant of melanoma. BRAF gene mutations have been poorly explored in this entity. OBJECTIVE: To detect BRAF gene mutation in a series of DM. METHODS: This is a single-center retrospective study of ten patients with DM, with a biomolecular analysis of BRAF mutation. RESULTS: The male:female ratio was 2.3:1, with a mean patient age of 66.5 years. Melanoma arose in the head and neck region in 3 cases. The mean tumor thickness was 7.97 mm, Clark level was IV or V in all cases. Six melanomas were of the pure DM variant. Three patients had at least one local recurrence, two had regional node metastases, and two experienced systemic metastases which they died of (average follow-up 34.1 months). A V600E BRAF mutation was detected in only one patient. CONCLUSION: BRAF mutation seems to be a rare event in DM contrary to other melanoma variants.

[Proxy lymphomatoid contact dermatitis]. / Dermite de contact lymphomatoïde par procuration.

BACKGROUND: Lymphomatoid contact dermatitis is a delayed hypersensitivity reaction predominantly featuring T-cell infiltration. We report a case mainly involving B-cell infiltration associated with eczema and resulting from an indirect proxy contact with an allergen in a conjugal setting. PATIENTS AND METHODS: A 32-year-old man had an infiltrated cutaneous lesion on the interior aspect of the left arm with eczematous lesions of the waist and the anterior aspect of the left arm which were present for 6 months. All of these lesions were unresponsive to strong local steroids. Biopsy of the infiltrated lesion showed a dense lymphoid dermal infiltration chiefly comprising B cells. Histological examination of a waist lesion revealed chronic eczema. Patch testing was performed with the ECDRG test battery. A PPD (paraphenylenediamine) patch test was the only examination yielding a positive result. Detailed questioning revealed use of a hair dye by the patient's spouse and withdrawal of the allergen resulted in complete remission of the two types of lesion. DISCUSSION: This case highlights the ability of a single allergen to induce different lymphoid phenotypes. It also underscores the value of detailed questioning in allergology.

[Efficacy of infliximab in the treatment of follicular occlusion triad]. / Efficacité de l'infliximab dans le traitement d'une triade d'occlusion folliculaire.

BACKGROUND: Dermatological treatments for the follicular occlusion triad have only partial and transient efficacy. PATIENTS AND METHODS: A 20-year-old patient presented folliculitis of the scalp, acne and hidradenitis suppurativa, associated with spondyloarthritis. Treatment with infliximab for rheumatologic symptoms induced complete and lasting dermatological and rheumatological remission. COMMENTS: The efficacy of anti-TNF-alpha in follicular occlusion triad provided confirmation that infection is not at the heart of the aetiological process. However, efficacy data is still sparse and additional studies are required.

[Prognostic value of the LRINEC score (Laboratory Risk Indicator for Necrotizing Fasciitis) in soft tissue infections: a prospective study at Clermont-Ferrand University hospital]. / Valeur pronostique du score Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) dans les dermohypodermites infectieuses : étude prospective au CHU de Clermont-Ferrand.

BACKGROUND: The LRINEC score was developed in a retrospective study in order to distinguish necrotizing fasciitis from severe soft tissue infections using laboratory data. AIM: To evaluate the prognostic value of the LRINEC score in infectious cellulitis. PATIENTS AND METHODS: A prospective study was performed at the departments of infectious diseases and dermatology of the Clermont-Ferrand University Hospital. The three evaluation criteria were: time from initiation of antibiotics to regression of erythema, duration of fever and occurrence of complications (abscess, surgery, septic shock, necrotizing fasciitis, death, transfer to intensive care). Potential predictive variables were: LRINEC score>6 at admission, comorbidities, local appearance, clinical presentation and soft tissue ultrasound results. RESULTS: Fifty patients were included. The rate of complications was higher for patients with a LRINEC score>6 (54%) than for patients with a score<6 (12%, P=0.008). However, a LRINEC score>6 on admission was not significantly associated with increased duration of erythema or of fever. Prior lymphoedema was associated with a better prognosis. DISCUSSION: The LRINEC score may be a useful tool for the detection of complicated forms of soft tissue infections. Patients with a LRINEC score>6 on admission should be carefully evaluated (hospitalization, surgical assessment, close monitoring).

[Abdominal manifestations of Henoch-Schönlein purpura in adults. A retrospective study of 23 cases]. / Atteintes digestives du purpura rhumatoïde de l'adulte. Etude d'une série rétrospective de 23 patients.

INTRODUCTION: Gastrointestinal manifestations of Henoch-Schönlein purpura (HSP) in adults may be severe. Data about treatment are controversial and the outcome is seldom described. METHODS: Twenty-three patients with gastrointestinal manifestations of HSP (ACR criteria) were retrospectively studied. We proposed to use clinical and radiological (CT scan) severity scores to assess the usefulness and the efficacy of corticosteroid therapy. RESULTS: Age at onset ranged from 16 to 80 years (median 39). Gastrointestinal manifestations included abdominal pain (96%), vomiting (52%), gastrointestinal bleeding (39%), diarrhoea (13%) and intestinal obstruction (9%). Scores of disease clinical severity were calculated in 21 patients. Fourteen, three and four had severe, intermediate and mild disease, respectively. Ten patients in the group with severe clinical involvement underwent CT scan that showed severe radiological involvement (parietal thickening of several bowel loops of the same segment or several segments with ileal involvement). Eleven patients out of 14 with clinically severe disease were given corticosteroids. Two patients out of three with intermediate and three patients out of four with mild scores also received corticosteroids. The others received supportive care. In-patients with severe clinical scores, gastrointestinal symptoms improved within 2 days when they were given corticosteroids and within 12.3 days without corticosteroids (p<0.0002). No side effect was observed with steroid therapy. CONCLUSION: These results suggest that corticosteroids may reduce abdominal symptoms of HSP in adults with clinically severe disease. They are safe when CT scan is performed.

[Photopheresis: an alternative therapeutic approach in corticoresistant erosive oral lichen planus]. / La photophérèse: une alternative thérapeutique aux corticoïdes pour le lichen érosif muqueux corticorésistant.

BACKGROUND: The immunomodulatory effect of extracorporeal photochemotherapy (photopheresis) coupled with its efficacy in lymphocytic skin diseases provides a rationale for its use for erosive lichen planus. We report two cases of chronic oral erosive and corticoresistant lichen planus successfully treated with photopheresis. PATIENTS AND METHODS: Case 1. A 61-years-old man had erosive oral lichen planus for four years. Oral steroids were contra-indicated due to iatrogenic pancreatitis. After nine photopheresis sessions, subjective improvement occurred and the oral lesions were stabilized. Case 2. A 17-years-old woman presented corticodependent oral and genital erosive lichen planus and cutaneous lesions. After seven sessions of photopheresis, the patient was able to eat again and the pain decreased. After 20 sessions, the cutaneous lichen planus disappeared and complete remission of the mucous lesions was obtained without corticotherapy. DISCUSSION: These two cases, together with 23 other cases reported elsewhere, strongly suggested the value of photopheresis in the treatment of erosive lichen planus. However, relapses after treatment withdrawal appear extremely frequent.