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.

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).

[Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis: role of gadolinium?]. / Dermopathie néphrogénique fibrosante traitée par photochimiothérapie extracorporelle: rôle du gadolinium?

BACKGROUND: Nephrogenic fibrosing dermopathy is a cutaneous and systemic sclerosis affecting patients with renal failure. CASE-REPORT: A 68-year-old man with renal insufficiency and on dialysis developed hardening of the skin and severe joint contractions. He had previously undergone angiography with gadolinium-containing contrast agents. A skin biopsy confirmed nephrogenic fibrosing dermopathy. The patient was treated by oral steroids followed by extracorporeal photopheresis. An improvement was seen after 12 cycles. DISCUSSION: Treatment of nephrogenic systemic fibrosis is not codified and is normally based on the methods used for other forms of systemic sclerosis. Six cases of patients showing improvement under extracorporeal photopheresis have been published. The physiopathology of the disease is unknown. Gadolinium could act as a triggering agent by attracting circulating fibrocytes in the dermis of patients. Medical authorities recommend avoidance of gadolinium in patients with advanced kidney failure unless strictly necessary.

[Regression in primary cutaneous melanoma is not predictive for sentinel lymph node micrometastasis]. / Absence de valeur prédictive des signes de régression histologique sur l'envahissement du ganglion sentinelle.

BACKGROUND: The predictive value of regression in melanoma is debated. AIM OF THE STUDY: A retrospective single-centre study to evaluate the correlation between regression in primary skin tumor and the presence of micrometastases in sentinel lymph nodes. PATIENTS AND METHODS: Histological signs of regression in 84 melanomas (>1 mm) with corresponding sentinel lymph nodes were studied by two independent pathologists. RESULTS: Regression was seen in 40 skin melanoma tumors while micrometastasis was seen in 24. Of the tumors with micrometastasis, only 10 were regressive (RR: 0.47, p=0.49). Breslow value>2 mm and male sex were predictive for node micrometastasis (RR: 4.6, p=0.03 and RR: 7.6, p=0.006, respectively). On multivariate analysis, these two factors were independent. COMMENTS: These data suggest that regression in primary cutaneous melanoma is not predictive for lymph node metastasis.

[Chronic actinic dermatitis: treatment with topical tacrolimus (two cases)]. / Dermatite actinique chronique traitée par le tacrolimus topique (deux cas).

BACKGROUND: Chronic actinic dermatitis is usually controlled under systemic immunosuppressive drugs. We report herein two patients successfully treated with topical tacrolimus. CASE REPORTS: Two men aged 60 and 70 years were treated for chronic actinic dermatitis for two years using systemic immunosuppressive drugs. Due to drug intolerance and treatment resistance, systemic treatment was substituted by topical tacrolimus. Cutaneous lesions improved dramatically within two months but withdrawal of daily application was immediately followed by recurrence of the lesions. DISCUSSION: Our cases, together with the 10 others reported in the literature, confirm the efficacy of topical tacrolimus in the treatment of chronic actinic dermatitis. Since tacrolimus chiefly targets activated T lymphocytes, it has been successfully deployed in various inflammatory dermatoses and its use is logical in the treatment of chronic actinic dermatitis. While dramatic improvement is achieved within a few weeks in this indication with twice-daily applications of tacrolimus ointment 0.1%, symptoms recur rapidly on dosage reduction, and extremely long-term, or even lifelong, treatment is thus probably needed. Topical tacrolimus application has been shown to be safe for periods of three years. However, the peculiar mechanism of chronic actinic dermatitis with a pronounced imbalance in T-cell subsets raises the question of theoretical risk of carcinogenicity of tacrolimus applications, and this, together with the prolonged duration of treatment, calls for long-term follow-up of chronic actinic dermatitis patients.

[Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome]. / Xanthogranulome nécrobiotique sans gammapathie monoclonale d'évolution rapidement fatale.

BACKGROUND: Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. CASE REPORT: A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.