[Creation, implementation and objectives of CARADERM, a national network for rare skin carcinomas - Adnexal neoplasm part]. / Développement, mise en œuvre et objectifs de CARADERM, réseau national cancers cutanés rares ­ partie carcinomes annexiels.

INTRODUCTION: CARADERM is a French national network that includes patients with rare skin adnexal neoplasms. The present paper describes only the adnexal neoplasm part of this network. The primary objective of CARADERM is to improve medical care for malignant skin adnexal neoplasms. A multidisciplinary review group and a centralized pathological review group have been set up. PATIENTS AND METHODS: A dual network of clinicians and pathologists has been set up. Data are recorded in a secure database. RESULTS: The CARADERM network comprises of 38 clinical centres and 22 pathology centres. Between 2014 and 2017, 1598 patients with an adnexal neoplasm were included. Data of interest were documented in 80% of cases. Median patient age was 72 years. Major histological subtypes were sweat gland carcinomas (50%), hair follicle carcinomas (37.7%), and sebaceous gland carcinomas (9.8%). Surgery was the first-line treatment for 81% of patients, including 76.9% with standard surgical margin analysis, and 5.5% with exhaustive margin analysis. 920 patients (57.6%) underwent a national pathology review process. DISCUSSION: The CARADERM network aims at providing assistance in difficult situations concerning diagnosis and care in skin adnexal neoplasms. Analysis of the CARADERM data should allow the creation of a prognostic classification of these rare neoplasms together with recommendations. A national multidisciplinary consensus exists. Translational and therapeutic research is ongoing. CONCLUSION: The CARADERM network is currently recruiting and more data should lead to improved knowledge of these tumours in the coming years.

[Cutaneous granular bacteriosis occurring in Staphylococcus aureus septicaemia]. / Bactériose à grains cutanée au cours d'une septicémie à Staphylococcus aureus.

BACKGROUND: Herein we report a case of cutaneous granular bacteriosis, with discussion of the nosological setting of this entity based upon the clinical and histological findings. PATIENTS AND METHODS: A 62-year-old woman receiving methotrexate for Sezary syndrome was admitted for fever of 38.5̊C and overall impairment of her health. She presented a fistulous nodule on her right knee, and skin biopsy revealed a focus of ulcerated suppuration with quantities of Gram+ and Grocott+ granules containing no filament, enclosed by eosinophilic matter (Splendore-Hoeppli phenomenon). A sample of effusion from the sole of her right foot revealed a methicillin-sensitive strain of Staphylococcus aureus, which was also found in several blood cultures. Two abscessed nodules on the middle right lobe were visible on a thoracic CT scan despite the initial absence of respiratory symptoms. In view of this bacteraemia of cutaneous origin with sepsis caused by methicillin-sensitive S. aureus complicated by pulmonary abscesses, dual antibiotic treatment against staphylococci (cloxacillin-gentamicin followed by rifampicin-ofloxacin) was given over a two-month period. DISCUSSION: The histological picture of granular bacteriosis suggested the possibility of botryomycosis or mycetoma. Botryomycosis involves chronic, relapsing, weeping and ulcero-vegetating abscesses. Mycetoma consists of fistulous swellings that secrete a discharge composed of blood and serum and containing grains made up of filaments. Although the staphylococcal organism identified was evocative of botryomycosis, the clinical findings were not consistent with either of these entities, since they revealed an acute bacterial abscess. The most adequate term is thus the more generic name of septic cutaneous granular abscess.

Assessment of diagnostic criteria between primary cutaneous anaplastic large-cell lymphoma and CD30-rich transformed mycosis fungoides; a study of 66 cases.

BACKGROUND: Transformed mycosis fungoides (TMF) large cells may express CD30 antigen, and because of this, the differential diagnosis between CD30-rich TMF and primary cutaneous anaplastic large-cell lymphoma (cALCL) may be difficult, and especially in distinguishing cALCL associated with MF vs. CD30-rich TMF. OBJECTIVES: To find clinical, histological and molecular diagnostic features useful for differential diagnosis between cALCL and CD30-rich TMF. To analyse and compare the prognostic value of clinical and pathological factors in these two diseases. MATERIAL AND METHODS: We conducted a retrospective study (1999-2012) of 32 patients with cALCL and 34 with CD30-rich TMF, seen in reference centres of the French Study Group of Cutaneous Lymphoma. Clinical, histological and molecular features were analysed and compared to determine their diagnostic and prognostic value. RESULTS: Comparison of the two groups showed that age ˃ 60 years, ≥ 5 skin lesions, early progression, absence of spontaneous regression and trunk involvement were significantly associated with the diagnosis of TMF. Abnormal T-cell phenotype and perforin expression were significantly more frequent in cALCL (both P < 0·001). Overall survival (OS) at 5 years was 77·4% for cALCL and 20·7% for CD30-rich TMF. Stage T3, ≥ 5 skin lesions, lower limb involvement for cALCL and stage T4, extracutaneous involvement, B symptoms, high levels of lactate dehydrogenase for CD30-rich TMF were associated with poor OS and progression-free survival. DUSP22 gene rearrangement had no diagnostic or prognostic value. CONCLUSIONS: Clinical features and outcome are the most discriminative to differentiate the two entities. Even histological and molecular markers were not fully specific; abnormal vs. normal T-cell phenotype and perforin expression may constitute helpful tools.

Pulmonary aspergilloma in a cavity formed after percutaneous radiofrequency ablation.

We report two cases of pulmonary aspergilloma (PA) in a cavity formed after percutaneous radiofrequency ablation (PRFA), a rare complication that has only been described once in the literature. The first patient was a 59-year-old white woman treated for a secondary lung nodule of an advanced hepatocellular carcinoma. One month after PRFA, a consolidation of a cavity was noticed with an "air crescent sign," and aspergilloma serology was highly positive. A bisegmentectomy was performed due to the proximity of the lesion to mediastinal vessels and the absence of significant regression after antifungal treatment. Histological examination confirmed the diagnosis of PA. The second patient was a 61-year-old white man followed-up for a non-small-cell lung cancer. A cavitation with thick margins in the ablation zone was noticed 6 months after PRFA. A biopsy was performed, and aspergilloma was diagnosed. Medical treatment with itraconazole was administered for 13 months, and there was significant regression.