Acquired generalized lipodystrophy under immune checkpoint inhibition.

Immune checkpoint inhibitors are now the standard of care in the treatment of several types of cancer. Cutaneous immune-related adverse events (irAEs) are usually of low grade and reversible, while endocrine irAEs are generally irreversible and managed with hormone replacement therapy. We report a 47-year-old patient, treated with the anti-programmed cell death (PD)1 antibody pembrolizumab for a metastatic melanoma, who developed severe lipodystrophy after 10 months of treatment, characterized by the loss of subcutaneous fat tissue, central obesity and insulin resistance with a decreased leptin level. Histological analysis of a cutaneous biopsy revealed subcutaneous fat cell destruction associated with oedema, the presence of lipophages, and a CD3+ lymphocytic infiltrate involving the panniculus. This led to the diagnosis of anti-PD-1-induced acquired generalized lipodystrophy, after ruling out differential diagnoses (i.e. genetic and systemic autoimmune diseases). No corticosteroids were introduced considering the high risk of inducing severe metabolic complications, and pembrolizumab was discontinued as complete response of the melanoma was achieved. However, after 12 months of follow-up, lipodystrophy and its severe metabolic complications are still ongoing. What's already known about this topic? Anti-programmed cell death (PD)1 agents are now a standard of care in the treatment of several cancers, including melanoma. Endocrine and cutaneous immune-related adverse events (irAEs) are among the most frequent irAEs (14-30% and 30-40%, respectively) in patients treated with immune checkpoint inhibitors. What does this study add? Acquired generalized lipodystrophy can occur during anti-PD1 therapy and is associated with severe metabolic complications. With the increase in anti-PD1 prescription in several cancer types, clinicians must be aware of the whole range of irAEs that may occur.

Accuracy of computed tomographic colonography in a nationwide multicentre trial, and its relation to radiologist expertise.

OBJECTIVE: Reports on the accuracy of computed tomographic colonography (CTC) mainly involve series from expert institutions. The aims of this study were to assess CTC accuracy in a nationwide population and to relate it to radiologist performance in their initial training. DESIGN: Nationwide multicentre trial. SETTING: Twenty-eight radiologists, working in 26 mostly academic clinical units, were involved in the study after having attended a formal specialised 2-day training session on CTC. They worked through a training set of 52 cases with automatic feedback after an attempt at each case. PATIENTS: The study enrolled 845 patients with average and high risk of colorectal cancer, 737 of whom had both complete CTC and videocolonoscopy data, which constituted the dataset. INTERVENTIONS: Patients underwent same-day CTC followed by videocolonoscopy with segmental unblinding of CTC results. MAIN OUTCOME MEASURES: Sensitivity, specificity and positive and negative predictive values for detection of polyps ≥ 6 mm in per-patient and per-lesion analyses of CTC without computer-aided detection. RESULTS: Sensitivity, specificity and positive and negative predictive values for patients with polyps ≥ 6 mm were 69% (95% CI 61% to 77%), 91% (95% CI 89% to 94%), 67% (95% CI 59% to 74%) and 92% (95% CI 90% to 94%), respectively. Univariate analysis showed that the detection rate for polyps ≥ 6 mm was linked to neither radiologist case volume nor number of polyps, but was related to sensitivity achieved in the training set. Pooled sensitivity was 72% (95% CI 63% to 80%) versus 51% (95% CI 40% to 60%) for radiologists achieving above and below median sensitivity in the training set (61%), respectively. Multivariate analysis showed that sensitivity for polyps ≥ 6 mm in the training set was the only remaining significant predictive factor for subsequent performance. CONCLUSIONS: Radiologist sensitivity CTC for detection of polyps ≥ 6 mm in training was the sole independent predictor for subsequent sensitivity in detection of such polyps.

[Diffusion-weighted MR imaging of the breast]. / Diffusion en IRM mammaire.

Diffusion-weighted imaging is helpful to further characterize lesions that remain indeterminate after morphological and dynamic MR evaluation. Suspicious lesions are hyperintense on diffusion-weighted images with corresponding low ADC values, indicating restricted diffusion and hypercellularity. Benign lesions and tumors responding to treatment usually have no diffusion restriction. ADC maps are useful for T2W hyperintense lesions that could mask the presence of restricted diffusion. Image fusion is sometimes needed to accurately localize enhancing lesions on ADC maps. For indeterminate lesions, a hypocellular appearance suggests a lower ACR category whereas the presence of restricted diffusion suggests a higher category.

[Pneumatosis cystoides intestinalis complicating paraneoplastic dermatomyositis]. / Pneumatose kystique intestinale compliquant une dermatomyosite paranéoplasique.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of gas-filled cysts within the wall of the digestive tract. Classically, it occurs in lung or colon diseases but rarely in patients with collagen disorders. We report a new case of PCI occurring during the course of paraneoplastic dermatomyositis. PATIENTS AND METHODS: A 53-year-old woman was diagnosed with dermatomyositis two years ago. Relapse of dermatomyositis preceded the discovery of metastases for which chemotherapy was initiated with 5-fluorouracil and vinorelbine. Three months later, she was admitted to our department for abdominal pains. On physical examination, the abdomen was distended with normal peristalsis. There was no evidence in favour of active dermatomyositis. Abdominal computed tomography scan showed gas collection in the mesentery, revealing the PCI. There was also pneumoperitoneum. The patient slowly improved with symptomatic treatment. DISCUSSION: PCI is uncommon in systemic diseases and extremely rare in dermatomyositis. The pathogenesis and aetiology of PCI are unknown in most cases. In collagen diseases, several hypotheses have been suggested: digestive hypokinesia, corticosteroid-induced ulceration and intestinal vasculitis. In our patient, two factors contributed to PCI: corticosteroid administration and a chemotherapeutic agent (vinorelbine), resulting in severe constipation. Diagnosis of PCI is based on abdominal computed tomography. Pneumoperitonitis is frequent. Although rare, the diagnosis of PCI must be evoked in collagen disorder patients presenting nonspecific abdominal symptoms.

[Diagnosis of a malignant lymph node]. / Le diagnostic d'un ganglion tumoral.

There are several imaging techniques, each with advantages and limitations. Standard or computed radiography is always useful. CT diagnosis of an enlarged lymph node is easy but it is very difficult to conclude about its reactive or metastatic nature: subtle signs can help. MRI has similar pitfalls but sometimes it may be possible to identify fibrotic scarred nodes. US with Doppler can evaluate the abnormal angioarchitecture of a metastatic lymph node. Sentinel lymph nodes are easily identified by nuclear medicine. CT-PET provides morphologic and metabolic information which increases the diagnostic accuracy. Imaging work-up strategies for selected malignancies are discussed.

Thoracic disk herniation mimicking renal crisis.

A 45-year-old-woman presented with a mimicking renal stone colicky pain that was finally attributed to radiculalgia T12 caused by a calcified T11-T12 disk herniation.

[The value of radiological follow-up for stage III melanoma]. / Intérêt du suivi radiologique pour les mélanomes au stade III.

INTRODUCTION: The modalities of follow-up (frequency of consultations and interest of repeated radiological examinations) of patients presenting with glandular metastases of melanoma (stage III of the AJCC classification) have not reached a consensus. PATIENTS AND METHODS: Since 1995, we have proposed clinical follow-up every two months and radiological controls with a thoracic-abdominal-pelvic scan every 4 months, to patients at high risk of relapse for the early screening of an infra-clinical relapse. RESULTS: The median follow-up was of 16 months (range: 1 to 82 months). Eight patients out of 24 (33 p. 100) followed-up in this manner, had asymptomatic metastases discovered by the radiological examinations. Among these 8 patients, three presented with a an operable, single, metastatic localization and two patients underwent surgery. One patient relapsed 3 months later, the other was still alive without relapse 24 months later. DISCUSSION: Surgery remains the treatment of choice for all stages of melanoma. In the absence of clearly effective treatment of metastatic melanoma, the early discovery of an infra-clinical metastatic relapse presents two major advantages. The first is the discovery of a single, operable metastasis, as was the case in two of the patients out of 24. The second is to be able to suspend an eventual adjuvant therapy with interferon alpha, as soon as a relapse has been discovered.

[Fatty replacement of the pancreas in a patient with asymptomatic pulmonary cystic fibrosis]. / Infiltration graisseuse du pancréas dans le cadre d'une mucoviscidose asymptomatique sur le plan pulmonaire.

The authors report the case of a 26-year-old woman with hepatomegaly, who had recurrent and progressive nausea and abdominal pain. Computed tomography and magnetic resonance imaging demonstrated fatty replacement of the entire pancreas resulting from cystic fibrosis. The pulmonary disease was totally asymptomatic. Fatty replacement of the pancreas is the most frequent pattern in older patients with cystic fibrosis. This pattern correlates with pancreatic exocrine dysfunction.

[Hereditary multiple exostoses after 40 years of development: a case report]. / Maladie des exostoses multiples après 40 ans d'évolution: à propos d'un cas.

INTRODUCTION: Hereditary multiple exostoses is an autosomal dominant skeletal disorder with genetic heterogeneity and an estimated prevalence of 1/50,000 in western countries. Malignant degeneration is a rare (about 2%) but classical complication in patients with hereditary multiple exostoses. At least 3 loci identified as EXT 1, EXT 2 and EXT 3 are involved in this skeletal disease. EXEGESIS: The case of a 45-year old man is described with 15 years follow-up after resection of a well-differentiated chondrosarcoma (grade I), which arose from a right posterior pelvic exostosis. The observed radiological lesions remained relatively stable until now. The genetic mutation which is responsible for the disease was determined at the locus EXT 1. CONCLUSION: The present case report illustrates the natural history of hereditary multiple exostoses, especially since the patient underwent a malignant degeneration which could be resected without recurrence. The results of the genetic analysis contributed to the understanding of the pathophysiology of the disease.

Imaging of MALT lymphomas.

The broad category of non-Hodgkin's lymphoma includes a large variety of different diseases including indolent as well as aggressive lymphomas. Mucosa-associated lymphoid tissue (MALT) lymphoma arises in the extranodal mucosal lymphoid tissue and has only been recognised as a distinct entity in recent years. It affects one or several extranodal structures such as the stomach, the lung, the eye and salivary glands. The lymphoma is generally of low grade and has indolent course. The aim of this article is to exemplify the most common radiological patterns of MALT lymphoma.

Cerebral and spinal cord involvement resulting from invasive aspergillosis.

Although central nervous system involvement in disseminated aspergillosis is known to occur in immunocompromised patients, particularly after bone marrow transplantation, localized involvement of the spinal cord is exceedingly rare. In this report we present and illustrate detailed imaging findings of central nervous system invasion by Aspergillus fumigatus in a 30-year-old woman, with emphasis on the spinal cord involvement.

Use of voriconazole to successfully treat disseminated Trichosporon asahii infection in a patient with acute myeloid leukaemia.

Trichosporon spp. is an emerging fungal pathogen in immunocompromised hosts, and disseminated infection is often fatal in neutropenic patients. Reported here is a case of disseminated infection in a neutropenic patient with acute leukaemia. After failure of amphotericin B and fluconazole therapy, the course of infection dramatically improved with voriconazole treatment. A literature search revealed 69 additional cases of disseminated Trichosporon spp. infections in neutropenic patients, and these are also reviewed. Clinical symptoms that suggest infection include fever, disseminated papulopustular cutaneous lesions and pulmonary involvement. Despite treatment with antifungal agents (amphotericin B, fluconazole), 78% of patients died. Voriconazole may represent a promising therapy for this life-threatening infection.

[Digital thoracic radiology: devices, image processing, limits]. / Radiographie thoracique numérisée: appareillages, traitements d'images, limites.

In a first part, the different techniques of digital thoracic radiography are described. Since computed radiography with phosphore plates are the most commercialized it is more emphasized. But the other detectors are also described, as the drum coated with selenium and the direct digital radiography with selenium detectors. The other detectors are also studied in particular indirect flat panels detectors and the system with four high resolution CCD cameras. In a second step the most important image processing are discussed: the gradation curves, the unsharp mask processing, the system MUSICA, the dynamic range compression or reduction, the soustraction with dual energy. In the last part the advantages and the drawbacks of computed thoracic radiography are emphasized. The most important are the almost constant good quality of the pictures and the possibilities of image processing.

[Infection of a pneumonectomy site 16 years after intervention]. / Infection d'une cavité de pneumonectomie 16 ans après l'intervention.

BACKGROUND: Infection of the residual cavity after pneumonectomy generally occurs early after surgery. CASE REPORT: A 67-year old patient was hospitalized with fever 16 years after pneumonectomy for lung cancer. Investigations led to the diagnosis of infection of the pneumonectomy cavity. DISCUSSION: Late infection several years after pneumonectomy is exceptional and usually occurs in a setting of bacteriemia. Diagnosis is difficult due to modifications of the thoracic signs but should be entertained whenever unexplained fever or an inflammatory syndrome occurs in a pneumonectomized patient. The thoracic CT scan shows an abnormally enlarged cavity. Bacteriological examination of evacuated fluid provides the key to diagnosis. Drainage-lavage is indicated. Surgery may be needed exceptionally.

Image-guided core-needle biopsy in patients with suspected or recurrent lymphomas.

BACKGROUND: It is now commonly admitted that the diagnosis of recurrence of lymphoma can be assessed by image-guided needle biopsy (IGNB). However, the means of obtaining tissue for the original diagnosis of lymphoma is often surgery. The aim of this study was to compare the accuracy of IGNB at the time of diagnosis and at the time of recurrence or progression. METHODS: The authors performed 212 IGNBs on 194 patients who eventually had a diagnosis of lymphoma. One hundred three IGNBs were obtained at original diagnosis and 109 at recurrence or progression. Large-cutting core-biopsy needles, ranging in size from 20 gauge to 14 gauge, were used. Immunohistochemistry studies were performed in all lymphoma cases. RESULTS: A diagnosis of lymphoma with subtyping was obtained in 88% of all cases, in 85% at initial diagnosis, and in 89% at follow-up. Therapy was initiated on the basis of IGNB in 93% of all cases, in 91% at initial diagnosis, and in 94% at follow-up. Benign complications occurred in 7.5% of cases and did not require specific treatment. IGNB was equally effective for making a specific diagnosis of lymphoma and initiating therapy at the time of original diagnosis and at follow-up. CONCLUSIONS: The authors recommend that IGNB be performed as the initial procedure for the diagnosis of lymphoma in the absence of peripheral lymph nodes, either at presentation or at recurrence.