Can 18F-FDG PET/CT findings be used to predict orbital tumor histology?

PURPOSE: To investigate whether 18F-FDG PET/CT might be useful to predict the histology of various orbital tumors based on the maximum standard uptake value (SUVmax) and the OMSUV (orbital max SUV)/MLSUV (mean liver SUV) ratio. PATIENTS AND METHODS: A retrospective single-center study was conducted between May 2019 and December 2020. Patients with an orbital mass who underwent preoperative 18F-FDG PET/CT followed by an orbital biopsy were included. Tumor histology was classified as follows: orbital inflammation, solid tumor, low-grade lymphoid tumor, and high-grade lymphoid tumor. Orbital tumors were also classified as indolent or aggressive. Data recorded included the orbital SUVmax, OMSUV/MLSUV ratio and additional extra-orbital SUV sites. RESULTS: Forty-five patients (24 men) were included. There were 15 (33.3%), 14 (31.1%), 9 (20%), and 7 (15.5%) cases of orbital inflammation, solid tumor, low-grade lymphoid tumor, and high-grade lymphoid tumor, respectively. No correlation was found between the OMSUV/MLSUV ratio and orbital SUVmax and tumor histology (Z = -0.77, Z = -0.6, Z = -1.6, and Z = 0.94, all P > 0.05, respectively). No correlation was found between the OMSUV/MLSUV ratio (Z = -1.42, P > 0.05) and orbital SUVmax (Z = -0.82, P > 0.05) and tumor aggressiveness (indolent versus aggressive). Subgroup analyses showed that SUVmax was predictive of lymphoma aggressiveness (P = 0.05) and was able to distinguish orbital cancers (all lymphomas+solid tumors) from benign tumors (P = 0.02). CONCLUSION: 18F-FDG PET/CT could not be used to predict the underlying orbital tumor histology. However, more aggressive tumors, especially high-grade lymphomas and cancers, tended to have a higher orbital SUVmax compared to indolent lesions.

Eye amputation following lifitegrast treatment for ocular graft-versus-host disease: First case report.

Graft-versus-host disease (GVHD) is a common complication in patients undergoing allogeneic stem cell transplantation for acute myeloblastic leukemia that could be very difficult to treat. Lifitegrast 5% (Xiidra@, Novartis), a new immunosuppressive eye drop, was recently approved by the FDA for the treatment of severe dry eye and is currently under review by the European Medicines Agency. In France, lifitegrast has been approved by the French authorities for temporary use in refractory dry eye syndrome resistant to tear substitutes and topical cyclosporine. To date, serious complications have been reported only exceptionally. In this article, we report the case of a 65-year-old patient with a medical history of acute myeloid leukemia (AML) diagnosed in 2015 who received a first matched related donor transplant. In 2019, this patient developed chronic GVH involving the skin, oral mucosa and eye. Despite taking topical and systemic medications for 3 months, the patient did not report relief of ocular symptoms. Therefore, lifitegrast was prescribed. To our knowledge, we report the first case of corneal perforation in which evisceration was required following treatment with topical lifitegrast for chronic GVH. In the case presented here, it can be assumed that the underlying mechanisms leading to corneal perforation are multifactorial. Using drug accountability criteria, lifitegrast appears to be strongly associated with the development of bacterial keratitis and corneal perforation.

[Clinical, radiological, pathological features, treatment and follow-up of periocular and/or orbital amyloidosis: Report of 6 cases and literature review]. / Caractéristiques cliniques, radiologiques, anatomopathologiques, thérapeutiques et évolutives de l'amylose périoculaire et/ou intraorbitaire : à propos de 6 cas et revue de la littérature.

PURPOSE: To assess demographic, clinical, radiological, pathological features, treatment and follow-up of periocular or/and orbital amyloidosis. PATIENTS AND METHODS: We conducted an observational retrospective monocentric study from January 2004 to April 2017 in patients diagnosed with histologically proven periocular or/and orbital amyloidosis. RESULTS: Six patients were included (2 females, 4 males). Mean age was 76.8 years (range 66-88 years). Mean time between first ophthalmological symptoms and diagnosis was 27 months (range 11-36 months). The main symptoms were subconjunctival infiltration (6 patients; 100%), periocular pain or discomfort (4 patients; 66.6%) and subconjunctival hemorrhage (1 patient; 16.6%). Clinical findings included ptosis (4 patients; 66.6%), keratitis (3 patients; 50%) leading to corneal perforation in one patient, and proptosis (3 patients; 50%). One-half of the patients showed bilateral involvement. AL amyloidosis was identified on immunohistochemistry in 5 patients (83.3%). One case of B cell marginal zone orbital lymphoma was diagnosed. Systemic work-up was negative for all patients. Treatment consisted of simple monitoring (1 patient; 16.6%), surgical debulking (3 patients; 50%), ptosis surgery (1 patient; 16.6%), eyelid or eyelash malposition surgery (2 patients; 33.3%) and orbital radiation beam therapy (2 patients; 33.3%). Mean follow-up was 14.6 months (range 6-36 months), and no progression nor recurrence were noted. CONCLUSION: Periocular or/and orbital amyloidosis is rarely encountered. Diagnosis is based on pathological examination, and immunohistochemistry analysis should always be performed to guide systemic work-up. Orbital lymphoma and multiple myeloma should be ruled out if AL amyloidosis is diagnosed. Progression is slow, and surgery is the mainstay of treatment in symptomatic patients. Long-term multidisciplinary follow-up is advocated.

[Uveitis treated with biotherapy and/or DMARD: Analysis from the French Pharmacovigilance Study Base]. / Uvéites sous biothérapies et/ou DMARDs en rhumatologie : analyse de la base de données déclarative de la pharmacovigilance nationale française.

PURPOSE: To report the characteristics of uveitis cases occurring while on biologic therapy or disease-modifying antirheumatic drugs (DMARDs) reported to the French national pharmacovigilance database. METHODS: All the uveitis cases occurring in patients with chronic rheumatologic diseases, chronic inflammatory intestinal diseases or connective tissue diseases, while treated with DMARDs and/or biologic therapies between 2000 and 2015 and reported to the French National Pharmacovigilance Database were collected. RESULTS: During the study period, 32 cases of uveitis were reported (15 men, 17 women). Two patients were treated with one DMARD alone, 24 with biologic therapy alone, and six with both treatments. Anterior uveitis was diagnosed in 19 patients (8 cases were bilateral); intermediate uveitis was found (unilaterally) in one patient; posterior and diffuse uveitis occurred in 5 and 2 cases respectively. Five cases were inconclusive with regard to the anatomical type of uveitis. The uveitis was of infectious origin in 5 cases: 2 toxoplasmosis, 2 herpes virus and 1 tuberculosis. In the 27 other cases, it was not possible to state whether the uveitis was associated with the underlying disease (uncontrolled) or a side effect of the biologic/DMARD treatments. The occurrence of the uveitis led to 9 switches in biologic therapy and 13 discontinuations of treatment (8 complete discontinuations, 5 discontinuations only until uveitis remission was obtained). In 4 cases, the treatments were not modified. The database does not specify the ultimate course or rheumatologic disease activity at the time of the uveitis. CONCLUSIONS: The presence of uveitis while on biologic therapy must not be taken to indicate a therapeutic failure, especially if the ocular manifestation is isolated. In the case of uveitis occurring in patients treated with biologic therapies and/or DMARDs, infectious complications should be ruled out.

[Panuveitis associated with papillary carcinoma of the thyroid]. / Panuvéite bilatérale associée à un carcinome papillaire de la thyroïde.

Ocular involvement secondary to thyroid carcinomas is uncommon. Uveal metastasis may occur. More rarely, they can be responsible for paraneoplastic syndromes. We report the case of a 64-year-old woman who presented with a severe bilateral panuveitis with venous vasculitis associated with hyperthyroidism from a multinodular goiter, complicated by papillary carcinoma. Systemic steroid therapy was initiated; ocular symptoms resolved completely after total thyroidectomy. Other causes of panuveitis with venous vasculitis were ruled out. This is the first reported case of panuveitis associated with papillary thyroid carcinoma. The occurrence of the ocular symptoms with hyperthyroidism and their remission after surgery supports the possibility that this association may not be coincidental. A paraneoplastic phenomenon is suspected.

[Diagnosis of IgG4-related systemic disease in a patient with an ocular tumor associated with lung nodules]. / Diagnostic de syndrome d'hyper-IgG4 chez une patiente présentant une tumeur oculaire associée à des nodules pulmonaires.

The IgG4-related systemic disease is a recently described entity of fibro-inflammatory systemic damage. Although initially described in some forms of pancreatitis, the disease can affect all organs. The common histological features include a lymphoplasmacytic infiltration (especially to IgG4), fibrosis and phlebitis. Elevated serum level of IgG4 is also often present. This rare but certainly underdiagnosed disease must be kept in mind of all clinician faced to a non-specific inflammatory lesion. We report a case of ocular inflammation and lung tumors in a patient of 84 years for which the diagnosis was made through immunolabelling with IgG4 in lesions biopsied.

[Management of acute retinal necrosis syndrome with cystoid macular edema: a case report]. / Prise en charge du syndrome de nécrose rétinienne aiguë compliqué d'oedème maculaire cystoïde.

Acute retinal necrosis is a rare but devastating and rapidly progressive viral retinitis. We report the case of a healthy 46-year-old woman who presented bilateral acute retinal necrosis caused by varicella zoster virus (VZV). Intravenous and intravitreal antiviral therapy was given for 6 weeks and followed by oral therapy. After 3 months, the patient presented a bilateral macular edema which was treated first by laterobulbar injections of corticosteroids, then successfully by interferon alpha-2a. This clinical disease, which can cause major visual function impairment, must be known in order to treat it immediately.

Successful treatment of refractory generalized myasthenia gravis with rituximab.

OBJECTIVE: Myasthenia gravis (MG) is an autoimmune neuromuscular disorder for which current therapies carry a high risk of side-effects and may be insufficient in stabilizing the clinical status. Many therapeutic options can be ruled, such as thymectomy, corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil, methotrexate, intravenous immunoglobulin (IVIg) or less frequently plasmapheresis must be ruled. METHODS: We followed prospectively six patients with MG who presented with a poor response to two or three lines of immunosuppressive conventional drugs associated with oral corticosteroids. All but one were acetylcholine receptor negative and three were anti-MuSK positive. IVIg did not improved the neurological status and all patient required high doses of cholinesterase inhibitors. RESULTS: Rituximab was introduced with a mean follow-up of 1.5 years (375 mg/m(2), days 1, 8, 15, 28 during the first month and then one dose every 2 months). After 2 years of follow-up, all patients stopped corticosteroids and tapered off cholinesterase inhibitors from 60 to 180 mg/day without severe infectious events. CONCLUSION: Rituximab, a chimeric IgG k monoclonal antibody that target CD20 is used for the treatment of relapsing/refractory CD20 positive low-grade non-Hodgkin's lymphoma and other autoimmune neuromuscular diseases. Four previous short reports have described a good response of MG associated with lymphoma with rituximab. It appears to be a promising and effective drug for the treatment of MG without lymphoma, with a substantial benefit to the clinical status and good tolerability.

Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature.

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs. We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage. Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.