Paraganglioma of the head and neck region, treated with radiation therapy, a Rare Cancer Network study.

BACKGROUND: Paraganglioma of the head and neck (HNPGL) are rare often benign tumors. Surgery and radiation therapy (RT) are the main treatment choices. We present an analysis of outcome and toxicity after RT from 13 institutions of the Rare Cancer Network. METHODS: Data were collected using a questionnaire concerning patients' characteristics, treatment, and outcome. A total of 81 patients with 82 HNPGL were analyzed. RESULTS: The median follow-up was 48 months (1-456). Sixty-two lesions were treated with conventional RT and 20 lesions with stereotactic RT. Local control (LC) was achieved in 69 out of 77 lesions. Late toxicity occurred in 17 patients. Patients treated with stereotactic RT experienced neither disease progression nor late toxicity. Four patients with a follow-up longer than 20 years experienced disease progression. CONCLUSION: RT for HNPGL offered good local control with acceptable toxicity. Stereotactic RT might offer better results. Long-term follow-up is required.

Extraskeletal Osteosarcoma: An International Rare Cancer Network Study.

OBJECTIVES: To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS). MATERIALS AND METHODS: Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed. RESULTS: Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P<0.001), primary size >10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P<0.001). In multivariate analysis for DFS, primary size >10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome. CONCLUSIONS: Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS.

Is there a correlation between circulating levels of citrulline and intestinal dysfunction in the critically ill?

OBJECTIVE: The aim of this study was to assess the correlation between plasma citrulline and Sequential Organ Failure Assessment (SOFA) score, Acute Physiology and Chronic Health Evaluation II (APACHE II) score, survival, inflammation (C-reactive protein [CRP]), inotrope use, serum levels of prealbumin and albumin, and renal failure in the critically ill patient. METHODS: This prospective observational single-center controlled study included 91 adult patients over a 2-year period. Inclusion criteria were patients staying in the intensive care unit for >48 hours. Patients' renal status was categorized as those with a glomerular filtration rate (GFR) >60 mL/min without renal support, a GFR >60 mL/min with renal support, a GFR <60 mL/min without renal support, and a GFR <60 mL/min with renal support. Plasma citrulline concentrations were categorized into 3 groups: low (0-15 µmol/L), medium (16-35 µmol/L), and high (>36 µmol/L). The relationship between the recorded parameters and these different cut-off values of plasma citrulline concentrations was analyzed. RESULTS: Ninety-one patients (34% female and 66% male) with a mean (SD) age of 69.3 (11.9) years, a mean (SD) body mass index of 24.8 (5.34) kg/m(2), a mean (SD) APACHE II score of 22.4 (7.92), a mean (SD) SOFA score of 8 (4.4), and a mean (SD) plasma citrulline of 21.7 (13.1) µmol/L were enrolled. Only patients with intestinal dysfunction had low plasma citrulline level <15 µmol/L (P = .014). No correlations between serum levels of CRP, albumin, or prealbumin; renal failure; inotrope use; SOFA score; and APACHE II score were found with plasma citrulline level. CONCLUSION: Low plasma citrulline levels in patients correlate well with intestinal dysfunction.

A fatal case of cutaneous adverse drug-induced toxic epidermal necrolysis associated with severe rhabdomyolysis.

Toxic epidermal necrolysis represents an immunologic reaction to a foreign antigen and is most often caused by drugs. Atorvastatin, a blood cholesterol-lowering agent, is a recognized cause of rhabdomyolysis; while naproxen, a widely used nonsteroidal anti-inflammatory drug, is a known cause of photo-induced skin lesions. We report the first fatal case of drug-induced toxic epidermal necrolysis associated with severe muscle necrosis due to the use of a nonsteroidal anti-inflammatory drug and a statin with very high levels of creatine phosphokinase leading to acute kidney injury, disseminated intravascular coagulation, and complete skin necrosis leading to death.

A delayed fatal metastatic Hürthle cell carcinoma.

Hürthle (oxyphilic or oncocytic) cell carcinoma is a variant of follicular cell carcinoma of thyroid. Although this entity of thyroid cancer is well known, its occurrence in young patients has scarcely been reported. We report a case of a 26 year-old male patient, at the time of diagnosis, of Turkish origin, who developed a tracheal, pulmonary and mediastinal metastatic Hürthle cell carcinoma with bilateral cervical and mediastinal lymphadenopathies. This case illustrates an aggressive and metastatic cancer at the time of diagnosis and resistant to all treatment options including surgery, chemotherapy and radioactive iodine.

A study to determine the correlation between clinical, fiber-optic endoscopic evaluation of swallowing and videofluoroscopic evaluations of swallowing after prolonged intubation.

BACKGROUND: Clinical evaluation of swallowing disorders postextubation is often neglected. Videofluoroscopy is the gold standard with fiber-optic endoscopic evaluation of swallowing (FEES) having a high sensitivity. The aim of this study was to analyze the correlations between clinical, FEES, and videofluoroscopic evaluations in the intensive care unit. METHODS: Twenty-one patients extubated after prolonged intubation were subjected to a clinical evaluation of swallowing and FEES within 24 hours. This was repeated at 48 hours with a videofluoroscopic evaluation with identical swallowing-namely, boluses of liquid and thickened water. The patients were scored from 0 (normal) to 3 (worst). RESULTS: There was no correlation between the oral phase (bedside evaluation) and FEES. The correlation between pharyngeal phase (palatal and laryngeal elevation, pharyngeal rales, and gag reflex) before and after swallowing at 24 and 48 hours was statistically significant (liquid water P = .025 [24 hours] vs P < .001 [48 hours]; thickened water P < .001 [24 and 48 hours]). Clinical assessment, although not statistically significant, failed to detect silent aspiration (P = .58). There was a good correlation between FEES and videofluoroscopy as opposed to clinical assessment and videofluoroscopy (P < .001 vs P = .762). CONCLUSION: Cough is a reliable sign of swallowing disorder but does not exclude silent aspiration and contraindicates oral feeding. Cough induced by liquid water should lead to modification of diet in terms of consistency and viscosity with cough reassessment.

Late onset of Aspergillus aortitis presenting as femoral artery embolism following coronary artery bypass graft surgery.

Aspergillus sp. are ubiquitous mould infections and in most patients, the source is presumed to be air-borne infections during surgical procedures. Prevention of these infections requires special attention of ventilation systems in operating rooms. Post-operative aspergillosis occurs mainly in immunocompromised patients as well as those who receive corticosteroids temporarily. We report a case of a 71-year-old immunocompromised patient who developed multiple lower limb embolisms due to Aspergillus niger originating from an aortitis of the ascending aorta nine months following coronary artery bypass graft (CABG) surgery.

Retained foreign body in ethmoid with bilateral pneumoencephaly.

Intracranial penetrating injury through the nose is rare. We present a case of a 79 year-old patient who had intracranial penetrating injury with a wooden object accompanied by massive bilateral pneumoencephaly with the presence of a foreign body in the ethmoid bone with fracture and displacement of crista galli. This is a hitherto unreported retained foreign body with fractured ethmoid resulting in bilateral pneumoencephaly.