Faculty Bibliography

A 55-year-old man with a history of chronic lymphocytic leukaemia presented with diffuse skin lesions that began 1 week after starting a new chemotherapy regimen with bendamustine and rituximab. The lesions appeared as erythematous papules that were neither itchy nor tender, and did not blanch with pressure. Initially, they began on his scalp and flanks and, over the next few days, spread diffusely throughout his body, becoming darker in colour. Skin biopsy showed atypical clonal B-cell proliferation in a perivascular, periadnexal and dermal band-like distribution, which was further characterised by immunohistochemical evaluation. These findings were suggestive of leukaemia cutis and consistent with the patient's chronic lymphocytic leukaemia, which was previously confirmed by bone marrow biopsy. The bendamustine was stopped and the patient's chemotherapy regimen was switched to fludarabine, cyclophosphamide and rituximab. Shortly thereafter, the leukaemia cutis regressed significantly.

Tolosa-Hunt syndrome, an idiopathic granulomatous inflammation of the cavernous sinus, is primarily a diagnosis of exclusion. The majority of patients present with unilateral orbital pain and features suggestive of paralysis of one or more of the cranial nerves passing through the cavernous sinus and/or superior orbital fissure. MRI of the head may show unilateral enhancement of the cavernous sinus and orbital apex. Treatment is with high-dose intravenous steroids followed by tapering oral steroids. Rapid amelioration of pain within 24-48 h supports this rare diagnosis. Resolution of neuropathies may take longer. We describe a case of a young man who presented with left periorbital pain, complete ophthalmoplaegia and ptosis of the left eye. MRI showed enhancement of the left cavernous sinus and orbital apex. High dose steroids led to complete resolution of pain, while ptosis and ophthalmoplaegia improved gradually.

CONTEXT/BACKGROUND:Sarcomas are a rare group of malignancies. Very little is known about their risk factors. AIMS/OBJECTIVE:The aim was to evaluate different risk factors in patients with sarcomas and to determine the median age at diagnosis, differences in race, gender, histological grades and staging in sarcoma patients. SETTINGS AND DESIGN/METHODS:A retrospective case-control study was conducted in a tertiary care hospital in the USA. This included patients diagnosed with sarcomas from year 2000 to 2010. MATERIALS AND METHODS/METHODS:Data were extracted with the help of electronic medical records using International Classification of Diseases, Ninth revision codes. Healthy, matched controls were randomly selected from the same tertiary care hospital database. STATISTICAL ANALYSIS/METHODS:Univariate comparisons between cases and controls were done using a two-group independent t-test for age and using Chi-square tests for the categorical variables. In order to identify possible independent predictors of sarcomas, a multiple logistic regression model was constructed using sarcoma status as the dependent variable and using, initially, all variables with a univariate P < 0.2 as independent variables. Variables were reduced in a manual stepwise manner to arrive at a final model. Statistical significance was set at P < 0.05. All analyses were performed using SAS 9.4 (SAS Institute Inc., Cary, NC, USA). RESULTS:A total of 425 sarcoma patients and 429 age, sex and race matched healthy controls were analyzed in this study. We found that a history of smoking and alcoholism was significantly associated with sarcomas. We also found that the history of cancer in first-degree relatives had a significant relationship. In addition, patients with sarcomas are more likely to have a history of another malignancy when compared with controls. CONCLUSIONS:Smoking and alcohol are potential risk factors for sarcomas. In addition, a history of cancer in the first-degree relative is also a potential risk factor. Patients with sarcomas are likely to have a history of another malignancy when compared with controls.

A 26-year-old male without any significant past medical history presented to the hospital with shortness of breath, cough, pleuritic chest pain, and weight loss for the past 3 months. On chest CT, he was found to have extensive mediastinal and hilar lymphadenopathy and multiple pulmonary nodules. On physical examination, a right groin mass was noted which had been slowly growing for the past 2 years. Ultrasound of the groin showed complex solid mass with internal vascular channels. CT guided biopsy of the mass showed desmoplastic small round cell tumour. His hospital course was complicated by hypoxic respiratory failure requiring emergent intubation and ICU admission where he completed one cycle of vincristine, cyclophosphamide, and doxorubicin with subsequent improvement, followed by extubation. His condition continued to improve after second cycle of chemotherapy and he was ultimately discharged in a stable condition to continue outpatient chemotherapy after a 2-month inpatient stay.

A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis.