[Show abstract][Hide abstract] ABSTRACT: INTRODUCTION: Synovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma. CASE PRESENTATION: We describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented. CONCLUSION: Awareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.
Journal of Medical Case Reports 11/2012; 6(1):385.
[Show abstract][Hide abstract] ABSTRACT: Self-inflicted burns (SIB) are responsible for 2-6% of admissions to Burn Units in Europe and North America, and for as many as 25% of admissions in developing nations. Recently, a promising new tool was proposed to stratify SIB patients in the following subgroups: "typical", "delirious", and "reactive". However, as far as the authors know, the clinical usefulness of this instrument has not yet been validated by others. We retrospectively reviewed the clinical records of 56 patients admitted to our Burn Unit with the diagnosis of SIB injury in the past 14 years. The following parameters were evaluated: demographic features; psychiatric illness; substance abuse; mechanism of injury; burn depth, total body surface area (TBSA) involved, Abbreviated Burn Severity Index (ABSI); length of hospital stay, and mortality. All patients were followed up by a psychologist and a psychiatrist, and were classified according to the SIB-Typology Tool, into three classes: "typical", "delirious" and "reactive". There was a slight predominance of the "typical" type (44.6%), followed by the "delirious" type (30.4%), and, finally the "reactive" type (25.0%). Mortality was significantly higher in the "typical" subgroup. In conclusion, the SIB-Typology Tool appears to be a valuable instrument in the clinical management of SIB patients.
Burns: journal of the International Society for Burn Injuries 03/2011; 37(2):322-7. · 1.95 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Stevens-Johnson Syndrome (SJS), Overlap Syndrome (OS) and Toxic Epidermal Necrosis (TEN) are rare medical emergencies associated with a high morbidity and mortality. The literature on the characteristics of these diseases in Portugal is scarce. A retrospective study was conducted based on the clinical notes of the 20 patients admitted to São José Burn Unit in the previous 15 years with the diagnosis of SJS, OS or TEN. Most patients had TEN (65%), followed by OS (25%) and SJS (10%). Average age was 57,1 ± 19,0 years. The mean duration of stay in the Burn Unit was 12,6 ± 7,8 days. Mortality was 50%, being significantly higher than the 16,4% overall mortality in the general Burn Unit population in the same period (p < 0,01). The mean surface area involved was 43,9 ± 28,6 %. Allopurinol was the causal agent most frequently implicated (35%) followed by UV light exposure (15%). Fourteen patients (70%) were treated with steroids in the first days, whereas six patients were treated conservatively (30%). Mortality was inferior in the patients treated with steroids (42,8 vs. 66,7%), even though this difference did not reach statistical significance. Infection rates were not significantly different between the two groups. SCORTEN score proved to be a good predictor of mortality. Further studies are needed to reduce mortality in these diseases.
[Show abstract][Hide abstract] ABSTRACT: Ollier Disease and Maffucci Syndrome are two rare diseases that can cause tumors in several organs, having a special predilection for the hand. However, there have been very few reports in the literature focusing on hand manifestations of these diseases. We report the cases of three female patients: one with Ollier Disease, and two other with Maffucci Syndrome. All patients had hand involvement as their initial primary complaint. The Ollier Disease patient developed chondrosarcomas of two digits and had to have these fingers amputated. One of the Maffucci patients died one year after presentation from a brain glioblastoma. These cases emphasize the importance of early diagnosis of Ollier Disease and Maffucci Syndrome, as these two conditions are associated not only to crippling hand deformity, but also to a significant risk of chondrosarcoma, and other malignant tumors.