Desmoid Tumor In Patients With Familial Adenomatous Polyposis.

Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.47373-

[churg Strauss Syndrome: A Rare Vasculitis].

The aim of this report is to verify the demographic, clinical, laboratorial and histopathological findings of patients with CSS followed in a University Clinical Hospital. We reviewed the medical records of all patients with the diagnosis of CSS. Only patients who fulfilled ACR criteria were included. Five Caucasian patients were found, with a sex ratio M/F: 3/2. The mean age at disease onset was 46 years old (40 to 55) and the mean time of follow-up was 2.37 years (0.25 to 6). General symptoms, asthma, peripheral neuropathy and skin lesions were the most common clinical findings in all patients. Cardiovascular, gastrointestinal and renal involvement was found in one patient each. All patients presented hypereosinophilia (> 10%) and p-ANCA was present in 3 patients. The chest x-ray showed pulmonary parenchymal infiltrate in 2 cases and in 1 of them an unilateral pleural effusion. Skin biopsies showed extravascular eosinophylia in 3 out of 4. All patients were treated with prednisone and in 3 it was added immunosuppressive drug. No patient died during the follow-up. CSS is a rare disease with a multivisceral involvement and asthma is the main and the earliest symptom. General symptoms and involvement of skin and peripheral nerves were found in all patients. Renal, cardiovascular and gastrointestinal involvement was rare, differently from the literature series where these findings are more frequent. In spite of the CSS being a serious and life-threatening disease, these patients presented adequate response to the treatment and a favorable outcome.44179-8

Síndrome de Churg Strauss: uma vasculite rara

O objetivo deste trabalho é avaliar as características demográficas, clínicas, laboratoriais e histopatológica de pacientes com diagnóstico de síndrome de Churg Strauss (SCS) acompanhados no ambulatório de vasculites do Hospital das Clínicas da Universidade Estadual de Campinas (HC/Unicamp). Foi realizado estudo retrospectivo dos prontuários dos pacientes com vasculite primária, classificados como SCS pelos critérios do American College of Rheumathology (ACR). Foram observados cinco pacientes com diagnóstico de SCS (M/F:3/2, caucasóides). A média de idade do início da doença foi 46 anos (40-55). A média de tempo de seguimento foi de 2,37 anos (0,25-6). Sintomas sistêmicos, asma, neuropatia periférica e lesão cutânea foram observados em todos os pacientes. Envolvimento cardiovascular, renal e de trato gastrintestinal estiveram presentes cada qual em um paciente. Todos os pacientes apresentaram eosinofilia (>10%),e o p-ANCA foi positivo nos três casos investigados. A radiografia de tórax revelou infiltrado pulmonar intersticial em dois casos; em um, associado à derrame pleural. Na histopatologia, evidências de eosinófilos extravascular foram encontradas nas biópsias cutâneas de 3/4 pacientes. Todos os pacientes foram tratados com prednisona. Em 3, houve a necessidade de associar-se imunossupressor. Nenhum paciente evoluiu a óbito. A SCS é uma patologia rara, com acometimento sistêmico, sendo a asma uma das principais e mais precoce manifestação. Todos os pacientes apresentaram sintomas sistêmicos, acometimento de pele e sistema nervoso periférico. Envolvimento renal, cardíaco e gastrointestinal foi observado, cada qual em um único paciente, diferindo de outras séries nas quais esses acometimentos são mais freqüentes. Embora seja uma patologia grave e de prognóstico reservado, observamos evolução favorável com tratamento

Churg strauss Syndrome: a rare vasculitis

The aim of this report is to verify the demographic, clinical, laboratorial and histopathological findings of patients with CSS followed in a University Clinical Hospital. We reviewed the medical records of all patients with the diagnosis of CSS. Only patients who fulfilled ACR criteria were included. Five Caucasian patients were found, with a sex ratio M/F: 3/2. The mean age at disease onset was 46 years old (40 to 55) and the mean time of follow-up was 2.37 years (0.25 to 6). General symptoms, asthma, peripheral neuropathy and skin lesions were the most common clinical findings in all patients. Cardiovascular, gastrointestinal and renal involvement was found in one patient each. All patients presented hypereosinophilia (> 10%) and p-ANCA was present in 3 patients. The chest x-ray showed pulmonary parenchymal infiltrate in 2 cases and in 1 of them an unilateral pleural effusion. Skin biopsies showed extravascular eosinophylia in 3 out of 4. All patients were treated with prednisone and in 3 it was added immunosuppressive drug. No patient died during the follow-up. CSS is a rare disease with a multivisceral involvement and asthma is the main and the earliest symptom. General symptoms and involvement of skin and peripheral nerves were found in all patients. Renal, cardiovascular and gastrointestinal involvement was rare, differently from the literature series where these findings are more frequent. In spite of the CSS being a serious and life-threatening disease, these patients presented adequate response to the treatment and a favorable outcome.O objetivo deste trabalho é avaliar as características demográficas, clínicas, laboratoriais e histopatológica de pacientes com diagnóstico de síndrome de Churg Strauss (SCS) acompanhados no ambulatório de vasculites do Hospital das Clínicas da Universidade Estadual de Campinas (HC/Unicamp). Foi realizado estudo retrospectivo dos prontuários dos pacientes com vasculite primária, classificados como SCS pelos critérios do American College of Rheumathology (ACR). Foram observados cinco pacientes com diagnóstico de SCS (M/F:3/2, caucasóides). A média de idade do início da doença foi 46 anos (40-55). A média de tempo de seguimento foi de 2,37 anos (0,25-6). Sintomas sistêmicos, asma, neuropatia periférica e lesão cutânea foram observados em todos os pacientes. Envolvimento cardiovascular, renal e de trato gastrintestinal estiveram presentes cada qual em um paciente. Todos os pacientes apresentaram eosinofilia (>10%),e o p-ANCA foi positivo nos três casos investigados. A radiografia de tórax revelou infiltrado pulmonar intersticial em dois casos; em um, associado à derrame pleural. Na histopatologia, evidências de eosinófilos extravascular foram encontradas nas biópsias cutâneas de 3/4 pacientes. Todos os pacientes foram tratados com prednisona. Em 3, houve a necessidade de associar-se imunossupressor. Nenhum paciente evoluiu a óbito. A SCS é uma patologia rara, com acometimento sistêmico, sendo a asma uma das principais e mais precoce manifestação. Todos os pacientes apresentaram sintomas sistêmicos, acometimento de pele e sistema nervoso periférico. Envolvimento renal, cardíaco e gastrointestinal foi observado, cada qual em um único paciente, diferindo de outras séries nas quais esses acometimentos são mais freqüentes. Embora seja uma patologia grave e de prognóstico reservado, observamos evolução favorável com tratamento.17918

Desmoid tumor in patients with familial adenomatous polyposis

CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy

Tumor desmóide em pacientes com polipose adenomatosa familiar

CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.CONTEXTO: Os tumores desmóides representam uma das manifestações extraintestinais mais importantes na síndrome da polipose adenomatosa familiar. O aparecimento desta neoplasia está relacionada ao aumento da morbimortalidade nos doentes com polipose adenomatosa familiar. OBJETIVOS: Avaliar a ocorrência dos tumores desmóides nos casos de polipose adenomatosa familiar submetidos a colectomia profilática e avaliar o seguimento em ambulatório. MÉTODOS: Entre 1984 e 2008, 68 pacientes foram submetidos a colectomia por polipose adenomatosa familiar no Hospital das Clínicas da Faculdade de Ciências Médicas da Universidade de Campinas, SP. Os tumores desmóides ocorreram em nove pacientes (13.2%), que foram estudados retrospectivamente, por meio da análise de prontuários, buscando dados clínicos e cirúrgicos. RESULTADOS: Dos nove pacientes, sete (77,8%) foram submetidos a laparotomia para ressecção do tumor. A média de idade no momento da cirurgia foi de 33,9 anos (variando 22-51 anos). Os tumores desmóides da parede abdominal ocorreram em 3/9 casos (33.3%) e os intra-abdominais em seis casos (66,7%). O tempo médio entre a cirurgia do reservatório ileal e o diagnóstico do tumor desmóide foi de 37,5 meses (variando 14-60 meses), enquanto o tempo médio entre a cirurgia de colectomia com anastomose íleorretal e o diagnóstico foi de 63,7 meses (variando 25-116 meses). Em 6/9 (66,7%) pacientes com tumor desmóide, a doença estava controlada ou não havia evidência de recidiva do tumor em 63,1 meses (variando de 12 a 240 meses) de tempo médio de seguimento. CONCLUSÃO: Os tumores desmóides ocorreram em 13,2% dos casos de polipose adenomatosa familiar após a cirurgia do cólon; desta maneira, os pacientes com polipose adenomatosa familiar devem manter seguimento em ambulatório e o rastreamento deve incluir o exame abdominal minucioso a fim de identificar sinais e sintomas que possam conduzir ao diagnóstico de tumor desmóide. As opções de tratamento incluem cirurgia e manejo clínico com antiestrogênios, anti-inflamatórios ou quimioterapia.37337

Sporothrix Schenckii Infection Presented As Monoarthritis: Report Of Two Cases And Review Of The Literature.

Septic arthritis demands early diagnosis and correct treatment if the function of the joint is to be restored. Sometimes, as in fungal infection, signs and symptoms may be mild and the diagnosis delayed. Nevertheless, the outcome of fungal arthritis is severe and usually causes joint disability. The authors report two patients with chronic monoarthritis due to Sporothrix schenckii infection diagnosed by synovial fluid cultures. Their clinical presentation, laboratory and image findings, and their treatment and follow-up are analyzed and compared to previously reported cases. These cases illustrate the differential diagnosis of monoarthritis in immunocompetent adults and picture clinical features that could lead to early diagnosis and proper treatment.25926-