POEMS syndrome presenting with abdominal distension, lower limb edema and shortness of breath: A case report and literature review.

POEMS syndrome is a rare paraneoplastic disorder. A 60-year-old female patient was admitted to the Department of Gastroenterology, Xiangya Hospital of Central South University (Changsha, China), complaining of abdominal distension, severe edema of both lower limbs and shortness of breath for more than 1 year. After intensive and careful medical investigations, the patient manifested with polyneuropathy, M-proteinemia, splenomegaly, lymphadenopathy, hypothyroidism, extravascular volume overload, sclerotic bone lesions, elevated VEGF and pulmonary hypertension. According to the latest diagnostic criteria of POEMS syndrome, this patient met two mandatory major criteria, two other major criteria and three minor criteria, the diagnosis was clear after ruling out differential diagnosis. The patient was treated with dexamethasone and lenalidomide, which relieved her clinical symptoms. The pathogenesis of POEMS syndrome is not fully understood; however, increased levels of vascular endothelial growth factor may contribute to most of the clinical manifestations. This patient had been in physical discomfort for more than 14 months, which seriously affected her quality of life. Clinically, the awareness of early diagnosis and treatment of POEMS syndrome should be improved.

Clinical Characteristics and Prognostic Factors of Small Intestine Angiosarcoma: a Retrospective Clinical Analysis of 66 Cases.

BACKGROUND/AIMS: Primary angiosarcoma of the small intestine is a rare neoplasia, and there are limited data from systematic analyses. The aim of this study is to describe the clinical and pathological characteristics in addition to the prognostic factors for this rare neoplasia. METHODS: We retrospectively collected the clinical records and prognostic information of 66 patients with small intestine angiosarcoma reported between 1970 and 2017. We used the Chi-square test, the log-rank test, and Cox regression analyses to evaluate the data. RESULTS: There were 66 patients diagnosed with small intestine angiosarcoma. The onset age ranged from 24-92 years old. There were 24 patients diagnosed before the year 2000, and 42 patients were diagnosed after 2000. The data indicated that 49 cases were diagnosed as primary disease, and the remaining 15 cases were secondary disease. The main clinical symptoms were nonspecific and included gastrointestinal (GI) bleeding and abdominal pain. Additionally, we found multi-center foci were one of the characteristics of this disease. Radiation-induced small intestine angiosarcoma (RSIA) is a special type of disease with a similar prognosis. This type was more frequent in females and decreased after the year 2000. We also found that GI bleeding was less common in RSIA cases. The log-rank test results revealed that old-age, poor differentiation, and GI bleeding were associated with worse prognosis. Surgical treatment showed a trend toward a prolonged survival time. However, the result was not statistically significant. Our results show treatment with adjuvant therapy improved prognosis. The multivariate Cox analysis demonstrated adjuvant therapy was an independent indicator of a favorable outcome in small intestine angiosarcoma patients. CONCLUSION: Pay attention to the unexplained gastrointestinal bleeding could lead to a faster diagnosis and control of small intestine angiosarcoma. Furthermore, treatments including adjuvant therapy can effectively improve the prognosis.