PubMed · 9236619
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V L Wills, J C Graham, R P Eckstein. 1997. Aortic intimal sarcoma: a case report.. https://doi.org/10.1111/j.1445-2197.1997.tb02019.x
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PURPOSE: Appendiceal adenocarcinomas are very rare. We analyzed contemporary outcomes associated with surgical therapies for these malignancies. METHODS: Retrospective outcomes for patients treated at a tertiary academic medical center from 1981 through 2001 were analyzed. RESULTS: A total of 36 patients (22 females (61 percent) mean age, 52 years) with appendiceal adenocarcinoma were treated. Eighty-eight percent of patients presented with symptoms of acute appendicitis. Eighteen (50 percent) patients underwent curative resection (7 primary right hemicolectomies, 10 appendectomies + subsequent right hemicolectomy, and 1 appendectomy alone). Mean length of follow-up was 55 months. Overall five-year survival rate was 46 percent. The five-year survival rate after curative resection was 61 percent and after palliative surgery was 32 percent ( P < 0.05). Among patients who underwent curative resection, factors associated with improved five-year survival rates included histologic type (79 vs. 32 percent for colonic vs. mucinous types, respectively; P < 0.05), T stage (75 vs. 47 percent for T1 and 2 vs. T3 and 4, respectively; P < 0.05), and tumor grade (100 vs. 46 percent for well-differentiated tumors vs. moderately or poorly differentiated tumors, respectively; P < 0.05). CONCLUSIONS: Patients undergoing surgery for appendiceal adenocarcinoma can be stratified according to prognostic variables. The role of adjuvant therapies for patients with poor prognostic factors needs to be evaluated in a multi-institutional setting.
BACKGROUND: Biliary papillomatosis (BP) is a rare disease that is characterized by multiple numerous papillary adenomas in the biliary tree. The clinical features and outcome, however, are not well known. The authors retrospectively analyzed their clinicopathologic features and long-term follow-up results. METHODS: Between March 1995 and January 2003, 58 patients were diagnosed with BP by cholangioscopic and histologic findings at a tertiary referral center, Asan Medical Center (University of Ulsan College of Medicine, Seoul, Korea). The authors retrospectively reviewed the medical records to obtain demographic, radiologic, cholangioscopic, and pathologic data. RESULTS: The common clinical manifestations at the presentation of patients were repeated episodes of abdominal pain, jaundice, and acute cholangitis. Acute cholangitis was more common in patients with mucin-hypersecreting BP (MBP), whereas patients with nonmucin-producing BP (NMBP) were more asymptomatic (P < 0.05). Papillary adenocarcinoma and mucinous carcinoma were detected in 48 patients (83%) with papillary adenomas. Overall survival rates of NMBP and MBP were 89% and 69% at 1 year, 57% and 37% at 3 years, and 52% and 19% at 5 years, respectively. The mean survival period of NMBP and MBP was 52.27 +/- 6.72 months and 30.84 +/- 8.36 months, respectively. CONCLUSIONS: BP should be regarded as a premalignant disease with high malignant potential. The pathogenesis of progression from benign to malignant disease may follow the adenomacarcinoma sequence. Although clinical presentations were somewhat different for patients with NMBP and MBP, the long-term survival rate was similar.
We report a patient who presented with a purpuric rash and polyarthritis, but IgA deposits were not found in the skin. Abdominal pain and renal disease first emerged 1 and 2 weeks, respectively, after presentation, to reveal the classic tetrad of Henoch-Schönlein purpura. Our patient emphasises the need for careful follow-up in patients with cutaneous vasculitis, as they may develop systemic manifestations, of which renal involvement, particularly, may be asymptomatic.