Etanercept therapy in patients with advanced primary amyloidosis.

No effective treatment exists for primary amyloidosis, a plasma cell dyscrasia characterized by deposition of amyloid fibrils consisting of monoclonal light chains in various organs. TNF-alpha has been implicated in other amyloid disorders; therefore, we used etanercept to treat patients with advanced amyloidosis who had failed other therapies or were ineligible for other treatment regimens. Sixteen patients with amyloidosis that included patients with severe cardiac or multiple organ involvement were treated with etanercept and evaluated every 4-6 wk for evidence of toxicity and clinical response. Patients were treated with etanercept for a median of 42 wk. Eight of 16 patients (50%) experienced objective improvements and 14 patients (88%) experienced subjective improvements in symptoms. Only one patient experienced an adverse effect attributable to etanercept. For the entire group, improvement in performance status was statistically significant (p = 0.001), estimated median survival is 24.2 mo, 8 of whom are still alive with a median survival is 26.6 mo. The 12 patients with any cardiac involvement had an estimated median survival of 24.2 mo. Six of those 12 patients are still alive, with a median survival is 26.6 mo. The group of eight patients with severe cardiac involvement showed an estimated median survival of 13.2 mo, three of whom are still alive with a median survival is 25.9 mo. The clinical observations in this group of advanced and relapsed/refractory patients are highly encouraging. For the group as a whole, median survival was 24.2 mo and improvement in performance status was highly significant. Median survival for the patients with severe cardiac involvement was 13.2 mo with 3/8 patients are alive with a median survival of 25+ mo. Moreover, there was a statistically significant improvement in patients' performance status. These results, even though in a small group of patients, suggest that etanercept may provide a new therapeutic option for the management of amyloidosis that should be studied further.

A case of biliary carcinoid presenting with pancreatitis and obstructive jaundice.

A 43-yr-old man presented to the clinic with abdominal pain, jaundice, nausea, and vomiting and weight loss over a 6-month period. Physical exam was unrevealing other than mild epigastric tenderness. A computed tomographic scan of the abdomen revealed a mass in the head of the pancreas, which was resected at laparotomy by a Whipple's procedure. The histology showed a biliary tract carcinoid tumor. The patient had normal hydroxy-indole-acetic acid (HIAA) levels throughout. There has been no evidence of disease or tumor recurrence at 3.5 yr of follow up.

Squamous-cell carcinoma of the colon responsive to combination chemotherapy: report of two cases and review of the literature.

PURPOSE: The majority of colorectal neoplasms diagnosed are adenocarcinomas. Other histologies such as squamous, adenosquamous, carcinoid tumors, or lymphoid tumors are occasionally identified. Given the rarity of squamous-cell tumors, it is very difficult to study their natural course and response to therapy. An attempt is made to describe the frequency, anatomic location, and response to therapy with a review of the literature. METHODS: From the Cancer Registry at the University of Missouri-Columbia Ellis Fischel Cancer Center, tumors of the colon identified above the dentate line were selected for chart review. Data were extracted from cases between the years 1940 and 1996. The key terms used to identify cases were epidermoid, squamous cell, and cancer of the rectum or colon. Using this approach, forty patients were identified and each record was reviewed. RESULTS: The majority of these cases were anal cancers with proximal extension into the rectum and were excluded. Of 4,561 cases of epithelial colon and rectal cancers identified, only one additional case of squamous-cell cancer could be verified. In this report we describe a patient with a primary squamous-cell carcinoma of the sigmoid colon with metastatic disease to the liver at diagnosis who responded to systemic chemotherapy. We believe this to be the first reported case of this rare tumor type in which the patient's tumor responded to systemic chemotherapy. Two cases with a thorough review of literature are presented. CONCLUSIONS: Primary squamous-cell carcinoma of the colon is a rare malignancy of unknown cause and pathogenesis. Metastatic tumors to the colon should be ruled out in all cases before therapy. Early detection and surgery remain the main therapeutic options, but as presented in our case, response to chemotherapy in advanced disease is encouraging.