Desmoplastic small round-cell tumor: an adult with previous exposure to agent orange.

Desmoplastic small round-cell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. It is very unusual in the elderly population. Although Agent Orange has been associated with soft-tissue sarcoma, an association with desmoplastic small round-cell tumor has not been reported. A 52-year-old male presented with abdominal distention, dyspnea, and a 9 kg weight loss. Prior history was significant for hepatitis C and diabetes. He was a Vietnam veteran and he admitted being exposed to Agent Orange. On physical examination, the abdomen was distended and tense. Computed tomography scan of the chest, abdomen and pelvis demonstrated extensive mediastinal and retroperitoneal adenopathy, diffuse omental masses and extensive pleural, intra-abdominal and pelvic ascites. Omental core needle biopsy was consistent with desmoplastic small round-cell tumor based on morphology and immunohistochemistry. He responded poorly to chemotherapy with high-dose cyclophosphamide, doxorubicin and vincristine and died 5 months after presentation secondary to neutropenic sepsis despite G-CSF support and antibiotics.

A phase I/II trial of fixed-dose docetaxel plus irinotecan and escalating doses of estramustine phosphate for second-line or greater treatment of selected advanced solid tumors.

This phase I/II study evaluated the safety of the combination of irinotecan, docetaxel, and estramustine for selected advanced solid tumors and also obtained initial efficacy data. Twenty-two patients were enrolled in the study. The regimen consisted of docetaxel 30 mg/m(2) and irinotecan 60 mg/m(2) both given intravenously on days 1 and 8 every 21 days in combination with escalating doses of estramustine (500 mg/m(2)/day escalated to 750 mg/m(2)/day on days 0, 1, 2, 7, 8, and 9 given every 21 days) during phase I. Dose escalation was continued until the maximum planned dose level of estramustine (750 mg/m(2)/day) was reached. After the appropriate phase II dose of estramustine was found additional patients were enrolled. Twenty-one of the 22 patients were evaluable for toxicity and 17 for tumor response. The recommended phase II dose of estramustine was found to be 750 mg/m(2)/day orally on days 0, 1, 2, 7, 8, and 9 given every 21 days. Hematologic toxicity was fairly mild, with only one episode of grade 3 neutropenia. Diarrhea was the most common nonhematologic toxicity with grade 3 toxicity occurring in five of 21 patients. Only one episode of venous thrombosis was observed. Objective response rate was 15.8%, overall clinical benefit rate was 63%, and median time to progression was 15 weeks. Estramustine in combination with the doublet of docetaxel and irinotecan is a well-tolerated regimen with minimal hematologic toxicity, mild to moderate nonhematologic toxicity, and promising initial antitumor activity in previously treated patients with advanced solid tumors.

Achievement of three year remission with bevacizumab and irinotecan in recurrent glioblastoma multiforme: a case report.

A 34-year-old man presented to the hospital with right-sided headache. He was diagnosed with GBM. He underwent resection of the tumor with placement of carmustine impregnated wafers. Then he underwent adjuvant chemotherapy with temozolamide. Before the completion of chemotherapy he had a recurrence. He underwent re-resection with placement of carmustine impregnated wafers. Subsequently he had eighteen cycles of salvage biochemotherapy with bevacizumab and irinotecan. To date, routine MRI scans of the brain have not shown evidence of recurrence. He continues to be in remission three years after treatment with bevacizumab and irinotecan.

A case of metastatic mucoepidermoid carcinoma complicated by resistant hypercalcemia.

Tumors of salivary glands are uncommon and comprise of about 2%-4% of all head and neck tumors. About 75%-80% of these tumors are benign and include pleomorphic adenoma, monomorphic adenoma, oncocytoma, and papillary cystadenoma lymphomatosum. Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands, representing 5-10% of all salivary gland tumors. Although known to be metastatic to local lymph nodes, distant metastases are rare (especially, with low and intermediate grade tumors). Histologic grade and the expression of various mucin glycoproteins are useful prognostic indicators. We present a case of mucoepidermoid carcinoma of parotid gland origin with distant metastases which is an uncommon occurrence with intermediate grade tumors. Also, this is the first reported case of humoral hypercalcemia of malignancy secondary to mucoepidermoid carcinoma.

Precursor B-cell acute lymphoblastic leukemia presenting as obstructive jaundice: a case report.

INTRODUCTION: Acute leukemias very rarely present with jaundice. Herein we report a case of precursor B-cell acute lymphoblastic leukemia that presented with jaundice in an adult. CASE PRESENTATION: A 44-year-old Hispanic man presented with right upper quadrant abdominal pain and jaundice. His initial blood work revealed pancytopenia and hyperbilirubinemia. Direct bilirubin was more than 50% of the total. His imaging studies were unremarkable except for hepatomegaly. All blood screening tests for various hepatocellular etiologies were normal. A diagnosis of precursor B-cell acute lymphoblastic leukemia was made upon liver biopsy. It also showed lymphocytic infiltration of the hepatic parenchyma leading to bile stasis. The diagnosis was subsequently confirmed upon bone marrow biopsy. The patient was treated with a hyperfractionated cyclophosphamide/vincristine/doxorubicin/dexamethasone regimen. CONCLUSION: Acute lymphoblastic leukemia should be one of the differential diagnoses that should be considered when initial work-up for jaundice is inconclusive. Some cases of acute lymphoblastic leukemia have been reported in both adults and children to have presented with the initial manifestation of jaundice, but only a few had no radiographic evidence of biliary obstruction. Such presentation can pose a serious diagnostic dilemma for clinicians. This manuscript attempts to highlight it. Moreover, we believe that if acute lymphoblastic leukemia presentations similar to this case continue to be reported in adults or children, a specific immunophenotypic expression and cytogenetic abnormality may be found to be associated with hepatic infiltration by leukemia. This may substantially contribute to the further understanding of the pathophysiology of this hematologic disease.

Fasting hyperglycemia upon hospital admission is associated with higher pneumonia complication rates among the elderly.

BACKGROUND: Hyperglycemia is an independent predictor of adverse outcomes during hospitalization. In patients who have pneumonia, significant hyperglycemia is associated with poor outcomes. This study evaluates the interaction of the degree of hyperglycemia and complication rates stratified by age in non-critically ill patients admitted to the hospital for care of community-acquired pneumonia. METHODS: Retrospective review of patient records coded for pneumonia. Analysis included 501 non-critically ill patients admitted to a tertiary care hospital in New York City. Data were stratified by diabetes status, age (less than 65 and 65 and over), and fasting blood glucose (FBG) within the first 24 hours of hospitalization. Among patients with no history of diabetes, FBG was stratified as "normal" [FBG /=126 mg/dl (7 mmol/l)]. The diabetic group included known diabetics regardless of FBG. The Pneumonia Severity Index (PSI) was calculated for all patients. Complications rates, hospital length of stay and mortality were compared among the groups. RESULTS: In patients age 65 and older, complication rates were 16.7% in normoglycemics, 27.5% in the "mild-hyperglycemia" group, 28.6% in the "severe hyperglycemia" group, and 25.5% in those with known diabetes. The mild and severe-hyperglycemics had similar complication rates (p = 0.94). Compared to the normal group, mild and severe groups had higher rates of complications, p = 0.05 and p = 0.03, respectively. PSI tended to be higher in those over the age of 65. PSI was not significantly different when the normal, mild, severe, and known diabetes groups were compared. PSI did not predict complications for new hyperglycemia (normals' mean score 87, mild 84.7, severe 93.9, diabetics 100). Hospital mortality did not differ among groups. Length of stay was longer (p = 0.05) among mild-hyperglycemics (days = 8.4 s.e. 14.3) vs. normals (days = 6.2 s.e.6.5). CONCLUSION: This study shows that FBS between 101-125 mg/dl (5.7-6.9 mmol/l) on hospital admission increases pneumonia complication rates among the elderly with no previous diagnosis of diabetes.