A Cribriform Cancer Metastatic to Liver: Case Report and Literature Review.

Metastasis from salivary gland tumors to liver is exceedingly uncommon. Reported is the first case of a mammary analog secretory carcinoma (MASC) of salivary gland origin metastasized to the liver, even after complete surgical resection. A 76 year old female, with past history of a completely extirpated right parotid gland MASC, presented 2 years after right superficial parotidectomy and right neck dissection, with back and flank pain. Subsequent abdominal and pelvic CT revealed multiple small hepatic lesions. Biopsy of the largest hepatic lesion confirmed metastatic MASC of primary parotid gland origin. Both the parotid primary and the hepatic metastases had the confirmatory ETV6 rearrangement by fluorescence in situ hybridization. Although high-grade malignancy and distant metastases of MASC of salivary gland origin to liver is rare, recognizing metastatic MASC potentially alters prognosis and determines therapeutic options.

Acroparesthesia in a female: diagnostic dilemma.

Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of a-galactosidase A (also known as ceramide trihexosidase) and resultant accumulation of globotriaosylceramide (Gb3) and related glycophospholipids. The disease affects nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems leads to renal and heart failure; debilitating pain as a result of nervous system involvement also occurs.

Irinotecan, carboplatin, and imatinib in untreated extensive-stage small-cell lung cancer: a phase II trial of the Minnie Pearl Cancer Research Network.

INTRODUCTION: The tyrosine kinase KIT has variable expression in small-cell lung cancer (SCLC) and may be a prognostic factor. Imatinib targets KIT expression, providing rationale for studying its role in combination with chemotherapy in SCLC in a multicenter phase II trial. METHODS: Patients with untreated extensive-stage SCLC received carboplatin area under the concentration-time curve of 4 on day 1; irinotecan 60 mg/m2 on days 1, 8, and 15; and imatinib 600 mg/day. Treatment cycles were 28 days. Patients remained on imatinib until progressive disease or significant toxicity. RESULTS: Between September 2002 and May 2004, 68 patients were enrolled in this multicenter trial. Median age was 60 years (range, 37-81). The objective response rate was 66% (95% confidence interval: 54%-76%). Median progression-free survival was 5.4 months (95% CI: 4.3-6.0 months). Median overall survival was 8.4 months (95% CI: 6.3-10.5 months). Thirty-five percent of patients were alive at 1 year. Grade 3/4 hematologic toxicity included neutropenia (43%), anemia (16%), and thrombocytopenia (9%). Grade 3 nonhematologic toxicity included diarrhea (19%), fatigue (24%), and nausea (26%). Forty-eight of 56 patients (86%) with available tumor specimens had KIT expression detected. KIT expression did not appear to correlate with progression-free survival or overall survival in a retrospective analysis. CONCLUSIONS: Irinotecan, carboplatin, and imatinib is a safe and generally well-tolerated regimen in patients with SCLC. However, the addition of imatinib did not improve results from those expected with chemotherapy alone.

Hyperhomocysteinemia and cobalamin deficiency in young Asian Indians in the United States.

Hyperhomocysteinemia, a risk factor for vascular disease, may be a particular problem in Asian Indians, but information is limited, especially in the U.S., despite its growing Asian population. Moreover, suggestions have been made that folate deficiency is responsible for the hyperhomocysteinemia in Indians. Therefore, we studied homocysteine status in healthy Asian Indians in the U.S. prospectively, determined the frequency of cobalamin and folate deficiency as contributors to it, and examined whether food-cobalamin absorption contributed to cobalamin deficiency. Homocysteine levels were higher in Asian Indian men than in 4 other ethnic groups (P < 0.0001); 10/39 Indian men (25.6%) were hyperhomocysteinemic. Cobalamin levels were lower in Indian men (P = 0.000005) and women (P = 0.03) than in non-Indians; low levels were found more frequently in both Indian men (23/39; 59.0%) and women (5/21; 23.8%) than in others. Measuring methylmalonic acid in 10 selected subjects showed that the low cobalamin levels reflected cobalamin deficiency, and high methylmalonic acid levels were found in some subjects without hyperhomocysteinemia. Evidence of folate deficiency was not found in any subjects. Food-cobalamin absorption was normal in all 13 Indian subjects tested, including those with Helicobacter pylori infection. The results show that hyperhomocysteinemia is strikingly common in apparently healthy, young Asian Indian men. The cause appears to be cobalamin deficiency, which affected more than half of the Indian men, may be largely subclinical, is underestimated by homocysteine levels alone which were not always abnormal, and is probably largely dietary in origin. Folate deficiency is rare. This public health problem is amenable to prevention and treatment in this growing segment of the U.S. population. It was, parenthetically, noteworthy that many of the affected subjects were young physician trainees.