BackgroundPrimary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.MethodsFrom 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.ResultsOne patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.ConclusionWe have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.
Primary undifferentiated embryonal sarcoma of the liver is a rare tumor with a peak incidence between the ages of 6 and 10 years. We report a case of a primary hepatic undifferentiated embryonal sarcoma arising in a 21-year-old male mistaken for hydatid disease of the liver. The rapid recurrence of this tumor along the site of attempted percutaneous drainage illustrates some important management points regarding this malignancy.
Celiac disease (CD) rarely presents with life-threatening complications in older individuals. We report a 64-year-old woman who presented with profuse diarrhea, weight loss, hemodynamic instability, hypokalemia, hypoproteinemia, acidosis, and vitamin and iron deficiency. Pathologic and serologic studies confirmed CD presenting with celiac crisis with extensive and severe intestinal disease. Although celiac crisis occurs mostly in childhood and early adulthood, it should be considered in adults presenting with acute severe diarrheal illness, electrolyte abnormalities, and malabsorption.
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