Background: Hypereosinophilic syndrome is a rare condition, which is connected with a variety of diseases. Very infrequently, it occurs in association with malignancy denoting widespread metastases and poor prognosis. We describe a patient with hypereosinophilia and hypercalcemia as a manifestation of disseminated cholangiocarcinoma.
Case description: A 54-year-old man with a history of alcoholic hepatitis for the last 10 years was admitted due to severe fatigue, diffuse abdominal pain, flatulent abdomen and dyspnoea. The symptoms had gradually deteriorated over the past 20 days. Examination showed tense ascites, expiratory wheezing and lower extremity oedema. The following laboratory parameters were of note: total white blood cell count of 14010/μl, 12.4% eosinophils (absolute count: 1737/μl), elevated serum calcium (11.13 mg/dl), normal serum phosphate, low parathyroid hormone (PTH) levels (4.40 pg/ml), negative hepatitis B and C virus serology, normal Alpha-Fetoprotein and extremely elevated CA 19.9 (3666 U/ml). Imaging techniques (chest X-rays, CT of the thorax, abdominal ultrasound, CT and MRI) revealed cirrhotic liver with a 13.5×12.5 cm lesion in the right lobe, ascites, enlarged intra-abdominal lymph nodes, peritoneal carcinomatosis, diffuse lung metastases and left-sided pleural effusion. Both diagnostic thoracentesis and abdominal paracentesis yielded bloody effusion with positive cytology for adenocarcinoma. Cholangiocarcinoma was diagnosed by liver biopsy. The hypereosinophilic syndrome responded to corticosteroid treatment. The patient died of acute respiratory failure on the 20th day of hospitalization.
Discussion: The association of cholangiocarcinoma with hypereosinophilic syndrome and hypercalcaemia is extremely rare and might account for the poor prognosis in this case. Hypercalcaemia combined with low PTH levels could be explained by the PTH-related protein secretion from cancer cells.
01 - 05 Apr 2006
European Society of Endocrinology