Remission of paraneoplastic dermatomyositis associated with hepatocellular carcinoma under prednisolone and azathiopin, and concommittant sorafenib.

BACKGROUND: Hepatocellular carcinoma (HCC) is one of the most common tumor diseases worldwide. Dermatomyositis is a known paraneoplastic syndrome that can complicate the course of a variety of different cancers, however, the association with HCC is extremely rare. CASE REPORT: Here, we report on a patient with the rare concurrence of dermatomyositis and non-hepatitis-associated advanced HCC with intra-abdominal and intrathoracal lymph node metastases. The HCC was treated with sorafenib. The dermatomyositis responded well to treatment with prednisolone and azathioprin although sorafenib did not lead to a response in the underlying HCC. CONCLUSIONS: Paraneoplastic dermatomyositis can be associated with non-hepatitis-associated HCC. The potential pathogenetic links between these two diseases are discussed, as well as a potential immunomodulatory effect of sorafenib independent of its antineoplastic potential.

Symptomatic hyponatraemia caused by cylophosphamide.

BACKGROUND: Acute hyponatraemia after administration of alkylating agents such as cyclophosphamide or ifosfamide has been documented as an infrequent but life-threatening complication. CASE REPORT: A 69-year-old female patient with metastatic adenocarcinoma of the salivary glands presented with severe symptomatic hyponatraemia (nadir 112 mmol/l) after chemotherapy with cyclophosphamide, cisplatin, and doxorubicin. Serum sodium was carefully corrected at the intensive care unit. The patient recovered completely from her neurological symptoms within a couple of days. A literature review showed only few cases with cyclophosphamide-induced acute hyponatraemia, and to our knowledge this is the first case where hyponatraemia was seen with a dose of only 500 mg/m(2) of cyclophosphamide. CONCLUSION: Oncologists should be aware of cyclophosphamide-induced acute hyponatraemia as a rare but life-threatening side-effect, especially since its clinical features may mimic those of chemotherapy-induced nausea.

Ep-CAM expression in squamous cell carcinoma of the esophagus: a potential therapeutic target and prognostic marker.

BACKGROUND: To evaluate the expression and test the clinical significance of the epithelial cellular adhesion molecule (Ep-CAM) in esophageal squamous cell carcinoma (SCC) to check the suitability of esophageal SCC patients for Ep-CAM directed targeted therapies. METHODS: The Ep-CAM expression was immunohistochemically investigated in 70 primary esophageal SCCs using the monoclonal antibody Ber-EP4. For the interpretation of the staining results, we used a standardized scoring system ranging from 0 to 3+. The survival analysis was calculated from 53 patients without distant metastasis, with R0 resection and at least 2 months of clinical follow-up. RESULTS: Ep-CAM neo-expression was observed in 79% of the tumors with three expression levels, 1+ (26%), 2+ (11%) and 3+ (41%). Heterogeneous expression was observed at all expression levels. Interestingly, tumors with 3+ Ep-CAM expression conferred a significantly decreased median relapse-free survival period (log rank, p = 0.0001) and median overall survival (log rank, p = 0.0003). Multivariate survival analysis disclosed Ep-CAM 3+ expression as independent prognostic factor. CONCLUSION: Our results suggest Ep-CAM as an attractive molecule for targeted therapy in esophageal SCC. Considering the discontenting results of the current adjuvant concepts for esophageal SCC patients, Ep-CAM might provide a promising target for an adjuvant immunotherapeutic intervention.

Direct genetic analysis of single disseminated cancer cells for prediction of outcome and therapy selection in esophageal cancer.

The increasing use of primary tumors as surrogate markers for prognosis and therapeutic decisions neglects evolutionary aspects of cancer progression. To address this problem, we studied the precursor cells of metastases directly for the identification of prognostic and therapeutic markers and prospectively analyzed single disseminated cancer cells from lymph nodes and bone marrow of 107 consecutive esophageal cancer patients. Whole-genome screening revealed that primary tumors and lymphatically and hematogenously disseminated cancer cells diverged for most genetic aberrations. However, we identified chromosome 17q12-21, the region comprising HER2, as the most frequent gain in disseminated tumor cells that were isolated from both ectopic sites. Survival analysis demonstrated that HER2 gain in a single disseminated tumor cell but not in primary tumors conferred high risk for early death.