[Adult T-cell leukemia/lymphoma with multiple intracranial masses and CMV and HHV-6 reactivation at initial presentation].

A 55-year-old male was referred to our hospital after complaining of a sore throat for a month. Physical examination revealed a disturbance in consciousness, nuchal rigidity, painful multiple ulcers in the oral cavity, and erythema, the size of rice grains on the body. Hematological examination showed the following results: white blood cells, 7,910/µl (abnormal lymphocytes 2%), LDH, 203 U/l, corrected calcium, 11.2 mg/dl, soluble IL-2 receptor, 11,800 U/ml, and cytomegalovirus antigenemia assay (C10, C11) 43/49. Abnormal lymphocytes (CD4+CD25+) were discovered in the peripheral blood, bone marrow, and skin samples. Southern blotting of peripheral blood revealed monoclonal integration of human T-cell leukemia virus type 1 (HTLV-1) provirus DNA; and consequently, he was diagnosed with adult T-cell leukemia/lymphoma (ATLL). Multiple tumors with ringed contrast effect were observed in the brain parenchyma using contrast-enhanced computed tomography. The cell number in the cerebrospinal fluid was 1,320/mm3 (ATLL cells were 79% in flow cytometry), and the protein level was 244 mg/dl; moreover, the examination revealed a positive result for human herpesvirus 6 DNA. Despite herpesvirus genus treatment and modified LSG15 therapy combined with intrathecal chemotherapy, the patient became comatose and died on day 21 of hospitalization. A better understanding of the pathogenesis of ATLL, and the involvement with the central nervous system is needed along with the development of standard treatment.

Neuroendocrine carcinoma of the pancreas with similar genetic alterations to invasive ductal adenocarcinoma.

Neuroendocrine carcinoma (NEC) of the pancreas is very rare, and its origin is not fully elucidated. Here, we present a case of a small-size NEC of the pancreas that is genetically similar to invasive ductal adenocarcinoma (IDA). A 65-year-old man was referred to our hospital due to obstructive jaundice and found to have a 12-mm solid tumor in the pancreas head. The tumor exhibited low vascularity on enhanced computed tomography, and endoscopic retrograde pancreatographic imaging revealed an irregular obstruction in a branch duct of the pancreas. The patient was thereby diagnosed with a pancreatic ductal cancer, and stomach-preserving pancreaticoduodenectomy with regional lymph node resection was performed. Histochemical analysis of the resected tumor showed that the neoplastic cells with scanty cytoplasm and hyperchromatic nuclei strongly expressed chromogranin A and synaptophysin. The Ki-67 index was 40 % in the most proliferative tumor regions, and the tumor was diagnosed as a NEC of the pancreas. However, in the analysis of genetic alterations of the tumor tissue, the neoplastic cells showed altered KRAS, TP53, and SMAD4/DPC4, suggesting that the NEC in our case is genetically related to IDA. Our data suggest that poorly differentiated IDAs may transform into NECs.