Nested stromal epithelial tumor of liver presenting with cushing syndrome: A rare case report.

Nested stromal and epithelial tumor of the liver is an extremely rare pediatric hepatic tumor. To the best of our knowledge, about 25 cases have been reported in the English literature so far, few of which accompanied with Cushing syndrome. Herein we report our experience with an 8-year-old boy presented with Cushing's syndrome because of ectopic ACTH production by this tumor.

Placental morphology in pre-eclampsia and eclampsia and the likely role of NK cells.

Background: Placenta has long been a neglected organ as far as its pathology is concerned. This study is an attempt to observe the morphological features of placentae both gross and microscopic in normal pregnancy, preeclampsia, and eclampsia. Materials and Methods: A total of 150 placentae were collected; of these, 50 belonged to normal pregnancy, 50 belonged to pre-eclamptic women, and 50 were from patients of eclampsia. Results: Placental trimmed weight was seen to be decreased in patients of preeclampsia and eclampsia. Placental infarcts were more commonly seen in the diseased group and they were more centrally located. Hypertrophy of the spiral arterioles was observed in the decidual portion found in placental disc and membranes. Distal villous hypoplasia was also frequently seen in the diseased group. Fetal membranes were thickened and showed infarcts in preeclampsia and eclampsia. Conclusions: Morphological features seen in eclamptic placentae were similar but exaggerated compared to preeclampsia. In conclusion, the pathological changes were found to be more severe and frequent in preeclampsia and eclampsia, but more so in eclamptic placentae as compared with placenta of normal pregnancy. CD56 immunomarker was also used to identify NK cells. They were found to be present only in the diseased group and were located in the decidual portion of the basal plate, implicating their role in the development of the disease.

Extrapulmonary Small Cell Carcinoma of the Liver: Clinicopathological and Immunohistochemical Findings

Patients with primary small cell carcinoma of the liver have rarely been described in medical literature. Knowledge of clinical, pathological and immunohistochemical properties remains limited. We described an 82-year-old female patient with primary small cell carcinoma of the liver. Histologically, the tumor showed typical morphology of a pulmonary small cell carcinoma. Immunohistochemically, the tumor revealed neuroendocrine differentiation; positive reaction for chromogranin, synaptophysin, CD56, and neuron specific enolase. The tumor was also positive for TTF-1 and c-kit but completely negative for hepatocyte, carcinoembryonic antigen, cytokeratin 7; 19; and 20. Herein, we discussed the clinical, pathological and immunohistochemical findings of extrapulmonary small cell carcinoma of the liver and reviewed the relevant literature.

CD4+CD56+Lineage Negative Hematopoietic Neoplasm: So Called Blastic NK Cell Lymphoma

Blastic natural killer (NK) cell lymphoma is a rare neoplasm characterized by blastoid tumor cells expressing CD4 and CD56, with predominant skin involvement. Although this tumor has been regarded as a neoplasm related to NK cell, recent studies suggested that it is derived from plasmacytoid dendritic cells, but not from NK cell. Herein we report 4 cases of CD4+CD56+ lineage marker- blastic NK cell lymphomas with a review of literatures. The patients were 3 men and one woman. Three of them were young (17, 18, and 22 yr old). Three patients had skin lesions, at initial presentation in two patients and during the course of disease in other patient. Histologically, tumors consisted of monotonous medium to large blastoid cells showing no necrosis, angiocentric growth or epidermotrophism. All four tumors were CD4+ and CD56+. Three expressed CD68 antigen. Lineage specific markers for B- and T cell were negative. All tumors did not express myeloperoxidase. T-cell receptor gene rearrangement, EBV, CD13 and CD33 were negative. In one patient, tumor cells arranged in Homer-Wright type pseudorosette and expressed terminal deoxynucleotidyl transferase(TdT). Despite the standard lymphoma chemotherapy, the tumors, except one lost during follow-up, progressed and relapsed. The patients died 8-60 months after diagnosis.

Extranasal T/NK-cell lymphoma presenting as intestinal diverticulum

A case of intestinal angiocentric T/NK-cell lymphoma in a 58-year-old man is reported. The patient presented initially with panperitonitis because of perforation of sigmoid colon diverticulum. He underwent segmentectomy of involved bowel. Histologically, the intestinal wall showed diffuse infiltration of medium or large size lymphoma cells with angiocentric growth and necrosis. The lymphoma cells were CD56+, CD45RO+, CD3+, CD4-, CD8-, CD20-, and CD30- in paraffin sections with germline configuration of TCR-gamma gene, consistent with T/NK-cell lymphoma. Further staging revealed splenomegaly. Intestinal angiocentric T/NK cell lymphoma represents a distinct etiology of diverticulum with perforation.