Poorly differentiated, neuron-specific enolase positive round cell tumor with two translocations t(11;22) and t(21;22).

The authors describe a highly malignant, disseminated round cell tumor originating in the ninth rib of a 14-year-old boy. Extensive studies by means of light and electron microscopic examination, histocytochemistry and immunocytochemistry and cytogenetic analysis revealed an undifferentiated, neuron-specific enolase positive round cell tumor with a unique karyotype: 45,XY,-21,t(11;22)(q23;q11), der(22)t(21;22)(q11.2;p11).

Immunocytochemical markers of uncommon pancreatic tumors. Acinar cell carcinoma, pancreatoblastoma, and solid cystic (papillary-cystic) tumor.

Nine acinar cell carcinomas of the pancreas, 2 pancreatoblastomas, 16 solid-cystic (papillary-cystic) tumors, and 20 ductal adenocarcinomas were immunocytochemically investigated using antisera against four pancreatic enzymes (alpha-amylase, lipase, trypsinogen, chymotrypsinogen), four pancreatic hormones, neuron specific enolase (NSE), alpha-1-antitrypsin (AAT), carcinoembryonic antigen (CEA), and CA 19-9. Lipase, trypsinogen, and chymotrypsinogen, but no alpha-amylase were detected in all acinar cell carcinomas and pancreatoblastomas. In contrast, solid-cystic tumors (SCT) were negative for pancreatic enzymes but 2 of 16 stained with NSE.

Interferon-alfa corrects thrombocytosis in patients with myeloproliferative disorders.

During previous therapeutic trials with interferon, decreased levels of peripheral platelet counts have been observed. Taking advantage of this effect, we investigated the efficacy of recombinant interferon (rec-IFN) in the treatment of thrombocytosis in myeloproliferative diseases. A total of 15 patients with polycythemia vera, essential thrombocytosis, or chronic myeloid leukemia received rec-IFN-alfa at initial doses of 25-70 x 10(6) units/week; maintenance therapy following week 8 of treatment consisted of 20-35 x 10(6) units/week rec-IFN.

Neuron specific enolase demonstration in the diagnosis of a solid-cystic (papillary cystic) tumour of the pancreas.

Immunoreactivity to neuron specific enolase (NSE) was demonstrated in a solid-cystic (papillary cystic) tumour of the human pancreas, employing immunohistochemical methods. Positive staining for NSE was found with two different antisera. In addition, sodium-dodecyl-sulphate-polyacrylamide-gel-electro-phoresis (SDS-PAGE) of tumour homogenate revealed a distinct band reacting with a NSE antiserum.

[Prognosis of malignant tumors of the exocrine pancreas: effect of clinical and pathologico-anatomic variables on patient survival].

Survival patterns of 112 patients with histologically ascertained pancreatic cancer were analysed retrospectively in an attempt to determine the relationship to various clinical and pathologic-anatomic prognostic factors. Stage of disease, localisation of the primary tumor, as well as histologic grade were found to influence the patient's survival significantly. A limited anatomic involvement with tumor, localisation within the head of the pancreas, and high-grade differentiation were associated with an increased median survival.

[Angiolymphoid hyperplasia with eosinophilia (Kimura disease)].

A case of a rare disease, known as angiolymphoid hyperplasia with eosinophilia, or Kimura's disease, is described in order to discuss the histopathology, aetiology and differential diagnosis by means of literature. The clinical symptomatology consisting of the appearance of cutaneous or subcutaneous tumours is reported as well as the histological features. The therapy of choice is the surgical treatment of the tumour, whereas in most cases radiation or large-dosed therapy with corticosteroids leads to remissions only.

[DiGeorge syndrome--significance of early diagnosis in cellular immunodeficiency].

Di George syndrome is caused by anomalous development of the organs arising from the third and fourth pharyngeal pouches and results in congenital aplasia of the thymus, aplasia or hypoplasia of the parathyroid glands and cardiovascular malformations. Clinically, affected children show hypoparathyroidism and, because of depressed cell-mediated immunity, serious bacterial, viral and fungal infections. We present an infant, aged 6 weeks, with convulsions due to hypocalcemia, in which cell-mediated immunodeficiency was detected.