muramidase and Teratoma

The relatively frequent association of hematologic neoplasia and primary mediastinal germ cell tumors has been reported. Of these hematologic malignancies, nine were classified as malignant histiocytosis or acute monoblastic leukemia, and all occurred in males. We now report on a patient who was phenotypically female, with 46XY gonadal dysgenesis, and who developed a true histiocytic malignancy that presented as a large hepatic tumor and also involved the spleen, right kidney, and lymph nodes. Twenty-six months before the development of the histiocytic malignancy, an ovarian malignant teratoma with yolk sac elements was removed; the patient subsequently received chemotherapy. The neoplasm was composed of large pleomorphic cells and the histiocytic nature was established by cytologic, cytochemical, immunologic, and ultrastructural studies. In the course of her illness, the patient developed classic acute monoblastic leukemia 8 months after the diagnosis of histiocytic malignancy. Karyotypic analysis of the hepatic tumor, bone marrow, and blood showed 46XY gonadal dysgenesis. We believe that this is the first reported case of a phenotypically female patient who developed these two rare malignancies. It suggests that the association between germ cell tumors and histiocytic malignancy in genotypically male individuals may not be coincidental or secondary to therapy, but may be a phenomenon related to dysgenetic gonads in the presence of a Y chromosome.

Topics: Adolescent; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Biomarkers, Tumor; CD11 Antigens; DNA, Neoplasm; Female; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; Gonadal Dysgenesis, 46,XY; Histiocytic Sarcoma; Humans; Immunohistochemistry; Karyotyping; Liver; Lymphocytes; Muramidase; Ovarian Neoplasms; Phenotype; S100 Proteins; Teratoma

A testicular malignant teratoma containing embryoid bodies and other embryonic and extra-embryonic structures in various stages of development has been examined by several histochemical and immunohistological techniques to study the distribution of various substances in the teratomatous elements. The substances demonstrated included various types of mucins; argyrophil, argentaffin, Paneth cells and haemosiderin granules; alpha-fetoprotein, alpha-l-anti-trypsin, lysozyme, beta-HCG and CEA. The significance of the findings is discussed in relation to early embryonic development.

Topics: Adult; alpha 1-Antitrypsin; alpha-Fetoproteins; Carcinoembryonic Antigen; Chorionic Gonadotropin; Chorionic Gonadotropin, beta Subunit, Human; Histocytochemistry; Humans; Immunologic Techniques; Male; Mucins; Muramidase; Peptide Fragments; Teratoma; Testicular Neoplasms

Paneth cells occurring in intestinal and extraintestinal sites were studied using various light microscopic techniques, including standard hematoxylineosin (HE)-stained histologic sections; a number of histochemical reactions, including the phosphotungstic acid-hematoxylin (PTAH) method; and immunohistochemical labeling for the presence of lysozyme using the peroxidase-antiperoxidase (PAP) method. Cells, identified on HE as Paneth cells, uniformly stained with PTAH and PAP-lysozyme. Further, all of the cases demonstrating epithelial cell lysozyme showed eosinophilic cytoplasmic granules, typical of Paneth cells, on HE sections. Lysozyme was demonstrated in benign and malignant ovarian tumors, as well as in a variety of ectopic intestinal sites and benign and malignant intestinal tumors, supporting the concept that the elaboration of lysozyme is a nonspecific feature of intestinal-cell differentiation and is not a response to a specific stimulus.

Topics: Cystadenocarcinoma; Cystadenoma; Cytoplasmic Granules; Digestive System; Female; Humans; Intestinal Mucosa; Muramidase; Ovarian Neoplasms; Rectum; Staining and Labeling; Teratoma

Topics: Adenocarcinoma; Cystadenoma; Female; Humans; Muramidase; Ovarian Cysts; Ovarian Neoplasms; Parovarian Cyst; Teratoma