Partial excision of schwannomatosis with fascicular invasion occurring in the ulnar nerve above the elbow: A 1-year follow-up study.

Although not as common as solitary lesions, multiple schwannomas do occur, even in single nerve lesions. We report a rare case of a 47-year-old female patient who presented with multiple schwannomas with inter-fascicular invasion in the ulnar nerve above the cubital tunnel. Preoperative MRI revealed a 10-cm multilobulated tubular mass along the ulnar nerve above the elbow joint. During excision under 4.5° loupe magnification, we separated three ovoid yellow-colored neurogenic tumors of different sizes, but there were still remaining lesions as it was difficult to completely separate lesions from the ulnar nerve due to the risk of iatrogenic nerve ulnar nerve injury. The operative wound was closed. Postoperative biopsy confirmed the diagnosis of the three schwannomas. During the follow-up, the patient recovered without neurological symptom or limitations in range of motion, and there were no neurological abnormalities. At 1year after surgery, small lesions remained in the most proximal part. However, the patient had no clinical symptoms and was satisfied with the surgical results. Although a long-term follow-up is necessary for this patient, we were able to obtain good clinical and radiological results.

Ultrastructural and phenotypic analysis of in vitro erythropoiesis from human cord blood CD34+ cells.

Erythropoietin (EPO) induces erythropoiesis in vitro as well as in vivo, and the process of erythroid differentiation has been explored phenotypically and morphologically. However, morphological analysis of in vitro erythropoiesis of human hematopoietic progenitor cells at the ultrastructural level has not been reported before. In the present study, we have traced the ultrastructural changes of erythroid differentiation during ex vivo expansion of human cord blood (CB) CD34(+) cells in the presence of EPO by electron microscopy (EM), along with concurrent phenotypic analysis. CD34(+) cells purified from ten CBs by immunomagnetic selection were cultured in serum-free essential media in the presence of a combination of the several cytokines including EPO, thrombopoietin, flt3-ligand (FL), stem cell factor (SCF), granulocyte colony-stimulating factor, interleukin (IL)-3 and/or IL-11. Phenotypic analysis was performed by flow cytometric analysis for erythroid markers, including glycophorin C (GPC), Kell-related, glycophorin A (GPA), band 3, Lu(b), and RhD. Ultrastructural analysis was performed by electron-microscopic examination of the cultured cells stained with uranyl acetate and lead citrate. Phenotypic analysis revealed that in the absence of EPO, genuine erythroid fraction expressing the typical pattern of erythroid markers did not appear. The order of the above markers expressed in the cultured cells in the presence of EPO was GPC, Kell-related, GPA, band 3, Lu(b), and RhD, irrespective of the type of cytokine added. Of the cytokines used in combination with EPO, FL + IL-3 was the most efficient in inducing erythroid differentiation, which was followed by SCF + IL-3. EM examination demonstrated complete process of erythroid development from pronormoblasts to reticulocytes with nuclei having been extruded and mature erythrocytes. These results suggest that morphologically intact erythrocytes could be produced by ex vivo expansion of CB CD34(+) cells using EPO.

Megakaryothrombopoiesis during ex vivo expansion of human cord blood CD34+ cells using thrombopoietin.

Thrombopoietin (TPO) is one of the most promising stimulants for ex vivo expansion of haematopoietic stem cells. Previously, we have found that TPO induces a characteristic pattern of apoptosis during ex vivo expansion of human cord blood (CB) CD34+ cells and that the TPO-induced apoptotic cells belong to megakaryocyte (MK) lineage. In this study, we have examined the maturation of MK and platelet production in association with the TPO-induced apoptosis. CD34+ cells, purified from human CB, were expanded in serum-free conditions stimulated with TPO. Apoptosis was confirmed by terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate-biotin nick end labelling (TUNEL) assay and electron microscopy (EM). Simultaneous measurement of DNA content and immunophenotyping revealed that the cells with higher DNA content (>8 N) constituted less than 5% of the CD41+ fractions until day 14, implying premature apoptosis of MKs before full polyploidization. Nevertheless, EM observation showed not only platelet territories but also newly produced platelets in which granules and microfilaments could be identified. Furthermore, flow cytometry demonstrated that the platelet fraction expressed P-selectin and an activation motif on GPIIb/IIIa recognized by monoclonal antibody PAC-1 upon stimulation with adenosine diphosphate (ADP). In addition, periodic acid-Schiff (PAS)-positive materials and nonspecific esterase activities could be demonstrated. Therefore, it is suggested that platelet production and the accompanying processes, rather than apoptosis only, be hastened during the ex vivo expansion of CB CD34+ cells when using TPO.

Expression of CD40 and apoptosis related molecules in autoimmune thyroid diseases.

Apoptosis is responsible for the loss of thyrocytes in autoimmune thyroiditis. Recent investigations into the pathogenesis of apoptosis have revealed that the important roles of suicide molecules expression on both thyrocytes and cytotoxic T-lymphocytes. To study the mechanism of thyrocyte loss in various forms of thyroiditis, we evaluated in situ expression patterns of CD40, Fas, and Fas-L on thyrocytes and infiltrating inflammatory cells by immunohistochemical staining of thyroid samples obtained from 49 patients (Graves' disease, n=10: Hashimoto's thyroiditis, n=14; nonspecific lymphocytic thyroiditis, n=11; subacute granulomatous thyroiditis, n=11; normal, n=3). The role of cytotoxic T-lymphocytes was also evaluated by analyzing the expression of granzyme B along with their phenotypic characteristics. CD40 was not expressed on thyrocytes of normal controls while they showed a diffuse expression of Fas and a scattered focal expression of Fas-L. The plump thyrocytes proximal to the inflammatory infiltrates showed more intense expressions of these three molecules in various forms of thyroiditis and a close correlation was found between CD40 and Fas-L expression on thyrocytes. Unlike Fas, which was expressed on infiltrating lymphocytes in all groups, Fas-L was not expressed on infiltrating lymphocytes, except those in subacute granulomatous thyroiditis. Granzyme B expressing activated cytotoxic T-lymphocytes occupied a negligible proportion of CD8+ T-lymphocytes in various forms of thyroiditis, and no difference was found in terms of their proportions according to the type of thyroiditis. These results show the acquisition of CD40, Fas and Fas-L molecules on thyrocytes proximal to inflammatory cell aggregates and the negligible expression of granzyme B and Fas-L on the infiltrating lymphocytes, and suggest that Fas and Fas-L mediated apoptosis of thyrocytes (fratricide) may be more important than T cell-mediated cytotoxicity in various forms of thyroiditis.

Acinic cell carcinoma of the lacrimal gland with intracranial extension: a case report.

PURPOSE: To describe a patient with acinic cell carcinoma of the lacrimal gland presenting with atypical manifestations including erosion of the orbital roof and intracranial extension. We compare his clinical manifestations with two previously reported cases. METHODS: Case report. RESULTS: A 51-year-old man presented with a firm, tender, palpable mass in the superior temporal portion of his right orbit. CT scan demonstrated a large homogeneously enhancing mass displacing the globe inferiorly and eroding the roof of the orbit. MRI of the orbit showed intracranial extension of tumor. Biopsy revealed an acinic cell carcinoma of the lacrimal gland. CONCLUSIONS: Acinic cell carcinoma is a rare neoplasm occurring primarily in the major and minor salivary glands.

Primary carcinoid tumor of the testis: immunohistochemical, ultrastructural and DNA flow cytometric study of two cases.

Primary testicular carcinoid tumor, occupying 0.23% of testicular neoplasm, is a rare and indolent neoplasm with the potential for distant metastasis. We present two cases of primary pure carcinoid tumor of the testis. Both patients were 36 years old. Physical examination revealed testicular mass with and without tenderness. The preoperative serum levels of beta-human chorionic gonadotropin and alpha-fetoprotein were normal and neither patient had carcinoid syndrome. The tumors measured 7.5x6x4 cm and 5.5x5x4 cm in size. Histologically, immunohistochemically and ultrastructurally, the tumors showed typical features of the carcinoid tumor. Case 1 showed extensive tumor necrosis and vascular invasion. DNA flow cytometric analysis showed aneuploidy with DNA index of 1.47 and S+G2M of 14.0% in case 1 and tetraploidy with DNA index of 1.96 and S+G2M of 22.1% in case 2. Both patients have been well without any signs of metastasis after operation for 24 months in case 1 and for 16 months in case 2.

A case of bleeding from the Dieulafoy lesion of the jejunum.

Dieulafoy lesion is an uncommon cause of gastrointestinal bleeding, reported to be only 2% of acute or chronic upper gastrointestinal bleeding episodes. Bleeding occurs from a small mucosal erosion involving an unusually large submucosal artery in an otherwise normal mucosa. It is associated with massive, life threatening hemorrhage and is difficult to diagnosis. In most cases the lesion is encountered in the proximal stomach, antrum, duodenum, colon and rectum. In particular, extragastric Dieulafoy lesion is an extremely rare source of intestinal bleeding. In Korea, no case of bleeding from a Dieulafoy lesion of the small intestine has been previously reported. We experienced one case of bleeding from a jejunal Dieulafoy lesion, which was confirmed by the pathologic examination of the resected specimen, and report here.

Myxoma: life-threatening benign nonepithelial tumor of the larynx.

Myxoma is a rare nonepithelial neoplasm of the larynx frequently misdiagnosed as a large vocal polyp due to its slow-growing nature. Myxoma is a benign but often infiltrating neoplasm of uncertain mesenchymal cell origin, characterized by irregular round, spindle or stellate cells within a matrix containing abundant mucoid material, scant vascularity and a variable meshwork of reticulum and collagen. We report one case of myxoma with life-threatening dyspnea requiring tracheotomy.