A Case of Malignant PEComa of the Uterus Associated with Intramural Leiomyoma and Endometrial Carcinoma

Perivascular epithelioid cell tumors (PEComas) refers to a family of mesenchymal neoplasms composed of angiomyolipomas, clear cell “sugar” tumors of the lung, and lymphangioleiomyomatoses. These tumors have a distinctive and common component of perivascular epithelioid cells that show an association with blood vessel walls and immunohistochemically display myomelanocytic differentiation. The unique neoplasms have been shown to have an expanded range through a variety of case reports, including visceral, intra-abdominal, soft tissue, and bone tumors. The retroperitoneum, abdominopelvic region, and uterus have been reported to be the most common sites. Most PEComas follow a benign course. However, reports of malignant PEComas are increasing. Many papers have described uterine PEComas, but to our knowledge, there have not yet been any reports of a malignant PEComa arising concomitant with another epithelial tumor and mesenchymal tumor. We report herein the case of a 67-year-old woman who experienced a malignant uterine PEComa infiltrating a preexisting intramural leiomyoma with synchronous well differentiated endometrial carcinoma and multiple liver and lung metastases.

Analysis of Human Tissue Management Models for Medical Research: Preparation for Implementation of the 2012 Revision of the Bioethics and Safety Act of Korea

Efficient management of human tissue samples is a critical issue; the supply of samples is unable to satisfy the current demands for research. Lack of informed consent is also an ethical problem. One of the goals of the 2012 revision of Korea's Bioethics and Safety Act was to implement regulations that govern the management of human tissue samples. To remain competitive, medical institutions must prepare for these future changes. In this report, we review two tissue management models that are currently in use; model 1 is the most common system utilized by hospitals in Korea and model 2 is implemented by some of the larger institutions. We also propose three alternative models that offer advantages over the systems currently in use. Model 3 is a multi-bank model that protects the independence of physicians and pathologists. Model 4 utilizes a comprehensive single bioresource bank; although in this case, the pathologists gain control of the samples, which may make it difficult to implement. Model 5, which employs a bioresource utilization steering committee (BUSC), is viable to implement and still maintains the advantages of Model 4. To comply with the upcoming law, we suggest that physicians and pathologists in an institution should collaborate to choose one of the improved models of tissue management system that best fits for their situation.

Proposal for the development of a human biological material management system for research hospitals

In this paper, the authors suggest more effective conditions for human tissue preparation in research hospitals. Because recent genetic and molecular studies have contributed to the rapid development of molecular and genetic medicine, human tissue is now again being considered as a valuable research resource. Basically, high-quality research-oriented tissue bank organizations are a very important part of a research hospital. The current management system for human tissue, however, is not very effective from either legal or practical perspectives. In this article, the authors propose some improvements on the human tissue management system. The laws on human tissue such as the Bioethics and Safety Act should be changed to contain suitable language applying on-site and efficient multi-dimensional information. Informed consent should be an essential requirement before surgery. Pathologists should be supported as the essential manpower of human tissue banks by law. A committee composed of a clinician, researcher, pathologist, information manager, and coordinator should be established to manage human tissue banks in hospitals. The Institutional Review Board should pay more attention to preventing the leakage of the private information of donors, and researchers should know about the review process and guidelines. This suggestion will create a more stable and effective system for the management of human tissue banks, and it will also create a complete and integrated system for research institutions. Therefore, human tissue banks can play an important role in improving research hospitals' competitiveness by providing a valuable collection of material for research.

Carcinosarcoma in the Cecum

Carcinosarcoma of the colon is rare. Seventeen cases have been reported in the English literature. Most cases occurred in the left side of the colon. Indeed, there is only one reported case of cecal carcinosarcoma. Carcinosarcoma has a tendency to distantly metastasize and shows dismal prognosis. We report a case of carcinosarcoma in the cecum and review the literature describing colonic carcinosarcoma.

Reappraisal of 'The Korean Journal of Pathology, 2010; 44: 111-6' by Youngjoon Ryu et al. and Comments on 'The Korean Journal of Pathology, 2010; 44: 343-5' by Kyu Won Jung and Soong Deok Lee

No abstract available.

Legal and Ethical Consideration in the Use of Human Biological Material

Traditionally, pathologists have used human biological material primarily for diagnostic purposes. More recently, advances in biomedical technology and changes in the research environment have placed new demands on pathologists and their handling of human materials. Moreover, these technological advances have required pathologists to be not only experts in diagnosis, but also managers of biobanks storing human biological material. Consequently, pathologists might now be confronted with unanticipated legal and ethical questions. We investigated seven examples of South Korean legislation concerning human biological material, including "The Bioethics and Safety Act" (2005), and we considered possible conflicts of interest between donors and researchers. We also reviewed international bioethical guidelines and legal precedents from several countries with special regard to pathologic glass slides, paraffin blocks, remaining specimens and other guidelines. We conclude that a better understanding of the legal and ethical questions concerning human biological material leads pathologists to safer and more conscientious management of these samples.

Extramedullary B Lymphoblastic Crisis of CML, Presenting as a Leptomeningeal Tumor: A Case Report

We report here on a rare case of a patient who presented with an extramedullary B lymphoblastic crisis as an initial manifestation of chronic myelogenous leukemia (CML). A 71-year-old man visited the emergency room due to suddenly developed dysarthria and right side weakness. Emergency craniotomy was done under the presumptive diagnosis of subdural hemorrhage. During the operation, a poorly demarcated firm mass was identified in the leptomeningeal space. Microscopically, the majority of the tumor was composed of monotonous immature cells with blast morphology, and these cells were immunoreactive for TdT, CD34, CD10 and CD20, indicating the precursor B-cell phenotype. The peripheral area of the tumor consisted of myeloid cells in various stages of maturation, and these cells were reactive for myeloperoxidase, chloroacetate esterase, CD43 and CD15. FISH analysis using the LSI bcr-abl dual color probe showed gene fusion signals in both the B-lymphoblasts and myeloid cells. The peripheral blood and bone marrow findings were consistent with CML with no evidence of a blast crisis. Cytogenetic study of the bone marrow demonstrated the 46, XY, t(9;22)(q34;q11) chromosome. A diagnosis of extramedullary B lymphoblastic blast crisis in a patient with Philadelphia chromosome-positive CML was made. Despite treatment, the patient died 3 months after he was diagnosed.

Pathological Characteristics of 20 Cases of Langerhans Cell Histiocytosis and Specificity of Immunohistochemical Stain of Langerin (CD207)

BACKGROUND: Langerhans cell histiocytosis (LCH) is a well-known neoplastic disorder of Langerhans cells which has characteristic findings, however, LCH has not been adequately studied in Korea. METHODS: We analyzed the clinicopathologic features of 20 patients with LCH who were diagnosed between 1997 and 2006 at the Korea University Guro and Anam Hospitals. RESULTS: The M:F ratio was 3:1 and the age ranged from 2-60 years (mean, 23.8 years [4 in 1st decade, 6 in 2nd decade, 2 in 3rd decade, 5 in 4th decade and 3> or =40 years of age). The cases were classified as unifocal unisystemic in 13 patients, multifocal unisystemic in 4 patients, and multifocal multisystemic in 3 patients. The bone was the most commonly involved organ (14), followed by lymph node (5), lung (2), skin (2) and ureter (1). The Langerhans cells were immunohistochemically stained with Langerin, CD1a, S-100 protein, and CD68. Langerin and CD1a were specific for Langerhans cells. CONCLUSIONS: The distribution of the involved organs in patients with LCH was similar to the distribution in Western countries, but lymph node involvement was more frequent, whereas lung involvement was less common. Langerin is considered to be a specific marker for Langerhans cells.

The Clinicopathological Characteristics of Gastrointestinal Neuroendocrine Tumors; An Analysis of 65 Cases

Background : This study was designed to investigate gastrointestinal neuroendocrine tumors with an emphasis on their clinicopathological characteristics. Methods : Sixty-five cases were reviewed and classified as typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SmCC). We performed immunohistochemistry to characterize the expression of the immunoreactivity for synaptophysin, chromogranin, gastrin, somatostatin, thyroid transcription factor-1, p53 and Ki-67. Results : Most commonly, the tumors were located in the rectum (54%), followed by the stomach (23%) and colon (9%). Histologically, the tumors were classified as 49 TCs, 4 ACs, 6 LCNECs and 6 SmCCs. Most tumors were stained positive for synaptophysin and/or chromogranin. Four LCNECs and one SmCC were p53-positive. The carcinoids revealed a low level ( or =30% of the cells showed reactivity for Ki-67 in the majority of LCNECs and SmCCs. Six patients with metastatic carcinoids were older than those patients without metastasis (64 vs 48 years, respectively, p=0.004). Furthermore, the size of tumors was larger for the patients with metastatic carcinoids than for the patients with nonmetastatic carcinoids (2.3 vs 0.5 cm, respectively, p=0.005). Conclusion : Old age, large tumor size and muscle invasion are associated with high grade neuroendocrine tumor and lymph node metastasis for those patients with carcinoids.