A Proposal for Creating a Guideline for Cancer Registration of the Fibromatosis, PEComa Group, Malignant Lymphoma In Situ and Dendritic Cell Tumors (III)

BACKGROUND: Understanding the biologic behavior of a tumor is a prerequisite for tumor registration code assignment. The aim of this report was to propose appropriate behavior codes of the International Classification of Disease Oncology 3 (ICD-O3) to rare, yet pathologically interesting hematopoietic and soft tissue tumors. METHODS: The Study Group for Hematopathology, the Bone and Soft Tissue Pathology Study Group, and the Cancer Registration Committee prepared the questionnaire containing provisional behavior codes of selected diseases. RESULTS: In situ lesions of mantle cell and follicular lymphomas, dendritic cell tumors, and neoplasms with perivascular epithelioid cell differentiation (PEComa), not otherwise specified were classified as malignant (-/3). The fibromatosis group, with the exception of lipofibromatosis, was proposed as benign (-/0). Lipofibromatosis and several diseases that belong to the PEComa group were proposed as uncertain malignant potential (-/1). For the hematologic and soft tissue tumors, 274 and 288 members of the Korean Society of Pathologists, respectively, provided opinions through questionnaire, and most responders showed agreement with the provisional behavior code proposed. CONCLUSIONS: The determination of behavior codes for the rare diseases described in this study, especially those of the PEComa group or malignant lymphoma, could be viewed as impractical and premature, but this study provides the basis for future research on this topic.

A case of peritoneal desmoplastic small round cell tumor / 대한산부인과학회지

Desmoplastic small round cell tumor is a rare and aggressive tumor that affects young males. It is usually an abdominopelvic malignancy that demonstrates distinct histological appearances and a unique cytogenetic profile. There have been many different approaches to the treatment of desmoplastic small round cell tumor, but unfortunately it remains incurable and has poor long-term survival rates. However, with an aggressive approach to the treatment using multiple modalities, some temporary benefit to survival can be achieved. There has not yet been a case in which treatment has led to a curative outcome. Recently, we also experienced a case of very poor outcome of 31-year-old female with small round cell tumor in peritoneum. Here, we report the case with a brief review of literatures.

Cytopathology of Hurthle Cell Adenoma: A Cese Report by Fine Needle Aspiration / 대한세포병리학회지

The increased use of thyroid fine needle aspiration (FNA) has refocused on Hurthle cell lesions. The cytologic diagnosis of Hurthle cell tumor is a challenge due to the presence of Hurthle cells in non-neoplastic lesions and the inability to differentiate between benign and malignant Hurthle cell tumor. We report a case of Hurthle cell adenoma(HCA) in a 68-year old woman, with reivew of the cytopathologic findings. FNA revealed losely cohesive or sheets of sheets of large oval to polygonal Hurthle cells containing bundant granular cytoplasm. The histopathologic examination confirmed the diagnosis of HCA with follicular growth pattern, Ultrastructurally, the cytoplasm was packed with variable sized mitochondria.