Korean J Pathol.  2012 Oct;46(5):436-442.

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

  • 1Department of Pathology, St. Vincent's Hospital, The Catholic University of Korea College of Medicine, Suwon, Korea.
  • 2The Society of Bone and Soft Tissue Study Group, Seoul, Korea.
  • 3Department of Pathology, Yeouido St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea.
  • 4The Korean Study Group of Hematopathology, Seoul, Korea.
  • 5Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 6Department of Pathology, Bundang CHA Hospital, CHA University College of Medicine, Seongnam, Korea.
  • 7Department of Pathology, Chungnam National University School of Medicine, Daejeon, Korea.
  • 8The Cancer Registration Committee of the Korean Society of Pathologist, Seoul, Korea. jhpath.sohn@samsung.com
  • 9Department of Pathology, Kyung Hee University School of Medicine, Seoul, Korea.
  • 10Department of Pathology, Korea Cancer Center Hospital, Seoul, Korea.
  • 11Department of Pathology, Korea University School of Medicine, Seoul, Korea.
  • 12Department of Pathology, Kangbuk Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.


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.
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.
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.
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.


ICD-O3; Behavior code; Hematologic malignancy; Soft tissue neoplasms

MeSH Terms

Dendritic Cells
Epithelioid Cells
Hematologic Neoplasms
Lymphoma, Follicular
Perivascular Epithelioid Cell Neoplasms
Surveys and Questionnaires
Rare Diseases
Soft Tissue Neoplasms
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