Aggressive systemic mastocytosis

• Functional activity
• All neoplasms are classified in this chapter, whether they are functionally active or not. An additional code from Chapter 4 may be used, to identify functional activity associated with any neoplasm.
• Morphology [Histology]
• Chapter 2 classifies neoplasms primarily by site (topography), with broad groupings for behavior, malignant, in situ, benign, etc. The Table of Neoplasms should be used to identify the correct topography code. In a few cases, such as for malignant melanoma and certain neuroendocrine tumors, the morphology (histologic type) is included in the category and codes.
• Primary malignant neoplasms overlapping site boundaries
• A primary malignant neoplasm that overlaps two or more contiguous (next to each other) sites should be classified to the subcategory/code .8 ('overlapping lesion'), unless the combination is specifically indexed elsewhere. For multiple neoplasms of the same site that are not contiguous, such as tumors in different quadrants of the same breast, codes for each site should be assigned.
• Malignant neoplasm of ectopic tissue
• Malignant neoplasms of ectopic tissue are to be coded to the site mentioned, e.g., ectopic pancreatic malignant neoplasms are coded to pancreas, unspecified (C25.9).

• Kaposi's sarcoma of lymph nodes (C46.3)
• secondary and unspecified neoplasm of lymph nodes (C77.-)
• secondary neoplasm of bone marrow (C79.52)
• secondary neoplasm of spleen (C78.89)

personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues (Z85.79)

• indolent mastocytosis (D47.02)
• mast cell leukemia (C94.30)
• mastocytosis (congenital) (cutaneous) (Q82.2)