D47.02

aggressive systemic mastocytosis (C96.21) mast cell leukemia (C94.3-)

, if applicable, any associated hematological non-mast cell lineage disease, such as: acute myeloid leukemia (C92.6-, C92.A-) chronic myelomonocytic leukemia (C93.1-) essential thrombocytosis (D47.3) hypereosinophilic syndrome (D72.1) myelodysplastic syndrome (D46.9) myeloproliferative syndrome (D47.1) non-Hodgkin lymphoma (C82-C85) plasma cell myeloma (C90.0-) polycythemia vera (D45)

Indolent systemic mastocytosis Isolated bone marrow mastocytosis Smoldering systemic mastocytosis Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)

congenital cutaneous mastocytosis (Q82.2) histiocytic neoplasms of uncertain behavior (D47.Z9) malignant mast cell neoplasm (C96.2-)

neoplasms of unspecified behavior (D49.-)

Categories D37-D44, and D48 classify by site neoplasms of uncertain behavior, i.e., histologic confirmation whether the neoplasm is malignant or benign cannot be made.

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). (C00-C96) Malignant neoplasms (C00-C96) (C00-C75) Malignant neoplasms, stated or presumed to be primary (of specified sites), and certain specified histologies, except neuroendocrine, and of lymphoid, hematopoietic and related tissue (C00-C75)