Chapter II
Neoplasms
(C00-D48)

Rules Conventions GCS DChS.II.1 DChS.II.2 DChS.II.4 Guidance

Malignant neoplasms
(C00-C97)

Coding-Hint
Use additional code ( U85) , if desired, to identify resistance, non-responsiveness and refractive properties of the neoplasm to antineoplastic drugs.

Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue
(C81-C96)

Excl.:
secondary and unspecified neoplasm of lymph nodes ( C77.-)

C81 Hodgkin lymphoma
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C81.0 Nodular lymphocyte predominant Hodgkin lymphoma
C81.1 Nodular sclerosis (classical) Hodgkin lymphoma
C81.2 Mixed cellularity (classical) Hodgkin lymphoma
C81.3 Lymphocyte depleted (classical) Hodgkin lymphoma
C81.4 Lymphocyte-rich (classical) Hodgkin lymphoma
Excl.:
nodular lymphocyte predominant Hodgkin lymphoma ( C81.0)
C81.7 Other (classical) Hodgkin lymphoma
Incl.:
Classic Hodgkin lymphoma, type not specified
C81.9 Hodgkin lymphoma, unspecified

C82 Follicular lymphoma
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

Incl.:
follicular lymphoma with or without diffuse areas
Excl.:
mature T/NK-cell lymphoma ( C84.-)
C82.0 Follicular lymphoma grade I
C82.1 Follicular lymphoma grade II
C82.2 Follicular lymphoma grade III, unspecified
C82.3 Follicular lymphoma grade IIIa
C82.4 Follicular lymphoma grade IIIb
C82.5 Diffuse follicle centre lymphoma
C82.6 Cutaneous follicle centre lymphoma
C82.7 Other types of follicular lymphoma
C82.9 Follicular lymphoma, unspecified
Incl.:
Nodular lymphoma NOS

C83 Non-follicular lymphoma
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C83.0 Small cell B-cell lymphoma
Incl.:
Lymphoplasmacytic lymphoma
Nodal marginal zone lymphoma
Non-leukaemic variant of B-CLL
Splenic marginal zone lymphoma
Excl.:
Chronic lymphocytic leukaemia ( C91.1)
Waldenström macroglobulinaemia ( C88.0)
mature T/NK-cell lymphomas ( C84.-)
C83.1 Mantle cell lymphoma
Incl.:
Centrocytic lymphoma
Malignant lymphomatous polyposis
C83.3 Diffuse large B-cell lymphoma
Incl.:
  • Anaplastic
  • CD30-positive
  • Centroblastic
  • Plasmablastic
  • Immunoblastic
  • Subtype not specified
  • T-cell rich
  • diffuse large B-cell lymphoma
Excl.:
mediastinal (thymic) large B-cell lymphoma ( C85.2)
mature T/NK-cell lymphomas ( C84.-)
C83.5 Lymphoblastic (diffuse) lymphoma
Incl.:
B-cell precursor lymphoma
Lymphoblastic B-cell lymphoma
Lymphoblastic lymphoma NOS
Lymphoblastic T-cell lymphoma
T-cell precursor lymphoma
C83.7 Burkitt lymphoma
Incl.:
Atypical Burkitt lymphoma
“Burkitt-like” lymphoma
Excl.:
mature B-cell leukaemia Burkitt-type ( C91.8)
C83.8 Other non-follicular lymphoma
Incl.:
Primary effusion B-cell lymphoma
Intravascular large B-cell lymphoma
Lymphoid granulomatosis
Excl.:
mediastinal (thymic) large B-cell lymphoma ( C85.2)
T-cell rich B-cell lymphoma ( C83.3)
C83.9 Non-follicular (diffuse) lymphoma, unspecified

C84 Mature T/NK-cell lymphomas
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C84.0 Mycosis fungoides
C84.1 Sézary disease
C84.4 Peripheral T-cell lymphoma, not elsewhere classified
Incl.:
Lennert’s lymphoma
Lymphoepithelioid lymphoma
C84.5 Other mature T/NK-cell lymphomas
Note:

If T-cell lineage or involvement is mentioned in conjunction with a specific lymphoma, code to the more specific description.

Excl.:
angioimmunoblastic T-cell lymphoma ( C86.5)
blastic NK-cell lymphoma ( C86.4)
enteropathy-type T-cell lymphoma ( C86.2)
extranodal NK-cell lymphoma, nasal type ( C86.0)
hepatosplenic T-cell lymphoma ( C86.1)
primary cutaneous CD30-positive T-cell proliferations ( C86.6)
subcutaneous panniculitis-like T-cell lymphoma ( C86.3)
T-cell leukaemia ( C91.-)
C84.6 Anaplastic large cell lymphoma, ALK-positive
Incl.:
Anaplastic large cell lymphoma, CD30-positive
C84.7 Anaplastic large cell lymphoma, ALK-negative
Excl.:
primary cutaneous CD30-positive T-cell proliferations ( C86.6)
C84.8 Cutaneous T-cell lymphoma, unspecified
C84.9 Mature T/NK-cell lymphoma, unspecified
Incl.:
NK/T cell lymphoma NOS
Excl.:
mature T-cell lymphoma, not elsewhere classified ( C84.4)

C85 Other and unspecified types of non-Hodgkin lymphoma
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C85.1 B-cell lymphoma, unspecified
Note:

If B-cell lineage or involvement is mentioned in conjunction with a specific lymphoma, code to the more specific description.

C85.2 Mediastinal (thymic) large B-cell lymphoma
C85.7 Other specified types of non-Hodgkin lymphoma
C85.9 Non-Hodgkin lymphoma, unspecified
Incl.:
Lymphoma NOS
Malignant lymphoma NOS
Non-Hodgkin lymphoma NOS

C86 Other specified types of T/NK-cell lymphoma
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

Excl.:
anaplastic large cell lymphoma, ALK negative ( C84.7)
anaplastic large cell lymphoma, ALK positive ( C84.6)
C86.0 Extranodal NK/T-cell lymphoma, nasal type
C86.1 Hepatosplenic T-cell lymphoma
Incl.:
Alpha-beta and gamma-delta types
C86.2 Enteropathy-type (intestinal) T-cell lymphoma
Incl.:
Enteropathy associated T-cell lymphoma
C86.3 Subcutaneous panniculitis-like T-cell lymphoma
C86.4 Blastic NK-cell lymphoma
C86.5 Angioimmunoblastic T-cell lymphoma
Incl.:
Angioimmunoblastic lymphadenopathy with dysproteinaemia [AILD]
C86.6 Primary cutaneous CD30-positive T-cell proliferations
Incl.:
Lymphomatoid papulosis
Primary cutaneous anaplastic large-cell lymphoma
Primary cutaneous CD30-positive large T-cell lymphoma

C88 Malignant immunoproliferative diseases
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C88.0 Waldenström macroglobulinaemia
Incl.:
Lymphoplasmacytic lymphoma with IgM-production
Macroglobulinaemia (primary)(idiopathic)
Excl.:
small cell B-cell lymphoma ( C83.0)
C88.2 Other heavy chain disease
Incl.:
Franklin disease
Gamma heavy chain disease
Mu (µ) heavy chain disease
C88.3 Immunoproliferative small intestinal disease
Incl.:
Alpha heavy chain disease
Mediterranean lymphoma
C88.4 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lyphoma]
Note:

Use additional code (C83.3) if desired, to specify transition to high malignant (diffuse large cell) lymphoma

Incl.:
Lymphoma of skin-associated lymphoid tissue (SALT-lymphoma)
Lymphoma of bronchial-associated lymphoid tissue (BALT-lymphoma)
C88.7 Other malignant immunoproliferative diseases
C88.9 Malignant immunoproliferative disease, unspecified
Incl.:
Immunoproliferative disease NOS

C90 Multiple myeloma and malignant plasma cell neoplasms
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C90.0 Multiple myeloma
DCS.VI.4
Incl.:
Kahler disease
Medullary plasmacytoma
Myelomatosis
Plasma cell myeloma
Excl.:
solitary plasmacytoma ( C90.3)
C90.1 Plasma cell leukaemia
Incl.:
Plasmacytic leukaemia
C90.2 Extramedullary plasmacytoma
C90.3 Solitary plasmacytoma
Incl.:
Localized malignant plasma cell tumour NOS
Plasmacytoma NOS
Solitary myeloma

C91 Lymphoid leukaemia
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C91.0 Acute lymphoblastic leukaemia [ALL]
Note:
This code should only be used for T-cell and B-cell precursor leukeamia
C91.1 Chronic lymphocytic leukaemia of B-cell type
Incl.:
Lymphoplasmacytic leukaemia
Richter syndrome
Excl.:
lymphoplasmacytic lymphoma ( C83.0)
C91.3 Prolymphocytic leukaemia of B-cell type
C91.4 Hairy-cell leukaemia
Incl.:
Leukaemic reticuloendotheliosis
C91.5 Adult T-cell lymphoma/leukaemia [HTLV-1-associated]
Incl.:
  • Acute
  • Chronic
  • Lymphomatoid
  • Smouldering
  • variant
C91.6 Prolymphocytic leukaemia of T-cell type
C91.7 Other lymphoid leukaemia
Incl.:
T-cell large granular lymphocytic leukaemia (associated with rheumatoid arthritis)
C91.8 Mature B-cell leukaemia Burkitt-type
Excl.:
Burkitt lymphoma with little or no bone marrow infiltration ( C83.7)
C91.9 Lymphoid leukaemia, unspecified

C92 Myeloid leukaemia
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

Incl.:
leukaemia:
  • granulocytic
  • myelogenous
C92.0 Acute myeloblastic leukaemia [AML]
Incl.:
Acute myeloblastic leukaemia, minimal differentiation
Acute myeloblastic leukaemia (with maturation)
AML1/ETO
AML M0
AML M1
AML M2
AML with t(8;21)
AML (without a FAB classification) NOS
Refractory anaemia with excess blasts in transformation
Excl.:
acute exacerbation of chronic myeloid leukaemia ( C92.1)
C92.1 Chronic myeloid leukaemia [CML], BCR/ABL-positive
Incl.:
Chronic myelogenous leukaemia:
  • Philadelphia chromosome (Ph1) positive
  • t(9:22)(q34; q11)
  • with crisis of blast cells
Excl.:
atypical chronic myeloid leukaemia, BCR/ABL-negative ( C92.2)
chronic myelomonocytic leukaemia ( C93.1)
unclassified myeloproliferative disease ( D47.1)
C92.2 Atypical chronic myeloid leukaemia, BCR/ABL- negative
C92.3 Myeloid sarcoma
Note:
a tumour of immature myeloid cells
Incl.:
Chloroma
Granulocytic sarcoma
C92.4 Acute promyelocytic leukaemia [PML]
Incl.:
AML M3
AML Me with t(15; 17) and variants
C92.5 Acute myelomonocytic leukaemia
Incl.:
AML M4
AML M4 Eo with inv(16) or t(16;16)
C92.6 Acute myeloid leukaemia with 11q23-abnormality
Incl.:
Acute myeloid leukaemia with variation of MLL-gene
C92.7 Other myeloid leukaemia
Excl.:
chronic eosinophilic leukaemia [hypereosinophilic syndrome] ( D47.5)
C92.8 Acute myeloid leukaemia with multilineage dysplasia
Note:
Acute myeloid leukaemia with dysplasia of remaining haematopoesis and/or myelodysplastic disease in its history.
C92.9 Myeloid leukaemia, unspecified

C93 Monocytic leukaemia
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

Incl.:
monocytoid leukaemia
C93.0 Acute monoblastic/monocytic leukaemia
Incl.:
AML M5a
AML M5b
AML M5
C93.1 Chronic myelomonocytic leukaemia
Incl.:
Chronic monocytic leukaemia
CMML-1
CMML-2
CMML with eosinophilia
C93.3 Juvenile myelomonocytic leukaemia
C93.7 Other monocytic leukaemia
C93.9 Monocytic leukaemia, unspecified

C94 Other leukaemias of specified cell type
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

Excl.:
leukaemic reticuloendotheliosis ( C91.4)
plasma cell leukaemia ( C90.1)
C94.0 Acute erythroid leukaemia
Incl.:
Acute myeloid leukaemia M6 (a)(b)
Erythroleukaemia
C94.2 Acute megakaryoblastic leukaemia
Incl.:
Acute myeloid leukaemia, M7
Acute megakaryocytic leukaemia
C94.3 Mast cell leukaemia
C94.4 Acute panmyelosis with myelofibrosis
Incl.:
Acute myelofibrosis
C94.6 Myelodysplastic and myeloproliferative disease, not elsewhere classified
C94.7 Other specified leukaemias
Incl.:
Aggressive NK-cell leukaemia
Acute basophilic leukaemia

C95 Leukaemia of unspecified cell type
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C95.0 Acute leukaemia of unspecified cell type
Incl.:
Acute bilineal leukaemia
Acute mixed lineage leukaemia
Biphenotypic acute leukaemia
Stem cell leukaemia of unclear lineage
Excl.:
acute exacerbation of unspecified chronic leukaemia ( C95.1)
C95.1 Chronic leukaemia of unspecified cell type
C95.7 Other leukaemia of unspecified cell type
C95.9 Leukaemia, unspecified

C96 Other and unspecified malignant neoplasms of lymphoid, haematopoietic and related tissue
DCS.II.1 DCS.II.7 DCS.XIII.4 Guidance

C96.0 Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis [Letterer-Siwe disease]
Incl.:
Histiocytosis X, multisystemic
C96.2 Malignant mast cell tumour
Incl.:
Aggressive systemic mastocytosis
Mast cell sarcoma
Excl.:
indolent mastocytosis ( D47.0)
mast cell leukaemia ( C94.3)
mastocytosis (congenital) (cutaneous) ( Q82.2)
C96.4 Sarcoma of dendritic cells (accessory cells)
Incl.:
Interdigiting dendritic cell sarcoma
Langerhans-cell sarcoma
Sarcoma of follicular dendritic cells
C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
Incl.:
Hand-Schüller-Christian disease
Histiocytosis X, multifocal
C96.6 Unifocal Langerhans-cell histiocytosis
Incl.:
Eosinophilic granuloma
Histiocytosis X, unifocal
Histiocytosis X NOS
Langerhans-cell histiocytosis NOS
C96.7 Other specified malignant neoplasms of lymphoid, haematopoietic and related tissue
C96.8 Histiocytic sarcoma
Incl.:
Malignant Histiocytosis
C96.9 Malignant neoplasm of lymphoid, haematopoietic and related tissue, unspecified