Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes (D37-D48)
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.
D37Neoplasm of uncertain behavior of oral cavity and digestive organs
Excludes 1: stromal tumors of uncertain behavior of digestive system (D48.1-)
D37.0Neoplasm of uncertain behavior of lip, oral cavity and pharynx
Excludes 1: neoplasm of uncertain behavior of aryepiglottic fold or interarytenoid fold, laryngeal aspect (D38.0)
› neoplasm of uncertain behavior of epiglottis NOS (D38.0)
› neoplasm of uncertain behavior of skin of lip (D48.5)
› neoplasm of uncertain behavior of suprahyoid portion of epiglottis (D38.0)
D37.01Neoplasm of uncertain behavior of lip
› Neoplasm of uncertain behavior of vermilion border of lip
D37.02Neoplasm of uncertain behavior of tongue
D37.03Neoplasm of uncertain behavior of the major salivary glands
D37.030Neoplasm of uncertain behavior of the parotid salivary glands
D37.031Neoplasm of uncertain behavior of the sublingual salivary glands
D37.032Neoplasm of uncertain behavior of the submandibular salivary glands
D37.039Neoplasm of uncertain behavior of the major salivary glands, unspecified
D37.04Neoplasm of uncertain behavior of the minor salivary glands
› Neoplasm of uncertain behavior of submucosal salivary glands of lip
› Neoplasm of uncertain behavior of submucosal salivary glands of cheek
› Neoplasm of uncertain behavior of submucosal salivary glands of hard palate
› Neoplasm of uncertain behavior of submucosal salivary glands of soft palate
D37.05Neoplasm of uncertain behavior of pharynx
› Neoplasm of uncertain behavior of aryepiglottic fold of pharynx NOS
› Neoplasm of uncertain behavior of hypopharyngeal aspect of aryepiglottic fold of pharynx
› Neoplasm of uncertain behavior of marginal zone of aryepiglottic fold of pharynx
D37.09Neoplasm of uncertain behavior of other specified sites of the oral cavity
D37.1Neoplasm of uncertain behavior of stomach
D37.2Neoplasm of uncertain behavior of small intestine
D37.3Neoplasm of uncertain behavior of appendix
D37.4Neoplasm of uncertain behavior of colon
D37.5Neoplasm of uncertain behavior of rectum
› Neoplasm of uncertain behavior of rectosigmoid junction
D37.6Neoplasm of uncertain behavior of liver, gallbladder and bile ducts
› Neoplasm of uncertain behavior of ampulla of Vater
D37.8Neoplasm of uncertain behavior of other specified digestive organs
› Neoplasm of uncertain behavior of anal canal
› Neoplasm of uncertain behavior of anal sphincter
› Neoplasm of uncertain behavior of anus NOS
› Neoplasm of uncertain behavior of esophagus
› Neoplasm of uncertain behavior of intestine NOS
› Neoplasm of uncertain behavior of pancreas
Excludes 1: neoplasm of uncertain behavior of anal margin (D48.5)
› neoplasm of uncertain behavior of anal skin (D48.5)
› neoplasm of uncertain behavior of perianal skin (D48.5)
D37.9Neoplasm of uncertain behavior of digestive organ, unspecified
D38Neoplasm of uncertain behavior of middle ear and respiratory and intrathoracic organs
Excludes 1: neoplasm of uncertain behavior of heart (D48.7)
D38.0Neoplasm of uncertain behavior of larynx
› Neoplasm of uncertain behavior of aryepiglottic fold or interarytenoid fold, laryngeal aspect
› Neoplasm of uncertain behavior of epiglottis (suprahyoid portion)
Excludes 1: neoplasm of uncertain behavior of aryepiglottic fold or interarytenoid fold NOS (D37.05)
› neoplasm of uncertain behavior of hypopharyngeal aspect of aryepiglottic fold (D37.05)
› neoplasm of uncertain behavior of marginal zone of aryepiglottic fold (D37.05)
D38.1Neoplasm of uncertain behavior of trachea, bronchus and lung
D38.2Neoplasm of uncertain behavior of pleura
D38.3Neoplasm of uncertain behavior of mediastinum
D38.4Neoplasm of uncertain behavior of thymus
D38.5Neoplasm of uncertain behavior of other respiratory organs
› Neoplasm of uncertain behavior of accessory sinuses
› Neoplasm of uncertain behavior of cartilage of nose
› Neoplasm of uncertain behavior of middle ear
› Neoplasm of uncertain behavior of nasal cavities
Excludes 1: neoplasm of uncertain behavior of ear (external) (skin) (D48.5)
› neoplasm of uncertain behavior of nose NOS (D48.7)
› neoplasm of uncertain behavior of skin of nose (D48.5)
D38.6Neoplasm of uncertain behavior of respiratory organ, unspecified
D39Neoplasm of uncertain behavior of female genital organs
D39.0Neoplasm of uncertain behavior of uterus
D39.1Neoplasm of uncertain behavior of ovary
Use Additional Code: code to identify any functional activity.
D39.10Neoplasm of uncertain behavior of unspecified ovary
D39.11Neoplasm of uncertain behavior of right ovary
D39.12Neoplasm of uncertain behavior of left ovary
D39.2Neoplasm of uncertain behavior of placenta
› Chorioadenoma destruens
› Invasive hydatidiform mole
› Malignant hydatidiform mole
Excludes 1: hydatidiform mole NOS (O01.9)
D39.8Neoplasm of uncertain behavior of other specified female genital organs
› Neoplasm of uncertain behavior of skin of female genital organs
D39.9Neoplasm of uncertain behavior of female genital organ, unspecified
D40Neoplasm of uncertain behavior of male genital organs
D40.0Neoplasm of uncertain behavior of prostate
D40.1Neoplasm of uncertain behavior of testis
D40.10Neoplasm of uncertain behavior of unspecified testis
D40.11Neoplasm of uncertain behavior of right testis
D40.12Neoplasm of uncertain behavior of left testis
D40.8Neoplasm of uncertain behavior of other specified male genital organs
› Neoplasm of uncertain behavior of skin of male genital organs
D40.9Neoplasm of uncertain behavior of male genital organ, unspecified
D41Neoplasm of uncertain behavior of urinary organs
D41.0Neoplasm of uncertain behavior of kidney
Excludes 1: neoplasm of uncertain behavior of renal pelvis (D41.1-)
D41.00Neoplasm of uncertain behavior of unspecified kidney
D41.01Neoplasm of uncertain behavior of right kidney
D41.02Neoplasm of uncertain behavior of left kidney
D41.1Neoplasm of uncertain behavior of renal pelvis
D41.10Neoplasm of uncertain behavior of unspecified renal pelvis
D41.11Neoplasm of uncertain behavior of right renal pelvis
D41.12Neoplasm of uncertain behavior of left renal pelvis
D41.2Neoplasm of uncertain behavior of ureter
D41.20Neoplasm of uncertain behavior of unspecified ureter
D41.21Neoplasm of uncertain behavior of right ureter
D41.22Neoplasm of uncertain behavior of left ureter
D41.3Neoplasm of uncertain behavior of urethra
D41.4Neoplasm of uncertain behavior of bladder
D41.8Neoplasm of uncertain behavior of other specified urinary organs
D41.9Neoplasm of uncertain behavior of unspecified urinary organ
D42Neoplasm of uncertain behavior of meninges
D42.0Neoplasm of uncertain behavior of cerebral meninges
D42.1Neoplasm of uncertain behavior of spinal meninges
D42.9Neoplasm of uncertain behavior of meninges, unspecified
D43Neoplasm of uncertain behavior of brain and central nervous system
Excludes 1: neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system (D48.2)
D43.0Neoplasm of uncertain behavior of brain, supratentorial
› Neoplasm of uncertain behavior of cerebral ventricle
› Neoplasm of uncertain behavior of cerebrum
› Neoplasm of uncertain behavior of frontal lobe
› Neoplasm of uncertain behavior of occipital lobe
› Neoplasm of uncertain behavior of parietal lobe
› Neoplasm of uncertain behavior of temporal lobe
Excludes 1: neoplasm of uncertain behavior of fourth ventricle (D43.1)
D43.1Neoplasm of uncertain behavior of brain, infratentorial
› Neoplasm of uncertain behavior of brain stem
› Neoplasm of uncertain behavior of cerebellum
› Neoplasm of uncertain behavior of fourth ventricle
D43.2Neoplasm of uncertain behavior of brain, unspecified
D43.3Neoplasm of uncertain behavior of cranial nerves
D43.4Neoplasm of uncertain behavior of spinal cord
D43.8Neoplasm of uncertain behavior of other specified parts of central nervous system
D43.9Neoplasm of uncertain behavior of central nervous system, unspecified
› Neoplasm of uncertain behavior of nervous system (central) NOS
D44Neoplasm of uncertain behavior of endocrine glands
Excludes 1: multiple endocrine adenomatosis (E31.2-)
› multiple endocrine neoplasia (E31.2-)
› neoplasm of uncertain behavior of endocrine pancreas (D37.8)
› neoplasm of uncertain behavior of ovary (D39.1-)
› neoplasm of uncertain behavior of testis (D40.1-)
› neoplasm of uncertain behavior of thymus (D38.4)
D44.0Neoplasm of uncertain behavior of thyroid gland
D44.1Neoplasm of uncertain behavior of adrenal gland
Use Additional Code: code to identify any functional activity.
D44.10Neoplasm of uncertain behavior of unspecified adrenal gland
D44.11Neoplasm of uncertain behavior of right adrenal gland
D44.12Neoplasm of uncertain behavior of left adrenal gland
D44.2Neoplasm of uncertain behavior of parathyroid gland
D44.3Neoplasm of uncertain behavior of pituitary gland
Use Additional Code: code to identify any functional activity.
D44.4Neoplasm of uncertain behavior of craniopharyngeal duct
D44.5Neoplasm of uncertain behavior of pineal gland
D44.6Neoplasm of uncertain behavior of carotid body
D44.7Neoplasm of uncertain behavior of aortic body and other paraganglia
D44.9Neoplasm of uncertain behavior of unspecified endocrine gland
D45Polycythemia vera
Excludes 1: familial polycythemia (D75.0)
› secondary polycythemia (D75.1)
D46Myelodysplastic syndromes
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
Excludes 2: drug-induced aplastic anemia (D61.1)
D46.0Refractory anemia without ring sideroblasts, so stated
› Refractory anemia without sideroblasts, without excess of blasts
D46.1Refractory anemia with ring sideroblasts
› RARS
D46.2Refractory anemia with excess of blasts [RAEB]
D46.20Refractory anemia with excess of blasts, unspecified
› RAEB NOS
D46.21Refractory anemia with excess of blasts 1
› RAEB 1
D46.22Refractory anemia with excess of blasts 2
› RAEB 2
D46.ARefractory cytopenia with multilineage dysplasia
D46.BRefractory cytopenia with multilineage dysplasia and ring sideroblasts
› RCMD RS
D46.CMyelodysplastic syndrome with isolated del(5q) chromosomal abnormality
› Myelodysplastic syndrome with 5q deletion
› 5q minus syndrome NOS
D46.4Refractory anemia, unspecified
D46.ZOther myelodysplastic syndromes
Excludes 1: chronic myelomonocytic leukemia (C93.1-)
D46.9Myelodysplastic syndrome, unspecified
› Myelodysplasia NOS
D47Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
D47.0Mast cell neoplasms of uncertain behavior
Excludes 1: congenital cutaneous mastocytosis (Q82.2)
› histiocytic neoplasms of uncertain behavior (D47.Z9)
› malignant mast cell neoplasm (C96.2-)
D47.01Cutaneous mastocytosis
› Diffuse cutaneous mastocytosis
› Maculopapular cutaneous mastocytosis
› Solitary mastocytoma
› Telangiectasia macularis eruptiva perstans
› Urticaria pigmentosa
Excludes 1: congenital (diffuse) (maculopapular) cutaneous mastocytosis (Q82.2)
› congenital urticaria pigmentosa (Q82.2)
› extracutaneous mastocytoma (D47.09)
D47.02Systemic mastocytosis
› Indolent systemic mastocytosis
› Isolated bone marrow mastocytosis
› Smoldering systemic mastocytosis
› Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)
Code Also: , 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)
Excludes 1: aggressive systemic mastocytosis (C96.21)
› mast cell leukemia (C94.3-)
D47.09Other mast cell neoplasms of uncertain behavior
› Extracutaneous mastocytoma
› Mast cell tumor NOS
› Mastocytoma NOS
› Mastocytosis NOS
D47.1Chronic myeloproliferative disease
› Chronic neutrophilic leukemia
› Myeloproliferative disease, unspecified
Excludes 1: atypical chronic myeloid leukemia BCR/ABL-negative (C92.2-)
› chronic myeloid leukemia BCR/ABL-positive (C92.1-)
› myelofibrosis NOS (D75.81)
› myelophthisic anemia (D61.82)
› myelophthisis (D61.82)
› secondary myelofibrosis NOS (D75.81)
D47.2Monoclonal gammopathy
› Monoclonal gammopathy of undetermined significance [MGUS]
D47.3Essential (hemorrhagic) thrombocythemia
› Essential thrombocytosis
› Idiopathic hemorrhagic thrombocythemia
› Primary thrombocytosis
Excludes 2: reactive thrombocytosis (D75.838)
› secondary thrombocytosis (D75.838)
› thrombocythemia NOS (D75.839)
› thrombocytosis NOS (D75.839)
D47.4Osteomyelofibrosis
› Chronic idiopathic myelofibrosis
› Myelofibrosis (idiopathic) (with myeloid metaplasia)
› Myelosclerosis (megakaryocytic) with myeloid metaplasia
› Secondary myelofibrosis in myeloproliferative disease
Excludes 1: acute myelofibrosis (C94.4-)
D47.ZOther specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
D47.Z1Post-transplant lymphoproliferative disorder (PTLD)
Code First: complications of transplanted organs and tissue (T86.-)
D47.Z2Castleman disease
Code Also: , if applicable, human herpesvirus 8 infection (B10.89)
Excludes 2: Kaposi's sarcoma (C46.-)
D47.Z9Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
› Histiocytic tumors of uncertain behavior
D47.9Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified
› Lymphoproliferative disease NOS
D48Neoplasm of uncertain behavior of other and unspecified sites
Excludes 1: neurofibromatosis (nonmalignant) (Q85.0-)
D48.0Neoplasm of uncertain behavior of bone and articular cartilage
Excludes 1: neoplasm of uncertain behavior of cartilage of ear (D48.1-)
› neoplasm of uncertain behavior of cartilage of larynx (D38.0)
› neoplasm of uncertain behavior of cartilage of nose (D38.5)
› neoplasm of uncertain behavior of connective tissue of eyelid (D48.1-)
› neoplasm of uncertain behavior of synovia (D48.1-)
D48.1Neoplasm of uncertain behavior of connective and other soft tissue
› Neoplasm of uncertain behavior of connective tissue of ear
› Neoplasm of uncertain behavior of connective tissue of eyelid
› Stromal tumors of uncertain behavior of digestive system
Excludes 1: neoplasm of uncertain behavior of articular cartilage (D48.0)
› neoplasm of uncertain behavior of cartilage of larynx (D38.0)
› neoplasm of uncertain behavior of cartilage of nose (D38.5)
› neoplasm of uncertain behavior of connective tissue of breast (D48.6-)
D48.11Desmoid tumor
D48.110Desmoid tumor of head and neck
D48.111Desmoid tumor of chest wall
D48.112Desmoid tumor, intrathoracic
D48.113Desmoid tumor of abdominal wall
D48.114Desmoid tumor, intraabdominal
› Desmoid tumor of pelvic cavity
› Desmoid tumor, peritoneal, retroperitoneal
D48.115Desmoid tumor of upper extremity and shoulder girdle
D48.116Desmoid tumor of lower extremity and pelvic girdle
› Desmoid tumor of buttock
D48.117Desmoid tumor of back
D48.118Desmoid tumor of other site
D48.119Desmoid tumor of unspecified site
D48.19Other specified neoplasm of uncertain behavior of connective and other soft tissue
D48.2Neoplasm of uncertain behavior of peripheral nerves and autonomic nervous system
Excludes 1: neoplasm of uncertain behavior of peripheral nerves of orbit (D48.7)
D48.3Neoplasm of uncertain behavior of retroperitoneum
D48.4Neoplasm of uncertain behavior of peritoneum
D48.5Neoplasm of uncertain behavior of skin
› Neoplasm of uncertain behavior of anal margin
› Neoplasm of uncertain behavior of anal skin
› Neoplasm of uncertain behavior of perianal skin
› Neoplasm of uncertain behavior of skin of breast
Excludes 1: neoplasm of uncertain behavior of anus NOS (D37.8)
› neoplasm of uncertain behavior of skin of genital organs (D39.8, D40.8)
› neoplasm of uncertain behavior of vermilion border of lip (D37.0)
D48.6Neoplasm of uncertain behavior of breast
› Neoplasm of uncertain behavior of connective tissue of breast
› Cystosarcoma phyllodes
Excludes 1: neoplasm of uncertain behavior of skin of breast (D48.5)
D48.60Neoplasm of uncertain behavior of unspecified breast
D48.61Neoplasm of uncertain behavior of right breast
D48.62Neoplasm of uncertain behavior of left breast
D48.7Neoplasm of uncertain behavior of other specified sites
› Neoplasm of uncertain behavior of eye
› Neoplasm of uncertain behavior of heart
› Neoplasm of uncertain behavior of peripheral nerves of orbit
Excludes 1: neoplasm of uncertain behavior of connective tissue (D48.1-)
› neoplasm of uncertain behavior of skin of eyelid (D48.5)
D48.9Neoplasm of uncertain behavior, unspecified