Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue (C96) ICD-10-CM
The C96 code range covers other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue with 13 ICD-10-CM diagnosis codes. 11 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues Z85.79
Codes in the C96 Range 13 codes · 11 billable
- C96 Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissueNon-billable
- C96.0 Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
- C96.2 Malignant mast cell neoplasmNon-billable
- C96.20 Malignant mast cell neoplasm, unspecified
- C96.21 Aggressive systemic mastocytosis
- C96.22 Mast cell sarcoma
- C96.29 Other malignant mast cell neoplasm
- C96.4 Sarcoma of dendritic cells (accessory cells)
- C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
- C96.6 Unifocal Langerhans-cell histiocytosis
- C96.9 Malignant neoplasm of lymphoid, hematopoietic and related tissue, unspecified
- C96.A Histiocytic sarcoma
- C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the C96 range.
Churg-Strauss Syndrome
Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.
Eosinophilic Granuloma
The most benign and common form of Langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by EOSINOPHILS.
Eosinophils
Granular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin.
Herpesvirus 2, Gallid
The type species of the genus MARDIVIRUS in the family HERPESVIRIDAE. It is the etiologic agent of MAREK DISEASE, infecting domestic fowl and wild birds.
Histiocytic Sarcoma
Malignant neoplasms composed of MACROPHAGES or DENDRITIC CELLS. Most histiocytic sarcomas present as localized tumor masses without a leukemic phase. Though the biological behavior of these neoplasms resemble lymphomas, their cell lineage is histiocytic not lymphoid.
Kimura Disease
A chronic inflammatory disease characterized by benign enlargement of cervical LYMPH NODE and SALIVARY GLANDS with increased levels of IMMUNOGLOBULIN E. Unlike ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA it involves eosinophil infiltrates in lymph node and salivary glands and mostly found in Asian males.
Langerhans Cell Sarcoma
Rare malignant neoplasm of dendritic LANGERHANS CELLS exhibiting atypical cytology, frequent mitoses, and aggressive clinical behavior. They can be distinguished from other histiocytic and dendritic proliferations by immunohistochemical and ultrastructure studies. Cytologically benign proliferations of Langerhans cells are called LANGERHANS CELL HISTIOCYTOSIS.
Neurolymphomatosis
Infiltration of the nervous system by malignant lymphoma cells.
Polyarteritis Nodosa
A form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized ARTERIES, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called CHURG-STRAUSS SYNDROME.
About the C96 Code Range
ICD-10 code C96 covers a range of rare and unspecified malignant tumors affecting lymphoid, hematopoietic, and related tissues, including various uncommon lymphomas, mast cell cancers, and sarcomas. These codes are essential for identifying specific malignancies that do not fit more common lymphoma classifications.
This section includes codes for conditions like Langerhans cell histiocytosis (C96.0, C96.5, C96.6), which may be known as disseminated or unifocal eosinophilic granuloma. It also covers malignant mast cell neoplasms, such as aggressive systemic mastocytosis (C96.21) and mast cell sarcoma (C96.22), diseases sometimes referred to clinically as malignant mastocytosis or mast cell malignancy of lymph nodes. Additionally, sarcomas of dendritic or histiocytic cells (C96.4, C96.A) are included, covering rare tumors like dendritic cell sarcoma or malignant histiocytosis. The code C96.9 is used when the specific type is unspecified but still malignant. Medical coders use these codes to accurately categorize uncommon and complex malignancies of the blood and immune system, helping clinicians distinguish these diseases from more typical lymphoma codes.
Questions About This Page
How many billable codes are in the C96 range?
Of the 13 codes in this range, 11 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the C96 range classify?
The range classifies other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
