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  2. ICD-10-CM Codes List
  3. C00–D49
  4. C81-C96
  5. Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue (C96)

Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue (C96)

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.

Instructional Notations

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

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

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.