C96.4

Sarcoma of dendritic cells (accessory cells)

A neoplasm composed of spindle to ovoid cells which have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It usually behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy. Recurrences have been reported in up to half of the cases. Cases which present with abdominal involvement and a high morphologic grade usually have a poorer prognosis.

A neoplastic proliferation of langerhans cells with overtly malignant cytologic features. It can be considered a higher grade variant of langerhans cell histiocytosis lch) and it can present de novo or progress from antecedent lch. who, 2001)

A neoplastic proliferation of spindle to ovoid cells which show phenotypic features similar to those of interdigitating dendritic cells. The clinical course is generally aggressive. who, 2008)

A rare sarcoma of interdigitating cells found in the lymph nodes and non-lymphoid organs. They exhibit a variable immunophenotype and lack birbeck granules.

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.

Sarcoma of follicular dendritic cells most often found in the lymph nodes. This rare neoplasm occurs predominately in adults.

C96.4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

This is the American ICD-10-CM version of C96.4 – other international versions of ICD-10 C96.4 may differ.