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Name
LCH, disseminated
ICD-O-1 Morphology
9720/3: Malignant histiocytosis
Effective
1978 - 1991
ICD-O-2 Morphology
9720/3: Malignant histiocytosis
Effective
1992 - 2000
ICD-O-3 Morphology
9751/3: Langerhans cell histiocytosis
Effective
2010 and later
Reportable
for cases diagnosed
1978 - 2000,
2010 and later
Primary Site(s)
See Module 7
Most common sites of involvement: bone, lung, soft tissue, lymph nodes, bone marrow
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Reportability for Langerhans Cell Histiocytosis changed in 2021. For cases with diagnosis date before 2021, make sure you choose the "Help me code for diagnosis years" to 2020 or earlier.
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, only LCH disseminated/multisystem is now assigned /3 behavior (malignant). All other forms of LCH are no longer malignant (/3 behavior) and are not reportable except for benign Brain and CNS tumors (9751/1).
LCH, disseminated/multisystem is a combined pathological/clinical diagnosis. Microscopic (histological) confirmation of LCH may be done, but may not document that the tumor is disseminated/ multisystem. The disseminated/multisystem diagnosis is based on clinical evaluation by the managing/treating physician and is based on multiple areas of involvement. Not all suspected sites of involvement need to be biopsied or surgically resected.
If the only information you have is a pathology report that has LCH, NOS with no clinical evaluation on the extent of involvement, do not abstract the case.
Langerhans cell histiocytosis presents in several different forms
1. DISSEMINATED/MULTISYSTEM: Occurs in infants who present with fever, cytopenia, skin and bone lesions, and hepatosplenomegaly. Pulmonary disease in childhood is clinically variable. The skin, bone, and marrow are preferential sites of involvement. The liver and spleen are also common sites; however, they are typically metastatic and not the primary site. Involvement of bone marrow, liver, and lung are regarded as high-risk factors. (9751/3)
2. SOLITARY/UNIFOCAL form: Usually occurs in older children or adults who most commonly present with a lytic bone lesion eroding the cortex. Solitary lesions may occur at other sites as mass lesions or enlarged lymph nodes. Other sites include adjacent soft tissue (skull, femur, vertebra, pelvic bones, and ribs) and less commonly lymph nodes, skin and lung. (Only reportable if benign Brain and CNS, See 9751/1)
3. MULTIFOCAL/UNISYSTEM multifocal disease-Usually occurs in young children who present with multiple or sequential destructive bone lesions often associated with adjacent soft tissue masses. Skull and mandibular involvement is common. Diabetes insipidus follows cranial involvement. (Only reportable if benign Brain and CNS, See 9751/1)
Self-healing Langerhans cell histiocytosis (LCH) of the skin means that the disease regressed without treatment. This is a known phenomenon and is non reportable.
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, only LCH disseminated/multisystem is now assigned /3 behavior (malignant). All other forms of LCH are no longer malignant (/3 behavior) and are not reportable except for benign Brain and CNS tumors (9751/1).
LCH, disseminated/multisystem is a combined pathological/clinical diagnosis. Microscopic (histological) confirmation of LCH may be done, but may not document that the tumor is disseminated/ multisystem. The disseminated/multisystem diagnosis is based on clinical evaluation by the managing/treating physician and is based on multiple areas of involvement. Not all suspected sites of involvement need to be biopsied or surgically resected.
If the only information you have is a pathology report that has LCH, NOS with no clinical evaluation on the extent of involvement, do not abstract the case.
Langerhans cell histiocytosis presents in several different forms
1. DISSEMINATED/MULTISYSTEM: Occurs in infants who present with fever, cytopenia, skin and bone lesions, and hepatosplenomegaly. Pulmonary disease in childhood is clinically variable. The skin, bone, and marrow are preferential sites of involvement. The liver and spleen are also common sites; however, they are typically metastatic and not the primary site. Involvement of bone marrow, liver, and lung are regarded as high-risk factors. (9751/3)
2. SOLITARY/UNIFOCAL form: Usually occurs in older children or adults who most commonly present with a lytic bone lesion eroding the cortex. Solitary lesions may occur at other sites as mass lesions or enlarged lymph nodes. Other sites include adjacent soft tissue (skull, femur, vertebra, pelvic bones, and ribs) and less commonly lymph nodes, skin and lung. (Only reportable if benign Brain and CNS, See 9751/1)
3. MULTIFOCAL/UNISYSTEM multifocal disease-Usually occurs in young children who present with multiple or sequential destructive bone lesions often associated with adjacent soft tissue masses. Skull and mandibular involvement is common. Diabetes insipidus follows cranial involvement. (Only reportable if benign Brain and CNS, See 9751/1)
Self-healing Langerhans cell histiocytosis (LCH) of the skin means that the disease regressed without treatment. This is a known phenomenon and is non reportable.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Langerhans cell histiocytosis, disseminated
Definition
Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type cells that express CD1a, langerin and S100 protein and show Birbeck granules by ultrastructural examination.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
TR genes are clonally rearragned
Immunophenotyping
CD1a expression
CD30 absent
CD45 expression
CD68 positive
HLA-DR positive
Langerin (CD207) expression
S100 Protein expression
Vimentin positive
Treatments
Chemotherapy
Hormone therapy
Immunotherapy
Other therapy
Radiation therapy
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
202.5 Letterer-Siwe Disease
Corresponding ICD-10 Codes
C96.0 Letterer-Siwe Disease
Corresponding ICD-10-CM Codes (U.S. only)
C96.0 Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Progression and Transformation
Progression from initial focal disease multisystem involvement can occur, mostly in infants
Systemic and (rarely) multifocal disease can be complicated by hemophagocytic syndrome
Epidemiology and Mortality
Age: most cases occur in childhood
Incidence: 5 per million population per year
Race: more common in whites of northern Europena descent, rare in blacks
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 470-472
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 470-472
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Langerhans Cell Histiocytosis (LCH)
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
Section: General Information About Langerhans Cell Histiocytosis (LCH)
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
