Table of Contents
Definition / general | Radiology description | Radiology images | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Electron microscopy description | Electron microscopy images | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/boneLCH.html. Accessed July 14th, 2017.
Definition / general
- Formerly called Histiocytosis X
- Either solitary bone involvement, multiple bone involvement (variable skin involvement) or multiple organ involvement (bone, liver, spleen, other sites)
- Ages 5 - 15 years, 60% male
- Neoplastic, although cause unknown
- Sites: skull, jaw, humerus, rib, femur; metaphysis or diaphysis
Radiology description
- Lytic masses that may extend into soft tissue
Radiology images
Case reports
- 2 year old boy with tender, palpable mass in right parietal region (Case of the Week #72)
Treatment
- Often recurs after excision (Pediatr Blood Cancer 2007;48:696)
Gross description
- Sharply circumscribed
Microscopic (histologic) description
- Infiltration by Langerhans cells (polygonal cells with eosinophilic cytoplasm, oval nuclei with longitudinal grooves resembling coffee beans)
- Eosinophils, giant cells, neutrophils, foam cells, lymphocytes, plasma cells, fibrosis, necrosis; may have typical and atypical mitotic figures
Microscopic (histologic) images
Images hosted on PathOut server:
Images hosted on other servers:
Contributed by Dr. Saroona Haroon, The Aga Khan University Hospital (Pakistan):
Images hosted on other servers:
Contributed by Dr. Saroona Haroon, The Aga Khan University Hospital (Pakistan):
Cytology description
- Highly cellular with large, polygonal cells with ample cytoplasm, nuclei are round, oval or bean shaped with fine and even chromatin and prominent longitudinal grooves
- Inconspicuous nucleoli, mild pleomorphism
- No / minimal mitotic figures
- Scattered eosinophils and neutrophils and multinucleated osteoclast-like giant cells
- Necrosis common
Cytology images
Electron microscopy description
- Birbeck granules (electron dense cross striations)
Electron microscopy images
Molecular / cytogenetics description
- Often loss of DNA sequences involving several chromosomes and 1p (Hum Pathol 2002;33:555)
Differential diagnosis
- Acute myelomonocytic leukemia
- Lymphoma: lacks nuclear features
- Macrophages
- Mastocytosis
- Osteomyelitis
- Sinus histiocytosis with massive lymphadenopathy
Additional references
































