Bone
Other tumors of bone
Langerhans cell histiocytosis


Topic Completed: 1 June 2005

Revised: 11 November 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed search: langerhans cell histiocytosis [title] bone

Nat Pernick, M.D.
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Cite this page: Pernick N. Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/boneLCH.html. Accessed November 15th, 2019.
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 of the Week #72

Involvement of both parietal bones with soft tissue extension #1 (plain film)

#2 (CT scan)


AFIP images

Eosinophilic granuloma


Contributed by Dr. Mark R. Wick

Skull xray

Case reports
Treatment
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

AFIP images

Eosinophilic granuloma


Contributed by Dr. Mark R. Wick

Langerhans cell disease



CD1a

Langerin stain

S100



Images hosted on other servers:

(a) H&E;
(b) CD1a



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

Case of the Week #72

Pap stain of parietal bone


Contributed by Dr. Mark R. Wick

Touch preparation,
Diff-Quik stain


Images hosted on other servers:

B: Diff Quik; C: Pap (arrow at eosinophil); D: CD1a; E: H&E

Positive stains
Negative stains
Electron microscopy description
  • Birbeck granules (electron dense cross striations)
Electron microscopy images

Contributed by Dr. Mark R. Wick

Birbeck granules

Electron micrograph

Molecular / cytogenetics description
Additional references
Solitary bone involvement
Definition / general
  • Also called eosinophilic granuloma
  • Accounts for most cases
  • Usually young adults with localized pain
  • Lesions may spontaneously regress

Sites
  • Any bones but hands and feet; most common is cranial vault, jaw, humerus, rib, femur

Radiology description
  • Osteolytic lesion of metaphysis of long bones, with variable periosteal proliferation
  • May resemble metastatic carcinoma or Ewing / PNET
  • May extend into soft tissue if bone fracture
  • Skull lesions have area of lucency with "hole in hole" appearance due to different rates of destruction of two tables of bone in skull

Case reports
Treatment
  • Excision but may recur; also small doses of radiation
  • Excellent prognosis
Multiple bone involvement
Definition / general
  • Also called multiple or polyostotic eosinophilic granuloma, Hand-Schüller-Christian disease
  • May cause proptosis, diabetes insipidus, chronic otitis media
  • Prolonged clinical course with regression and relapse
  • Usually favorable outcome
Multiple organ involvement
Definition / general
  • Lung and skin most commonly involved in addition to bone; also lymphadenopathy, diabetes insipidus

Prognostic factors
  • Poor prognosis if < 18 months old at diagnosis, hepatomegaly, anemia, bone marrow involvement, thrombocytopenia, hemorrhagic skin lesions
  • Histologic features not predictive of clinical course
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