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RESIDENT
CASE
STUDIES
November 12,
2003: Case 2 Table
of Contents | List
of Diagnoses | Case 1 | Case 2 9 year old female with
forehead lesion after falling
Discussion by Michelle Mudge, DO
Langerhans cell histiocytosis (LCH) is an uncommon lesion. It is a
neoplastic proliferation of langerhans cells. It is also known as eosinophilic granuloma, histiocytosis X. Clinical variants of the disease are Hand-Schuller-Christian disease and Letterer-Siwe disease.
Incidence: LCH having bone involvement has a wide age distribution, from the first month to the 8th decade of life.
It is commonly seen in patients less than 10 years of age.
There is a tendency for LCH to involve the bone of the skull notably the calvarium (as in this patient), where it usually presents with pain and
swelling of the affected bone. It can however affect other bones. Radiology shows a well demarcated lytic lesion. Involvement of the vertebra may lead to collapse.
Prognosis: Usually good. Disseminated disease with visceral
involvement may have bad prognosis.
Histology:
- Recognition of Langerhans cells – intermediate-sized cells with indistinct cytoplasmic
borders; clear to eosinophilic cytoplasm with oval, frequently indented nuclei. The nuclei are irregular and typically possess nuclear grooves (bean shaped).
- Inflammatory infiltrate -- large numbers of eosinophils, as well as lymphocytes, neutrophils and plasma cells.
- There may be necrosis -- no effect on prognosis.
- Multinucleated osteoclast -- like giant cells and occasional lipid laden histiocytes may be present.
Immnohistochemistry:
- CD1a – membrane based positivity (image 3)
- S100 – cytoplasmic and nuclear positivity (image 4)
- CD45 – negative
Differential Diagnosis:
- Xanthogranuloma
- Reticulohistiocytoma
- Cutaneous T-cell lymphoma
References:
- Fletcher CDM, Unni KK, Mertens F. (Eds.): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press: Lyon 2002.
- Haber M. et al, Differential diagnosis in surgical pathology, Copyright 2002.
- Stephen S Sternberg et al. (Eds): Diagnostic surgical pathology, Volume 1, 3rd edition. Lippincott William & Wilkins.
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