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Joseph Junewick, MD FACR
over 7 years ago
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Langerhans Cell Histiocytosis

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: US
Activities:
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History

A 2 year old male presenting with papular rash on scalp of face, back and extremities, chronic otitis media, difficulty breathing, jaundice and failure to thrive.


Case Images


Diagnosis

Langerhans Cell Histiocytosis (LCH) of the Thyroid Gland

Clinical Notes

Laboratory:
Tbil 10.2 mg/dL
AST 184 IU/L
ALT 112 IU/L
GGT 525 IU/L
Hepatitis, CMV, EBV (-)

Abdominal US:
Hepatomegaly and periportal edema.

Pathology:
Skin biopsy demonstrated monotonous cells staining positive for S100 and CD1a consistent with LCH.

Findings

CT of the chest at initial presentation demonstrates an enlarged heterogeneous thymus, reticulonodular pulmonary infiltrates with scattered air cysts and a hypodense thyroid gland related to LCH. CT 2 months after initiation of therapy shows some restoration of normal thyroid architecture. Ultrasound demonstrates interstitial hypoechogenicity which is more focally prominent centrally.

Discussion

LCH is a disorder of immune dysfunction related to clonal proliferation of Langerhans cells. The typical patient is a young male caucasion although all ages, genders and races can be affected. Prognosis is related to the extent of systemic involvement. LCH can involve any organ. LCH of the thyroid is almost always associated with multisystem disease but is occasionally a unifocal process. Histiocytic infiltration results in thyromegaly and disorganized parenchyma or masses on imaging.

Reference

-Schmidt S, et al. Extraosseous involvement of Langerhan’s Cell Histiocytosis in Children. Ped Radiol 2004 34:313-322.
-Behrens RJ, et al. Langerhan’s Histiocytosis of the Thyroid: A Report and Review of the Literature. Thyroid 2001 11:697-705.
-Mrad K, et al. Langerhan’s Cell Histiocytosis of the Thyroid: A Rare Disease not to be Ignored. Ann Pathol 2002 22:35-38.



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