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Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Presentation of LCH in Children

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Oral cavity

In the mouth, presenting symptoms include gingival hypertrophy and ulcers of the soft or hard palate, buccal mucosa, or on the tongue and lips. Hypermobile teeth (floating teeth) and tooth loss usually indicate involvement of underlying bone.[6,7] Lesions of the oral cavity may precede evidence of LCH elsewhere.

Bone

LCH can occur in any bone of the body, although the hands and feet are often spared. Sites of LCH in children include the following:

  • Lytic lesion of the skull: The most frequent site of LCH in children is a lytic lesion of the skull vault,[8] which may be asymptomatic or painful. It is often surrounded by a soft tissue mass that may extend internally to impinge on the dura.
  • Femur, ribs, humerus, and vertebra: Other frequently involved skeletal sites are femur, ribs, humerus, and vertebra. Spine lesions may involve any vertebra, although involvement of the cervical vertebrae is most common and spine lesions are frequently associated with other bone lesions. Spine lesions may result in collapse of the vertebral body (vertebra plana). Vertebral lesions with soft tissue extension often present with pain and may present with significant neurologic deficits,[9] an indication for an urgent magnetic resonance imaging (MRI) scan.
  • CNS-risk bones: Proptosis from an LCH mass in the orbit mimics rhabdomyosarcomas, neuroblastoma, and benign fatty tumors of the eye.[10]

    Lesions of the facial bones or anterior or middle cranial fossae (e.g., temporal, orbit, sphenoid, ethmoid, zygomatic) with intracranial tumor extension comprise a CNS-risk group. These patients have a threefold increased risk of developing diabetes insipidus and other CNS disease. Because of the increased risk of diabetes insipidus, treatment is recommended for these patients.

Lymph nodes and thymus

The cervical nodes are most frequently involved and may be soft- or hard-matted groups with accompanying lymphedema. An enlarged thymus or mediastinal node involvement can mimic an infectious process and may cause asthma-like symptoms. Accordingly, biopsy with culture is indicated for these presentations. Mediastinal involvement is rare (<5%) and usually presents with respiratory distress, superior vena cava syndrome, or cough and tachypnea. The 5-year survival is 87%, with deaths mostly attributable to hematologic involvement.[11]

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