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[Orbital location of multivisceral Langerhans-cell histiocytosis]

M Abdellaoui1, A-I Benatiya, S Bhallil

  • 1Service d'ophtalmologie, hôpital Omar Drissi, CHU Hassan II, Fès, Maroc. mari2082002@yahoo.fr

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|May 17, 2011
PubMed

Insights

Langerhans cell histiocytosis (LCH) is a rare orbital tumor. This case highlights successful chemotherapy treatment for a multivisceral LCH presentation in an infant.

Area of Science:

  • Pediatric Oncology
  • Ophthalmology
  • Histiocytosis

Background:

  • Langerhans cell histiocytosis (LCH) is a rare neoplastic proliferation of Langerhans cells.
  • Orbital tumors are uncommon in LCH, representing less than 1% of cases.

Observation:

  • An 18-month-old infant presented with left exophthalmos, polydipsia, and polyuria.
  • Imaging revealed an orbital mass with bone destruction and soft tissue infiltration, alongside widespread secondary lesions.
  • Biopsy confirmed Langerhans cell histiocytosis.

Findings:

  • The infant had multivisceral LCH involving the orbit, skull, ribs, skin, liver, and spleen.
  • Chemotherapy with vinblastine and steroids was administered.
  • The child showed a positive response to treatment.

Implications:

  • This case underscores the importance of considering LCH in infants with orbital masses and systemic symptoms.
  • Multivisceral LCH requires prompt and aggressive multimodal therapy.
  • Successful treatment can lead to long-term remission.

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