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[Langerhans cell histiocytosis of the temporal bone in children: 7 cases analysis]
Insights
Langerhans cell histiocytosis (LCH) in children's temporal bone presents varied symptoms. Single-system LCH shows a better prognosis than multisystem LCH, highlighting distinct clinical outcomes.
Area of Science:
- Pediatric Oncology
- Otolaryngology
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder affecting children.
- Temporal bone involvement in LCH presents unique diagnostic and therapeutic challenges.
Observation:
- Seven pediatric cases of temporal bone LCH were analyzed retrospectively.
- Common symptoms included temporal tumors, otorrhea, otalgia, hearing loss, and external auditory canal granulation.
- CT imaging revealed osteolytic destruction without sclerotic borders.
Findings:
- Four cases regressed, one remained stable, and two progressed after 1-5 years of follow-up.
- Prognosis significantly differed between single-system (better outcome) and multisystem LCH (P < 0.05).
Implications:
- Early diagnosis and tailored therapy are crucial for managing pediatric temporal bone LCH.
- Distinguishing between single and multisystem disease is vital for predicting patient outcomes.
- Further research into optimal treatment strategies for temporal bone LCH is warranted.
Objective:
To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone in children.
Method:
Seven children with LCH of the temporal bone n our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations, imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed.
Result:
Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external auditory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were. received different therapies. Followed up for 1-5 years, 4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly different from the single system group (P < 0.05).
Conclusion:
The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.
