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Fibrous histiocytoma of the nose in an 8-year-old girl
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  1. Gargi Pandey,
  2. Kiran Varadharajan,
  3. Sevasti Konstantinidou and
  4. Benjamin Hartley
  1. ENT, Great Ormond Street Hospital for Children NHS Trust, London, UK
  1. Correspondence to Dr Gargi Pandey; gargi.pandey{at}nhs.net

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Description

A fit and well 8-year-old girl was referred to a tertiary paediatric centre due to a slow growing swelling around the nasal dorsum. Examination revealed no skin changes or pits and she reported no associated nasal symptoms. At her local district general hospital, a differential diagnosis of a possible nasal dermoid was made, however, a subsequently organised MRI scan suggested an extra nasal glioma. She had a biopsy of the lesion at the tertiary centre which was in keeping with non-langerhans histiocytosis. After a multidisciplinary discussion and CT imaging (figure 1) to aid surgical planning, the patient proceeded for excision of the non-malignant tumour via a Lynch Howarth incision. The final histology concluded a benign fibrous histiocytoma (BFH) (figure 2).

Figure 1

CT showing a nasal lesion centred on the superior nasal dorsum, slightly right eccentric. It is causing partial erosion of adjacent nasal bones, nasal septum and right frontal sinus.

Figure 2

H&E stained section (×10 magnification) showing spindled cells admixed with Touton-like giant cells and foamy macrophages.

BFH is known to occur in the skin and deep soft tissues of the extremities and retroperitoneum.1 Most frequently BFH is found in sun exposed areas of the skin or can be associated with areas of trauma or recurrent infections.2 Involvement of the nose is rare; less than five cases have been found in children, all symptomatic.1–4 Our patient was asymptomatic, only presenting due to a nasal swelling. BFH is usually a painless and slow growing mass with symptoms secondary to the invasion of anatomy around it. Differential diagnoses for a child with a nasal swelling include nasal dermoid, glioma or meningoencephalocele. CT or MRI is warranted to delineate the anatomy but also intracranial connections. Management involves biopsy for definitive diagnosis and surgical excision in a tertiary paediatric hospital, with good long-term prognosis. There is also a role for chemotherapy.5 We opted for surgical excision. The patient’s mother was made aware that if local recurrence were to occur then along with surgical management, chemotherapy would be reconsidered.

Learning points

  • Fibrous histiocytoma should be added to this list of differentials for slow growing head and neck masses in children.

  • CT and MRI are commonly done to aid surgical planning and further characterise the lesion for diagnosis.

  • Management of malignant fibrous histiocytoma can include radiotherapy, chemotherapy and surgery, however, the mainstay of management for benign fibrous histiocytoma is surgical excision.

Acknowledgments

We would like to thank Dr Mohammad Haini (Registrar in Paediatric Pathology) and Dr Thivya Sekar (Consultant Paediatric pathologist) for helping us with the pathology image and report.

References

Footnotes

  • Contributors GP is the main author of this manuscript. The case was identified by KV and is under the care of BH. SK was involved in the care of the patient. All listed authors were involved in the care of the patient, gave suggestions and reviewed the manuscript prior to submission.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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