The riddle of multinucleated "floret-like" giant cells and their detection in an extensive gluteal neurofibroma: a case report.

Stanger, Katrin; de Kerviler, Sora; Vajtai, Istvan; Constantinescu, Mihai Adrian (2013). The riddle of multinucleated "floret-like" giant cells and their detection in an extensive gluteal neurofibroma: a case report. Journal of medical case reports, 7(189), p. 189. BioMed Central 10.1186/1752-1947-7-189

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INTRODUCTION The neurofibromatoses are inherited tumor predisposition syndromes involving two major clinical phenotypes: neurofibromatosis type 1 (von Recklinghausen's disease) is linked to chromosome 17q, and tends to occur seven times more frequently than neurofibromatosis type 2. Neurofibromatosis type 1 entails a distinctive cutaneous manifestation prevailed upon by benign neurofibromas, which may vary in size, number and distribution. On the histological level, neurofibromas are composed of an admixture of neurilemmal cells, including Schwann cells, fibroblasts, and - to a lesser extent - perineurial cells. CASE PRESENTATION The case of a 39-year-old Caucasian man with a voluminous recurrent neurofibroma of 27×15cm extending from the left gluteal region to thoraco-lumbar levels Th6 through L4 is reported. Within the soft tissue tumor a pseudocyst of 7.3×9.3cm was found preoperatively. CONCLUSION Histopathological study of the excised mass was conspicuous for revealing a large number of multinucleated floret-like giant cells within an otherwise classical soft tissue neurofibroma.Previous reports on neurofibromas with multinucleated floret-like giant cells are distinctly scant. Available evidence from the literature does not suggest any consistent correlation of multinucleated floret-like giant cells in neurofibromas with gender, age, traumatic antecedents, size of the lesion, recurrence, or malignant transformation. Furthermore, the presence of such cells may not be specific for neurofibromatosis type 1, as they occasionally are encountered in some unrelated mesenchymal neoplasms as well.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Orthopaedic, Plastic and Hand Surgery (DOPH) > Clinic of Plastic and Hand Surgery
04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Department of Orthopaedic, Plastic and Hand Surgery (DOPH) > Clinic of Plastic and Hand Surgery > Hand Surgery

UniBE Contributor:

de Kerviler, Sora; Vajtai, Istvan and Constantinescu, Mihai Adrian

Subjects:

600 Technology > 610 Medicine & health
500 Science > 570 Life sciences; biology

ISSN:

1752-1947

Publisher:

BioMed Central

Language:

English

Submitter:

Ekkehard Hewer

Date Deposited:

15 Feb 2017 14:02

Last Modified:

19 Feb 2017 02:16

Publisher DOI:

10.1186/1752-1947-7-189

PubMed ID:

23890233

BORIS DOI:

10.7892/boris.95904

URI:

https://boris.unibe.ch/id/eprint/95904

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