Sclerosing Rhabdomyosarcoma of the Lip in a Child; A Case Report

Özgür İlhan Çelik, Yelda Dere, Serkan Yaşar Çelik, Burçin Tuna

Özet


Abstract

Rhabdomyosarcoma is the most common malignancy of soft tissue in childhood and adolescence. It has been traditionally classified into 3 main subtypes: embryonal, alveolar and pleomorphic. However a new, rare subtype called Spindle cell/sclerosing Rhabdomyosarcoma has been defined recently and classified as the fourth variant by the World Health Organization in 2013. We report a case of a 4 year-old girl who had a solid mass in her lower lip with a size of 1cm.  In the histopathologic examination the lesion was predominantly composed of mitotic active spindle-shaped cells with hyperchromatic nuclei in a sclerotic stroma and some rhabdomyoblastic cells with eosinophilic cross-striated cytoplasm. All of these cells were stained positive with Vimentin, Desmin, Myogenin, CD 99 and CD 56. Therefore the lesion was diagnosed as Spindle cell/sclerosing Rhabdomyosarcoma. This case reminds pathologists and the clinicians that this rare entity should be remembered in the differential diagnosis of spindle-cell tumors in childhood.

Keywords: Childhood,  Lip, Sclerosing rhabdomyosarcoma

Özet

Rabdomyosarkom; çocukluk ve adolesan çağda yumuşak dokunun en sık görülen malignitesidir.  Klasik olarak embriyonel, alveoler ve pleomorfik olarak 3 ana subtipe ayrılmaktadır.  Ancak son olarak, yeni ve nadir görülen, İğsi hücreli/Skrerozan Rabdomyosarkom olarak tanımlanan bir subtipi, 2013 yılında Dünya Sağlık Örgütü 4. varyant  olarak sınıflamaya dahil etmiştir. Yazımızda alt dudağında 1cm çaplı solid bir kitlesi olan 4 yaşında bir kız çocuğunu tartışmaktayız. Bu kitle histopatolojik olarak sklerotik stromada çoğunlukla mitotik olarak aktif, hiperkromatik nükleuslu, iğsi- hücreli ve bir kısmı da eozinofilik, çizgili sitoplazmalı rabdomyoblastik görünümlü,  Vimentin, Desmin, Myogenin, CD 99 ve CD 56 ile pozitif boyanan hücrelerden oluşmaktaydı. Bu nedenle olgu İğsi hücreli/Sklerozan Rabdomyosarkom olarak tanı aldı. Bu olgu patolog ve klinisyenlere çocukluk çağının İğsi hücreli tümörlerinin ayırıcı tanısında nadir bir varyant olarak akılda bulundurulması gerektiğini vurgulamak amacıyla sunulmuştur.

Anahtar Kelimeler: Çocukluk çağı, Dudak, Sklerozan rabdomyosarkom


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Referanslar


References

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