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

Öz

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 pathologists and the clinicians that this rare entity should be remembered in the differential diagnosis of spindle-cell tumors in childhood

Anahtar Kelimeler

• Childhood, Lip, Sclerosing rhabdomyosarcoma

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

Öz

AbstractRhabdomyosarcoma 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 rhabdomyosarcomaÖzetRabdomyosarkom; 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