Primary epithelioid rhabdomyosarcoma of the stomach: a case report and review of literature

Runde Jiang,Yangkun Wang,Zhishang Zhang,Pei Guo,Zuguo Li

doi:10.1186/s13000-019-0917-y

Runde Jiang, Yangkun Wang + Show 3 more

Open Access

https://doi.org/10.1186/s13000-019-0917-y

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Abstract

BackgroundEpithelioid rhabdomyosarcoma is a rare tumor that generally occurs in the bladder, the parotid gland, or the skin of the neck. We describe an unusual case of primary epithelioid rhabdomyosarcoma of the stomach and review the literature.Case presentationA 64-year-old woman presented with a lesion at the gastroesophageal junction. Histopathological examination showed irregularly sized round cells with low cytoplasmic content and eccentric nuclei. Mitotic figures were present. Fibrovascular septa and areas of necrosis were observed between tumor cells. Tumor cells were strongly positive for MyoD1, desmin, and myogenin, and weakly positive for actin, CD56, and PGP9.5. The ki-67 index was ≥90%.ConclusionsPrimary epithelioid rhabdomyosarcoma of the stomach is extremely rare. Better awareness of this entity is necessary for early diagnosis and treatment.

Highlights

Epithelioid rhabdomyosarcoma is a rare tumor that generally occurs in the bladder, the parotid gland, or the skin of the neck
We report an unusual case of Epitheloid rhabdomyosarcoma (ERMS) of the esophagogastric junction and discuss the differential diagnosis
Embryonal rhabdomyosarcoma accounts for 60% of cases in children and occurs mostly in the genitourinary or head and neck regions

Summary

Introduction

Epithelioid rhabdomyosarcoma is a rare tumor that generally occurs in the bladder, the parotid gland, or the skin of the neck. The World Health Organization (WHO) classification includes four types: embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, spindle cell rhabdomyosarcoma, and pleomorphic rhabdomyosarcoma [1,2,3]. We report an unusual case of ERMS of the esophagogastric junction and discuss the differential diagnosis. Case presentation A 64-year-old woman came to the Outpatient Department of Shenzhen Hospital of Southern Medical University with numbness and weakness in the limbs.

Results

Conclusion