title: Clear cell myomelanocytic tumor (PEComa) of the duodenum in a child with a history of neuroblastoma.
abstract: We report herein a case of digestive clear cell myomelanocytic tumor (PEComa) that is unique in its location and presentation. The lesion, located in the duodenal wall, was diagnosed in a child with a history of cervical neuroblastoma that was in remission after surgical resection and chemotherapy. The diagnosis was obtained by examination of a biopsy specimen taken during laparoscopy. The decision was made to perform surgical resection. Examination of the surgical specimen confirmed the diagnosis of PEComa. No metastasis was found. After 2 years of follow-up, the patient is alive, without evidence of metastasis or recurrence. This case highlights the distinctive characteristics of the cells in PEComa, recognizable even on limited biopsy material. It also suggests a possible association between PEComa and neuroblastoma, 2 unusual tumors that belong to the spectrum of lesions known to occur in patients with tuberous sclerosis and that may share a possible common pathogenetic mechanism.
authors.affiliation: The Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Lyon, France.
authors.forename: Tony
authors.initials: T
authors.lastname: Mhanna
authors.forename: Dominique
authors.initials: D
authors.lastname: Ranchere-Vince
authors.forename: Valérie
authors.initials: V
authors.lastname: Hervieu
authors.forename: Dominique
authors.initials: D
authors.lastname: Tardieu
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Scoazec
authors.forename: Christian
authors.initials: C
authors.lastname: Partensky
mesh_terms: D000230:Adenocarcinoma / Q000175:diagnosis; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D003937:Diagnosis, Differential; D004379:Duodenal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D004701:Endocrine Gland Neoplasms / Q000175:diagnosis; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D006801:Humans; D008297:Male; D008544:Melanocytes / Q000737:chemistry / Q000473:pathology*; D016609:Neoplasms, Second Primary; D009447:Neuroblastoma / Q000473:pathology*; D016577:Pancreaticoduodenectomy; D018227:Sarcoma, Clear Cell / Q000175:diagnosis; D018235:Smooth Muscle Tumor / Q000737:chemistry / Q000473:pathology*; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor