title: Malignant Peritoneal Mesothelioma in an Adolescent Male With BAP1 Deletion.
abstract: Diffuse malignant peritoneal mesotheliomas in children are uncommon, aggressive tumors with a grave prognosis. We herein report the clinical, radiologic, and pathologic findings of a 16-year-old male. The adolescent presented with a history of abdominal pain, nausea and daily, nonbilious, nonbloody emesis for 3 weeks. Radiographic imaging suggested small bowel obstruction. The diagnostic work-up and differential diagnoses are discussed. Histologically, the tumor was composed of epithelioid cells with a papillary and glandular architectural pattern. A few glands appeared to produce mucinous material. Histochemistry revealed PAS diastase resistant mucin, an inconspicuous finding in diffuse malignant peritoneal mesothelioma. An extensive immunohistochemistry panel (calretinin, WT-1, D2-40, CK 7, CAM 5.2, CK 5/6, CEA, B72.3, CK 20, CD10, CD30, CD15, CD117, PLAP, S100, TFE3, and EMA) confirmed the diagnosis. Of special interest, BAP1 staining was cytoplasmic and consistent with 3p deletion detected by conventional cytogenetics. The ultrastructural analysis demonstrated long microvilli, desmosomes, and intercellular junctions which further supported the diagnosis.
authors.affiliation: Departments of *Pathology and Laboratory Medicine †Hematology and Oncology ‡Surgery §Radiology, Phoenix Children's Hospital, Phoenix, AZ.
authors.forename: Steve
authors.initials: S
authors.lastname: Taylor
authors.forename: David
authors.initials: D
authors.lastname: Carpentieri
authors.forename: James
authors.initials: J
authors.lastname: Williams
authors.forename: Juan
authors.initials: J
authors.lastname: Acosta
authors.forename: Richard
authors.initials: R
authors.lastname: Southard
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D017353:Gene Deletion; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000235:genetics* / Q000473:pathology*; D008297:Male; D008654:Mesothelioma / Q000235:genetics* / Q000473:pathology*; D000086002:Mesothelioma, Malignant; D010534:Peritoneal Neoplasms / Q000235:genetics* / Q000473:pathology*; D025521:Tumor Suppressor Proteins / Q000235:genetics*; D043222:Ubiquitin Thiolesterase / Q000235:genetics*
chemical_list: C400134:BAP1 protein, human; D014408:Biomarkers, Tumor; D025521:Tumor Suppressor Proteins; D043222:Ubiquitin Thiolesterase