Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021843 (bowel obstruction)
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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.
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PMID:Malignant Peritoneal Mesothelioma in an Adolescent Male With BAP1 Deletion. 2522 65