Eriko Kinoshita

Adenomatoid tumor of the pleura.

A case of adenomatoid tumor of the pleura is reported, and its differential diagnosis from benign and malignant pleural lesions is discussed. A small pleural nodule was incidentally found during a thoracic operation in a 54‐year‐old woman with esophageal cancer. The patient had no history of exposure to asbestos, and was well with no sign of recurrence 14 months after the operation. A 7 mm, circumscribed tumor had characteristic features of adenomatoid tumor. The tumor was composed of an aggregation of irregularly shaped tubulocystic spaces with fibrous stoma. The spaces were lined by flattened and occasional cuboidal epithelioid cells with cytoplasmic vacuolization, and several spaces contained pale blue mucinous fluid. On immunohistochemistry the tumor cells were positive for AE1/AE3, CAM5.2, vimentin, cytokeratin 5/6, D2‐40, calretinin, thrombomodulin, and WT‐1, but negative for CEA, Leu M1 (CD15), thyroid transcription factor‐1, epithelial membrane antigen, desmin, glucose transporter‐1 (GLUT‐1), CD31, and CD34. The MIB‐1 (Ki‐67) labeling index was 1–2%, indicating low proliferative activity. Adenomatoid tumor of the pleura is rare, and the pathogenesis has not been elucidated. Recognition of these benign mesothelial lesions in the pleura is important to avoid misdiagnosis. The immunohistochemistry in the present case supports its mesothelial origin.

Secondary malignant giant cell tumor of the clivus: Case report

Giant cell tumors (GCTs) of bone are rare primary bone neolasms, representing approximately 5% of skeletal tumors. The piphyses of the long bones, particularly the distal femur, proxial tibia, and distal radius, account for 75–90% of GCTs [1]. GCTs arely occur in the skull, and comprise less than 1% of all reported hese tumors of bone GCTs and also preferentially involve the spheoid and temporal bones [2]. Malignancy arising in a GCT can be xpected in less than 2% of GCTs. Malignant transformation of GCT n the skull is exceptionally rare [3]. We report a case of GCT of the livus, which underwent malignant transformation 10 years after nitial treatment. We discuss the diagnosis and therapeutic considrations of GCT of the sphenoid, including the clivus, based on a eview of the pertinent literature.

Solitary intrapulmonary cystic lymphangioma in an infant: A case report with literature review

Lymphangioma rarely presents as a solitary pulmonary lesion. We encountered a case of solitary cystic lymphangioma and present its clinicopathologic and immunohistochemical findings. A 2-month-old boy was referred to the hospital after developing a persistent cough. Chest X-ray showed a large cyst in the right lung. Under the preoperative diagnosis of bronchogenic cyst, he underwent right lower lobectomy at the age of 11 months. The resected specimen contained a 5.5-cm septate cystic lesion. Microscopically, the lesion consisted of a large cystic space and interconnected slit-like spaces surrounding bronchovascular islands. The cyst was lined by a monolayer of flat cells with focal multinucleated giant cells. Immunohistochemically, the cells lining the cystic lesion were positive for D2-40, Prox1, CD34, and CD31, and weakly positive for VEGFR-3, but were negative for AE1/3, HMB45, VEGF-A, VFGF-C, VEGFR-1. Differential diagnoses included lobar or interstitial emphysema, bronchogenic cyst, congenital pulmonary airway malformation and alveolar adenoma. D2-40 and Prox1 were useful in differentiation and in determining the extent of the lesion. A review of the literature found only 15 cases of solitary pulmonary lymphangioma. In younger patients, the lesions tend to occupy more of the lung. Focal giant cell reaction has not been described in the reported papers.

Adult-onset renal failure in a family with Alagille syndrome with proteinuria and a novel JAG1 mutation

Alagille syndrome (AGS) is an autosomal-dominant multi-organ disorder involving the liver, heart, eyes, face and skeleton. In addition, various renal abnormalities have also been reported in several cases. We describe a patient with a novel frameshift mutation in exon 12 of the JAG1 gene who presented with chronic renal failure. In this family, five members of three generations had clinical features implicated in AGS. Three members had adult-onset renal dysfunction with proteinuria, and two of them required haemodialysis therapy. AGS should be considered in the differential diagnosis of proteinuric renal disease, even in adult patients.

Localized Pigmented Villonodular Synovitis of the Hip: Sudden-Onset Pain Caused by Torsion of the Tumor Pedicle

Pigmented villonodular synovitis is a rare, benign, but potentially locally aggressive disease that should be considered in younger patients who present with monoarticular joint symptoms and pathology. We present the case of a 33-year-old woman with a mass arising from her right hip joint that was examined using a multimodal radiological approach. Because her clinical presentation mimicked that of synovial osteochondromatosis of the hip, surgical dislocation was performed. Histopathological examination of the resected specimen confirmed the diagnosis of localized pigmented villonodular synovitis, with the mass consisting of proliferation of fibrohistiocytic cells, abundant hemosiderin, foamy histiocytes, and occasional giant cells. Because of the presence of tumor necrosis, we hypothesize that torsion of the tumor pedicle was the cause of acute presentation.