Nested stromal epithelial tumor is a recently described primary neoplasm of the liver. This tumor is characterized by well-demarcated nests of spindle and epithelioid cells with occasional calcification and bone formation. An association between these tumors and Cushing syndrome has been described. Herein we report a case of a recurrent nested stromal epithelial tumor of the liver in a 17-year-old female with aggressive clinical behavior and an extrahepatic lymph node metastasis. Also, we provide the first detailed clinical, histologic, immunohistochemical, and cytogenetic comparison of the original and recurrent tumors. Initially, the patient presented with Cushingoid symptoms and epigastric pain, radiating to her back. A computed tomographic (CT) scan revealed a large lesion in the liver. After a partial hepatectomy, the Cushingoid features were resolved. A year later, a CT scan revealed multiple lesions within the liver, and positron emission tomographic/CT imaging showed a hypermetabolic lymph node. The patient underwent a cadaveric liver transplant. Histologically, both the original and recurrent tumors had similar characteristics, with different immunoreactivity, correlating with the absence of systemic hormonal symptoms. Electron microscopy of the original neoplasm revealed an abundance of rough cytoplasmic reticulum and mitochondria. No evidence of endocrine differentiation was found. Cytogenetics of the primary tumor was complex with an abnormal hypotriploid karyotype. Our data indicate that patients with nested stromal epithelial tumor of the liver must be carefully followed with imaging to detect hepatic recurrence and extrahepatic metastases.

Introduction: Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium. Case: A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT) post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status. Conclusion: In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI) are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial sarcoma is not well established. Surgery is the cornerstone of treatment. In non-resectable tumors, aggressive neo-adjuvant chemotherapy with ifosfamide followed by surgical resection and EBRT may lead to improved outcome.

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Bronchopulmonary involvement is a rare but well documented extraintestinal manifestation of inflammatory bowel disease (IBD). IBD-related pulmonary disease can range from subglottic stenosis to tracheobronchitis to interstitial lung disease and is often misdiagnosed on initial presentation. We present a case of tracheobronchitis with stridor in a 23-year-old-woman with well controlled ulcerative colitis (UC).