Sclerosing Pneumocytoma: a Carcinoma Mimicker. A Case Report and Literature Review.

OBJECTIVES: Sclerosing pneumocytoma (SP) is a rare entity. Diagnosis of SP can be problematic especially on biopsy specimens, in particular, when the sample is obtained from the papillary component. METHODS: An asymptomatic 33 year-old female with no smoking history was incidentally found to have a lung mass. She was diagnosed to have well-differentiated adenocarcinoma with papillary features on biopsy. A lobectomy was performed. RESULTS: The circumscribed mass was composed predominately of papillary structures, with the "surface cuboidal cells" located on the surface of the papillae and "round cells" in the stalks of the papillae. Immunohistochemical staining demonstrated nuclear reactivity for TTF-1 in both cell types and Napsin A positivity only in "surface" cells. CONCLUSIONS: In evaluation of a lung biopsy from a mass with a predominantly papillary pattern, sclerosing pneumocytoma (sclerosing hemangioma in previous classifications) should always be in the differential diagnoses.

Tumefactive foreign body giant cell reaction following high-pressure paint injection injury: A case report and review of literature.

High-pressure paint injection injury is an uncommon but well-described injury. The histologic features of long-term paint injection injury with retained material are less recognized. A 46-year-old male presented clinically as "recurrent giant cell tumor of tendon sheath." The right index finger demonstrated fusiform enlargement by a pigmented mass with diffuse infiltration into the soft tissue of the hand. Histologically the tumor showed multiple giant cells in a fibrotic stroma extending into the dermis. There were multiple types of foreign material including diffuse brown black pigment, weakly optically polarizing foreign material and white inclusions with a "train track" appearance. The cells were positive for CD68 and negative for S100 antigen. Further investigation revealed that the patient had a history of high-pressure paint injection injury to his digit 6 years prior. Foreign material injected under high pressure into tissues may result in a pseudo-neoplastic foreign body granulomatous reaction that can mimic giant cell tumor of tendon sheath. Our case demonstrates that this reaction can be florid and can have slow growth over years. A high index of suspicion, a good clinical history and careful examination can distinguish these 2 entities.

Morphoproteomics provides support for TGF-ß pathway signaling in the osteoclastogenesis and immune dysregulation of osteolytic Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) has a challenging and still unclear pathogenesis. A body of literature points to impaired maturation of the lesional dendritic cells, and to immune dysregulation in the form of increased FoxP3 cells. Various cytokine abnormalities such as expression of transforming growth factor (TGF)-ß have been reported, as well as abnormalities in lipid content in LCH cells. Morphoproteomic techniques were applied to identify the signal transduction pathways that could influence histogenesis and immune regulation in osteolytic LCH. Five pediatric cases of osteolytic LCH were examined, using antibodies against CD1a, S100, CD68, CD8, FoxP3, phosphorylated (p)-STAT3 (Tyr705), protein kinase C (PKC)-α, phospholipase (PL)D1, fatty acid synthase (FASN), and zinc finger protein, Gli2. Positive and negative controls were performed. A FoxP3(+)/CD8(+) cell ratio was calculated by counting the FoxP3+ and CD8+ cells in 10 high power fields for each case. There is induction of sonic hedgehog (SHH) mediators consistent with TGF-ß signaling pathway through Smad3-dependent activation of Gli2, findings supported by the plasmalemmal and cytoplasmic expression of PKC-α and PLD1, and nuclear expression of Gli2, in lesional cells. The FoxP3+/CD8+ cell ratio is increased, ranging from 1.7-7.94. There is moderate cytoplasmic expression of FASN in most of the Langerhans cells, a finding that supports previously published phospholipid abnormalities in LCH and is consistent with PKC-α/PLD1/TGF-ß signaling. With our study, we strongly suggest that the TGF-ß cell signaling pathway is a major player in the pathogenesis of LCH, leading to non-canonical induction of nuclear Gli2 expression, thereby contributing to osteoclastogenesis in LCH histiocytes. It could also cause a state of immune frustration in LCH, by inducing the transformation of CD4(+)CD25(-) cells into CD4(+)/FoxP3(+) cells. This coincides with the clinical evidence of a response to thalidomide in patients with osteolytic LCH, given its reported ability to reduce TGF-beta 1 and FoxP3 cells. Such TGF-ß signaling in osteoclastogenesis and immune dysregulation, and the presence of FASN in the majority of cells, have additional therapeutic implications for osteolytic LCH.

Inflammatory aneurysm of the ascending aorta.

Inflammatory aneurysms of the aorta are usually seen in the infrarenal abdominal aorta and very rarely in the ascending aorta. We present the case of a 76-year-old male with inflammatory aneurysm of the ascending aorta.

Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity.

We describe a distinctive tumor of the liver in four children composed of nested spindled and epithelioid cells with extensive desmoplasia that we have termed "desmoplastic nested spindle cell tumor of the liver." All four patients were previously healthy. One patient had a presumptive diagnosis of hepatic hemangioma 11 years prior to presentation. Grossly, the tumors were well circumscribed, lobular white masses, ranging from 2.8 to 15 cm in diameter. These tumors were characterized by the presence of cohesive nests of plump, bland spindle cells arranged in short fascicles with an accompanying desmoplastic stroma. Epithelioid areas ranging from palisading epithelioid cells at the periphery of some nests to pseudoglandular and polygonal cells with intercellular bridges were invariably present. Mitotic activity was low. Calcification and ossification were present. Non-neoplastic bile ducts and hepatic elements were seen both within and surrounding the tumor cell nests. Each tumor displayed cytoplasmic reactivity for vimentin, pan-cytokeratin, CD57, and nuclear staining for WT1. Neuroendocrine markers were negative. Ultrastructurally, the tumor cells showed focally well-developed cell junctions, basal lamina, and few cytoplasmic organelles. All tumors were confined to the liver and were resected without complication. Two patients received postoperative adjuvant therapy for presumed hepatoblastoma. The patients are doing well without recurrence at 7.5 years, 7 years, 5 years, and 8 months post-surgery. The morphologic appearance and immunohistochemical profile of these lesions are unique in our experience and represent a new category of pediatric liver tumor.

Serum albumin concentration and clinical assessments of nutritional status in hospitalized older people: different sides of different coins?

OBJECTIVES: Malnutrition is common in hospitalized older people, and some have advocated routine nutritional screening. Serum albumin and clinically based measures such as the Subjective Global Assessment (SGA) are two potential methods of assessing nutritional status in hospitalized older people. Although both measures are strongly associated with prognosis, it is not clear whether they measure similar or different clinical constructs. Our goal was to assess the degree of clinical concordance between these measures. DESIGN: Cross-sectional study. SETTING: The inpatient medical service of a university teaching hospital. PARTICIPANTS: Three hundred eleven older (aged > or =70) patients. MEASUREMENTS: We independently measured serum albumin and performed the SGA on 311 older medical patients (aged > or =70) shortly after hospital admission. The SGA classified patients as well nourished, moderately malnourished (generally 5% weight loss with mild examination findings), or severely malnourished (generally >10% weight loss with marked findings) based on findings from a directed history and examination. We compared the distribution of clinical rating in patients with differing albumin levels and examined diagnostic test characteristics of albumin as a predictor of malnutrition as diagnosed on clinical examination. RESULTS: The mean age of subjects was 79.9; 64% were women, 42% were African American. Discordance between albumin and the SGA was common. For example, 38% of patients with albumin levels of 4.0 g/dL or higher were at least moderately malnourished on the SGA, whereas 28% of patients with albumin levels lower than 3.0 g/dL were rated as well nourished. No choice of albumin level was associated with simultaneously acceptable sensitivity and specificity as a predictor of SGA ratings. The area under the receiver operating characteristic curve for albumin level as a predictor of SGA rating was 0.58, suggesting that the ability of either measure to predict the other measure is only marginally better than chance. CONCLUSIONS: Albumin levels and clinical assessments, two possible measures of nutritional status in hospitalized older people, are often discordant. To some extent, this reflects limitations in both measures as markers of nutritional status. However, it also demonstrates that, in this population, albumin and clinical assessments of nutritional status reflect fundamentally different clinical processes.