This study is one of the few to characterize immunohistochemically the distribution and localization of Receptor-Associated Protein (RAP) in human autopsy brain. The results show prominent cortical neuronal localization. RAP is clearly identified in large neuronal dendritic/axonal processes. RAP is expressed in both large pyramidal and smaller interneurons. Occasional, much less frequent RAP is detectable in glial cells in white matter, which appear to be predominantly astrocytic. Although RAP is detectable immunohistochemically in Alzheimer's disease autopsy brain, the level of expression appears significantly reduced relative to age-matched control brains. These results suggest, at the immunohistochemical level, that there is a reduction of RAP protein in Alzheimer's disease brain (cortex). In terms of Alzheimer's disease pathophysiology, a reduction of neuronal RAP could then lead to reduced membrane expression of LRP, since RAP has also been shown to be an LRP antagonist.

John Provias

Brian Jeynes

Immunohistochemical Detection of Receptor-Associated Protein in Normal Human Brain and Alzheimer's Disease

Cystic lesions of the gallbladder are very rare and they are generally lined by single columnar or mucinous epithelium. A ciliated cyst of foregut origin is extremely rare in gallbladder. To our knowledge, only five cases have been reported so far. Here, we present the sixth case found incidentally in ultrasonographic examination in a 41-year-old woman suffering from chronic right upper quadrant pain. Laparoscopic cholecystectomy was performed for the gallstones and a benign appearing cyst in ultrasonography. Macroscopically, a submucosal unilocular cyst was located in the neck of the gallbladder. There was no communication with the lumen. Histologically, the cyst was lined by pseudostratified ciliated epithelium containing goblet cells and had a muscular wall.  The postoperative course was uneventful. Patient was discharged on the second day of the operation and was well after 2 months.

Asiye &#350;afak Bulut

Kaan Karayal&#231;&#305;n

Ciliated Foregut Cyst of the Gallbladder: Report of a Case and Review of Literature

There are few pathologic descriptions of fatal coronary artery disease in the young. The morphologic characteristics of sudden coronary deaths in 47 hearts from patients younger than 40 years were studied. Numbers of plaques with necrotic cores were quantitated in each heart. Compared to 194 sudden coronary deaths &#x3E;40 years, heart weight was lower, acute plaque erosions more frequent, and extent of disease less in the <svg style="vertical-align:-1.29163pt;width:15.175px;" id="M1" height="11.25" version="1.1" viewBox="0 0 15.175 11.25" width="15.175"  xmlns="http://www.w3.org/2000/svg">
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</svg>40 years group. Plaque burden was less in hearts with erosions, and healed infarcts more common in hearts with stable plaque. The numbers of fibroatheromas increased with age until the 6th decade (<svg style="vertical-align:-0.30096pt;width:63.962502px;" id="M2" height="11.0625" version="1.1" viewBox="0 0 63.962502 11.0625" width="63.962502"  xmlns="http://www.w3.org/2000/svg">
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				<tspan style="font-size: 12.50px; " x="22.930502" y="0">.</tspan>
				<tspan style="font-size: 12.50px; " x="26.056252" y="0">0</tspan>
				<tspan style="font-size: 12.50px; " x="32.307751" y="0">0</tspan>
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</svg>) as well as the proportion of total plaques that were atheromatous. Plaques in younger patients have fewer lipid-rich cores. Most thrombi show areas of organization, with layering frequent in erosions, suggesting a possible method of plaque enlargement in the absence of necrotic core formation. 

Fabio Tavora

Ling Li

Mary Ripple

David Fowler

Allen Burke

Morphologic Characteristic of Coronary Artery Disease, with Emphasis on Thromboses, in Patients Younger Than 40 Years of Age

Undifferentiated endometrial sarcomas (UESs) of the ovary are very rare tumors. This paper presents a case of a 56-year-old patient with a history of hysterectomy and bilateral salpingectomy seven years ago for uterine leiomyomata. Intraoperatively, a tumor originating from the left ovary, adherent to the sigmoid colon, with infiltration of the small intestine and the vaginal apex was found. Histologically, the tumor was composed of pleomorphic round and oval to spindled cells with polymorphous vesicular nuclei with coarse chromatin and large nucleoli. Mitotic activity was brisk. There were large necrotic areas. Adjacent to the tumor tissue endometrium-like glands surrounded by fibrous stroma with macrophages corresponding to ovarian endometriosis were noted. Tumor cells showed diffuse strong immunoreactivity for vimentin and patchy strong staining for CD10; no reactivities were found for AE1/AE3, desmin, S-100, LCA, CD20, c-kit, and CD31. The patient died of her neoplastic disease four months postoperatively. CD10 is frequently expressed in different gynecopathological as well as other lesions, and, thus, nonspecific without relevance to the classification of this case. Morphological features, extensive sampling, and appropriate immunohistochemistry including markers for cytokeratins and myogenic differentiation are mandatory to arrive at the correct diagnosis.

Hermann Brustmann

Ingrid M. Geiss

Susanne Hinterholzer

Undifferentiated Endometrial Sarcoma of the Ovary: A Case Report with Review of Recent Literature and Discussion of Lacking Specificity of CD10 Immunoreactivity

The provision of dynamic splicing events constitutes the reflected nature of neoplasia that locally infiltrates and systemically spreads in terms of evolutionary attributes of the primary and various secondary pathways in malignant transformation. The significant diversity in molecular characterization of the given tumor lesion would adaptively conform to dynamics of splicing as enhanced or silenced exons of the premessenger RNA molecule. The proteins synthesized are in turn potential modifiers in further gene expression within such contexts as RNA:protein and RNA:DNA binding events. The recognition of pathways of incremental scope would underline the development of lesions, such as tumors, as multiple alternative splicing phenomena primarily affecting molecular physicochemical identity. It is within contexts of operative intervention and modification that the real identity of the malignant neoplastic process arises, within terms of reference of contextual splicing events. Disrupted gene expression is thus a referential pathway in the modification of splicing that may prove constitutive or alternative, in first instance, but also aberrant as the lesion progresses locally and systemically.

Lawrence M. Agius

Given Dimensions of Neoplastic Events as Aberrantly Operative Alternative Splicing

Primary or idiopathic hypertrophy of the pyloric muscle (IHPM) is a rare entity with uncertain pathogenesis which both clinically and pathologically mimics gastric cancer. We present a rare late-occurring case of IHPM in a 71-year-old Caucasian man with no apparent predisposing factor. Imaging studies demonstrated gastric distension with air fluid levels and no evidence of extrinsic compression. At upper endoscopy, massive gastric distension and no evidence of any ulcer or other mucosal defects were observed. Microscopically, marked hypertrophy of muscularis mucosa with smooth muscle cells arranged in whorls and fascicles was present which gradually transitioned to normal areas. The muscle fibers stained with smooth muscle actin and trichrome stain highlighted fibrosis between the muscle fibers. Although uncommon, IHPM can clinically and histologically mimic other proliferations in the gastric wall, such as  gastrointestinal stromal tumor or a spindle cell neoplasm. The recent advances in understanding the pathogenesis of IHPM are discussed.

Alireza Zarineh

Marino E. Leon

Reda S. Saad

Jan F. Silverman

Idiopathic Hypertrophic Pyloric Stenosis in an Adult, a Potential Mimic of Gastric Carcinoma

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