bluuebunny/crossref_metadata_2025 huggingface dataset:
Thymic Tissue in the Skin: A Clue to the Diagnosis of the Branchio-oculo-Facial Syndrome
Authors: Drut Ricardo , Galliani Carlos
Published: June 2007

The branchio-oculo-facial (BOF) syndrome is a rare autosomal dominant disorder with a peculiar phenotype related to a combination of craniofacial abnormalities. Rare examples presenting with dermal thymic tissue have been described. We are reporting 2 children, 15 and 4 months old, respectively, with BOF syndrome in whom surgical repair/excision of skin cervical lesions showed thymic tissue. In the first patient the thymic tissue was exposed to the surface, without epithelial coverage and contained areas suggestive of all stages of thymus formation. A dermohypodermal nodule of fully developed thymic tissue superficially covered by nonkeratinizing squamous epithelium was found in the second patient. Since pharyngeal pouches are not exposed to the surface during normal embryogenesis and the thymus originates from the third pharyngeal pouch, both examples seem to indicate that in BOF syndrome there is a more complex malformation than simply ectopic thymus tissue. Thymic tissue at the skin appears to be unique for BOF syndrome and in a given case may provide the clue for the accurate diagnosis of the malformation complex.
Intraosseous Rhabdomyosarcoma of the Mandible: A Case Report
Authors: Loducca Silvia Vanessa L. , Mantesso Andrea , De Oliveira Eder Magno Ferreira , De Araújo Vera Cavalcanti
Published: June 2007

Rhabdomyosarcoma is the most common soft-tissue sarcoma of the head and neck region in children and adolescents. Oral cavity involvement is relatively uncommon, with tongue, soft palate, hard palate, and buccal mucosa being the sites of predilection. This report presents a rare case of intraosseous oral rhabdomyosarcoma arising in the mandibular bone of a 6-year-old child. Clinical, radiologic, and histopathologic features and possible pathogenesis are discussed.
Leiomyoma of the Urethra: Report of 3 Cases of a Rare Entity
Authors: Saad A. G. , Kaouk J. H. , Kaspar H. G. , Khauli R. B.
Published: June 2007

Leiomyomas are benign tumors of smooth muscle origin that can occur at any location in the urinary tract. Although rare, they are the most common mesenchymal neoplasms of the urethra. They are more frequent in females than in males. Only 3 cases of leiomyomas of the male urethra and multiple cases in the female have been reported in the English-language literature. We report 2 additional cases of leiomyoma of the male urethra and 1 more case of the female urethra and describe their differential diagnoses and management.
Tumor to Tumor Metastasis
Authors: Petraki Constantina , Vaslamatzis Michael , Argyrakos Theodoros , Petraki Kalliopi , Strataki Maria , Alexopoulos Constantinos , Sotsiou Flora
Published: June 2007

Tumor-to-tumor metastases are uncommon. The most frequent donor tumor is lung cancer, while renal cell carcinoma (RCC) is by far the most common recipient. In this report, a carcinoma of the uterine cervix metastasizing to an RCC and a urothelial carcinoma of the urinary bladder metastasizing to a solitary fibrous tumor of the pleura are described. No similar cases have been found in the accessible literature. These cases are discussed and the findings are correlated with the data of the literature.
Transitional Cell Carcinoma Arising in the Gastric Remnant FollowingGastrocystoplasty
Authors: Qiu Honghui , Kordunskaya Svetlana , Yantiss Rhonda K.
Published: June 2007

Urinary bladder augmentation with segments of the stomach (gastrocystoplasty), small bowel, or large intestine (enterocystoplasty) improves capacity and compliance in patients with bladder dysfunction. Although malignant complications of enterocystoplasty have been reported, the risk of malignancy in the setting of gastrocystoplasty is not knovvn. We describe the case of a 73-year-old woman who developed a transitional cell carcinoma associated with transitional cell metaplasia and dysplasia of the gastric epithelium 14 years following gastrocystoplasty. To our knowledge, this is the first reported case of a malignant complication of this surgical procedure. We conclude that patients who have undergone gastrocystoplasty are at an increased risk for the development of malignancy in the neobladder and require close long-term follow-up, similar to patients who have undergone enterocystoplasty.
Reactive Neuroepithelial Aggregates of the Skin
Authors: Chen Karl T. K.
Published: June 2007

A peculiar, apparently reactive, microscopic structure of the skin was observed in 5 patients. It is characterized histologically by a bundle of nerve fibers partially or completely ensheathed by bland nonkeratinizing squamous epithelium. In 2 patients, the specimens were reexcision specimens, in which this structure was found amid active postbiopsy repair. In the remaining 3 patients, there was no history of previous excision or trauma at the lesional sites, and the background changes were consistent with either folliculitis or prurigo nodularis. This structure, when viewed in isolation, resembles carcinomatous perineural invasion. This structure and comparable structures observed in extracutaneous sites have often been referred to as “perineural invasion” in the literature, but the author proposes the term reactive neuroepithelial aggregate (RNEA) to denote its reactive nature
Ductal Adenocarcinoma of the Pancreas with Huge Cystic Degeneration: A Lesion to Be Distinguished from Pseudocyst and Mucinous Cystadenocarcinoma
Authors: Lee Li-yu , Hsu Hui-Ling , Chen Han-Ming , Hsueh Chuen
Published: June 2007

Cystic neoplasms of the pancreas are rare and often mistaken for pseudocyst by imaging studies and macroscopic examination. We describe an unusual tumor of the pancreas composed of a mural nodule of anaplastic carcinoma arising from a huge ductal adenocarcinoma undergoing cystic degeneration. The cyst measured 27 x 13 x 4 cm. Light microscopy showed that the cyst was partly lined by a single layer of cuboidal to columnar tumor cells with focal mucin production and was surrounded by hyalinized connective tissue. Most lining epithelial cells were absent owing to extensive degenerative process. Immunohistochemical studies showed positive staining of cytokeratin and vimentin for pleomorphic giant tumor cells, which were negative for leukocyte common antigen (CD45), KP-1 (CD68), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The ductal adenocarcinoma stained strongly positive for cytokeratin and EMA, and negative for vimentin, CD45, CD68, and CEA. The clinical course of the current case was extremely poor and the prognosis resembled that of an anaplastic carcinoma. Therefore, we like to emphasize the importance of complete excision and extensive sampling of any cystic neoplasms in the pancreas including those with large cystic component to avoid missing the malignant elements
Nodular Pulmonary Amyloidosis Following Liver Transplantation
Authors: Goldar-Najafi Atoussa , Dugan John M. , Khettry Urmila
Published: June 2007

Nodular pulmonary lesions seen in liver transplant recipients have a broad differential diagnosis including both infectious and noninfectious etiologies. Here, we report the first case of nodular pulmonary amyloidosis, an uncommon and benign localized form of amyloidosis occurring after orthotopic liver transplantation for end-stage primary biliary cirrhosis
Testicular Ischemia Due to Intravascular Large B-Cell Lymphoma: A Novel Presentation in an Immunosuppressed Individual
Authors: Tranchida Paul , Bayerl Michael , Voelpel Mary Jo , Palutke Margarita
Published: June 2007

A 56-year-old man presented with fever, disorientation, and testicular pain. He was receiving azathioprine immunosuppression for autoimmune hepatitis. Orchiectomy identified occlusion of spermatic cord vessels by intravascular large B-cell lymphoma (IVLBL) and ischemic changes in the testis. Tumor cells were positive for CD 10, CD 20, CD 30, and Epstein-Barr virus (EBV) latent membrane protein 1 (LMP-1) and early region RNA (EBER). He was treated with the cessation of azathioprine, chemotherapy, anti-CD 20 immunotherapy, and radiotherapy. Twenty months after diagnosis, he is alive with no evidence of lymphoma or hepatitis. This is the first report of IVLBL presenting with testicular ischemia. It highlights the importance of prompt diagnosis and intervention to achieve durable response. That this lymphoma arose in the setting of immunosuppressive therapy introduces additional complexity relating to pathogenesis, clinical behavior, and treatment.
Unusual Morphologic Features of Uterine Leiomyomas Treated with Gonadotropin Releasing Hormone Agonists: Massive Lymphoid Infiltration and Vasculitis
Authors: McClean G. , McCluggage W. G.
Published: June 2007

This report describes 2 unusual morphologic features of leiomyomas in patients who had been treated preoperatively with gonadotropinreleasing hormone (GnRH) agonists. In 1 case there was extensive and widespread infiltration of the leiomyoma by numerous small mature lymphocytes, in keeping with a leiomyoma with massive lymphoid infiltration. In the other leiomyoma there were fibrin and foamy histiocytes within the walls of many arterioles, in keeping with a vasculitis. These 2 features, massive lymphoid infiltration and vasculitis, have rarely been described in association with GnRH agonists. Since GnRH agonists are increasingly being used in the management of uterine leiomyomas, pathologists should be aware of these unusual morphologic features in order to avoid diagnostic confusion.
Desmoplastic Melanoma of the Head and Neck: Histopathologic and Immunohistochemical Study of 28 Cases
Authors: Kay Paul A. , Pinheiro A. Daniel , Lohse Christine M. , Pankratz V. Shane , Olsen Kerry D. , Lewis Jean E. , Nascimento Antonio G.
Published: June 2007

Studies of the immunohistochemical profiles and clinical course of desmoplastic melanoma have produced conflicting results. We identified 28 cases of desmoplastic melanoma after a search of our files for spindle cell neoplasms of the head and neck from 1960 through 1995. The 17 male (61%) and 11 female (39%) patients averaged 65 years of age. The cheek was the most common location (12 cases, 43%). The average length of follow-up was 5 years. Overall 5-year survival rate was 46%. Melan A and tyrosinase positivity (P = 0.0195), smooth muscle actin positivity (P = 0.0328), tumor size (P = 0.0297), and tumor thickness (P = 0.0419) were significantly associated with local progression-free survival. No histologic or immunohistochemical marker was associated with overall or metastasis-free survival.
T-Cell-Rich B-Cell Lymphoma Presenting in the Spleen: A Clinicopathologic Analysis of 3 Cases
Authors: Li Shiyong , Mann Karen P. , Holden Jeannine T.
Published: June 2007

We review the clinical, pathologic, and molecular genetic features of 3 splenic T-cellrich B-cell lymphomas and discuss their differential diagnosis. All patients presented with symptomatic splenomegaly and underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen in all cases showed a micronodular proliferation of lymphoid cells. A proportion of the nodules demonstrated central hyalinization or sclerosis. There was also an exuberant extramedullary hematopoiesis. On immunohistochemical stain, the nodules consisted predominantly of small T cells with scattered large atypical B cells. The clonal nature of the atypical B cells was confirmed by polymerase chain reaction assays for immunoglobulin heavy-chain gene rearrangement. In the H&E sections, the differential diagnoses included Hodgkin's lymphoma, follicular lymphoma, peripheral T-cell lymphoma, and nonneoplastic granulomatous process. The presence of exuberant extramedullary hematopoiesis also raised the possibility of a chronic myeloproliferative disorder. The combined morphologic, immunohistochemical, and molecular genetic data are essential for a correct diagnosis of splenic T-cell-rich B-cell lymphoma.
Adenocarcinoma and Dysplasia in an Ileal Neobladder After Ileocystoplasty for Interstitial Cystitis
Authors: Robles M. W. , Rutgers J. K. L. , Shanberg A. M.
Published: June 2007

We report a 54-year-old woman with an adenocarcinoma of an ileal neobladder arising upon a background of ileal mucosal dysplasia. We believe that no case study or report has previously documented neobladder ileal mucosal dysplasia adjacent to an ileal neobladder adenocarcinoma. This observation supports the current hypothesis that ileal neobladders are dynamic environments for potential malignancy, and moreover, suggests a sequence of morphologic and molecular derangements similar to that seen in colorectal carcinoma. Those patients status post ileal neobladder are at risk for glandular dysplasia and malignancy and should be followed closely.
Splenic Hamartoma: Immunohistochemical and Ultrastructural Profile of Two Cases
Authors: Ali Tehmina Z. , Beyer Ginine , Taylor Michelle , Volpe Carmine , Papadimitriou John C.
Published: June 2007

Splenic hamartoma (SH) is a rare, benign lesion. We present 2 cases, both in females (2 and 30 years, respectively) with multiple urinary tract infections, and left upper quadrant abdominal pain. Immunohistochemical staining with factor VIII displayed intense diffuse staining in the SH with corresponding weak staining in the adjacent spleen. CD31 showed a reverse pattern from that of factor VIII. CD34 staining pattern was identical in both the spleen and the SH. Ultrastructurally, the SH showed endothelial cells with relatively empty cytoplasm, scattered Weibel Palade bodies, and lining by basement membrane surrounded by fibrous long-spacing collagen. Our study highlights the unique immunohistochemical profile of SH. The ultrastructural features are interesting, although their diagnostic significance remains to be confirmed in future studies.
Genetics, Pathology, and Clinics of Familial Gastric Cancer
Authors: Oliveira Carla , Seruca Raquel , Carneiro Faitima
Published: June 2007

Gastric cancer is relatively common worldwide, mainly in its sporadic form, but familial aggregation of the disease may be seen in approximately 10% of the cases. This suggests a genetic cause for the cancer in those families that has not been identified in most cases. Despite all efforts to determine its genetic basis, a single syndrome has been characterized-the hereditary diffuse gastric cancer (HDGC)-which is specifically associated with CDH1 (E-cadherin) germline mutations in one third of the families. The other two thirds and all the gastric cancer families not fulfilling the HDGC criteria remain without molecular diagnosis. In this article we review the state of the art of familial gastric cancer regarding the molecular aspects, the clinical criteria, the pathology features, and the management recommendations described so far to be associated with this cancer disease.
Expression of Serine Protease Matriptase in Renal Cell Carcinoma: Correlation of Tissue Microarray Immunohistochemical Expression Analysis Results with Clinicopathological Parameters
Authors: Jin Jong-Shiaw , Chen Ann , Hsieh Dar-Shih , Yao Chen-Wen , Cheng Ming-Fang , Lin Yeh-Feng
Published: June 2007

Serine protease matriptase (matriptase) cleaves and activates proteins implicated in the progression of cancer and represents a potential therapeutic target. Immunohistochemical analysis of matriptase was performed in tissue microarrays of 168 renal cell carcinomas (RCCs). All subtypes of RCC showed significant immunohistochemical expression of matriptase. In contrast, no expression occurred in areas of RCC with sarcomatous differentiation (SRCC) and in normal collecting tubules. The matriptase scores were significantly higher in papillary RCC (341) and clear cell RCC with granular cell differentiation (GRCC; 324) than in other histologic subtypes of RCC. In GRCC, matriptase scores were correlated with TNM staging and nuclear grading. Matriptase was overexpressed in all subtypes of RCC, and matriptase scores could distinguish between conventional clear cell RCC, GRCC, and SRCC.
Enterocolic Lymphocytic Phlebitis: Statistical Analysis of Histology Features in Viable and Ischemic Bowel
Authors: Medlicott Shaun A. C. , Guggisberg Kelly A. , DesCôteaux Jean-Gaston , Beck Paul
Published: June 2007

Enterocolic lymphocytic phlebitis is a rare cause of segmental ischemic enterocolitis. This artery-sparing transmural vasculitis is classically a circumferential phlebitis with perivenular lymphocyte cuffing and thrombi in the absence of systemic manifestations. Myointimal hyperplasia may represent a chronic phase of enterocolic lymphocytic phlebitis. Subclinical or early stage enterocolic lymphocytic phlebitis is not well delineated. We analyzed 600 submucosal and subserosal veins from both ischemic and intact bowel segments to discern if vascular morphology varied between sites. Crescentic and circumferential lymphocytic phlebitis is more common in viable bowel than in the ischemic segment. A nonsignificant trend was found for increased crescentic morphology between intact bowel remote from the ischemic focus compared with that adjacent to the ischemic focus. Hallmarks of ischemic bowel are necrotizing phlebitis and thrombi formation. Thrombophlebitis morphology is distinctly different in viable and ischemic bowel, changing from the classic lymphocytic to necrotizing lesions respectively.
Rhabdomyoblastic Differentiation in Malignant Melanoma in Adults: Report of 2 Cases
Authors: Gharpuray-Pandit Deepa , Coyne John , Eyden Brian , Banerjee S. Sankar
Published: June 2007

Two cases of malignant melanoma are reported in adults exhibiting rhabdomyoblastic differentiation to alert pathologists to this rare variant of malignant melanoma. One of the cases presented as a metastasis in a submandibular lymph node, and the other was a primary skin melanoma. There are only a few published reports on melanocytic tumors with rhabdomyoblastic differentiation, mainly occurring in giant congenital nevi. Both cases reported here were confirmed by immunohistochemistry. Both cases were also studied by electron microscopy, and one showed distinctive ultrastructural features of striated muscle.
High-Grade Neuroendocrine Carcinoma Arising in a Gastric Duplication Cyst
Authors: Horne Garnet , Ming-Lum Corey , Kirkpatrick Andrew W. , Parker Robin L.
Published: June 2007

Gastric duplication cysts are rare congenital anomalies that are mostly discovered in children but can remain asymptomatic and undetected into adulthood. Malignant transformation in gastric duplications is extremely rare, with most reported cases being adenocarcinomas. Herein, the authors report the first case of a high-grade neuroendocrine carcinoma arising in a gastric duplication cyst in a 40-year-old Asian male. This case highlights that duplication cysts should be included in the differential diagnosis of cystic masses of the gastrointestinal tract in adult, as well as pediatric, patients and emphasizes the importance of complete surgical resection and meticulous pathologic examination of these congenital anomalies.
Oncocytic Meningioma: Report of a Case With Progression After Radiosurgery
Authors: Marucci Gianluca , Betts Christine M. , Frank Giorgio , Foschini Maria P.
Published: June 2007

Oncocytic meningioma is an uncommon variant of meningioma, characterized histologically by cells rich in mitochondria. This subtype of meningioma needs to be distinguished from other types of meningioma because of its more aggressive behavior. A case of oncocytic meningioma showing rapid clinical progression with diffuse intracranial dissemination after radiosurgery is described. Resistance to radiotherapy has been previously reported in oncocytic tumors of various body sites. This new case is a further example of the greater aggressiveness of oncocytic meningioma, suggesting that radiation therapy might worsen the course of disease.
Intramucosal Ganglion Cells in Normal Adult Colorectal Mucosa
Authors: Tunru-Dinh Vonny , Wu Mark Li-cheng
Published: June 2007

Intramucosal ganglion cells are incompletely understood. Reviewed were 100 normal specimens from colorectal biopsies of nonconstipated adults to firmly establish the existence of intramucosal ganglion cells in normal adult colorectal mucosa, determine whether intramucosal giant ganglia exist, and compare the prevalence of colorectal intramucosal ganglion cells between men and women. Fifty specimens from each gender were examined. Twenty specimens contained intramucosal ganglion cells, including 15 from women (75%) and only 5 from men (25%). Intramucosal ganglion cells occurred singly or in clusters. One woman had large disciform and globular clusters with 7 ganglion cells, resembling giant ganglia. Awareness of ganglion cells and large ganglia in normal mucosa is necessary to avoid potential confusion with ectopic ganglion cells and submucosal giant ganglia seen in adult intestinal neuronal dysplasia. Intramucosal ganglion cells in normal colorectal mucosa are particularly common in women. This disparity might contribute to colorectal neurophysiologic differences between genders.
Comparison Between Echo-Color Doppler Sonography Features and Angioarchitecture of Thyroid Nodules
Authors: Foschini Maria P. , Ragazzi Moira , Parmeggiani Alfredo L. , Righi Alberto , Flamminio Federica , Meringolo Domenico , Castaldini Luciano
Published: June 2007

This study compared echo-color Doppler sonography features of thyroid nodules with the 3-dimensional reconstruction to find parameters useful for the preoperative diagnosis. Forty nodules relative to 29 patients were examined with echo-color Doppler before surgery. After histologic diagnosis, blocks were deparaffinized and prepared for 3-dimensional examination using a stereomicroscope. Echo-color Doppler type I nodules (8 nodules) always corresponded to colloid goiter. Type II and III nodules corresponded to colloid goiter with intralesional hemorrhage or were associated with hyperplastic nodules, follicular adenoma, follicular carcinoma minimally invasive, papillary carcinoma, and medullary carcinoma. Of interest was that 9 of 11 follicular lesions were characterized by a large central vessel, which was also evident in echo-color Doppler images. This architectural pattern is not seen in benign nodules or in papillary carcinomas. Comparison with histology suggests that echo-color Doppler images can visualize vessels showing a muscular wall.
Sinonasal Rhabdomyosarcoma in Children and Young Adults
Authors: Atif Ali Ahmed  , Tsokos Maria
Published: June 2007

Rhabdomyosarcoma is an aggressive malignant tumor often developing in the head and neck in children. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of the difficulty of surgical resection and its generally poor prognosis. We reviewed the archival pathology materials of 39 cases of rhabdomyosarcoma of the head and neck in children and young adults. The diagnosis was made through light microscopy, immunohistochemistry, electron microscopy, and/or reverse-transcriptase polymerase chain reaction (RT-PCR) molecular testing. We identified 14 tumors in the nose and paranasal sinuses. Patients' ages ranged from 9 to 40 years. Thirteen of the tumors were of the alveolar subtype. In 11 cases, the tumor cells were poorly differentiated, forming a solid alveolar pattern. In 2 cases, there was evidence of rhabdomyoblastic differentiation. Only one case was classified as embryonal rhabdomyosarcoma. A significant number of tumor cells in these cases had clear or vacuolated cytoplasm. Four alveolar rhabdomyosarcoma tumors were tested by RT-PCR; all showed PAX3/FKHR chromosomal translocation. We conclude that sinonasal rhabdomyosarcoma is predominantly of the alveolar subtype and frequently shows clear cells. A review of the literature shows that these tumors carry a poor prognosis, partly because of their parameningeal location and partly because of their “undifferentiated” alveolar histology.
Intraabdominal Calcifying Fibrous Pseudotumor
Authors: Chen Karl T. K.
Published: June 2007

Calcifying fibrous pseudotumor was recently described as a rare, distinctive, benign, fibrous lesion characterized by the presence of abundant hyalinized collagen with psammomatous or dystrophic calcification and a lymphoplasmacytic infiltrate. The lesions reported to date in the literature involved subcutaneous and deep soft tissues of the extremities, trunk, scrotum, groin, neck, and axilla of children and young adults. Four cases in which the lesion arose intraabdominally in elderly patients are reported. This report expands the age spectrum and anatomic topography of this lesion.
Gastric Epithelioid Stromal Tumor (Leiomyoma) With Granular Changes
Authors: Yao Takashi , Aoyagi Kunihiko , Hizawa Kazuoki , Kimura Yutaka , Tsuneyoshi Masazumi
Published: June 2007

A unique case of gastric epithelioid stromal tumor (leiomyoma) demonstrating granular changes is reported. Microscopically, the tumor was composed of polygonal or fusiform cells with a coarse, granular eosinophilic cytoplasm, focally admixed with fascicles of spindle-shaped cells. A marked lymphoid infiltrate forming follicles was also a characteristic finding. Immunohistochemically, these cells were positive for alpha-smooth-muscle actin, HHF-35 (muscle-specific actin), and NKI/C3 but negative for desmin, S100 protein, vimentin, lysozyme, and Mac-387. This case demonstrates that granular cell changes and prominent lymphoid aggregates are not limited to gastric stromal tumors with neural differentiation.
Periosteal Osteosarcoma of the Digits
Authors: Dominguez-Malagon Hugo , Ro Jae Y. , Ayala Alberto G.
Published: June 2007

A periosteal osteosarcoma arose in the first phalanx of the fourth finger of a 32-year-old woman's right hand. She had no predisposing factors for this cancer. The tumor was initially locally resected, but as recurrences developed, the finger and later the hand were amputated. Despite therapy with doxorubicin and cisplatin, distant metastases occurred and ultimately caused the patient's death. Primary conventional osteosarcomas of the short tubular bones of the hands and feet are rare, but surface lesions are rarer. This case constitutes the first reported periosteal osteosarcoma to arise in a phalanx.
Observations on Tumor and Metastatic Suppressor Gene Status in Human Fibrous Neoplasms with Particular Emphasis on p53 and nm23
Authors: Jialiang Li , Guanghua Yang , Gandi Li
Published: June 2007

Eighty-two soft-tissue sarcomas, including 50 patients with dermatofibrosarcoma protuberans (DFSP), 16 patients with fibrosarcomas (FS), and 16 patients with malignant fibrous histiocytoma (MFH), were investigated for the presence of p53 mutations and for p53, nm23 immunoreactivity with silver-staining polymerase chain reaction-single-strand conformation polymorphism and immunohistochemistry methods. The total mutation rate of p53 gene was 23% (3 of 50 DFSP, 7 of 16 FS and 9 of 16 MFH). Of 82 tumor samples, 22% (2 of 50 DFSP, 6 of 16 FS, and 10 of 16 MFH) were positive for p53 immunostaining. p53 gene mutation and expression were low in DFSP (6.0%; 4.0%) but high in FS (43.8%; 37.5%) and in MFH (56.3%; 62.5%). NDPK/nm23 expression was high in DFSP (84.0%) but was low in FS (31.3%) and in MFH (18.8%). High levels of p53 gene mutation and expression correlated with metastasis and degree of malignancy. On the contrary, low levels of NDPK/nm23 expression correlated with metastasis and degree of malignancy of human fibrous neoplasms. p53 mutations in soft-tissue sarcomas have a similar spectrum to those in carcinomas. Determination of p53 gene mutation and expression in combination with low nm23/NDPK expression may help predict metastasis and degree of malignancy of fibrous neoplasms. The precise association between the p53 gene and nm23 gene remains to be established.
Incidental Extrauterine Stromal Sarcoma in a Patient With Ovarian Carcinoma
Authors: Schnell Brian R. , Winston Janet S. , Brooks John S. J.
Published: June 2007

Most low-grade endometrial stromal sarcomas are intrauterine in origin, and a primary extrauterine location in the mesentery or omentum is extraordinarily rare. Hence, we report a well-documented, low-grade endometrial stromal sarcoma of extrauterine origin as an incidental finding in a patient who had extensive ovarian intra-abdominal carcinomatosis. This case adds to the literature experience in this entity and illustrates that such lesions may occur as incidental and unexpected findings in patients with other gynecologic neoplasms.
Stroma-Rich Castleman's Disease with Superimposed Kikuchi's Lymphadenitis-Like Changes
Authors: Chan John K. C. , Luk S. C. , Ho P. L.
Published: June 2007

The authors report a retroperitoneal lesion showing the hitherto undescribed coexistence of hyaline-vascular Castleman's disease and Kikuchi's lymphadenitis-like changes, the only feature in common for these two disease processes being the consistent presence of plasmacytoid monocytes (plasmacytoid T-cells). The Castleman's disease was classifiable as the stroma-rich variant, with broad interfollicular zones occupying 90% of the cross-sectional areas. The interfollicular regions were rich in atypical plump spindly cells with indistinct cell borders, oval nuclei, vesicular chromatin, and distinct nucleoli; they were disposed in fascicles and whorls around and between the hyaline-vascular follicles. The exact nature of these atypical stromal cells could not be ascertained because they were immunohistochemically inert except for vimentin expression. Scattered throughout the interfollicular areas were multiple discrete foci comprising plasmacytoid monocytes, karyorrhectic debris, activated lymphoid cells, histiocytes with twisted nuclei and crescentic histiocytes, as characteristic of Kikuchi's lymphadenitis. Although the coexistence of Castleman's disease and Kikuchi's lymphadenitis-like changes can be coincidental, it is also possible that the latter might represent an unusual aberrant local immune reaction to the stromal overgrowth in the Castleman's lesion.
Resident Neuroendocrine Cells in the Normal Ectoendocervical Epithelium
Authors: Remadi Sami , MacGee William , Megevand Eric , Chappuis Pierre , Redard Mireille , Seemayer Thomas A.
Published: June 2007

Among the major difficulties in determining the histogenesis of small-cell neuroendocrine carcinomas of the cervix is the issue of identifying the cell from which the neoplasm arises. In this study we performed an immunohistochemical analysis of the neuroendocrine cells in the normal ectoendocervical epithelium by using a microwave heating technique. In ectocervical epithelium, positivity for chromogranin and/or neuron-specific enolase was detected in 18% of the cases, whereas 46% of the endocervical mucosae demonstrated immunoreactive cells for these antibodies. Isolated endocervical neuroendocrine cells showed immunoreactivity for antigastrin (1 case), anti-VIP (3 cases), and antisynaptophysin (14 cases) antibodies. A comparative analysis of 4 diagnosed cases of small-cell carcinoma of the cervix demonstrated immunoreactivity for neuron-specific enolase and chromogranin, respectively, in 3 cases and for synaptophysin in 1 case. Thus, small-cell carcinoma of the cervix likely originates from cells with neuroendocrine differentiation localized mainly in the endocervical glands. These findings offer an explanation for the paraneoplastic syndromes described in some cases of small-cell carcinoma of the cervix.
Recurrent Hepatitis C in Liver Allografts: Early Histologic Indicators
Authors: Khettry Urmila , Robiou Cristian , Jenkins Roger L. , Loda Massimo , Lewis W. David
Published: June 2007

Our purpose was to establish the earliest histologic parameters of acute hepatitis that correlated with recurrent hepatitis C virus (HCV) infection in liver allografts. Histologic reviews of posttransplant liver biopsies from eight patients with pretransplant chronic HCV (group I) and eight with pretransplant non-HCV liver disease (group II) were performed and correlated with concurrent or subsequent presence or absence of HCV-RNA by polymerase chain reaction (PCR). Six of eight group I patients developed HCV-RNA positivity in liver and serum. Of the histologic parameters studied, the presence of Kupffer cell hyperplasia, spotty hepatocellular necrosis (acidophil bodies), or both, correlated the most with the earliest diagnosis of acute recurrent hepatitis. The postoperative time period for the earliest diagnosis of recurrent HCV was variable (34 to 123 days). These early indicators and the time of their appearance were not predictive of the course of the disease process or the final outcome of the patient. The recurrence rate of HCV infection in liver allografts is high. The presence of Kupffer cell hyperplasia and diffusely scattered acidophil bodies are reliable, early histologic indicators of an acute hepatitic process.
Primary Liposarcoma of Bone in an Adolescent
Authors: Rabah Raja , Lucas David R. , Farmer Diana L. , Ryan James R. , Ravindranath Yaddanapudi
Published: June 2007

We report the clinicopathologic and cytogenetic findings in an unusual case of primary liposarcoma of bone. A 16-year-old girl presented with a destructive, osteolytic tumor of the proximal humerus. Upon biopsy, the tumor was highly anaplastic and necrotizing and was initially considered to be consistent with osteosarcoma. Following chemotherapy, there was 100% tumor necrosis in the resection specimen. Twentyeight months later, solitary supraclavicular and pulmonary metastases appeared with the morphologic features of pleomorphic liposarcoma. Retrospective review of the bone biopsy showed scattered lipoblasts and no unequivocal malignant osteoid. Ghost-like remnants of necrotic liposarcoma were seen in the resection specimen. Cytogenetic analysis of a metastatic lesion showed complex karyotypic abnormalities. Awareness of the existence of primary bone sarcomas with lipogenic differentiation expands the differential diagnosis of malignant bone tumors including cases in the pediatric population. This is the first report of cytogenetic findings in this tumor.
Ewing's Sarcoma and Peripheral Primitive Neuroectodermal Tumor of Bone and Soft Tissue
Authors: Llombart-Bosch Antonio
Published: June 2007

The histological diagnosis of Ewing's sarcoma (Es) continues to be a difficult task for pathologists. A number of new Es varieties has been described, leading to further complexity. Conventional Es, atypical Es, and peripheral neuroectodermal tumor (pPNET), including peripheral neuroepithelioma, belong genetically to the same family of neoplasms, displaying common chromosomal rearrangements and analogous gene reorganizations. The main translocations are t(11;22) and t(21;22), with genes EWS, FLI-1 and ERG being involved, as well as other members of the ETS family of transcription factors. The prevalence of morphology should be maintained with the use of conventional histological techniques and supported with the analysis of the expression of CD99 Mic2 (HBA 71, 0.13) antigen, which is present in over 90% of cases, as well as with FLI-1 rearranged gene derived protein. Moreover, FISH and RT-PCR in paraffin-embedded tumor tissue may be of use in extremely difficult cases for differential diagnosis. Nevertheless, a number of mixed tumor types, such as primitive sarcoma or biphenotypic sarcoma, may show structural and genetic diversity, thus rendering their diagnosis even more complex.
Infants Fed Docosahexaenoic Acid- and Arachidonic Acid-Supplemented Formula Have Decreased Incidence of Bronchiolitis/Bronchitis the First Year of Life
Authors: Pastor Nitida , Soler Begoña , Mitmesser Susan Hazels , Ferguson Paul , Lifschitz Carlos
Published: June 2007

To assess the effect of docosahexaenoic acid and arachidonic acid supplementation in infant formula on the incidence of respiratory illnesses, pediatricians assigned infants to receive docosahexaenoic acid/arachinonic acid-supplemented formula or control formula. Anthropometrics, medical history, and illnesses were reported. Among 1342 infants, there was a higher incidence of bronchiolitis in control versus docosahexaenoic acid/arachinonic acid-supplemented groups at 5, 7, and 9 months ( P < .01). Weight, length, and head circumference were similar for both groups. Infants fed formula supplemented with 0.32% docosahexaenoic acid and 0.64% arachidonic acid experienced a lower incidence of bronchiolitis compared with infants fed formula supplemented with no docosahexaenoic acid/arachidonic acid or lower levels of docosahexaenoic acid/arachidonic acid in the first year of life.
The Effect of Agmatine Administration on Ischemic-Reperfused Isolated Rat Heart
Authors: Greenberg Shai , George Jacob , Wollman Yoram , Shapira Itchak , Laniado Shlomo , Keren Gad
Published: June 2007

Background: The natural polyamine Agmatine (Ag) plays a significant role in protection of nerve cell ischemic injury. A previous report indicated that Ag given intraperitoneally to rats enhanced the recovery of the heart from ischemic injury. Based on this initial observation, a larger investigation was undertaken to explore a dose-response effect and possible mechanisms underlying the protective effects.

Methods: Using the modified Langendorff model, 36 isolated hearts were divided into five groups: group 1, hearts receiving 100 μM/L Ag pre-ischemia (n=7); group 2, hearts receiving 100 μM/L Ag preand post-ischemia, (n=7); group 3, hearts receiving 250 μM/L Ag preischemia (n=7); group 4, hearts receiving 250 μM/L Ag preand postischemia (n=7); and group 5, hearts receiving Krebs-Hensleit solution served as control (n=8). The study design included 20 minutes of perfusion, 30 minutes of global ischemia, and 30 minutes of reperfusion.

Results: After ischemia, group 2 developed higher left ventricular pressure P(max) (P<0.01), improved first-derivative of the rise (dP/dt max; P<0.02), and fall (dP/dt min; P<0.04) in left ventricular pressure, and the area calculated under the left-ventricle developed pressure curve (pressure-time integral; P<0.015), but coronary flow was not significantly increased (P=0.06) compared to the control group. Group 1 had improved diastolic recovery: dP/dt min (P<0.05) and coronary flow (P<0.03), compared with the control group. Group 3 had improved P(max) (P<0.01), dP/dt min (P<0.01), and coronary flow (P<0.02); group 4 had no improvement in all hemodynamic parameters.

Conclusion: Low doses of Ag given preand post-ischemia, and high doses given only preischemia have favorable, protective effects on the hemodynamic recovery of isolated rat heart undergoing global ischemia and reperfusion.
Elevated Baseline Triglyceride Levels Modulate Effects of HMGCoA Reductase Inhibitors on Plasma Lipoproteins
Authors: Drmanac S. , Heilbron D. C. , Pullinger C.R. , Jafari M. , Gietzen D. , Ukrainczyk T. , Cho M. H. , Frost P. H. , Siradze K. , Drmanac R. T. , Kane J. P. , Malloy M. J.
Published: June 2007

Background: The response in levels of very-low-density (VLDL) and low-density (LDL) lipoproteins varies substantially among hyperlipidemic patients during treatment with HMGCoA reductase inhibitors. Apolipoprotein E genotype and gender are known to contribute to the regulation of steady state levels of plasma lipoproteins. This study explores the effect of these and other potential determinants of the response of VLDL and LDL to treatment with reductase inhibitors.

Methods: Using mixed linear statistical models, the response of lipoprotein lipid values was studied in 142 hyperlipidemic individuals who were treated with reductase inhibitors. Patients received one or more of the following drugs individually for a total of 623 treatment observations: lovastatin, pravastatin, simvastatin, or atorvastatin. For evaluation of the effects of treatment in the aggregate, actual doses were expressed as equivalent doses of atorvastatin, using factors based on random assignment comparisons in 16 reported studies. The analysis factors considered were apolipoprotein E genotype, baseline average triglycerides >170 mg/dL (vs less), and gender.

Results: Presence of an apo e4 allele was associated with a trend toward greater reduction of triglyceride levels and a diminished ability of the reductase inhibitors to reduce LDL cholesterol levels. Gender had only minimal effect on the response of either LDL cholesterol or triglycerides. However, the effect of elevated baseline triglycerides on the response of both triglycerides and LDL cholesterol was striking and was exerted in opposite directions. The triglyceride-lowering effect of reductase inhibitors was greater in patients with initial triglyceride levels above 170 mg/dL (P=0.0001). The effect was even greater in patients with initial triglyceride levels over 250 mg/dL (P=0.015). Conversely, for LDL cholesterol levels, elevated baseline triglycerides were associated with a significantly decreased response to the drugs (P=0.0015).

Conclusions: These findings indicate that baseline triglyceride levels are an important predictor of response of plasma lipoproteins to HMGCoA reductase inhibitors, perhaps reflecting fundamental differences in mechanism underlying the hyperlipidemic phenotype.
Importance of QT Interval Determination and Renal Function Assessment During Antiarrhythmic Drug Therapy
Authors: Reiffel James A. , Appel Gerald
Published: June 2007

A major concern associated with the use of antiarrhythmic drugs (AAD) is the occurrence of ventricular proarrhythmia, especially torsade de pointes (TdP). The AADs associated with TdP most commonly include quinidine, procainamide, disopyramide, sotalol, and the newer class III agents, such as ibutilide and dofetilide. It is not simply the administration and nature of an AAD but also several additional factors such as heart rate, ventricular hypertrophy, congestive heart failure, gender, age, concomitant drugs, and impaired drug clearance that influence TdP development. Dosing of these agents should be adjusted to take such factors into account to minimize the incidence of proarrhythmia.
L-Arginine Improves Post-Ischemic Vasodilation in Coronary Heart Disease Patients Taking Vasodilating Drugs
Authors: lannuzzi Arcangelo , Jannuzzo Gabriella , Sapio Cecilia , Pauciullo Paolo , Jorio Domenico , Spampinato Nicola , Mancini Mario , Rubba Paolo
Published: June 2007

Background: The hypothesis that intravenous L-arginine infusion improves the vasodilatory response to ischemia in the resistance vessels of human lower limbs in relatively young coronary heart disease patients taking vasodilating drugs was tested.

Methods: Twenty patients with onset of symptoms of coronary artery disease before age 50, operated for aortocoronary bypass and taking vasodilating drugs, were compared with 20 control subjects of comparable age and gender; neither group included heavy smokers (>10 cigarettes/day). Blood flow in the lower limbs was measured noninvasively with strain-gauge plethysmography, both at rest and during a reactive hyperemia test. Intravenous infusion of L-arginine was performed in nine coronary heart disease patients and in nine control subjects.

Results: Resting blood flow to the lower limbs was 2.3 mI,/minIlO0 mL in control subjects vs 3.4 mL/minIlO0 mL in patients (difference not statistically significant). Peak blood flow measured after a 3-minute arterial occlusion was 24.0 mL/min/lO0 mL in control subjects vs 20.3 mL/min/lO0 mL in coronary heart disease patients (P<0.05). Peripheral minimal vascular resistances were 4.28 and 5.46 peripheral resistances units (p.r.u.) in control subjects and patients, respectively (P<0.05). Intravenous infusion of L-arginine was followed by increased resting blood flow in cases and controls (P=0.009), with a parallel reduction in peripheral resting vascular resistances (P=0.009). Coronary heart disease patients showed increased peak blood flow (P=0.04) and reduced minimal vascular resistances (P=0.02), whereas no statistically significant changes in these parameters were detectable in control subjects. Intravenous glucose infusion, leading to increased serum insulin concentration, did not modify any hemodynamic parameter.

Conclusions: Hemodynamic responses in the skeletal muscle are impaired during a reactive hyperemia test in relatively young coronary heart disease patients taking vasodilating drugs. Intravenous L-arginine infusion corrects the impaired vasodilatory response of the lower limbs to an acute increase in flow following a cuff thigh occlusion.
The Relationship Between Low-Density Lipoprotein Cholesterol Goal Attainment and Prevention of Coronary Heart Disease-Related Events
Authors: Ito Matthew K. , Delucca Gina M. , Aldridge Martha A.
Published: June 2007

Background: According to the National Cholesterol Education Program (NCEP) treatment guidelines, patients with preexisting coronary heart disease (CHD) or other atherosclerotic vascular disease should lower low-density lipoprotein (LDL) cholesterol to < 100 mg/dL. Recent statin trials document the benefit of cholesterol lowering on CHD events but do not address the optimal goal of LDL cholesterol.

Methods: The pravastatin to simvastatin conversion-lipid optimization program (PSCOP) at the VA San Diego Healthcare System (VASDHS) was a formulary-conversion program designed to increase the percentage of patients who meet their recommended NCEP LDL cholesterol goal. We compared the incidence of clinical outcome and mortality between CHD patients from the original PSCOP cohort with postconversion LDL cholesterol greater than and < 100 mg/dL. A total of 524 patients were stratified by postconversion LDL cholesterol levels (greater than [N=183]) or < 100 mg/dL [N=341]) and observed for a mean duration of 27.7 months. Patients' VASDHS records were reviewed for postconversion mortality from any cause and CHD-related events. Patients were mailed a questionnaire to capture similar events that may have occurred outside of VASDHS, which might not be present in the patient's VASDHS record.

Results: Lipid-lowering therapy ≤ 100 mg/dL was associated with a significantly lower percentage of total deaths and CHD-related events (40% vs 61%, P=0.008). In patients with LDL cholesterol >100 mg/dL, the relative risk of unstable angina (relative risk, 2.2; 95% confidence interval, 1.3 to 3.8; P=0.004) and stroke (relative risk, 3.0; 95% confidence interval, 1.04 to 8.6; P=0.04) were significantly greater compared to patients meeting their LDL cholesterol goal.

Conclusions: Our study results support reducing LDL cholesterol to at least 100 mg/dL in the patient with CHD.
Effect of Chronic AT1 Receptor Antagonism on Postischemic Functional Recovery and AT1/AT2 Receptor Proteins in Isolated Working Rat Hearts
Authors: Moudgil Rohit , Xu Yi , Menon Vijayan , Jugdutt Bodh I.
Published: June 2007

To determine whether chronic angiotensin II (Ang II) type I receptor (ATR) antagonism improves recovery of left ventricular (LV) function after ischemia-reperfusion (IR) and increases AT,R and Ang II type 2 receptor (AT2R) protein expression in isolated working rat hearts, rats were randomized to pretreatment with either losartan (30 mg/kg/day) or UP269-6 (3 mg/kg/day), or no drug (control), for 1 week or 3 weeks before IR (50 min perfusion, 25 min ischemia, 40 min reperfusion). In vitro LV work and power and ex vivo AT,R and AT2R proteins (immunoblots) were measured. Compared to baseline perfusion, LV work and power showed variable recovery in control, losartan, and UP269-6 groups. Compared to control, losartan preserved recovery of LV work and power while UP269-6 showed less recovery after IR at both 1 week and 3 weeks. Both antagonists increased AT2R but not AT,R protein. The duration of pretreatment did not affect the expression of ATR or AT2R proteins. The results indicate that chronic AT,R blockade over 1 or 3 weeks increases AT2R (not ATIR) protein expression and may preserve but not improve postischemic functional recovery compared to controls in isolated working rat hearts.