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Sixty-three serum specimens from American patients with nasopharyngeal carcinoma were examined for antibodies to antigens associated with Epstein-Barr virus (EBV) and compared with 98 specimens from patients with other head and neck cancers, 133 from patients with benign head and neck diseases, and 96 from healthy donors. The level of antibody titers to EBV-associated antigens was correlated with nasopharyngeal carcinoma. The anti-EBV profile of elevated antibody titers directed against viral capsid antigen and early antigen was seen in undifferentiated and nonkeratinizing tumors but usually not in squamous cell tumors. Titers tended to rise with large increases in total tumor burden caused by distant metastases, often before clinical evidence of metastases. At the time of diagnosis, antibody-dependent cellular cytotoxicity testing was performed on serum samples from 46 of the patients with nasopharyngeal carcinoma. Pretreatment titers were usually low in patients in whom recurrence developed and were high in most of the patients who had a good response to treatment and have remained free of recurrence. [ABSTRACT FROM AUTHOR]

We present an unexpected outcome of 10 years of enzyme replacement therapy of a boy with mucopolysaccharidosis type II. Due to a positive familiar history (severe disease course in a sibling) the diagnosis was established in the first month of life. Treatment with Elaprase was introduced two months later. Since then normal physical and mental development is observed. The patient presents only relatively large head circumference (+2.1 SD) and slight decrease of joints mobility. In our opinion, early introduction of enzyme replecement therapy could attenuate the disease course., (© Polish Society for Pediatric Endocrinology and Diabetology.)

Objectives: Mucopolysaccharidoses (MPSs) are a group of rare, inherited metabolic disorders which result from the lack of one of the lysosomal enzymes responsible for the degradation of glycosaminoglycans. Early recognition of MPS is important as it enables prompt implementation of enzyme replacement therapy (ERT). Dipeptidyl peptidase-IV (DPP-IV) is a ubiquitous ectopeptidase which activity has been associated with the cell surface protein CD26. Our aims were to investigate plasma DPP-IV activity in untreated patients with MPS type II in comparison to control individuals and to evaluate changes of DPP-IV during ERT in MPS I or II patients., Design and Methods: One MPS I and five MPS II patients were treated with ERT for up to 19 months. DPP-IV activity was measured in plasma with a colorimetric method using Gly-Pro-p-nitroanilide as a substrate. The reference intervals were observed in 17 healthy donors and in 9 MPS II individuals before ERT implementation., Results: DPP-IV activity ranged from 557 to 1959 nmol/ml/h (median and interquartile range: 1453 [955– 1554], n = 17) in plasma of control samples. In 9 untreated MPS II individuals, DPP-IV activity was higher and ranged from 2565 to 5968 nmol/ml/h (median and interquartile range: 4458 [4031–5161]). In 6 MPS patients receiving ERT, DPP-IV activity ranged from 2984 to 8628 nmol/ml/h. No declining tendency was observed during the treatment., Conclusions: DPP-IV activity is a good, newa nd valuable biomarker distinguishing between MPS and healthy individuals. However, it is not a useful marker of treatment efficacy and is unsuitable for monitoring.

Introduction: Kawasaki disease is the number one cause of acquired heart disease among children in developed countries., Aim: The aim of the study was a retrospective analysis of the factors that may influence the persistence of coronary artery abnormalities in patients with Kawasaki disease., Materials and Methods: Analyzing the medical records of patients hospitalized in the University Children's Hospital of Krakow in the years 2005-2011 we collected the data of 28 patients diagnosed with Kawasaki disease. The group was divided into two subgroups, depending on the duration of the persistence of changes in the coronary arteries - A (n = 17) for up to 6 months, B (n = 11) - for more than 6 months. Both groups were analyzed for the presence of factors that may influence the course of the disease., Results: There were more boys in group A (11 boys (65%), 6 girls (35%)), whereas in group B the distribution was more uniform (6 boys (55%), 5 girls (45%)). The age of onset in group A was 37.9 months (SD 30.8), in group B 39.5 months (SD 16.7). 17.6% of patients in group A and 36.4% in group B were treated with glucocorticoids., Conclusions: In the group of patients in which coronary artery abnormalities disappeared more quickly, male and slightly older children dominated. The only difference observed between the 2 groups related to the frequency of the use of glucocorticoids, they were used more often in children, in whom coronary artery abnormalities persisted longer.

Cerebrospinal fluid (CSF) is the only body fluid in direct contact with the brain and thus is a potential source of biomarkers. Furthermore, CSF serves as a medium of endocrine signaling and contains a multitude of regulatory peptides. A combined study of the peptidome and proteome of CSF or any other body fluid has not been reported previously. We report confident identification in CSF of 563 peptide products derived from 91 precursor proteins as well as a high confidence CSF proteome of 798 proteins. For the CSF peptidome, we use high accuracy mass spectrometry (MS) for MS and MS/MS modes, allowing unambiguous identification of neuropeptides. Combination of the peptidome and proteome data suggests that enzymatic processing of membrane proteins causes release of their extracellular parts into CSF. The CSF proteome has only partial overlap with the plasma proteome, thus it is produced locally rather than deriving from plasma. Our work offers insights into CSF composition and origin.

A 21-month-old girl was admitted to our Department of Pediatrics to diagnose febrile states lasting for previous two weeks, anemia and increased erythrocyte sedimentation rate (ESR - erythrocyte sedimentation rate). The physical examination revealed the paleness of skin and oral mucosa, silent systolic murmur and hypotrophic constitution. The laboratory tests confirmed anemia and showed increased ESR and moderately increased C Reactive Protein (CRP - C Reactive Protein). The blood culture, the urine culture, the stool culture, the tests of the stool in direction of parasites and the serologic tests carried out in direction of infection caused by Toxoplasma ghondi, Mycoplasma pneumoniae, HAV, HCV, CMV, EBV and Parvovirus B19 were all negative. The chest X-ray picture and ultrasonographic examination of abdomen showed no abnormality. The consulting hematologist carried the bone marrow biopsy out--the bone marrow was poorly cellular. The urinary level of catecholamines and plasma level of neuron-specific enolase (NSE) were greatly increased. The computer tomography scan of head, neck, thorax and abdomen did not confirmed the presence of the tumor. Nevertheless the bone scintigraphy demonstrated the presence of foci of abnormally increased activity in left femur and the right hip-bone--pathognomonic of metastatic disease. During the hospitalization we did not observe the fever, but only the deepening anemia, weakness, irritability, limping and the presence of spectacle-shaped hematomas. The blood parameters temporarily were normal after blood transfusion. The patient was transmitted to the Department of Children's Oncology and Hematology. The trepanobiopsy of the bone marrow showed the presence of metastases of neuroblastoma. The magnetic resonance imaging (MRI) was made, but it did not revealed the presence of the primary tumor. The patient underwent a course of chemotherapy.

Background: The development of mass spectrometric (MS) techniques now allows the investigation of very complex protein mixtures ranging from subcellular structures to tissues. Body fluids are also popular targets of proteomic analysis because of their potential for biomarker discovery. Seminal plasma has not yet received much attention from the proteomics community but its characterization could provide a future reference for virtually all studies involving human sperm. The fluid is essential for the survival of spermatozoa and their successful journey through the female reproductive tract., Results: Here we report the high-confidence identification of 923 proteins in seminal fluid from a single individual. Fourier transform MS enabled parts per million mass accuracy, and two consecutive stages of MS fragmentation allowed confident identification of proteins even by single peptides. Analysis with GoMiner annotated two-thirds of the seminal fluid proteome and revealed a large number of extracellular proteins including many proteases. Other proteins originated from male accessory glands and have important roles in spermatozoan survival., Conclusion: This high-confidence characterization of seminal plasma content provides an inventory of proteins with potential roles in fertilization. When combined with quantitative proteomics methodologies, it should be useful for studies of fertilization, male infertility, and prostatic and testicular cancers.

The Aim of the Work: Tic disorders are differentiated in their symptomatology and time of duration. Assessing the courses of tics can be crucial for more successful treatment. The aim of the study was to identify the egzogenic and endogenic causes of tic disorders., Material and Methods: The group of 20 children and adolescents, 6 girls and 14 boys, aged 4-16 years, with tic disorders were analysed. 8 of them were identified as having transient tics 6 chronic motor or vocal tics and 6 Tourette's syndrome. WISC-R, Terman-Merrill, and Raven, Bender-Koppitz and Benton tests and questionnaire "Who are you", and projection tests, talk to children and their parents were used., Results: The analysed group consisted of 70% boys. Gestational and perinatal risk factors were confirmed in 5 children, in similar percentage of different types of tics. In 20% of patients CT or MRI examination showed mild abnormalities, whereas EEG examination excluded epileptic discharges. Majority of children with tics demonstrated significant emotional sesitivity (70%) and tendency to repress fear and anger (60%). Hyperkinetic disorders co-existing with Tourette's syndrome in 3 patients, while learning disabilities were confirmed in 30% children with tic disorders. The presence of negative environmental factors which may contribute to trigger tics showed a half of children from analysed group. Above all children experienced stress in family (40%) and/or in school (20%). The biological and/or environmental factors were detected in 80% children. They were not established in four patients: in one with transient tics and in three with Tourrete's syndrome., Conclusion: The identification of biological and environmental factors is necessary in children with tics, because they are present and significant in majority of children. In 20% of children these factors were not confirmed and in this group other causes should be considered. Considering the risk of disordered emotional and social functioning of children with tics, leading in many cases to tics exacerbation, complex therapeutic care should be provided.

The most abundant thiol in beans (Phaseolus vulgaris L. cv. Saxa) is the tripeptide homoglutathione (hGSH) rather than glutathione (GSH). At the whole-plant level the GSH content is less than 0.5% of the hGSH content. In the present study GSH was supplied to the roots of bean seedlings to test whether GSH can be taken up by roots and transported to the shoot. Therefore, 12-day-old plants were exposed to 1 mmol/L GSH for 4, 8 and 24 h prior to harvest. In response to this GSH exposure, elevated GSH contents were found in all tissues. After 4 h the GSH content increased in the roots from 1 +/- 1 to 22 +/- 2 nmol GSH g(-1) fresh weight (FW), in the leaves from 2 +/- 1 to 9 +/- 4 nmol GSH g(-1) FW, and in the apex from 30 +/- 5 to 75 +/- 4 nmol GSH g(-1) FW. These data indicate that GSH is taken up by bean roots and is transported to above above-ground parts of the plants. Roots exposed to GSH for 24 h contained 2-fold higher cysteine (Cys) and hGSH contents than the controls. Apparently, GSH taken up by the roots is not only loaded into the xylem but also partially degraded and used for hGSH synthesis.

We report a case of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type of the salivary gland arising in a background of chronic sclerosing sialadenitis. Chronic sclerosing sialadenitis is a common fibrosing chronic inflammatory lesion of the submandibular gland, which is thought to be the result of sialolithiasis, and is not associated with a systemic autoimmune disease. Salivary MALT lymphomas are typically associated with lymphoepithelial sialadenitis (LESA) in a patient with or without Sjögren's syndrome. Our case of salivary MALT lymphoma was neither preceded by Sjögren's syndrome nor accompanied by LESA. This case suggests that chronic inflammatory processes other than Sjögren's syndrome may provide a substrate for the development of MALT lymphoma.

Specific phosphorylation of serine- and arginine-rich pre-mRNA splicing factors (SR proteins) is one of the key determinants regulating splicing events. Several kinases involved in SR protein phosphorylation have been identified and characterized, among which human DNA topoisomerase I is known to have DNA-relaxing activity. In this study, we have investigated the mechanism of splicing inhibition by a glycosylated indolocarbazole derivative (NB-506), a potent inhibitor of both kinase and relaxing activities of topoisomerase I. NB-506 completely inhibits the capacity of topoisomerase I to phosphorylate, in vitro, the human splicing factor 2/alternative splicing factor (SF2/ASF). This inhibition is specific, because NB-506 does not demonstrate activity against other kinases known to phosphorylate SF2/ASF such as SR protein kinase 1 and cdc2 kinase. Importantly, HeLa nuclear extracts competent in splicing but not splicing-deficient cytoplasmic S100 extracts treated with the drug fail to phosphorylate SF2/ASF and to support splicing of pre-mRNA substrates containing SF2/ASF-target sequences. Native gel analysis of splicing complexes revealed that the drug affects the formation of the spliceosome, a dynamic ribonucleoprotein structure where splicing takes place. In the presence of the drug, neither pre-spliceosome nor spliceosome is formed, demonstrating that splicing inhibition occurs at early steps of spliceosome assembly. Splicing inhibition can be relieved by adding phosphorylated SF2/ASF, showing that extracts treated with NB-506 lack a phosphorylating activity required for splicing. Moreover, NB-506 has a cytotoxic effect on murine P388 leukemia cells but not on P388CPT5 camptothecin-resistant cells that carry two point mutations in conserved regions of topoisomerase I gene (Gly361Val and Asp709Tyr). After drug treatment, P388 cells accumulated hypophosphorylated forms of SR proteins and polyadenylated RNA in the nucleus. In contrast, neither SR protein phosphorylation nor polyadenylated mRNA distribution was affected in P388 CPT5-treated cells. Consistently, NB506 treatment altered the mRNA levels and/or splicing pattern of several tested genes (Bcl-X, CD 44, SC35, and Sty) in P388 cells but not in P388 CPT5 cells. The study shows for the first time that indolocarbazole drugs targeting topoisomerase I can affect gene expression by modulating pre-mRNA splicing through inhibition of SR proteins phosphorylation.

Background: Successful surgical management of primary hyperparathyroidism requires the ability to identify and distinguish normal from abnormal parathyroid tissue. Microscopic pathologic confirmation often helps with the diagnoses and decisions regarding the extent of parathyroid resection. Confocal reflectance microscopy (CRM) is an optical method of noninvasively imaging tissue without fixation, sectioning, and staining as in standard histopathology. The goal of this study was to determine if CRM imaging could be used to distinguish normal from diseased parathyroid tissue intraoperatively., Methods: In this study, 44 parathyroid glands from 21 patients undergoing operations for primary hyperparathyroidism were imaged immediately after excision. CRM images were compared with conventional hematoxylin-and-eosin stained sections obtained from the same gland. The percentage area occupied by fat cells was calculated in images of both normal and diseased glands., Results: Characteristic microscopic features of parathyroid glands were distinguishable by CRM and correlated well with histopathology. The stromal fat content of normal and diseased glands could easily be determined. The percentage area occupied by fat cells differed significantly (P <.00001) in normal glands (average, 23.0% +/- 10.9%) and adenomatous glands (average, 0.4% +/- 0.7%)., Conclusions: CRM imaging rapidly revealed microscopic features that reliably differentiated normal and diseased parathyroid glands. The success of this preliminary ex vivo study promotes interest in further development of an in situ probe for in vivo clinical diagnostic use.

Background: Papillary thyroid carcinoma is the most common malignant neoplasm of the thyroid gland, and fine-needle aspiration biopsy (FNAB) often is the initial diagnostic method used in its detection. Prior studies have shown that immunohistochemical staining for various cytokeratins in general, and cytokeratin 19 (CK19) in particular, can be applied as an ancillary technique for diagnosing papillary thyroid carcinoma in histologic specimens. In the current study the authors assessed the diagnostic utility of CK19 to detect papillary carcinoma effectively in cytologic preparations of thyroid FNABs., Methods: Immunocytochemical staining with CK19 was performed on cytologic aspirates from 37 papillary thyroid carcinomas and 36 other lesions of the thyroid (14 follicular adenomas, 10 multinodular goiters, 5 cases of Hashimoto thyroiditis, 6 oncocytic [Hürthle cell] neoplasms, and 1 follicular carcinoma). All cases included in the study had a corresponding histopathology specimen., Results: Positive immunocytochemical reactivity for CK19 was identified in 34 of 37 papillary carcinomas and in 1 of 36 other thyroid lesions (sensitivity of 92% and specificity of 97%). Although the strongest reactivity was obtained in methanol fixed thin layer preparations, the antibody also was effective in detecting papillary carcinoma in alcohol fixed and air-dried smears. The single false-positive case was a follicular adenoma with focal areas of papillary hyperplasia. All other aspirates including those from cases of Hashimoto thyroiditis, multinodular goiter, follicular adenoma, oncocytic neoplasms, and follicular carcinoma were negative., Conclusions: CK19 is an effective, highly sensitive, and specific ancillary tool for the diagnosis of papillary carcinoma in thyroid FNABs.

Purpose: This study attempted to identify differential cytokeratin expression in cystic jaw lesions using immunohistochemical staining., Patients and Methods: The charts from selected patients treated between 1983 and 1994 for jaw cysts were evaluated. Twenty-four paraffinized specimens were selected randomly for investigation with 5 immunohistochemical stains. The 4 diagnostic categories included ameloblastoma, dentigerous cyst, odontogenic keratocyst (OKC), and recurrent odontogenic keratocyst in patients with nevoid basal cell carcinoma (NBCC) syndrome. The 5 immunohistochemical stains included antibodies to cytokeratins 13, 17, and 18; CAM 5.2; AE 1/3; and carcinoembryonic antigen (CEA)., Results: Differential staining of OKCs from patients with and without NBCC syndrome was found only with the antibody to cytokeratin 17. Furthermore, staining of OKCs in syndromic patients appeared to be stronger and more uniform than in nonsyndromic patients., Conclusions: These findings suggest that immunohistochemical staining for cytokeratin 17 may aid in the diagnosis of OKCs and may be used to further subdivide these lesions based on the presence or absence of NBCC syndrome.

We present a case of dedifferentiated chondrosarcoma of the larynx in which the dedifferentiated component was initially diagnosed by fine-needle aspiration (FNA). The patient was a 74-yr-old man who presented with difficulty breathing and an anterior neck mass. A CT scan demonstrated a 4.5-cm cartilaginous lesion involving the left thyroid cartilage, with an anterior soft-tissue component. Nine years prior, the patient had an incomplete resection of a low-grade chondrosarcoma at the same site. FNA was performed on the current lesion, demonstrating a high-grade spindle-cell sarcoma with a storiform pattern. The cytomorphology together with immunocytochemistry and electron microscopy were diagnostic of malignant fibrous histiocytoma (MFH), and synthesis of the clinical, radiographic, and cytomorphologic features resulted in a diagnosis of dedifferentiated chondrosarcoma. The cytologic diagnosis was histologically confirmed by laryngectomy. Although rare, dedifferentiated chondrosarcoma should be included in the differential diagnosis of high-grade sarcomas of bone and cartilage assessed by FNA., (Copyright 2000 Wiley-Liss, Inc.)

Few large series compare lymphomas of the nasal cavity with those of the paranasal sinuses. We studied the cases of 58 patients, 34 males and 24 females, aged 7 to 92 years (mean, 57 years), who had lymphoma involving the nasal cavity or paranasal sinuses. Thirty-three patients had diffuse large B-cell lymphoma (DLBCL). Twenty-three were male and 10 were female, with an age range of 7 to 91 years (mean, 63 years); two were HIV-positive. Only 2 of 11 cases tested (one in an HIV-positive patient and one of lymphomatoid granulomatosis type) were Epstein-Barr virus (EBV)-positive. Thirty (91%) involved paranasal sinuses, 10 with nasal involvement, whereas three cases had nasal, but not sinus, involvement. At last follow-up, 16 (67%) were free of disease 7 to 169 months later (mean, 65 months), and 8 (33%) had died of disease 2 to 166 months later (mean, 45 months). Seventeen patients had nasal-type natural killer (NK)/T-cell lymphoma. There were 10 women and 7 men, aged 27 to 78 years (mean, 48 years). Thirteen of 14 were EBV-positive. Sixteen patients had nasal involvement, eight with sinus involvement. Eleven (73%) of 15 were alive and well 6 to 321 months later (mean, 139 months), three (20%) died of lymphoma 1, 11, and 12 months later, and one (7%) is alive with disease. There was one case each of marginal zone B-cell lymphoma, Burkitt's lymphoma, Burkitt-like lymphoma, peripheral T-cell lymphoma of unspecified type, and adult T-cell lymphoma/leukemia. In an additional three cases, the lymphomas were composed predominantly of large cells, but no immunophenotyping could be performed for subclassification. In 19 cases (17 DLBCLs, 1 Burkitt-like lymphoma, and 1 lymphoma of uncertain lineage), presenting symptoms included complaints related to the eyes. In 16 cases (13 DLBCLs, 1 Burkitt-like lymphoma, 1 nasal NK/T-cell lymphoma, and 1 lymphoma of uncertain lineage), the orbit was invaded by lymphoma. In our series, the most common lymphoma to arise in the sinonasal area is DLBCL, followed by nasal NK/T-cell lymphoma. Comparison of these two types of lymphoma showed that lymphomas involving sinuses without nasal involvement were predominantly DLBCLs (20 of 21), whereas nasal cavity lymphomas without sinus involvement were usually NK/T-cell type (8 of 11) (p = 0.000125). Compared with patients with DLBCL, patients with nasal NK/T-cell lymphoma were overall younger, with a lower male-to-female ratio. Lymphomas of B-cell lineage were more likely to be associated with symptoms related to the eyes (p < 0.0005) and to have extension to the orbit (p < 0.01) than were lymphomas of T- or NK-cell lineage. In contrast to results of Asian studies in which nasal NK/T-cell lymphoma has a very poor prognosis, our nasal NK/T-cell lymphomas had an outcome similar to that of DLBCL.

Microscopic evaluation of all adult tonsillar specimens has been considered essential despite the low incidence of unsuspected pathologic conditions. We evaluate whether routine histologic examination of clinically benign adult tonsillar specimens is indicated. We retrospectively reviewed pathology results from all tonsillectomies performed on patients ages 18 years or older at our institution from 1989 through 1996. Three groups were created on the basis of indications for tonsillectomy: (1) routine tonsillectomies for benign disease, (2) asymmetric tonsils, and (3) search for unknown primary lesions. Demographic data and pathologic findings in each group were analyzed. In 1280 tonsillectomies performed for benign disease there were no malignancies (0%) and 32 cases (2.50%) with clinically unsuspected benign pathologic conditions. In 31 cases of tonsillar asymmetry, two cases with malignant lymphoma (6.5%) and three cases with benign pathology (9.7%) were identified. In nine patients with squamous cell carcinoma metastatic to the neck, two occult primary lesions were identified in the ipsilateral tonsil. Our results suggest that histologic evaluation of adult tonsils removed for benign disease may be clinically unnecessary. The elimination of microscopic examination of tonsils removed from patients whose clinical presentation is entirely consistent with benign disease poses minimal risk of missing clinically significant pathologic conditions. Substantial costs for negative examinations may be avoided.

Increased expression of the cell adhesion molecule, CD44 standard form (CD44s), has been associated with papillary epithelial tumors, and decreased expression has been linked to tumor invasion and metastasis. Sinonasal inverted papillomas (SIPs) are the most common papillary tumors of the sinonasal tract. This study tests whether the development of squamous cell carcinoma (SCC) in situ and invasive SCC in SIP is associated with altered expression of CD44s. Seventy-six specimens of SIP from 68 patients, 2 specimens of SIP with focal SCC in situ, and 10 specimens of invasive SCC arising in SIP were studied. Automated immunohistochemistry was performed for CD44s expression on paraffin-embedded tissue sections using mouse antihuman CD44 antibody. All 76 SIPs (100%) expressed CD44 (strong membranous staining, 83%; moderate staining, 12%; weak staining, 5%). Two (100%) of 2 SIPs with SCC in situ maintained strong expression in benign and severely dysplastic foci. Six (60%) of 10 SIPs with SCC showed complete loss of CD44s expression, while 4 (40%) of 10 cases of SIP with SCC showed weak expression. Two SIPs with SCC (20%) featured weak diffuse staining of the SCC component, and 2 SIPs with SCC (20%) featured weak focal staining of the SCC component. The non-SCC SIP components of the 10 SIPs with SCC uniformly featured intact membranous CD44 staining. As in other papillary epithelial neoplasms, the typical benign SIP features diffuse membranous CD44s expression. In cases of SIP developing an invasive SCC, CD44s expression in the SCC component is frequently lost.

We describe two cases of a distinctive in situ and invasive cutaneous adnexal neoplasm occurring in the eyelid. Mucinous carcinoma represented the invasive portion of the tumor in one case, whereas the other infiltrated in small solid nests. The in situ component is identical to the recently described solid papillary carcinoma of the breast (endocrine ductal carcinoma in situ). Both tumors produced intra- and extracellular mucin, exhibited endocrine differentiation by immunohistochemistry and ultrastructural analysis, and were positive for estrogen and progesterone receptors.

Eosinophilic angiocentric fibrosis (EAF) is a rare condition of unknown etiology that causes stenosis of the upper respiratory tract. We report one case in which immunohistochemical studies were performed that involved the mucosa of the lateral nasal wall and septum of a 54-year-old woman. The lesion showed areas with vascular proliferation, small-vessel vasculitis, and dense inflammatory infiltrate consisting of T lymphocytes, macrophages, polyclonal plasma cells, and numerous eosinophils and neutrophils. Other areas were hypocellular and fibrotic, and in those, the collagen bundles showed perivascular onion-skin whorling. The clinical and histologic similarities between our case and those previously described, in addition to the immunohistochemical findings, support the hypothesis that eosinophilic angiocentric fibrosis is a chronic inflammatory condition with a peculiar and striking stromal response.

The term "endolymphatic sac tumor" (ELST) was coined to identify the likely origin of aggressive papillary tumors of the temporal bone. To evaluate the validity of this designation, the temporal bone collection at the Massachusetts Eye and Ear Infirmary was accessed in an effort to determine the pathologic relationship between these tumors and the endolymphatic sac. The search resulted in the identification of a de-novo papillary epithelial lesion arising within the confines of the endolymphatic sac in a patient with von Hippel-Lindau (VHL) disease who harbored a large, destructive ELST in the opposite temporal bone. This finding provides the most substantial evidence to date regarding the origin of the ELST and the accuracy of its nomenclature. Seven additional clinical cases of ELST were identified and analyzed in order to define the natural history of these tumors. All patients had a history of sensorineural hearing loss diagnosed an average of 10.6 years prior to tumor discovery. The presence of a polypoid external auditory canal mass, facial paralysis, and evidence of a destructive mass arising on the posterior fossa surface of the temporal bone were common physical and radiographic findings. The management of these patients, as well as those who are probably prone to such tumors (i.e., VHL patients), is discussed.

A 31-year-old woman had bilateral swelling of the parotid glands at 4 months of pregnancy. MR imaging showed marked enlargement of the parotid glands with increased signal on images with long repetition times. A diagnosis of polycystic disease of the parotid gland was made after biopsy and histologic examination. The radiographic and histologic features of this rare disease are discussed.

Although intraepithelial pigment in the prostate gland has been termed melanosis, the nature of the pigment is not entirely clear, and many pathologists are not aware of its existence. We examined 863 hematoxylin and eosin (H + E) stained slides from 150 surgical specimens of prostate (69 needle biopsies, 66 transurethral resections, 14 radical prostatectomies, and 1 suprapubic prostatectomy) from 149 patients (age range, 47 to 90 years; mean 70 years) in an effort to characterize this pigment. The 1-3 microns in diameter, predominantly subnuclear, yellow-brown to gray-brown granules with a dark blue rim (by H + E) stained positively with Fontana-Masson, periodic acid-Schiff with diastase, Congo red, luxol fast blue, and oil-red-O and exhibited yellow autofluorescence consistent with lipofuscin. H + E stained slides revealed pigment in the benign epithelium in 86 of 150 cases (57%), within stromal macrophages in eight cases, and in atypical epithelium in two cases of high-grade prostatic intraepithelial neoplasia. Ten cases of invasive adenocarcinoma without recognizable pigment in H + E stained sections were stained by the Fontana-Masson technique, and pigment was identified in malignant epithelium in three of these cases. Ultrastructural examination of intraepithelial pigment in KII-fixed tissue from three radical prostatectomy specimens demonstrated the typical appearance of lipofuscin. Although intraepithelial pigment in prostatic biopsy or resection specimens is usually considered characteristic of seminal vesicle epithelium, our study demonstrates that lipofuscin is commonly present in epithelial cells of benign prostatic hyperplasia and less frequently in those of prostatic intraepithelial neoplasia and adenocarcinoma. The recognition of this pigment is important in preventing diagnostic confusion with seminal vesicle epithelium and with melanocytic lesions.

We report three cases of an orbital soft tissue lesion that fulfills the histologic, immunohistochemical, and electron microscopic criteria for solitary fibrous tumor, an entity previously described as a pleural tumor, but recently reported to occur in other locations. All three patients presented with proptosis. Two of the patients were cured by simple excision, and one patient had two recurrences, the last recurrence incompletely excised. The findings indicate that solitary fibrous tumor can occur in the orbit and, like solitary fibrous tumors of other anatomic sites, may behave in a nonaggressive or occasionally, locally aggressive fashion, with as yet no metastatic potential demonstrated in orbital lesions.

Hamartomas are easily diagnosed entities when occurring in the lung and gastrointestinal tract. In the nose and nasopharynx, where such lesions are rare, biopsy of a hamartoma containing epithelial proliferation may lead to a misdiagnosis of cancer, with resultant radical and deforming surgery, particularly if they present in adulthood. We encountered three such lesions over 2 years in the Massachusetts Eye Ear Infirmary, and another was retrieved from the recent files. All presented with nonspecific obstructive symptoms of the nose or nasopharynx, and were treated by resection. Follow-up is short, (4 months to 1 year), but in no case has there been recurrence. The clinical diagnosis was malignancy in 2 cases, inflammatory polyp in one.

We report two solitary fibrous tumors of the nasal cavity and paranasal sinuses that were histologically and immunohistochemically virtually identical to solitary fibrous tumors (fibrous mesotheliomas) of the pleura. One tumor arose in a 48-year-old woman and the other in a 45-year-old woman. Both patients presented with nasal symptoms, and both patients are alive without evidence of disease 6 months and 1 year after excision. The tumors had a disorganized or "patternless" arrangement of spindle cells in a collagenous background and prominent vascular channels of varying size. Immunoperoxidase stains on paraffin sections showed staining of the cells for vimentin only; there was no staining for keratin, S-100 protein, desmin, and actin. Both cases presented some degree of diagnostic difficulty and had to be distinguished from other spindle cell tumors of the nasal cavity and paranasal sinuses, such as hemangiopericytoma, angiofibroma, and fibrous histiocytoma.

Hamartomas of the hypopharynx are rare. Detailed microscopic study is necessary to differentiate these tumors from the adult-type rhabdomyoma, pleomorphic rhabdomyosarcoma, and granular cell tumor. To our knowledge, our case of a hypopharyngeal rhabdomyomatous hamartoma removed by a lateral pharyngotomy is the only verified hypopharyngeal case in the literature, although a lesion reported by H. W. Smith in 1959 as a laryngeal rhabdomyoma most probably represents a rhabdomyomatous hamartoma, similar to that of the present case.

Laryngeal paraganglioma, carcinoid tumor, and small-cell carcinoma are rare. Histologically they are similar to analogous tumors in other locations but may be difficult to identify in small biopsy specimens. We compared the light microscopic, histochemical, immunohistochemical, and electron microscopic features of two laryngeal paragangliomas, one carcinoid tumor, and six small-cell carcinomas. The paraganglioma chief cells stained with Grimelius stain and for chromogranin and neuron-specific enolase. The carcinoid tumor cells stained with Grimelius stain and for chromogranin, serotonin, neuron-specific enolase, and keratin. The small-cell carcinoma cells stained for keratin and neuron-specific enolase. The patients with paragangliomas and carcinoid tumor remain healthy through 20 months of follow-up. Four of the patients with small-cell carcinomas have died. Distinction between these tumors is warranted by differing histologic appearances, staining characteristics, and biologic behavior.

Mucoepidermoid carcinoma of the lacrimal sac was diagnosed in a 62-year-old woman who presented with epiphora and a fixed painless mass in the area of the lacrimal sac. Histopathologic findings included nests and islands of neoplastic cells in a fibrotic stroma. The tumor nests contained both glandular spaces and solid epithelial areas. Treatment entailed dacryocystectomy with silastic intubation and subsequent medial maxillectomy and ethmoidectomy. The patient was tumor free on 1-year follow-up. Clinical features, histopathological findings, prognosis, and management of this rare tumor are reviewed in this article.