Subtype frequency, demographic features, treatment and outcome of Juvenile Arthritis in one Centre in Abu Dhabi in the United Arab Emirates.

BACKGROUND: Juvenile Idiopathic Arthritis is a chronic inflammatory disease that affects 1 in 1000 children worldwide. Our population in the United Arab Emirates is diverse. The objective of this study is to describe the subtype frequency, demographic features and treatments received and outcome of our patients. METHODS: Patients with the diagnosis of Juvenile Arthritis identified through the hospital electronic medical records system (EMR), which was implemented for all medical documentation in January 2011. All patients included are patients who presented to our center for treatment and follow up from 2011 to end of 2021. Retrospective case notes review of patients electronic medical records with the diagnosis of JIA was performed. RESULTS: One hundred thirty-eight patients in total. Oligoarticular subtype was the most represented with 75 patients (55%) followed by Rheumatoid factor negative polyarticular JIA with 32 patients (23%) then Enthesitis related arthritis (ERA) with 10 patients (7%) then psoriatic (6%) then systemic JIA (5%). Undifferentiated subtype of 2%. The most diagnostic delay is in enthesitis related arthritis subtype with a mean of 11.4 months (6-25) followed by undifferentiated JIA with a mean of 7.5 months (4-8.5). 131 (96%) out of 138 received steroid treatment. Only 6 patients did not receive steroids. Out of 138 patients, 101 (73%) were on synthetic disease modifying medication methotrexate. Sixty-eight patients out of the total 138 required biologic treatment (49%). In total 93 patients achieved clinical remission (67%). In remission on treatment 78 patients which is (56%) of the total number of patients with follow up ranging from 1 to 5 years and 84% of patients in remission. In remission off treatment 15 patients (11% of all patients and 16% of patients in remission). CONCLUSION: The most common subtype in our cohort of patients is oligoarticular JIA. Longest delay is for ERA subtype. All our patients with oligoarticular JIA received Intra articular steroid injection as first line treatment. 49% of our patients received biologic treatment similar to rate in Northern Europe. Our remission rate is 67% with 11% of patients are in remission off treatment. Access to care remains a priority to treat patients effectively.

Development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan).

AIMS: This study aims to describe the development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan) and to provide a baseline child and parent demographic information, birth history of the child participants, and distribution of the participants based on topographical distribution of cerebral palsy (CP) and functional classification systems. METHODS: The CPUP-Jordan was developed using a similar framework of a follow-up surveillance programme for persons with CP in Sweden (CPUP). Standard assessment forms were utilized to collect data related to child and family demographics, child birth history, and functional classifications and physiotherapy and occupational therapy assessments and interventions. Research assistants were trained to conduct the assessments. A secured web-based system was developed to store data and disseminate knowledge maintained in the registry. Children with CP were included in the registry if they have confirmed diagnosis of CP. The ascertainment age of inclusion and the minimum age of survival required are 4 years. RESULTS: One hundred sixty-seven children were registered between 2013 and 2015 (mean age is 3.6 ± 3.0 years). Forty-two percent were born premature, and 48% were less than the normal birthweight. Perinatal causes were reported for 54% of the participants. The most common type of CP based on tone disturbance was spastic type, and the most common topographical distributions of motor dysfunction were quadriplegia followed by diplegia. Fifty-six percent of the participants had severe limitation in ambulation; 48% had restricted manual abilities, and 47% had limited communication abilities even with familiar family members and partners. CONCLUSIONS: The development of CPUP-Jordan registry for children with CP proved to be both feasible and informative. The registry baseline descriptive data were similar to those reported in previous research in Jordan supporting validity of the data. The implementation of CPUP-Jordan at national level is expected to have a positive impact on children with CP, clinicians, policymakers, and researchers.

MRI and Ultrasonography for assessing multifocal disease and tumor size in breast cancer: Comparison with histopathological results.

UNLABELLED: The purpose of the study was to compare the accuracy of breast MRI and ultrasonography in assessing the tumor focality and tumor size of newly diagnosed non-high risk breast cancer patients. METHODS: The tumor focality status and the maximal tumor diameter by MRI and ultrasonography were retrospectively compared with the corresponding histopathological findings as reference. Test characteristics concerning the tumor focality status were calculated. Bland-Altman plots were generated to evaluate the agreement of the tumor size measurements by imaging and histopathology. The t-test for dependent samples and the Fisher exact test were used to test differences between groups for statistical significance. The Pearson correlation coefficient r was calculated to measure the degree of association between the tumor diameter by imaging and histopathology. RESULTS: Sixty-four patient diagnosed between 2011 and 2013 were analyzed. MRI showed a good sensitivity of 83% for detecting multifocal disease (ultrasonography, 75%). The positive predictive value was 67% and the ratio of true-positive to false-positive findings 2.0. MRI showed better limits of agreement (-21 to 26 mm versus -29 to 26 mm) and a better correlation (r=0.77 versus r=0.66) with the histopathological tumor diameter compared to ultrasonography. The mean differences between the tumor diameter by MRI and histopathology and ultrasonography and histopathology were not significantly different (p=0.09). The T classification (T1a, T1b, T1c, T2, T3) was correctly estimated by MRI in 43 patients (67.2%) and by ultrasonography in 39 patients (60.9%) (p=0.58). CONCLUSION: In our patient cohort only a modest diagnostic advantage of MRI compared to ultrasonography could be detected.

Use of the Measure of Processes of Care (MPOC-20) to evaluate health service delivery for children with cerebral palsy and their families in Jordan: validation of Arabic-translated version (AR-MPOC-20).

BACKGROUND: Family-centred service (FCS) is widely accepted now as best practice in paediatric rehabilitation. The Measure of Processes of Care-20 items set (MPOC-20) is a valid and reliable self-report measure of parents' perceptions of the extent to which health services are family-centred. Arabic-translated and validated version of the MPOC-20 (AR-MPOC-20) is used to examine Jordanian families' perception of service providers' caregiving behaviours as they receive rehabilitation services for their children with cerebral palsy (CP). METHODS: Parents of 114 children with CP who are receiving services at different settings in Jordan were interviewed using the AR-MPOC-20. Participating children aged 4.1 ± 4.4 years, 53.5% were males. Children varied across gross motor functional classification system (GMFCS). Parents were mostly mothers (76.3%), with at least high school education (71.9%). RESULTS: Factor analyses of the AR-MPOC-20 yielded a five-factor solution with items loaded differently from the original measure. All items correlated best and significantly with their own Arabic scale score (rs: 0.91-0.26, P < 0.01). Internal consistency values of AR-MPOC-20 scales were acceptable (Cronbach's α: 0.69-0.82). Scale 'Providing Written Information' has the lowest average score (1.9 ± 1.6), while scale 'Respectful & Coordinated Care' has the highest average score (5.2 ± 1.5). CONCLUSION: The AR-MPOC-20 is found to be a valid and reliable measure for use with Arabic-Jordanian families of children with CP. FCS is not yet well implemented in Jordan, with parents reporting more need for information about their children's health and available services. Service providers are encouraged to apply FCS in paediatric rehabilitation, and giving more attention to effective communication and information exchange with families. AR-MPOC-20 is recommended for use for program evaluation.

Family resources for families of children with cerebral palsy in Jordan: psychometric properties of the Arabic-family resources scale.

BACKGROUND: Resource-based, family-centred practices are associated with better health, emotional, and social well-being of children with disabilities. The adequacy of resources available for families of children with disabilities in Middle Eastern countries has not been described adequately in part because of lack of measures that are culturally adapted to be used in Arabic countries. Therefore, this study aims to (1) to evaluate the psychometric properties of the Arabic-Family Resource Scale (A-FRS) on a sample of families of children cerebral palsy (CP); (2) examine the adequacy of family resources as perceived by parents of children with CP in Jordan; and (3) examine the influence of child and family demographic variables on how parents report resources available to their families. METHOD: A cross-section design was applied. One-hundred fifteen parents of children with CP with mean age 4.6 years (SD = 4.4) and their parents participated in the study. Research assistants interviewed the participants to complete the A-FRS, and family and child demographic questionnaire, and determined the Gross Motor Function Classification System level of children. RESULTS: The principal axis factoring of the A-FRS yielded a six-factor solution that accounted for 67.39% of the variance and that is different than the factor structure reported by the developers of the FRS. Cronbach's coefficient alpha of the total score of family resources was 0.86 indicating a good internal consistency and the test­retest reliability for the total scale score was r = 0.92 (P = 0.000) indicating excellent test­retest reliability. Child health and family income were significantly associated with the total score of the A-FRS. CONCLUSIONS: The A-FRS is a valid and reliable measure of family resources for Jordanian families of children with CP. Service providers are encouraged to use A-FRS with families to plan resource-based interventions in which family resources are mobilized to meet family needs.

Family ecology of young children with cerebral palsy.

BACKGROUND: Family ecology in early childhood may influence children's activity and participation in daily life. The aim of this study was to describe family functioning, family expectations of their children, family support to their children, and supports for families of young children with cerebral palsy (CP) based on children's gross motor function level. METHODS: Participants were 398 children with CP (mean age = 44.9 months) and their parents residing in the USA and Canada. Parents completed four measures of family ecology, the Family Environment Scale (FES), Family Expectations of Child (FEC), Family Support to Child (FSC) and Family Support Scale (FSS). RESULTS: The median scores on the FES indicated average to high family functioning and the median score on the FSS indicated that families had helpful family supports. On average, parents reported high expectations of their children on the FEC and strong support to their children on the FSC. On the FES, higher levels of achievement orientation were reported by parents of children in Gross Motor Function Classification System (GMFCS) level II than parents of children in level I, and higher levels of control were reported by parents of children in level I than parents of children in level IV. On the FEC, parents of children with limited gross motor function (level V) reported lower expectations than parents of children at all other levels. CONCLUSIONS: Family ecology, including family strengths, expectations, interests, supports and resources, should be discussed when providing interventions and supports for young children with CP and their families.

Profiles of family needs of children and youth with cerebral palsy.

BACKGROUND: To identify profiles of family needs of families of children and youth with cerebral palsy (CP), and determine whether profile membership is related to child, family and service characteristics. METHODS: Participants were mostly mothers (80%) of 579 children and youth with CP. A family member completed modified version of the Family Needs Survey and questionnaires about their child, family and services. Research assistants determined the Gross Motor Function Classification System levels. K-means cluster analysis identified profiles of needs. Cluster membership was analysed to examine differences in clusters based on selected characteristics. RESULTS: Four profiles of needs were identified: Low needs, Needs related to community and financial resources, Needs related to child health condition and High needs. Profile membership was differentiated based on child/youth gross motor function, adaptive behaviour, family relationships, family income, access and effort to co-ordinate services. CONCLUSION: Despite heterogeneity among individuals with CP and their families, four profiles of family needs were identified. In total, 51% of families had low needs suggesting that they are effectively managing their children's health conditions while 11% of families had high needs that may require high levels of services and supports. Service providers are encouraged to partner with families, provide anticipatory guidance and co-ordinate services.

Oral and maxillofacial tumours in north Jordanian children and adolescents: a retrospective analysis over 10 years.

This study evaluates the types and distribution of oral and maxillofacial tumours in north Jordanian children and adolescents. The records of the Department of Pathology at Jordan University of Science & Technology, during the period 1991-2000, were reviewed for patients younger than 19 years with oral and maxillofacial tumours. The tumours were analysed for age, sex, site and type. Out of all maxillofacial tumours, 23% occurred in children and adolescents, of these 10% were malignant and 90% were benign. 73% of the latter were soft tissue tumours and 17% were jaw tumours (58% odontogenic and 42% non-odontogenic). The most common benign soft tissue and jaw tumours were haemangioma and odontoma, respectively. 58% of malignant tumours were sarcomas and 42% were carcinomas. The mean age was 11 years with a female to male ratio of 1:1.2. The intraoral and extraoral sites most commonly affected by benign soft tissue tumours were the lower lip and face, respectively. While the total number of benign jaw tumours was slightly larger in the maxilla than the mandible, odontogenic tumours were more in the maxilla, and nonodontogenic tumours were more in the mandible. Differences found in this study between Jordanian children and adolescents and those from other countries may be attributable to genetic and geographic differences. The majority of benign tumours in the young are probably developmental rather than true neoplasms.

Osteosarcoma of the urinary bladder occurring simultaneously with prostate and bowel carcinomas: report of a case and review of the literature.

Osteosarcoma is a very rare tumor of the urinary bladder, with only 28 well-documented cases reported in the literature to date. The tumors have characteristically been large and polypoid and most commonly have been located in the trigone. An abdominal mass has occasionally been palpated, and rectal examination has frequently revealed a mass in the region of the bladder. The prognosis for this tumor is very poor. In this report, a 75-year-old man with hematuria was found to have a primary osteosarcoma of the urinary bladder with superficial invasion. Radical cystoprostatectomy and ileal conduit urinary diversion were performed. During surgery, a synchronous mucinous adenocarcinoma of the ascending colon was found, and a right hemicolectomy was performed. Prostatic adenocarcinoma was discovered from the histology. The patient died 7 months after surgery with systemic spread of the osteosarcoma. Osteosarcoma of the bladder should be distinguished from other bladder tumors that may be associated with bone formation, such as carcinosarcomas and transitional cell carcinomas with osseous metaplasia of their stroma, both of which have a better prognosis than osteosarcoma.

Follicular lymphoma can be distinguished from benign follicular hyperplasia by flow cytometry using simultaneous staining of cytoplasmic bcl-2 and cell surface CD20.

The distinction between benign follicular hyperplasia (FH) and follicular lymphoma (FL) is sometimes problematic. We wanted to determine whether the expression of bcl-2 of FH was quantitatively different from that of FL, using surface CD20 expression as a discriminator of the various lymphoid compartments. Lymph node cell suspensions from 12 cases of FH and 17 cases of FL were analyzed by flow cytometry using a combined surface CD20 and intracellular bcl-2 staining. CD20- T cells in FH demonstrated the same bcl-2 expression as the CD20+ mantle cells, but the bright CD20+ germinal center cells showed near absence of bcl-2 expression. In contrast, the neoplastic cells of FL showed greater bcl-2 expression than the T cells of the same tumors and all cell populations of FH. This difference was particularly significant between the neoplastic B cells of FL and the germinal center cells of FH. The combined analysis of CD20 and bcl-2 should be useful for the differential diagnosis between FH and FL and particularly applicable to limited samples or when B-cell clonality is in question. Whether the quantitation of bcl-2 expression can be of further discriminatory value in malignant lymphomas remains to be determined.

Development of contralateral pleural effusion during chemotherapy for tuberculous pleurisy.

Paradoxical worsening of tuberculous lesions, despite effective chemotherapy, has been reported in intracranial tuberculomas, lymph nodes, pulmonary disease, and tuberculous pleurisy. However, development of contralateral pleural effusion during treatment of tuberculous pleurisy is very rare. We report the case of a 22 year old female patient who presented with right sided pleural effusion and was treated with antituberculous drugs. Four weeks later although her right sided pleural effusion was subsiding she developed a left sided pleural effusion. Closed pleural biopsy on the left side showed granulomatous inflammation with early caseation. Antituberculous drugs were continued and a short course of oral prednisolone was added. She recovered completely and her chest x-ray became normal after finishing her treatment.

Pulmonary mucosa-associated lymphoid tissue lymphoma: CT and pathologic findings.

OBJECTIVE: The objective of this study was to determine the CT findings in pulmonary mucosa-associated lymphoid tissue lymphoma and to correlate these CT findings with histologic specimens. CONCLUSION: In the 11 patients reviewed, the most common CT appearance of pulmonary mucosa-associated lymphoid tissue lymphoma was consolidation with air bronchograms, correlating histologically with a cellular lymphocytic infiltrate expanding the interstitium and compressing adjacent alveoli, producing air bronchograms.

Radiotherapy in the management of orbital lymphoma.

PURPOSE: This retrospective study reviews the treatment technique, disease outcome, and complications of radiotherapy used in the management of lymphoma involving the orbits. PATIENTS & METHODS: Thirty-eight patients were treated between May 1969 and January 1995, with a median follow-up of 8.3 years. All patients had biopsy-proven orbital lymphoma. Twenty patients who had limited disease were treated with curative intent, and 18 patients who had known systemic disease were treated with palliative intent. Of the 20 patients treated with curative intent, 14 had low-grade and 6 had intermediate- or high-grade disease. None received chemotherapy. Most patients received treatment with 250 kVP or 60Co radiation, using either an en face anterior field or wedged anterior and lateral fields. Median treatment dose was 25 Gy. Lens shielding was performed if possible. For patients treated for cure, cause-specific survival and freedom from distant relapse were calculated using the Kaplan-Meier method. RESULTS: Control of disease in the orbit was achieved in all but 1 patient, who developed an out-of-field recurrence after irradiation of a lacrimal tumor and was salvaged with further radiotherapy. In the patients treated curatively, the 5-year rate of actuarial freedom from distant relapse was 61% for those with low-grade and 33% for those with intermediate/high-grade disease (p = 0.08). Cause-specific survival at 5 years was 89% for patients with low-grade and 33% for those with intermediate/high-grade disease (p = 0.005). Two patients with low-grade disease had contralateral orbital failures; both were salvaged with further irradiation. Acute toxicity was minimal. Cataracts developed in 7 of 21 patients treated without lens shielding and 0 of 17 patients treated with lens shielding. No patient developed significant late lacrimal toxicity. CONCLUSION: Radiotherapy is a safe and effective local treatment in the management of orbital lymphoma.

Non-Hodgkin's lymphoma of the head and neck: a 30-year experience at the University of Florida.

BACKGROUND: Outcome in previously untreated patients with non-Hodgkin's lymphoma of the head and neck needed to be assessed. METHODS: A retrospective review was performed of 79 patients with stage I or II non-Hodgkin's lymphoma of the head and neck treated between 1964 and 1994 with radiotherapy (RT) or combined modality therapy (CMT) at the University of Florida. Freedom from relapse, cause-specific survival, and absolute survival were analyzed by the Kaplan-Meier method. Patterns of failure were defined, and the relationship between dose and infield recurrence was studied. Histology was classified as low grade or intermediate/high grade. RESULTS: At 10 years, absolute survival for patients with low-grade lymphoma treated with RT was 45%; absolute survival for patients with intermediate/high-grade lymphoma was 41% for those treated with RT and 57% for those who received CMT. Twenty-seven patients had a recurrence of lymphoma after initial treatment. Twenty patients (74%) had recurrences outside the radiation treatment field; 90% of these failures were in predictable sites that would be included in comprehensive lymphatic irradiation fields (Waldeyer's ring, mantle, and whole abdomen). No clear dose response was observed. Multivariate analysis showed that patients with tumors <5 cm in diameter had improved cause-specific survival, absolute survival, and freedom from relapse compared with patients with tumors > or = 5 cm in diameter. CONCLUSIONS: Patients with non-Hodgkin's lymphoma in the head and neck with tumors > or = 5 cm in diameter appear to have a worse prognosis than those with smaller tumors. The patterns of failure suggest that initial treatment with comprehensive lymphatic irradiation fields could potentially eliminate the majority of treatment failures.

Multiparametric flow cytometry in the diagnosis and characterization of low-grade pulmonary mucosa-associated lymphoid tissue lymphomas.

Primary mucosa associated lymphoid tissue (MALT) lymphomas are rare neoplasms that seem to have a better prognosis than nodal lymphomas. Morphologic diagnosis of these lesions may be difficult because of features that overlap with those of benign lymphoid infiltrates. In this study, we assessed the contribution of multi-parametric flow cytometry in demonstrating clonality and further characterizing pulmonary MALT lymphomas. Based on a clinical or pathologic suspicion of MALT-lymphoma, 3 transbronchial biopsies, 4 fine needle aspirates, 1 core needle biopsy, and 13 wedge excisions of lung were submitted fresh (unfixed) to our laboratory for evaluation. Among the 13 cases diagnosed as MALT lymphomas, B-cell monoclonality was established by identifying expression of a single immunoglobulin light chain on CD20 or CD19-positive cells in 12 cases. One case lacked expression of both light chains on B-cells. Of 11 lymphoma cases in which CD5 and CD10 surface antigens were assessed, no cases expressed CD10, and 1 case demonstrated weak CD5 expression. Nine of 10 cases studied were diploid and 1 case was hyperdiploid. All of the lymphomas displayed low (< or = 3%) S-phase fractions consistent with low grade processes. In 10 patients with short follow-up, none died of their disease and the majority had no evidence of lymphoma dissemination. In seven of the remaining eight cases, B-cells were polyclonal consistent with reactive processes. In one morphologically reactive case, flow cytometric analysis was unsuccessful because of poor cell viability. The pulmonary MALT lymphomas in this study represent a group of B-cell tumors with distinctive morphologic, immunophenotypic, and cell kinetic characteristics. Multi-parametric flow cytometry is useful for confirming B-cell monoclonality and illustrating an antigenic profile compatible with this diagnosis. Flow cytometry can be particularly helpful when working with small biopsies and cytologic samples with limited diagnostic material and may abrogate the need for more aggressive surgical procedures.

CD10 expression in follicular lymphoma and large cell lymphoma is different from that of reactive lymph node follicles.

BACKGROUND AND OBJECTIVES: CD10 is a proteolytic enzyme expressed on the surface of germinal center cells and lymphomas derived from these cells. There is a well-known association between CD10 expression and lymphomas of follicular center cell origin. However, the reported frequency of CD10 positivity in follicular lymphomas is widely variable, and no studies have addressed the importance of assessing the intensity of CD10 expression in the diagnosis of these tumors. In this study, we utilized flow cytometry to determine differences in CD10 expression in lymphomas and follicular hyperplasias. METHODS: Cell suspensions from 61 follicular lymphomas, 43 diffuse large B-cell lymphomas, and 44 lymph nodes with follicular hyperplasia were analyzed simultaneously with phycoerythrin-labeled anti-CD20 and fluorescein isothiocyanate-labeled anti-CD10. RESULTS: CD10 expression was mainly observed on B cells and was detected in 89% of follicular lymphomas, 56% of diffuse large B-cell lymphomas, and 55% of lymph nodes showing follicular hyperplasia. In follicular hyperplasia, two subpopulations of B cells displaying dim and bright CD20 expression were recognized. CD10 expression was restricted to the bright CD20-positive cells, which accounted for an average of 16% of B cells. In CD10-positive follicular and diffuse large B-cell lymphoma cases, a significantly higher proportion of B cells (73%) coexpressed CD10. Furthermore, the intensity of CD10 expression in follicular lymphoma and diffuse large B-cell lymphoma was much higher than that of follicular hyperplasia. CONCLUSIONS: Quantitative flow cytometry can detect significant differences in CD10 expression between normal follicular cells and follicular or diffuse large B-cell lymphoma. The use of CD10 intensity of expression as well as the fraction of CD10-expressing B cells should help distinguish reactive from neoplastic B-cell processes.

Contribution of flow cytometry to the diagnosis of gastric lymphomas in endoscopic biopsy specimens.

Gastric lymphomas seem to have unique clinical, pathologic, and immunophenotypic features that set them apart from nodal lymphomas. Microscopic examination of endoscopic biopsy specimens is the most frequent procedure used to diagnose gastric tumors, but it is very difficult, and sometimes impossible, to recognize lymphomas in endoscopic samples by histologic or even immunohistologic methods. Because most gastric lymphomas are of B-cell origin, we used flow cytometry to assess B-cell clonality in gastric biopsy specimens containing dense lymphocytic infiltrates thought to represent lymphoma. We prepared viable cell suspensions from unfixed specimens obtained from 29 consecutive patients who had a previous microscopic diagnosis of suspicious gastric lymphoid infiltrates. We performed immunophenotypic studies with multicolor flow cytometry, and we assessed clonality by examination of immunoglobulin (Ig) light-chain expression analyzed exclusively on B cells identified by anti-CD20 or CD19 antibodies. The mean number of cells recovered was 1.04 x 10(6), from an average of 5.5 gastric biopsy fragments per patient. In 26 of the 29 patients, the number of cells was adequate for analysis. We detected B-cell monoclonality in 16 cases, including 5 in which the percentage of clonal B cells was less than 5%. Of the 16 cases, only 8 could be diagnosed as lymphomas on morphologic grounds alone; the remaining 8 patients had either suspicious lymphoid infiltrates or chronic gastritis. The three cases with an insufficient number of cells were considered non-neoplastic either on histologic grounds alone or in conjunction with Southern analysis of Ig genes. We conclude that flow cytometric immunophenotypic analysis of freshly prepared cell suspensions obtained from endoscopic biopsy specimens can be used to evaluate gastric lymphocytic infiltrates. Specifically, the analysis of surface Ig light-chain expression on B cells distinguishes between monoclonal (lymphoma) and polyclonal (nonlymphoma) infiltrates. The rapidity, ease, quantitative properties, and sensitivity of this technique make it a supplement to the morphologic assessment of gastric lymphoid infiltrates.

Primary gastrointestinal lymphomas in Jordan are similar to those in Western countries.

It has been a common practice to divide gastrointestinal (GI) lymphomas into Western and Middle Eastern types; the former predominantly involves the stomach, whereas the latter are mostly intestinal. Recent studies suggested that these lymphomas are biologically different from their nodal counterpart and hence should be classified separately. In this retrospective study, we examined all of the primary GI lymphomas seen at Jordan University Hospital, Amman, Jordan, between 1983 and 1992 in an attempt to reclassify and immunophenotype these cases. We studied 53 cases of primary GI lymphomas for which available material was found. Lymphomas were morphologically reclassified according to current classification schemes. Immunoperoxidase stains were performed using the streptavidin biotin method using antibodies against leukocyte common antigen, B, and T-cells. The stomach was the most common site of involvement, accounting for 62% of the cases. The male-to-female ratio was 2.8:1. The three most common histologic types were, in order, diffuse large cell lymphoma, Burkitt's lymphoma, and lymphoma of mucosa-associated lymphoid tissue. Immunoproliferative small intestinal disease was seen in only one patient. The B-cell phenotype predominated in our cases; it was seen in 85% of the gastric and 100% of the intestinal cases. Unlike other series from the region, the stomach was the most commonly involved site by GI lymphoma. Immunoproliferative small intestinal disease seems to be a rare entity in Jordan, and the majority of intestinal and gastric lymphomas were diffuse large cell lymphomas. Histopathologic evidence of mucosa-associated lymphoid tissue origin was seen in at least 18% of the gastric cases. B-cell lymphomas were by far the most common type seen, and T-cell lymphomas were rare.

Soft tissue Langerhans' cell histiocytosis in an adult. A case presentation with flow cytometric analysis and literature review.

We report a case of solitary unifocal Langerhans' cell histiocytosis presenting as a large lower extremity soft tissue mass in a 48-year-old white man. Radiologically, this was an infiltrating mass involving all compartments of the right thigh; the mass had a maximum diameter of 18 cm. Extensive evaluation revealed no evidence of involvement of any other organ. An incisional biopsy was performed under the presumptive clinical impression of sarcoma. A definitive diagnosis of Langerhans' cell histiocytosis was established on the basis of characteristic morphologic features, cell surface marker findings by immunohistochemistry and flow cytometry, and electron microscopic studies. The patient was treated with four courses of chemotherapy, (etoposide and prednisone) and had no evidence of disease 3 years after the initial presentation. A review of the literature revealed that soft tissue Langerhans' cell histiocytosis usually presents in the head and neck area of children and usually has associated bone, lung, or reticuloendothelial system involvement. To our knowledge, this is the first reported case of solitary Langerhans' cell histiocytosis in an adult in which the presentation mimicked sarcoma.