Solitary fibrous tumour--everywhere, and a diagnosis in vogue.
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Diagnosis, Differential↗
Biomedical subjects
J K Chan
Publications and source records attributed to J K Chan.
Mammary myofibroblastoma with leiomyomatous differentiation.
An unusual example of mammary myofibroblastoma in a 36-year-old woman was reported. The central portion resembled mammary myofibroblastoma or solitary fibrous tumor of other sites and showed reactivity for CD34 and muscle-specific actin, with few cells staining for desmin. The peripheral portion resembled leiomyoma and showed strong reactivity for actin and desmin but only focal staining for CD34. We interpret this tumor as mammary myofibroblastoma showing frank peripheral leiomyomatous differentiation. Although closely related to solitary fibrous tumor, mammary myofibroblastoma appears slightly different because of its consistent slant toward myoid differentiation in the form of actin and variable desmin reactivity as opposed to the rare and sparse immunohistochemical expression of myoid features by solitary fibrous tumors of other sites. This case demonstrates this phenomenon carried to an extreme, a feature not previously documented in mammary myofibroblastoma. It is of interest to note the modulation of the antigenic profile with the differentiation process, that is, loss of CD34 and gain of desmin accompanying the myoid differentiation.
Adult↗
Cytokeratin 20 immunoreactivity distinguishes Merkel cell (primary cutaneous neuroendocrine) carcinomas and salivary gland small cell carcinomas from small cell carcinomas of various sites.
Cytokeratin 20 (CK20) is a low-molecular-weight cytokeratin (CK) that shows restricted expression in the gastrointestinal epithelium, urothelium, and Merkel cell. Recent studies have suggested that since Merkel cell (primary cutaneous neuroendocrine) carcinomas are consistently CK20-positive, this feature may help to distinguish it from pulmonary small cell carcinomas. However, only limited numbers of these tumors have been studied, and the pattern of CK20 expression in other small cell carcinomas has not been established. Therefore, we studied CK20 expression in small cell carcinomas from a wide variety of sites. Immunohistochemical study was performed on paraffin sections using CK20 antibody, coupled with antigen retrieval by pressure cooking in citrate buffer. The cases included 34 Merkel cell carcinomas and 89 small cell carcinomas from various sites (pulmonary, 37; gastrointestinal tract, nine; pharynx and tongue, two; sinonasal tract, three; salivary gland, five; larynx, nine; breast, two; thymus, three; uterine cervix and corpus, 12, prostate, three; urinary bladder, two; kidney, one; pancreas, one). In addition, all cases were immunostained with pan-CK (MNF-116) and low-molecular-weight CK (CAM5.2) antibodies to ascertain their epithelial nature. With the exception of one case, all Merkel cell carcinomas were CK20-positive; and 30 of the 33 cases showed a punctate pattern. Almost 100% of tumor cells were positive, except for two cases that showed staining of 10% and 30% of tumor cells, respectively. Among the other small cell carcinomas, only five cases were CK20-positive, including one of 37 pulmonary (40% cells positive in punctate pattern), one of 11 cervical (10% cells positive), and three of five salivary gland (100% cells positive). We conclude that CK20-positivity in a small cell carcinoma of uncertain origin strongly predicts a diagnosis of Merkel cell carcinoma, especially if the majority of tumor cells are positive. A negative CK20 reaction can practically rule out Merkel cell carcinoma, provided that an effective antigen retrieval technique is used and appropriate staining is obtained with other cytokeratin antibodies. The frequent CK20 positivity observed in salivary gland small cell carcinomas in this series suggests that at least some of them may be more closely related biologically to Merkel cell carcinoma than to pulmonary-type small cell carcinoma. This may explain why they are far less clinically aggressive than other small cell carcinomas.
Biomarkers, Tumor↗
Nodular histiocytic/mesothelial hyperplasia: a lesion potentially mistaken for a neoplasm in transbronchial biopsy.
This report describes two examples of nodular histiocytic/ mesothelial hyperplasia as seen in transbronchial biopsy that initially led to serious consideration of neuroendocrine neoplasm or meningioma. The biopsies showed nodular collections of cohesive polygonal or round cells with ovoid or deeply grooved nuclei and a moderate amount of finely granular cytoplasm. Nuclear pleomorphism was mild. Immunohistochemical studies showed few cells staining for cytokeratin and the mesothelial marker HBME-1, whereas most cells were decorated by the histiocytic marker PG-M1 (CD68). This lesion appears to be identical to nodular mesothelial hyperplasia as described in hernia sacs and mesothelial/monocytic incidental cardiac excrescences, and we propose modifying the designation to "nodular histiocytic/mesothelial hyperplasia" to take into account the marked predominance of histiocytes over mesothelial cells. The clues to recognition of the true nature of the lesion are clinicopathologic correlation and identification of strips of low cuboidal (mesothelial) cells in the vicinity, and the diagnosis can be further confirmed by immunohistochemical staining. Nodular histiocytic/mesothelial hyperplasia probably results from irritation to the mesothelial lining by various causes leading to focal aggregation of histiocytes within retraction pockets or crevices of the serosal cavity.
Biopsy↗
Thymic carcinomas, but not thymomas and carcinomas of other sites, show CD5 immunoreactivity.
Thus far, there are no immunohistochemical markers that are specific for thymic epithelial neoplasms, although demonstration of immature T cells in an epithelial tumor can indirectly support a diagnosis of thymoma. In this study, the usefulness of a paraffin section-reactive CD5 antibody (clone CD5/54/B4) for supporting the thymic origin of an epithelial neoplasm was evaluated. Antigen retrieval was effected by microwaving in citrate buffer. Sixteen of 24 thymic carcinomas (67%) were immunoreactive for CD5, including nine of nine squamous cell, two of two undifferentiated, two of four lymphoepithelioma-like, and one case each of basolid carcinoma, clear cell carcinoma, and unclassified thymic carcinoma, but none of four thymic small cell carcinomas. None of 17 cases of benign thymoma and 21 cases of invasive thymoma (including six cases classifiable as well-differentiated thymic carcinoma using the Muller-Hermelink criteria) was immunoreactive for CD5, in the presence of CD5-positive lymphocytes as an internal positive control. Two of three thymic neoplasms with features borderline between thymic carcinoma and invasive thymoma were immunoreactive for CD5. In contrast, none of 61 cases of other malignant neoplasms with a tendency to involve the mediastinum was immunoreactive for CD5, including 40 nonthymic carcinomas and 13 malignant germ cell neoplasma. CD5 staining of thymic epithelial tumors correlated with the absence of tumor-associated CD99-positive thymocytes, as demonstrated in our previous studies. We conclude that CD5 is a useful marker of primary thymic carcinomas. Taken together, CD5 and CD99 (or other immature T-cell markers such as TdT and Cd1a) should be particularly useful in evaluating mediastinal and other biopsy samples of possible thymic epithelial neoplasms and in the subtyping of these tumors.
12E7 Antigen↗
Thymoma with pseudosarcomatous stroma: report of an unusual histologic variant of thymic epithelial neoplasm that may simulate carcinosarcoma.
Six cases are described of an unusual type of primary thymic epithelial neoplasm characterized by a biphasic epithelial/spindle cell morphology that closely resembled a carcinosarcoma. The patients were two women and four men 28-70 years of age. The tumors presented clinically as asymptomatic anterior mediastinal masses found incidentally on routine chest radiographs. All patients were treated by complete surgical excision. Grossly, the tumors consisted of well-circumscribed, encapsulated masses that measured 6-14 cm in greatest diameter and showed a gray-white, homogeneous, rubbery cut surface. Histologically, the lesions were composed of anastomosing islands and cords of oval to polygonal epithelial cells displaying large nuclei with occasional prominent nucleoli and rare mitotic figures, separated by areas containing a highly cellular spindle cell proliferation without nuclear atypia. Thymic remnants could be identified in the periphery of the lesions in four cases. Immunohistochemical stains showed diffuse strong positivity for keratin and focally for epithelial membrane antigen (EMA) in the epithelial cell component, and strong positivity for vimentin and focally for actin in the spindle cell stromal component. Stains for keratin, EMA, desmin, S-100 protein, and CD34 were negative in the spindle stromal cells in all cases except one, in which EMA positivity was present; CD5 stains were negative in the epithelial cells in all cases examined. Electron microscopic examination in one case showed well-formed desmosomes and tonofilaments in the epithelial elements, as well as features indicative of fibroblastic differentiation in the spindle stromal cells. Because of the unusually florid spindle cell stromal component and the focally atypical features of the epithelial cells, some of these tumors initially were misinterpreted as examples of carcinosarcoma. Clinical follow-up in five cases showed that the patients were alive and without evidence of disease over a period of 5-20 years (mean follow-up 10 years), suggesting a benign or very low grade malignant biologic behavior. The present cases appear to represent an unusual, previously undescribed morphologic variant of thymoma characterized by a prominent pseudosarcomatous stromal component. Because of the distinctive histologic appearance and indolent clinical behavior, these lesions should be distinguished from other more aggressive anterior mediastinal neoplasms displaying a biphasic morphology.
Adult↗
Adult-to-adult living donor liver transplantation using extended right lobe grafts.
OBJECTIVE: The authors report their experience with living donor liver transplantation (LDLT) using extended right lobe grafts for adult patients under high-urgency situations. SUMMARY BACKGROUND DATA: The efficacy of LDLT in the treatment of children has been established. The major limitation of adult-to-adult LDLT is the adequacy of the graft size. A left lobe graft from a relatively small volunteer donor will not meet the metabolic demand of a larger recipient. METHODS: From May 1996 to November 1996, seven LDLTs, using extended right lobe grafts, were performed under high-urgency situations. All recipients were in intensive care units before transplantation with five having acute renal failure, three on mechanical ventilation, and all with hepatic encephalopathy. The median body weight for the donors and recipients was 58 kg (range, 41-84 kg) and 65 kg (range, 53-90 kg), respectively. The body weights of four donors were less than those of the corresponding recipients, and the lowest donor-to-recipient body weight ratio was 0.62:1. The extended right lobe graft was chosen because the left lobe volume was <40% of the ideal liver mass of the recipient. RESULTS: Median blood loss for the donors was 900 mL (range, 700-1600 mL) and hospital stay was 19 days (range, 8-22 days). Homologous blood transfusion was not required. Two donors had complications (one incisional hernia and one bile duct stricture) requiring reoperation after discharge. All were well with normal liver function 5 to 10 months after surgery. The graft weight ranged from 490 g to 1140 g. All grafts showed immediate function with normalization of prothrombin time and recovery of conscious state of the recipients. There was no vascular complication, but six recipients required reoperation. One recipient died of systemic candidiasis 16 days after transplantation and 6 (86%) were alive with the original graft at a median follow-up of 6.5 months (range, 5-10 months). CONCLUSIONS: When performed by a team with experience in hepatectomy and transplantation, LDLT, using an extended right lobe graft, can achieve superior results. The technique extends the success of LDLT from pediatric recipients to adult recipients and opens a new donor pool for adults to receive a timely graft of adequate function.
Acute Kidney Injury↗
Non-invasive ventilation in the management of respiratory failure.
Non-invasive positive pressure ventilation (NIPPV) has been used increasingly to treat various forms of respiratory failure, with benefits in terms of gas exchange improvement, avoidance of endotracheal intubation and a decreased mortality. This review will focus on the recent developments and recommendations in the use of NIPPV in the treatment of acute and chronic respiratory failure, the methodology in the application of NIPPV, and briefly on its proposed mechanism of action.
Acute Disease↗
Prevalence of asthma and allergy in Hong Kong schoolchildren: an ISAAC study.
Asthma and allergic disease in children is increasing in many Western countries but such trend has not been well-defined in Chinese populations. This paper aims to determine the prevalence of asthma and allergic disease in Hong Kong schoolchildren and compare it with previous data to identify a changing trend. We studied 4,665 schoolchildren aged 13-14 yrs using the International Study of Asthma and Allergy in Childhood (ISAAC) protocol to determine prevalence rates for asthma, wheeze, respiratory symptoms, rhinitis and eczema in 1994-1995. Additional questions on education levels of the parents and smoking status were also asked. Concordance between responses to the written and video questionnaires was good (76% for wheeze ever, 80% for current wheeze). Prevalence rates for asthma ever, wheeze ever, and current wheeze were 11, 20 and 12%, respectively, and were greater in boys (p < 0.05). Rhinitis affected slightly over half of the subjects (52%), and eczema was reported by a sixth (15%), whilst current rhinitis and current eczema were present in 44% and 3.6% of children, respectively. In multiple logistic regression: odds ratio male sex (OR) 1.47; (95% confidence interval (95% CI) 1.15-1.86); current rhinitis (OR 3.00; 95% CI 2.36-3.81); current eczema (OR 2.34; 95% CI 1.40-3.93); and active smoking (OR 2.00; 95% CI 1.38-2.89) were associated with current wheeze; whilst severe wheezing attack was associated with: current rhinitis (OR 2.72; 95% CI 1.47-5.02); current eczema (OR 6.13; 95% CI 2.82-13.33); and active smoking (OR 4.62; 95% CI 2.43-8.76). Age, parental education and passive smoking were not important factors. When compared to previous epidemiological data obtained in 1992, the prevalence rates for asthma ever and wheeze ever had increased by 71 and 255%, respectively, in Hong Kong schoolchildren. The severity of asthma and respiratory symptoms showed a similar increasing trend. Further studies should aim to identify the role of the environment in the pathogenesis of asthma.
Adolescent↗
Fractures attributable to osteoporosis: report from the National Osteoporosis Foundation.
To assess the cost-effectiveness of interventions to prevent osteoporosis, it is necessary to estimate total health care expenditures for the treatment of osteoporotic fractures. Resources utilized for the treatment of many diseases can be estimated from secondary databases using relevant diagnosis codes, but such codes do not indicate which fractures are osteoporotic in nature. Therefore, a panel of experts was convened to make judgments about the probabilities that fractures of different types might be related to osteoporosis according to patient age, gender, and race. A three-round Delphi process was applied to estimate the proportion of fractures related to osteoporosis (i.e., the osteoporosis attribution probabilities) in 72 categories comprised of four specific fracture types (hip, spine, forearm, all other sites combined) stratified by three age groups (45-64 years, 65-84 years, 85 years and older), three racial groups (white, black, all others), and both genders (female, male). It was estimated that at least 90% of all hip and spine fractures among elderly white women should be attributed to osteoporosis. Much smaller proportions of the other fractures were attributed to osteoporosis. Regardless of fracture type, attribution probabilities were less for men than women and generally less for non-whites than whites. These probabilities will be used to estimate the total direct medical costs associated with osteoporosis-related fractures in the United States.
Aged↗
Medical expenditures for the treatment of osteoporotic fractures in the United States in 1995: report from the National Osteoporosis Foundation.
Osteoporotic fractures are a significant public health problem, resulting in substantial morbidity and mortality. Previous estimates of the economic burden of osteoporosis, however, have not fully accounted for the costs associated with treatment of nonhip fractures, minority populations, or men. Accordingly, the 1995 total direct medical expenditures for the treatment of osteoporotic fractures were estimated for all persons aged 45 years or older in the United States by age group, sex, race, type of fracture, and site of service (inpatient hospital, nursing home, and outpatient). Osteoporosis attribution probabilities were used to estimate the proportion of health service utilization and expenditures for fractures that resulted from osteoporosis. Health care expenditures attributable to osteoporotic fractures in 1995 were estimated at $13.8 billion, of which $10.3 billion (75.1%) was for the treatment of white women, $2.5 billion (18.4%) for white men, $0.7 billion (5.3%) for nonwhite women, and $0.2 billion (1.3%) for nonwhite men. Although the majority of U.S. health care expenditures for the treatment of osteoporotic fractures were for white women, one-fourth of the total was borne by other population subgroups. By site-of-service, $8.6 billion (62.4%) was spent for inpatient care, $3.9 billion (28.2%) for nursing home care, and $1.3 billion (9.4%) for outpatient services. Importantly, fractures at skeletal sites other than the hip accounted for 36.9% of the total attributed health care expenditures nationally. The contribution of nonhip fractures to the substantial morbidity and expenditures associated with osteoporosis has been underestimated by previous researchers.
Aged↗
Lipoadenoma of the parotid gland with probable striated duct differentiation.
We report an example of lipoadenoma of the parotid gland, a previously undescribed tumor of the salivary gland. It is a well-circumscribed tumor comprising an intimate admixture of mature fat cells and branching narrow tubules. The tubules were elongated and had small or absent lumina, resulting in a remarkable Sertoliform pattern. They were lined by columnar cells, which were supported by a basal cell layer. The latter cells lacked evidence of myoepithelial differentiation but were best highlighted by immunostaining for high molecular-weight cytokeratin (34 beta E12). Comparison with the normal salivary gland components showed the greatest homology of the neoplastic tubules with the striated duct, which normally has an incomplete basal cell layer. The latter phenomenon is under-recognized, with some current textbooks still describing the presence of a single cell type only in the striated duct of the salivary gland.
Adenoma↗
Comparison of the ISAAC video questionnaire (AVQ3.0) with the ISAAC written questionnaire for estimating asthma associated with bronchial hyperreactivity.
BACKGROUND: A standardized protocol is essential for international comparisons of asthma prevalence and severity. The International Study of Asthma and Allergies in Childhood (ISAAC) used a standardized written questionnaire (WQ) and a video questionnaire (AVQ3.0) to survey the prevalence and severity of asthma in 13-14-year-old schoolchildren in different countries. OBJECTIVE: To compare the effectiveness of WQ and AVQ3.0 in predicting bronchial hyperresponsiveness (BHR), defined as having a provocation dose of inhaled methacholine causing a 20% fall in baseline FEV1 of 7.8 mumol. METHODS: One hundred and eighty-nine Chinese schoolchildren completed a written questionnaire followed by a video questionnaire on asthma symptoms. They then underwent bronchial challenge to methacholine. RESULTS: Fair correlations were seen between the first two corresponding questions (moderate wheezing at rest and exercise wheeze) in the two questionnaires with Kapper indices of 0.44 and 0.43, respectively. The ability to predict BHR, as indicated by the Youden's index, was similar between the corresponding questions of the two questionnaires, except for 'severe wheeze' which had a significantly higher Youden's index in AVQ3.0 (0.44) than the corresponding question in WQ (0.11, P < 0.05). CONCLUSION: The ISAAC International video questionnaire is at least as effective as the ISAAC written questionnaire in predicting BHR. It therefore provides a simple and valid tool for international comparisons of asthma prevalence and severity.
Adolescent↗
Minimum graft volume for successful adult-to-adult living donor liver transplantation for fulminant hepatic failure.
The major limitation of adult-to-adult living donor liver transplantation is the adequacy of the size of the graft that can be safely harvested from the donor. The present report describes a 22-year-old woman with stage 4 hepatic coma due to fulminant hepatic failure who was successfully treated using a small-for-size left lobe graft from her father. The graft weight was 0.6% of the recipient's body weight, or 25% of her ideal liver weight. Avoidance of warm ischemia, short cold ischemic time, and early treatment of rejection are important elements in optimizing small-for-size graft function. Since the left lobe represents 23-36% of the total liver volume of an adult, it is possible, in most cases, to harvest a left lobe graft of adequate size from a donor of similar size as the recipient.
Adult↗
Aggressive T/natural killer cell lymphoma presenting as testicular tumor.
BACKGROUND: Nonnasal CD56+ T/natural killer (NK) cell lymphomas with morphologic and immunogenetic features similar to those of the distinctive nasal T/NK cell lymphoma are uncommon and have been characterized only recently. They show predominantly extranodal presentation, high stage disease, a highly aggressive course, strong association with Epstein-Barr virus (EBV), and lack of T cell receptor gene rearrangement. Only one previously reported case had a testicular presentation, although the testis is not uncommonly involved during the course of disease in both nasal and nonnasal T/NK cell lymphomas. METHODS: Three patients with T/NK cell lymphoma who presented initially with a testicular mass are reported. RESULTS: The three patients underwent orchidectomy for testicular tumor. Histologically, the testes showed diffuse dense infiltration of medium-sized or large lymphoma cells. Antiocentric growth and necrosis were prominent in two cases. The lymphoma had the following immunophenotype: CD2+ CD3 epsilon+ CD56+ compatible with T/NK cell lymphoma; two lacked staining with Leu4 (CD3), and one had weak staining. With immunohistochemical preparations, it was noted that the rete testis stained consistently for CD56, and the Leydig cells and Sertoli cells showed patchy staining. The neoplastic cells harbored EBV, as demonstrated by in-situ hybridization. Additional sites of disease were detected at the time of the diagnosis in one patient (nose) or appeared soon afterwards in all three patients (skin or gastrointestinal tract). All three patients died within 5 months. CONCLUSIONS: This study confirms that testicular CD56+ T/NK cell lymphoma tends to disseminate early, pursues an aggressive course, and is strongly associated with EBV. CD56 recognizes the neural cell adhesion molecule (NCAM), which exhibits homophilic binding properties. The expression of CD56 in the normal testicular constituents can perhaps explain the tendency for T/NK cell lymphoma to localize in this organ.
Aged↗
Clarification of CD3 immunoreactivity in nasal T/natural killer cell lymphomas: the neoplastic cells are often CD3 epsilon+.
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Animals↗
Pituitary adenoma presenting as sinonasal tumor: pitfalls in diagnosis.
Pituitary adenomas may cause significant difficulties in histological diagnosis when presenting in unusual sites either as extension from an intrasellar lesion or as ectopic tumor. Three such cases, involving the sinonasal tract are described herein, and the differential diagnoses are discussed. Two of them were invasive intrasellar macroadenomas that presented as unilateral nasal polyp, and one was an ectopic pituitary adenoma involving the sphenoid sinus. There was notable cellular atypia in two cases, with nuclear pleomorphism, giant cells, chromatin clumping, and distinct nucleoli, leading to serious consideration of the possibility of poorly differentiated carcinoma. In none of the cases was the diagnosis of pituitary adenoma suspected clinically. The clues to diagnosis were an endocrine growth pattern comprising tumor cells arranged in packets, ribbons, or rosettes, with prominent delicate vascularized stroma; a high index of suspicion; and immunohistochemical showing of neuroendocrine markers and pituitary hormones in the tumor cells. A correct diagnosis is important because in contrast to neuroendocrine carcinoma as a whole or to poorly differentiated carcinoma, pituitary neoplasms have a much more favorable prognosis and a low metastatic potential.
Adenoma↗
A study of the possible etiologic association of Epstein-Barr virus with reactive hemophagocytic syndrome in Hong Kong Chinese.
The herpes group of viruses, particularly Epstein-Barr virus (EBV), has frequently been implicated in the causation of reactive hemophagocytic syndrome (RHS) in the Western populations. EBV has also been implicated in the rare fulminant form of RHS occurring in Oriental children. However, our previous adult-predominant study indicated little clinical and serological evidence of EBV infection in patients with RHS in Hong Kong. In the present study, we further examined this issue using a more sensitive and specific technique for the demonstration of EBV, ie, in situ hybridization for EBV encoded RNA (EBER). The 43 Chinese patients studied were mostly adults with a mean age of 44 years, and a male to female ratio of 1.5:1. About two-thirds (28) of patients had associated malignant lymphoma at the time of diagnosis. Five patients had documented infection (typhoid fever 2; systemic candidiasis 1; adenovirus pneumonia 1; viral encephalitis 1), and two had systemic lupus erythematosus. EBER signals were detected in only 11 cases (25.6%). All positive cases were associated with malignant lymphoma, and the positive signals were exclusively localized to the lymphoma cells but not in the histiocytes. On comparing the results (11 of 28 cases positive; 39.3%) with our previous data on EBER-expression in malignant lymphomas in Hong Kong, no significant difference is observed in the frequency of EBV-positivity between the two groups of lymphomas. Thus, a definite pathogenetic link between EBV and lymphoma-associated RHS cannot be established. However, the overrepresentation of T and T/NK lineage lymphoma in this sample of lymphoma-associated with RHS (61%) versus nonselected cases of lymphomas (31%) suggests that it is the T and T/NK cell origin of the lymphoma rather than the EBV positivity that predisposes to RHS. Notwithstanding the previous findings, EBER in situ hybridization may still serve as a useful adjunct in the investigation of patients with RHS, because the presence of EBER-positive cells should raise a strong suspicion of an underlying malignant lymphoma.
Adolescent↗