Multicentric reticulohistiocytosis. A case report.

A patient with multicentric reticulohistiocytosis is reported, and the clinical and pathological features of the condition are discussed.     

Multicentric reticulohistiocytosis: a proliferation of macrophages with tropism for skin and joints, part II

In this second part of the review of multicentric reticulohistiocytosis, the authors discuss its association with other diseases, in particular, cancer, and laboratory and therapeutic aspects of this incapacitating and disfiguring disease. Histopathologic aspects are characteristic: dense mononuclear infiltrate with typical multinucleated cells that contain periodic acid-Schiff-positive and diastasis-resistant material, conferring a "ground glass" aspect when stained with hematoxylineosin.     

Multicentric reticulohistiocytosis: a proliferation of macrophages with tropism for skin and joints, part I

The authors present part I of a review of multicentric reticulohistiocytosis, a rare systemic proliferative disease of histiocytes of unknown cause. It is clinically characterized by cutaneous and mucosal nodules and by osteoarticular lesions. The disease occurs in outbreaks that progress in severity, with spontaneous regression, but usually leaving incapacitating arthritis and disfiguring facial lesions. The authors discuss the historical, epidemiologic, and clinical aspects of this disease.     

Langerhans cell granulomatosis manifested as pigmented villonodular synovitis

We report an unusual case of Langerhans cell granulomatosis (LCG) manifested as a villous synovial proliferation in a 38-year-old female jogger. One year after the onset of joint symptoms, she had a classical LCG presentation with skin and visceral lymph node involvement. Review of the literature revealed only one case of synovial shoulder joint tenosynovitis associated with LCG in a middle-aged woman. Ours is the first reported case presenting clinically in the synovium of the hip joint as pigmented villonodular synovitis. Histiocytic/dendritic proliferations involving the synovial tissues are not uncommon. These lesions as well as the rare multicentric reticulohistiocytosis (MRH), a systemic monocytoid/histiocytic disorder with multinucleated giant cells, polyarthritis, and papulonodular skin lesions, should be considered in the differential diagnosis. Clinical and pathologic features will distinguish LCG from MRH.     

The value of blood transfusion in tumor surgery. Experiences from randomized trials

Since the first publication by Tartter in 1982 [3] multiple studies were performed concerning the association between blood transfusion and outcome in surgical oncology. The hypothesis was that blood transfusion-associated immunosuppression influences postoperative infection rates and long-term prognosis. Due to the fact that most of these studies were uncontrolled observational the decisive question about the causal relationship could not be answered so long. In randomized studies different methodological approaches were performed. Jeekel from Rotterdam and our group tried to control the allogeneic transfusion-associated immunosuppression by use of autologous transfusions. We found in our monocentric study with a very homogenous study population a significantly reduced infection rate in the autologous blood group which was not observed in the multicentric Dutch trial. The second approach was chosen by Jensen from Denmark and van de Velde from the Netherlands. They used leucocyte-depletion to create a less immunosuppressive blood product, which was then randomized and compared with conventional allogeneic transfusions. Whereas the monocentric Dutch study showed a significant effect in the pilot study and also in the later larger confirmational study, again this was not found in the multicentric Dutch trial. Concerning the association between prognosis and transfusion the results of two randomized studies are available. The multicentric study from Jeekel from Rotterdam and our monocentric study found also in this endpoint different results. Whereas in our study allogeneic transfusions were an independent risk factor demonstrated in the intention-to-treat comparison by a clear trend (p = 0.067), this could not be detected in the multicentric Dutch study. In conclusion, the results from actually available randomized studies are not compulsive. Whereas the homogeneous monocentric studies showed a significant transfusion effect for the postoperative infection rate and also for long-term prognosis, this could not be verified in two multicentric trials. Also the cumulative meta-analyses are not able to answer the decisive question concerning the causal relationship between transfusion and long-term prognosis. Therefore new and innovative study designs are mandatory.     

Tumormarker beim Blasenkarzinom

INTRODUCTION: The number of noninvasive diagnostic tests for bladder cancer has increased tremendously over the last years with a large number of experimental and commercial tests. Comparative analyses of tests for diagnosis, follow-up, and recurrence detection of bladder cancer were performed retrospectively as well as prospectively, unicentrically, and multicentrically. METHODS: An analysis of multicentric studies with large patient numbers compared with our own Kiel Tumor Bank data is presented. The Kiel Tumor Bank data looked prospectively at 106 consecutive bladder tumor patients from the year 2006. Special focus was put on urine cytology as a reference test, as well as the commercial NMP 22 Bladder Chek. RESULTS: The analysis of the NMP 22 Bladder Chek showed an overall sensitivity of 69% for all tumor grades and stages, with a specificity of 76%. Comparison to multicentric data with an overall sensitivity of 75% for all tumor grades and stages, with a specificity of 73%, showed results similar to those in the literature. Urine cytology showed a comparable overall sensitivity of 73% for all tumor grades and stages, with a specificity of 80%. CONCLUSIONS: A large number of noninvasive tests for bladder cancer follow-up with reasonable sensitivity and specificity can currently be used. Because of limited numbers of prospective randomized multicentric studies, no single particular marker for bladder cancer screening can be recommended at this point in time.     

Congenital Self-Healing Reticulohistiocytosis

Congenital self-healing reticulohistiocytosis, also known as congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease, is a Langerhans cell histiocytosis. It is characterized by skin lesions in the newborn period in an otherwise healthy infant that show a Langerhans cell infiltrate in the skin on histological analysis. These findings subsequently spontaneously involute. This report describes two newborns who presented at birth with differing presentations of congenital self-healing reticulohistiocytosis. A review of the disorder, including diagnosis and evaluation, is presented.     

The case against tylectomy for carcinoma of the breast. The factor of multicentricity.

Five hundred consecutive cases of breast carcinoma were studied to determine the incidence of multicentric lesions in the resected specimens. When residual tumor in juxtaposition to the primary tumor or biopsy cavity is excluded, 41.6 per cent of specimens exhibited multicentric foci of tumor; 31 per cent of such foci were in sectors or quadrants remote from the primary tumor. In more than half of these cases the lymph nodes were uninvolved and cure rate would have been maximal had these multicentric tumor foci been removed. These findings confirm previous similar studies and we consider tylectomy an inappropriate mode of therapy for breast cancer.     

Multifocal and Multicentric Breast Cancer is Associated with Increased Local Recurrence Regardless of Surgery Type

Multifocal and multicentric breast cancers have been correlated with poor prognostic factors and worse outcomes versus unifocal disease. We evaluated the impact of multifocal and multicentric disease versus case controls with unifocal disease, matching for age, grade, T‐, and N‐stage. A total of 110 patients with multifocal (n = 93) or multicentric (n = 17) disease and 263 matched case controls were identified with a median follow‐up of 53 months and 64 months, respectively. The actuarial local control rates for the multifocal/multicentric and unifocal group were 88% and 97%, respectively at both 5 and 10 years (p < 0.001). On multivariate analysis, multifocal/multicentric disease remained associated with higher local recurrence after controlling for other covariates including surgery type. The disease‐free survival rates in the multifocal/multicentric group at 5 and 10 years were 75% and 71%, respectively, versus 87% and 78% at 10 years (p = 0.01). On multivariate analysis, multifocal/multicentric disease was no longer associated with worse disease‐free survival. There was no difference in the cohorts in terms of regional control, overall survival, or cancer specific survival. Our findings suggest that multifocal/multicentric disease may be associated with worse outcomes versus unifocal disease regardless of type of surgery. This suggests a more biologically aggressive cancer and may be an important consideration when managing these patients. Further studies are needed to better understand the impact of multifocal/multicentric breast cancers on outcomes.     

An autopsy case of multicentric glioma of multiple histopathology

An autopsy case is described of an 66-year-old man with multicentric glioma of multiple histopathology, i.e. protoplasmic astrocytoma and glioblastoma. Enhanced CT scan revealed three separate lesions in the right cerebral hemisphere, pons, and cerebellar vermis. Initial diagnosis by CT included metastatic and primary brain tumor, multiple abscess, fungal infection, parasites, tuberculoma, and so on. Biopsy of the right frontal mass revealed astrocytoma grade-2. An autopsy revealed gelatinous, clear marginal mass in the right frontal, parietooccipital and cerebellar vermis; an opaque marginal mass with necrosis in dorsal pons was found. At microscopic examination, the right frontal tumor exhibited continuity with both the paraventricular and the right parietooccipital tumor. The right cerebral hemisphere and cerebellar vermis tumors showed protoplasmic astrocytoma; the dorsal pons tumor showed glioblastoma. CSF examination revealed no tumor cells. Tumor invasion of the internal capsule and the meninges was also not found. Accordingly, we diagnosed as multicentric astrocytoma of multiple histopathology. Only 11 case reports of multicentric glioma were recorded in Japan; only one of which was of multiple histopathology. Worldwide, only 7 case reports of multicentric glioma of multiple histopathology were recorded; this is the first case of protoplasmic astrocytoma and glioblastoma. Seen in terms of pathogenesis of multicentric glioma, this case is thought to be very interesting.     

Eight multicentric hepatocellular carcinomas occurring in the same segment of the liver

We present a rare case of eight multicentric hepatocellular carcinomas (HCCs) occurring in the same segment of the liver. In a 66-year-old Japanese man, multiple liver tumors were detected during follow-up of chronic hepatitis C infection, and he was admitted to our hospital in 1995. Ultrasonography (US) showed eight tumors, each measuring between 10 and 15 mm in diameter, in the right lobe, and a 10-mm tumor in the left lobe. Angio-ultrasonography (US) showed no enhancement of the tumors, and multicentric occurrence was suspected. Portal angio-US showed eight tumors in the right lobe located in the anterior segment. Accordingly, anterior segmentectomy and partial resection of the S3 subsegment were performed, in December, 1995. On histological examination, all eight tumors in the anterior segment and the tumor in the S3 subsegment were well differentiated HCC. The liver parenchyma showed cirrhosis. The grade and stage of hepatitis did not differ between the anterior segment and the S3 subsegment, but irregular regeneration of hepatocytes was more prominent in the anterior segment. The multicentric occurrence of HCCs in the anterior segment may be related to the more severe damage caused by chronic hepatitis in the anterior segment than in the left lobe of the liver. Received: November 13, 2000 / Accepted: April 16, 2001     

Sacral radiculopathy secondary to multicentric osteosarcoma

STUDY DESIGN: A case of multicentric osteosarcoma presenting with sacral radiculopathy is reported. OBJECTIVE: To present unusual clinical and radiologic findings of multicentric osteosarcoma. SUMMARY OF BACKGROUND DATA: Multicentric osteosarcoma is a rare variant of osteosarcoma. To the authors' knowledge, no cases of multicentric osteosarcoma presenting as sacral radiculopathy have been reported previously. METHODS: A 14-year-old boy had a large sacral tumor extending into the spinal canal, which was found to account for the initial symptoms, which mimicked those of herniated nucleus pulposus. At diagnosis, a bone survey showed multiple foci of osteosarcoma in the long bones. RESULTS: The patient was treated with chemotherapy, but died of the disease 8 months after the initial presentation. CONCLUSION: Multicentric osteosarcoma should be considered in the differential diagnosis for a pediatric patient with low back pain and sciatica.     

Significant influence of accompanying chronic hepatitis status on recurrence of hepatocellular carcinoma after hepatectomy. Result of multivariate analysis.

OBJECTIVE: The aim of this study was to evaluate the correlation between the histologic status of accompanying chronic hepatitis and the recurrence rate of hepatocellular carcinoma (HCC) after hepatectomy by multivariate analysis. SUMMARY BACKGROUND DATA: Recent studies have suggested that a considerable number of intrahepatic recurrence of HCC after hepatectomy might be the results of metachronous multicentric hepatocarcinogenesis. The authors hypothesized that the incidence of recurrence due to metachronous multicentric hepatocarcinogenesis would depend on the histologic status of accompanying chronic viral liver disease, which is a main promoter of HCC. METHODS: One hundred ten patients with HCC who underwent curative resection were studied. Histologic status of accompanying chronic hepatitis was classified into the three categories: 1) normal liver or chronic persistent hepatitis (CPH, n = 13), 2) chronic aggressive hepatitis (CAH, n = 50), and 3) liver cirrhosis (LC, n = 47). RESULTS: The Cox multivariate proportional hazard model showed that the accompanying chronic viral hepatitis status (p = 0.0133), extent of hepatectomy (p = 0.0078), and number of tumors (p = 0.0475) were significantly predictive variables for recurrence-free survival. By the log-rank test, recurrence-free survival rate in patients with CPH was significantly higher than those in patients with CAH (p = 0.0005) and LC (p = 0.0075). Patients with CAH had the lowest recurrence-free survival rate (vs. LC, p = 0.028). CONCLUSIONS: The results of this study indicated the significant influence of histologic activity of hepatitis on recurrence of HCC. This might support the concept of significant contribution of multicentric hepatocarcinogenesis to recurrence of HCC after hepatectomy.     

Specific Gene-Expression Profiles of Noncancerous Liver Tissue Predict the Risk for Multicentric Occurrence of Hepatocellular Carcinoma in Hepatitis C Virus–Positive Patients

Background Hepatitis C virus (HCV) infection produces chronic hepatitis, cirrhosis, and, ultimately, hepatocellular carcinoma (HCC). A molecular analysis of the damaged liver tissues infected with HCV may identify specific gene-expression profiles associated with a risk for liver carcinogenesis. Methods Forty patients with HCV-positive HCC were classified into two groups: single nodular HCC group (n = 28) and multicentric HCC group (n = 12). Using a complementary DNA microarray, we compared the gene-expression patterns of the noncancerous liver tissue specimens between the two groups. We also identified the differentially expressed genes related to multicentric recurrence in the liver remnant. We then evaluated whether a specific gene-expression profile can accurately estimate the risk for multicentric hepatocarcinogenesis. Results We selected the 230 differentially expressed genes in the multicentric HCC group. A hierarchical clustering analysis identified a cluster that might be closely associated with the multicentric occurrence of HCC. On the basis of the gene-expression profiling of the 36 genes commonly associated with both multicentric HCC and multicentric recurrence, we created a scoring system to estimate the risk for multicentric hepatocarcinogenesis. The prediction score of patients in the multicentric HCC group with multicentric recurrence (19.9 ± 9.2) was significantly higher (P < .05) than that in the single nodular HCC group without multicentric recurrence (−1.8 ± 12.7). Conclusions Specific gene-expression signatures in noncancerous liver tissue may help to accurately predict the risk for developing HCC. M. Okamoto and T. Utsunomiya contributed equally to this study.     

Epithelioid hemangioendothelioma of bone. A clinicopathologic, ultrastructural, and immunohistochemical study

In a review of 29 cases of solitary and multicentric hemangioendothelial sarcomas of bone, 14 were found to have histologic features of epithelioid hemangioendotheliomas. These were characterized by the presence of epithelioid or "histiocytoid" endothelial cells that were either round or spindle-shaped. Intracytoplasmic vacuolization was noted, and some showed nesting of cells, mimicking metastatic adenocarcinoma. The presence of a myxoid matrix suggested the diagnosis of chondrosarcoma in some cases. Some of the tumors strongly resembled the lesions formerly designated as intravascular bronchioloalveolar tumor, angioglomoid tumor of bone, or malignant myxoid angioblastoma of bone. Factor VIII-related antigen was detected in five of the six cases examined, and ultrastructural study in five cases confirmed the endothelial nature of the tumor cells; Weibel-Palade bodies were present in three cases. There were 11 men and three women, and the tumors were seen predominantly in patients who were under 30 years of age (10 cases). Four cases involved solitary tumors, and nine were multicentric in bone. The multicentric tumors had a predilection for the bones of one lower extremity (five cases). The total series had a protracted clinical course, and the multicentric tumors appeared to follow a less aggressive course.     

Neoadjuvant chemotherapy for the treatment of osteosarcoma of the extremities: a comparison of results obtained in single-institution and multicenter trials

Osteosarcoma is a rare tumor. In multicentric trials on the neoadjuvant treatment of this neoplasm many of the single institutions involved have the opportunity to treat a small number of cases (the average for all of the studies was less than 1 case per year). In order to verify whether this can change the percentage of amputations avoided and prognosis, a review of the current literature was carried out, concerning neoadjuvant treatment of osteosarcoma of the extremities. The results obtained in 9 multicentric trials and in 12 mono-institutional trials were evaluated. It was observed that in mono-institutional studies the percentage of patients treated by conservative surgery instead of aggressive surgery (73% vs 55%) and the disease-free survival 5 years after surgery (73% vs 55%; p < 0.0001) are significantly higher as compared to what was observed for patients treated in multicentric trials. Based on these differences, the authors conclude that it would be opportune to direct patients with osteosarcoma of the extremities to the best centers that each year treat large numbers of patients with musculoskeletal tumors.     

Management and outcome of high-grade multicentric gliomas: a contemporary single-institution series and review of the literature

Background

Multicentric malignant gliomas are well-separated tumours in different lobes or hemispheres, without anatomical continuity between lesions. The purpose of this study was to explore the clinical features, the pathology and the outcome according to the management strategies in a consecutive series of patients treated at a single institution. In addition, an analysis of the existing literature is presented.

Methods

For the institutional analysis, a retrospective review of all patients who underwent treatment for multicentric gliomas in the last 7 years was performed. For the analysis of the literature, a MEDLINE search with no date limitations was accomplished for surgical treatment of multicentric malignant gliomas.

Results

Two hundred and thirty-nine patients with glioma were treated in our department. Eighteen patients (7.5 %) with a mean age of 64 years (age range, 37–78 years) presented multicentric malignant gliomas. Thirteen patients (72 %) underwent surgical resection of at least one lesion that was followed by adjuvant treatment in all but one case. Five patients (28 %) underwent stereotactic biopsy and thereafter received chemotherapy. A survival advantage was associated with resection of at least one lesion followed by adjuvant treatment (median overall survival 12 months) compared with 4 months for stereotactic biopsy followed by chemotherapy. Similar results were obtained from the review of the literature.

Conclusions

Resection of at least one lesion seems to play a significant role in the management of selected patients with multicentric malignant gliomas. Multi-institutional studies on larger series are warranted to define how aggressively the patients with malignant multicentric gliomas should be treated.     

High Recurrent Rate of Multicentric Papillary Thyroid Carcinoma

Background  Multicentric papillary thyroid carcinoma (PTC) is not unusual in patients with PTC. However, its clinical features concerning cancer recurrence and mortality are not well described. Methods  A total of 1682 PTC patients at a single institution who underwent total thyroidectomy were retrospectively reviewed; the mean follow-up period was 7.7 ± 0.1 years. Postoperative radioactive iodide ablation for thyroid remnant was performed after surgery for most patients. Results  Of all the PTC cases reviewed, 337 cases (20.0%) were categorized as multicentric PTC. Compared with patients with unifocal PTC, multicentric PTC patients demonstrated older age, advanced TNM staging, and higher recurrence. A higher recurrence rate for multicentric PTC (20.2%) was observed compared with that for unifocal PTC; 45.8% of multicentric PTC cases with ≥ 5 foci experienced cancer recurrence. Mean tumor size of the largest nodule in patients with multicentric PTC was significantly smaller than that found in unifocal PTC. Patients with multicentric papillary microcarcinoma (≤1 cm) had higher recurrence rate and cancer mortality than those with unifocal papillary microcarcinoma. Of the recurrent multicentric PTC cases, 52.9% were persistent or diagnosed within the first year of thyroidectomy and had a cancer-related mortality of 27.8%. The 5-, 10-, and 20-year survival rates of multicentric PTC patients were 97.7%, 94.4%, and 84.7%, respectively, which were not statistically different from those of unifocal PTC patients. Conclusions  Multicentric PTC warrant postoperative adjuvant therapy and close surveillance within the first year. Patients with multicentric papillary thyroid microcarcinoma need to be treated as high-risk patients.     

Abnormal glomerular basement membrane in idiopathic multicentric osteolysis

The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of end-stage renal failure. We believe that this biopsy finding represents or is related to the unknown primary lesion causing nephropathy in idiopathic multicentric osteolysis.     

纵隔巨大淋巴结增生症的诊断及外科治疗

目的：总结纵巨大淋巴增生症（giant lymph node hyperplasia）诊断和外科治疗的经验。方法：６例纵隔巨大淋巴结增生症病人，男女各３例，按Frizzera分类标准分为局灶性，多中心性ＨＶ型各１例、多中心性Ｍix型３例，ＰＣ型１例，均行手术治疗.结果：除１例多中心性Ｍix型病人术后４年复发，再接受第２次后术治疗，余５例病人均长期生存。结论：该症只要无系统功能严重受累，应积极考虑手术治疗，放射治疗效果不明显，但对多中心性或术中无法完全切除者，术后可考虎辅助放射治疗。