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[Preliminary evaluation of a method for detection of individual malignant melanoma cells in peripheral blood while monitoring the course of the malignancy and its treatment].

Using reverse transcription and polymerase chain reaction (PCR) with primers specific for tyrosinase the individual melanoma cells were detected in peripheral blood of patients in different stages of disease, after excision of primary lesion and prior and after chemotherapy. No relation between stage of disease (including situations with overt generalized spread of melanoma) and probability of positive PCR reaction detecting transcript for tyrosinase gene was found. Many patients in III and IV stages were negative for prolonged periods. Therefore, this method cannot be used for monitoring of all patients, because many of them are negative prior as well as after chemotherapy. With regard to the effects of therapy, the patients differed one to another and although some persons positive prior treatment became negative thereafter, a similar number of initially negative patients became positive after treatment.

Adult↗

Review of a home-based palliative care program for children with malignant and non-malignant diseases.

This retrospective chart review presents the patient characteristics and utilization of the home-based palliative care program at The Hospital for Sick Children in Toronto. A total of 126 children dying from a broad spectrum of diseases was admitted during the period 1986-1994, referred from neurosurgery, genetic/metabolic, neurology, neonatology, nephrology, cardiology, general pediatrics, general surgery, and pulmonology. At the time of review, 15 patients remained alive and 18 had been discharged from the program. Mean age at the time of referral was 4.8 +/- 0.51 years and mean age at death was 5.3 +/- 0.55 years. The mean number of days in hospital was 26.5 +/- 14.6 while days spent at home averaged 98.4 +/- 15.2; thus 80% of the children's remaining time was spent at home. The average number of parent-team contacts was 3.5 +/- 0.9 by pager and 24.0 +/- 2.9 by telephone. Of the 93 patients who died in the program, 53% died at home, 18% died in community hospitals, and 29% died in a tertiary care facility. Analgesic medications were administered to 54% of the patients; 56% of these then required opioid analgesia for pain and symptom management. Home-based palliative care appeared to be an effective program for many children with a variety of terminal illnesses after adequate supports for the child and family had been established.

Adolescent↗

[An Italian multicenter case-control study on malignant neoplasms of the hematolymphopoietic system. Hypothesis and preliminary results on work-related risks. WILL (Working Group on Hematolymphopoietic Malignancies in Italy)].

A multicenter case-control study was conducted in Italy in twelve areas covering a population of 7 million persons with the aim of verifying the hypothesis of an association between exposure to herbicides and to organic solvents and occurrence of lymphoma, leukaemia and multiple myeloma. 3420 cases were recruited in a three-year period (1991-1993), through periodical surveys in the main hospitals in each area. A random sample of the general population (2317 subjects) constitutes the control group. Cases and controls were face to face interviewed by trained personnel. A questionnaire was used asking about personal habits, residential and occupational history, past health status. Preliminary analyses, concerning a total of 1388 cases and 1718 controls, focused risks by occupation. Elevated risks of non Hodgkin's lymphoma (OR: 1.7; IC 95%: 0.92-3.02; based on 28 exposed cases), and of multiple myeloma (OR: 3.6; IC 95%: 1.51-8.63; based on 9 exposed cases) were shown for welders. Among female hairdressers there were elevated risks of non Hodgkin's lymphoma (OR: 1.8; IC 95%: 0.76-4.30; based on 8 exposed cases), multiple myeloma (OR: 3.3; IC 95%: 0.77-14.15; based on 3 exposed cases) and Hodgkin's disease (OR: 2.0; IC 95%: 0.93-4.47; based on 16 exposed cases).

Adult↗

Characteristics of malignant tumors in young people, with particular emphasis on carcinomas and sarcomas.

The aims of the study are to investigate the characteristic patterns related to different aged subgroups of young Chinese patients with malignant tumors. The subjects were young Chinese patients (defined as age > 1 and < 40 years) who underwent autopsy for malignant tumors in a teaching hospital in Hong Kong from 1970 to 1999. They were divided into four age groups: 1 to 9 years, 10 to 19 years, 20 to 29 years, and 30 to 39 years. The malignant tumors were classified into subgroups according to the pathological classification. The clinicopathologic features of patients with carcinomas and sarcomas were reviewed in depth. Four hundred sixty-three (22%) of 2,080 young patients had malignant tumors detected at autopsies. The male-to-female ratio was 1.5. The two most common malignant tumors were malignant lymphoid tumors and carcinomas, accounting for 49.9% and 29.8%, respectively, of all malignant tumors. In children (ages 1-9 years), 78.9% of malignant tumors found were malignant lymphoid tumors and neuroendocrine tumors. In teenagers (ages 10-19 years), malignant lymphoid tumors were the most important group of malignant tumor, accounting for 69% of malignant tumors in this age group. Carcinomas and malignant lymphoid tumors were seen in more than 80% of malignancies in young adults (ages 20-39). Overall, carcinomas and sarcomas were noted in 29.8% and 4.1%, respectively, of young patients with malignant tumors. The common primary sites of carcinomas were liver, stomach, lung, and nasopharynx. Sarcomas were more common in females (11 females, seven males), and rhabdomyosarcoma was the most common sarcoma found. Compared with other malignant tumors, carcinomas were less often diagnosed before death. In conclusion, malignant tumor is a common cause of death in young patients. Different types of malignant tumors were seen in various groups of young patients.

Adolescent↗

Pheochromocytomas: can malignant potential be predicted?

OBJECTIVES: The presence of metastatic lesions is the only acceptable fact to confirm malignant pheochromocytoma. Patients with malignant pheochromocytomas, however, have a very poor survival rate. The aim of our study was to postulate predictive values for malignant pheochromocytomas. METHODS: We evaluated symptoms, diagnostic modalities, treatment, and long-term follow-up of 86 patients with 85 benign and 10 malignant pheochromocytomas. Parameters from the benign were compared with those of the malignant pheochromocytomas. RESULTS: Preoperative 24-hour urinary dopamine was in the normal range for benign pheochromocytomas but increased in malignant pheochromocytomas (P<0.0001). Vanillylmandelic acid was elevated in both benign and malignant pheochromocytomas but higher in malignant than in benign tumors (P = 0.01). No differences could be shown in urinary epinephrine and norepinephrine samplings. Tumor location was divided into 77 adrenal (81%) and 18 extra-adrenal (19%) sites. Malignant pheochromocytomas were located more often at extra-adrenal sites (P = 0.03). There was no increased incidence of malignancy in patients with familial bilateral pheochromocytomas or multiple endocrine neoplasia. Tumors greater than 80 g in weight corresponded to malignancy (P<0.0001). Dopamine tumor concentration was higher in malignant than in benign pheochromocytomas (P = 0.01). Persistent arterial hypertension occurred in 9 (13%) of 72 benign and 6 (60%) of 10 malignant pheochromocytomas (P = 0.001). The 10-year survival rate was 94% for benign pheochromocytomas. All patients with malignant pheochromocytomas died within this period (P = 0.0001). CONCLUSIONS: High preoperative 24-hour urinary dopamine levels, extra-adrenal tumor location, high tumor weight, elevated tumor dopamine concentration, and postoperative persistent arterial hypertension are all factors that increase the likelihood of malignant pheochromocytoma. Patients with these characteristics should have more frequent follow-up evaluations to identify malignancy at earlier states.

Adolescent↗

Modern diagnostics in chronic myeloproliferative diseases (CMPDs).

According to the new WHO classification a group of chronic myeloproliferative diseases (CMPDs) were defined: chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia and hypereosinophilic syndrome (CEL/HES), polycythemia vera (PV), chronic idiopathic myelofibrosis (with extramedullary hematopoiesis, CIMF), essential thrombocythemia (ET), and so called CMPD/unclassifiable. As clinical features and laboratory findings differ widely between these diseases several diagnostic approaches are mandatory at diagnosis for classification and are needed also for follow up studies, especially for the measurement of minimal residual disease (MRD). We here outline the laboratory set up at diagnosis and during follow up in CMPDs with specific focus on the respective therapeutical consequences. Only by using a comprehensive diagnostic panel including cytomorphology, cytogenetics, and molecular genetic methods establishing the correct diagnosis, optimizing treatment as well as evaluating treatment response is possible in CMPDs today.

Blood Cell Count↗

Pathologic, immunohistochemical, and molecular features of benign and malignant phyllodes tumors of the breast.

The histologic distinction between benign and malignant Phyllodes tumors (PT) is often difficult and arbitrary. We analyzed a group of benign and malignant PT to determine whether specific histologic features and expression of Ki-67 and p53 could be useful in distinguishing benign PT from malignant tumors. We also determined whether deletions in Chromosome 3p at the FHIT and hMLH1 loci are common abnormalities in PT. Twenty PT were histologically classified as benign (7) or malignant (13). Seven of the malignant PT were low grade, and six were high grade. Ki-67 and p53 immunohistochemistry was performed on all tumors and analyzed for the stromal and for the epithelial component. PCR-based loss of heterozygosity analyses were performed with the following markers on Chromosome 3p: D3S1478 (3p21.2--21.3), D3S1289 (3p21.1--21.2), and D3S1295 (3p14.3--21.1). The distribution of immunoreactivity for Ki-67 was analyzed by quantifying the percentage of positive nuclei and expressed as the labeling index (LI). Patients' ages ranged from 13 to 71 years (median: 51 y). After a mean follow-up period of 8 years, none of the PT metastasized, whereas three recurred locally. Although malignant PT were larger than benign PT (means, 7.1 versus 4.3 cm), this difference was not statistically significant. Five tumors had infiltrating margins, and 14 were circumscribed. The Ki-67 LI in low-grade malignant PT (16 +/- 25.5) was significantly higher than that in benign PT (3.6 +/- 4.8), whereas the LI in the high-grade malignant PT group (50 +/- 21.9) was significantly higher than that in low-grade malignant tumors (P =.012). The Ki-67 LI in the three tumors that recurred was less than 10%. Two of seven (29%) benign PT were focally positive for p53, whereas four of seven (57%) low-grade malignant and three of six (50%) high-grade malignant PT were diffusely positive for p53. The three tumors that recurred initially were histologically benign, as were two of the recurrences. One recurrent tumor evolved to a high-grade malignant PT. Margins were greater than 1 cm in all tumors except four, three of which recurred locally. No allelic loss of 3p was found. In summary, Ki-67 expression may assist in distinguishing benign from malignant PT in diagnostically difficult cases. 3p deletions do not play a significant role in the development of these tumors. Neither Ki-67 nor p53 can reliably predict recurrence. Histologically high-grade malignant PT have a favorable prognosis if widely excised. We emphasize the importance of adequate margins in the treatment of benign and malignant PT.

Breast Neoplasms↗

In vitro effects of 4-chloro-3-ethylphenol in skeletal muscle preparations from malignant hyperthermia susceptible and normal swine.

BACKGROUND AND OBJECTIVE: The in vitro contracture test with halothane and caffeine is the current gold standard for diagnosis of malignant hyperthermia. This test has a sensitivity of 99.0% but a specificity of only 93.6%. Therefore, an alternative drug is desirable which distinguishes between malignant hyperthermia-susceptible and malignant hyperthermia-normal subjects with a higher specificity and sensitivity. METHODS: 4-chloro-3-ethylphenol has recently been shown to trigger Ca2+-induced Ca2+-release in skeletal muscle terminal cisternae and to increase the myoplasmic free Ca2+ concentration in skeletal muscle fibres. The purpose of this study was to investigate the ability of 4-chloro-3-ethylphenol to distinguish between malignant hyperthermia-susceptible and malignant hyperthermia-normal porcine muscle specimen in the in vitro contracture test. Ten malignant hyperthermia-susceptible and 14 malignant hyperthermia-normal swine were anaesthetized and muscle biopsies were taken. For the in vitro contracture test muscle specimens were exposed to cumulative concentrations of 4-chloro-3-ethylphenol (12.5 to 200 micromol L(-1)). RESULTS: 4-chloro-3-ethylphenol produced contractures in a concentration-dependent manner in the malignant hyperthermia-susceptible muscle bundles. In contrast, cumulative 4-chloro-3-ethylphenol did not generate contractures in malignant hyperthermia-normal specimens. Contractures were significantly greater (P < 0.05) in the malignant hyperthermia-susceptible compared to the malignant hyperthermia-normal preparations in all 4-chloro-3-ethylphenol concentration steps from 50 micromol L(-1) to 200 micromol L(-1). There was no overlap between the two groups above a concentration of 75 micromol L(-1) in cumulative 4-chloro-3-ethylphenol in vitro contracture tests. CONCLUSIONS: It remains to be verified whether an in vitro contracture test with 4-chloro-3-ethylphenol can also discriminate between malignant hyperthermia-susceptible and malignant hyperthermia-normal humans. Since no prior tested agent revealed a clear differentiation in contracture development without overlap, the 4-chloro-3-ethylphenol test might be a promising new approach to the diagnosis of malignant hyperthermia.

Animals↗

Differential heparanase-1 expression in malignant and benign pheochromocytomas.

INTRODUCTION: Extracellular matrix (ECM) degradation is an essential step that allows tumor cells to penetrate a tissue barrier and become metastatic. Heparanase-1 (HPR) is an endoglycosidase that specifically degrades heparan sulfate proteoglycans, a chief component of the ECM. Previous studies have demonstrated HPR expression in various malignancies and that there is differential HPR expression between benign and malignant tumors. Currently, there is no technique that can reliably predict the malignant behavior of some pheochromocytomas. This study tests whether HPR is differentially expressed in malignant and benign pheochromocytomas. METHODS: Paraffin-embedded specimens from 29 pheochromocytomas were evaluated. The tissues were collected from surgical specimens over a 10-year period from 26 patients (8 males, 18 females) with a mean age of 47 years (range 19-78 years, median 47 years). One female patient underwent 3 separate operations for malignant pheochromocytoma and thus provided 3 specimens. Another female patient had both the primary tumor and a liver metastasis processed, and therefore provided 2 specimens. Patient charts and pathology reports were reviewed to classify the pheochromocytomas as either benign or malignant. Based on clinical behavior and/or pathological evidence of metastasis or invasion into surrounding tissues, 10 specimens were malignant and 19 had benign behavior. As a control, normal adrenal tissue from 3 nephrectomy specimens was included in the study, as was tissue from 1 adrenocortical adenoma. All 33 specimens were tested for HPR gene expression by in situ hybridization (ISH) with an antisense RNA probe and immunohistochemistry (IHC) with an anti-HPR antibody. Statistical analysis was done using the chi(2) test of proportions to determine if HPR expression correlated with malignancy using ISH, IHC, or both tests together. RESULTS: Using ISH, the percentage of HPR expression in the malignant pheochromocytomas was 50% while HPR expression in the benign tumors was 21% (P = 0.11). Using IHC, the percentage of HPR expression in the malignant pheochromocytomas was 80% while HPR expression in the benign tumors was 32% (P = 0.01). Considering both tests cumulatively, all 10 malignant pheochromocytomas stained positive for HPR by ISH and IHC, while only 37% of the benign tumors were positive for HPR expression (P = 0.001). The one adrenal adenoma and the 3 normal adrenal glands processed stained negative for HPR expression by both ISH and IHC. CONCLUSIONS: HPR expression is higher in malignant pheochromocytomas than in benign pheochromocytomas or normal tissue. HPR may contribute to the invasive characteristics of malignant pheochromocytomas and might be used as a marker to distinguish malignant from benign pheochromocytomas. HPR expression might also be used as a prognostic tool in guiding long-term patient follow-up.

Adrenal Gland Neoplasms↗

Mycophenolate mofetil and risk of developing malignancy after orthotopic heart transplantation: analysis of the transplant registry of the International Society for Heart and Lung Transplantation.

BACKGROUND: Malignancy after organ transplantation has been described as the "price of immunotherapy." Evolving strategies aimed at effective immunosuppression could have differing effects on the likelihood of developing malignancy. We analyzed data from the transplant registry of the International Society for Heart and Lung Transplantation (ISHLT) to ascertain which factors are associated with the development of malignancy after orthotopic heart transplantation (OHT). METHODS: Multivariate modeling was performed to determine factors predictive of first post-transplant malignancy in patients taking standard immunosuppressive regimens, defined as cyclosporine or tacrolimus and azathioprine or mycophenolate mofetil (MMF), who underwent OHT between January 1, 1995 and December 31, 1997. RESULTS: Of the 3,895 transplants described in the cohort, 703 (18%) developed post-transplant malignancy at any time during the follow-up period, and 549 (14%) developed malignancy within the first 5 years post-transplant. The breakdown of malignancy was as follows: skin: 47%; post-transplant lymphoproliferative disease: 10%; other malignancies: 24%; combination of types: 10%; and unreported: 10%. Multivariate modeling revealed that independent predictors of increased risk were prior malignancy and increased age, whereas the use of MMF as part of a standard immunosuppressive regimen was associated with an adjusted relative risk (RR) = 0.73 (95% confidence interval 0.56 to 0.95). Relative to a recipient age of 55 years, the risk of malignancy for 30, 45 and 60 years of age was 0.32, 0.46 and 1.37, respectively. Although the use of tacrolimus appeared protective in the univariate analysis, it was not significant according to multivariate analysis. Female gender appeared to be protective. Neither OKT3 nor anti-thymocyte globulin (ATG) use was associated with a significantly increased risk of malignancy. CONCLUSIONS: The choice of immunosuppressive regimen may affect the likelihood of developing malignancy after OHT. Induction immunosuppression does not appear to increase the risk of subsequent malignancy. The use of MMF in standard immunosuppressive regimens is associated with a significantly lower risk of developing malignancy.

Aging↗

Cardiac transplantation in patients with preexisting malignancies.

A history of preexisting malignancy has been considered a contraindication to cardiac transplantation. The reasons for this prejudice include concerns about potentially deficient intrinsic immunomodulation and fear of cancer recurrence (or development of second cancers) because of therapeutic immunosuppression. In the past four years at the Northern Indiana Heart Institute seven patients with preexisting malignancies underwent cardiac transplantation. Their two-year survival rate was 100%, which is comparable to a rate of 81% in non-malignancy patients. After an average 31 months of follow-up (range = 6-56 months), only one patient has had a recurrent tumor (basal cell carcinoma). Statistical comparison of immunosuppression dosages, incidences of rejection, and incidences of infections between patients with preexisting malignancy and those without preexisting malignancy was performed. We found that the only significant difference was an increased number of infections in preexisting malignancy patients. Additionally, we found no difference in the incidence of posttransplant coronary artery disease in the preexisting malignancy group when compared with those patients without preexisting malignancies. This study demonstrates that patients who have been successfully treated for malignancies have no greater incidence of rejection than those patients without preexisting malignancy. Furthermore, preexisting malignancy patients require no significant modulation of immunosuppression. Although preexisting malignancy patients have a higher incidence of infections than patients without preexisting malignancy, their two-year survival is not worse than the patients without preexisting malignancy.

Adult↗

Mycosis fungoides associated with B-cell malignancies.

BACKGROUND: The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual. Most descriptions are isolated case reports and case series are strikingly sparse. OBJECTIVES: To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms. METHODS: Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period. RESULTS: Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma). No case of Hodgkin's lymphoma was found. In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first. The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years). Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB). Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy. Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed. In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy. One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled. CONCLUSIONS: This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy. In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm. The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases. It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features.

Adult↗

Prospective study of biliary strictures to determine the predictors of malignancy.

BACKGROUND: There have been few prospective studies regarding the investigation of biliary strictures, principally because of rapid technological change. The present study was designed to determine the sensitivity of various imaging studies for the detection of biliary strictures. Serum biochemistry and imaging studies were evaluated for their role in distinguishing benign from malignant strictures. METHODS: Thirty-one patients with suspected noncalculus biliary obstruction were enrolled consecutively in the study. A complete biochemical profile, ultrasound, Disida scan and cholangiogram (endoscopic retrograde cholangiopancreatography [ERCP] or percutaneous cholangiogram) were obtained at study entry. Stricture etiology was determined based on cytology, biopsy and/or clinical follow-up at one year. RESULTS: Twenty-nine of 31 patients had biliary strictures, of which 15 were malignant. The mean age of the malignant cohort was 73.9 years versus 53.9 years in the benign cohort (P<0.001). Statistically significant differences between the malignant and benign groups, respectively, were as follows: alanine transaminase 235.2 versus 66.9 U/L (P=0.004), aspartate transaminase 189.8 versus 84.5 U/L (P=0.011), alkaline phosphatase 840.2 versus 361.1 U/L (P=0.002), bilirubin 317.8 versus 22.1 micromol/L (P<0. 001) and bile acids 242.5 versus 73.2 micromol/L (P=0.001). Threshold analysis using receiver operative characteristic (ROC) curves demonstrated that a bilirubin level of 75 micromol/L was most predictive of malignant strictures. Intrahepatic duct dilation was present in 93% of malignant strictures versus 36% of benign strictures (P=0.002). Common hepatic duct dilation was less discriminatory (malignant 13.5 versus benign 9.6 mm; P=0.11). Ultrasound was highly sensitive (93%) in the detection of the primary tumour in the bile duct or pancreas, or in the visualization of nodal or liver metastases. In benign disease, ultrasound failed to detect evidence of intrahepatic or extrahepatic biliary dilation in most cases. Disida scans were not able to distinguish between malignant or benign strictures and could not accurately localize the level of obstruction. The sensitivity of Disida scan for the diagnosis of obstruction was 50%. Cholangiographic characterization of strictures revealed an equal distribution of smooth (eight of 13) and irregular (five of 13) strictures in the malignant group. Ten of 13 benign strictures were characterized as smooth. Malignant strictures were significantly longer than benign ones - 30.3 versus 9.2 mm (P=0.001). Threshold analysis using ROC curves showed that strictures greater than or equal to 14 mm were predictive of malignancy (sensitivity 78%, specificity 75%, log odds ratio 11.23). CONCLUSIONS: A serum bilirubin level of 75 micromol/L or higher, or a stricture length of greater than 14 mm was highly predictive of malignancy in patients with a biliary stricture. Ultrasound was useful in predicting malignant strictures by detecting either intrahepatic duct dilation or by visualizing the tumour (primary or metastases). Strictures with a 'benign' cholangiographic appearance are frequently malignant. Disida scan did not add additional information. ERCP is necessary to diagnose benign strictures, which tend to be less extensive at presentation.

Adult↗

Multiple primary malignant tumors in a national cancer registry--reliability of reporting.

During the years 1958-1988, 808,522 individuals were registered in the Swedish national population-based cancer register with a total of 933,900 primary malignant tumors. Roughly 11% of the tumors reported to the Swedish Cancer Registry in 1988 were found in persons earlier registered for another primary malignancy. One hundred of the individuals registered with multiple primary malignant tumors were randomly selected for a study of the reliability of reporting of multiple malignancies. Medical records and when necessary histopathological slides and other relevant diagnostic material for each malignancy were collected and the diagnoses reevaluated. Three persons had to be excluded; thus 97 cases with 209 reported malignancies were analysed. Of these, 94% of the first, 98% of the second and 79% of the third malignancy were accepted. Twelve of the reported tumors were not accepted as malignant, five were benign or cancer in situ, five were incorrectly reported as new primaries and two were a second incorrect registration of a previously registered malignancy. All 97 persons had at least one malignant tumor, in 90% of the persons all reported diagnoses were accepted and 93% had multiple primary malignancies. The results of this quality control study indicate that suitable data are available in the Swedish Cancer Registry for investigations related to the occurrence of multiple primary malignancies in a large unselected population. The Swedish Cancer Registry, to which all newly diagnosed malignancies by law have to be reported, comprise today close to one million individuals with registered malignancies.

Breast Neoplasms↗

[Extramammary Paget's disease with internal malignancies].

Extramammary Paget's disease (EP) has frequently underlying adnexal cancer and perhaps has a tendency to be associated with internal malignancies. Chanda (1985) reviewed 197 cases of EP (196 cases reported in the literature from 1962 to 1982 and 1 case of his own) with regard to the prognosis and relationship with internal malignancy. We analysed 68 cases of EP experienced in our clinic over the last 30 years and compared our data with Chanda's review. Of our 68 patients, 10 cases (14.7%) had an associated internal malignancy, while of Chanda's 153 patients, 44 (28.8%) had the same malignancy. Thirty-nine patients who had an associated internal malignancy have been reported in Japan including our 10 cases (1932-1986). In 25 Japanese cases occurring on the penis, scrotum or groin, the following internal malignant tumors were found, 3 each of prostatic and bladder cancer, 2 testicular malignancies (1 sarcoma and 1 malignant lymphoma), 1 hypernephroma and 16 other malignancies. In EP of the vulvar area, 2 each of uterine and bladder cancer, and 1 each of breast, colon and anal cancer were observed. In Chanda's data, the location of the associated internal malignancy appeared to be closely related to the location of the EP, i.e., a penile-scrotum-groin location being associated with male genitourinary malignancy, a vulva location being associated with female genitourinary malignancy and a perianal location being associated with adenocarcinoma of the digestive system. In the Japanese data, however, the associated internal malignancy was more widely distributed to other organs. Our data suggest that a more direct search should be made for internal malignancy in Japanese patients with EP than in Caucasians.

Adult↗

[Incidence of malignant lymphomas in Western Maritime Provinces in the years 1980-1984].

Incidence rate of malignant lymphomas was determined in the population of Western Pomerania region. The period of studies covered the years from 1980 to 1984 and the patients being over 14 years old. In the Western Pomerania region malignant lymphoma was diagnosed during that period in 528 subjects: low grade malignancy lymphomas were diagnosed in 268, therein lymphocytic lymphomas 135, with high grade malignancy 54: malignant granulomatosis 125 cases and 77 plasmocytic myelomas. The following data were taken into consideration: sex, place of residence, type of lymphoma: in case of malignant granulomatosis also the histopathologic subtype, and in multiple myeloma the type of monoclonal protein. Szczecin Voivodeship was found to have the highest incidence rate of malignant lymphoma, namely 9,1/10(5) of inhabitants: in Koszalin and Gorzów Voivodeships respectively 6.9/10(5) and 6.1/10(5) of inhabitants annually. The difference in the incidence rate in Szczecin Voivodeship and two other ones was significant (p < 0.001). No significant differences in the morbidity existed between Koszalin and Gorzów Voivodeships. In the region there was a preporderance of malignant lymphomas in relation to malignant granulomatosis. Morbidity in malignant lymphoma more frequently involved men than women. Morbidity rate of lymphoma with low and high malignancy, as well as plasmocytic myeloma increases with the age. On the other hand, in malignant granulomatosis no statistically significant differences depending on the age and sex were recorded. The incidence rate in respective groups of malignant lymphoma was the highest in Szczecin, followed by other towns in the region, being the lowest in rural environment. In our region, with regard to all malignant lymphomas, the disease is more frequently diagnosed in the advanced clinical stage.

Adult↗