MOLES,MELANOMAS AND EPITHELIOMAS IN CHILDREN

The management of moles which occur in childhood is important from the standpoint of clinical diagnosis and treatment. Melanomas of both the malignant and juvenile types are sometimes mistaken for ordinary moles. Malignant epithelial tumors are rare in childhood as are congenital tumors. There is a possible relationship between congenital defects of various types and childhood neoplasms.     

Moles,melanomas and epitheliomas in children

The management of moles which occur in childhood is important from the standpoint of clinical diagnosis and treatment. Melanomas of both the malignant and juvenile types are sometimes mistaken for ordinary moles. Malignant epithelial tumors are rare in childhood as are congenital tumors. There is a possible relationship between congenital defects of various types and childhood neoplasms.     

Risk of melanoma arising in large congenital melanocytic nevi: a systematic review

Large congenital melanocytic nevi are cutaneous lesions regarded by many as premalignant; estimates of malignancy incidence range from 0 to 42 percent. Given the often complex and extensive nature of large congenital melanocytic nevi resection and reconstruction, the risk of malignant transformation is a crucial factor that surgeons and families must weigh when deciding whether or not to excise the lesion. The authors conducted a systematic analysis of data from the existing literature to critically evaluate the published studies and to establish a crude incidence rate for the risk of malignant melanoma transformation in large congenital melanocytic nevi. After a comprehensive literature search, they analyzed data from eight studies (containing a total of 432 large congenital melanocytic nevi patients) of sufficient scientific quality. Twelve patients (2.8 percent) in this sample developed cutaneous malignant melanoma during the reported follow-up periods. Using a subset of this data and comparing the incidence rates to those of the Surveillance, Epidemiology, and End Results population-based database using a standardized morbidity ratio, the authors found that the large congenital melanocytic nevi patients had an increased risk of melanoma (standardized morbidity ratio, 2599; 95 percent confidence interval, 844 to 6064) compared with the general population. Regarding treatment before developing melanoma in the 12 patients, 50 percent were observed before diagnosis, 17 percent had partial excision, 8.3 percent had dermabrasion, 8.3 percent had a chemical peel, and 17 percent did not have any treatment information. These combined data are clinically useful when consulting with the parents of children with large congenital melanocytic nevi and in the management of older patients with existing lesions. This study shows that there is a significantly increased risk of melanoma in large congenital melanocytic nevi patients. The data also reveal the need for a standardized definition of large congenital melanocytic nevi and a long-term, prospective outcomes study to determine the true lifetime risk of melanoma in patients with and without surgical excision.     

Congenital malignant melanoma.

The diagnosis and management of a 3-day-old infant with an exophytic pigmented growth on her back and chest are discussed. At 3 days of age, the child underwent a complete surgical excision of this lesion with immediate coverage by partial-thickness skin grafts. Pathologically, the lesion proved to be a thoracolumbar congenital melanocytic nevus with multiple focal areas of malignant melanoma. The child is now 3 years past surgery and shows no evidence of recurrence or distant metastases. The importance of this case lies in the presence of overt malignant melanoma at birth within a giant congenital nevocytic nevus.     

Carbon dioxide laser dermabrasion for giant congenital melanocytic nevi

The management of giant congenital melanocytic nevi remains controversial. There is a balance to be achieved between minimizing the disfiguring appearance of these lesions, both before and after surgical treatment, and limiting the risk of malignant change. A series of seven patients who were treated in the same manner, with carbon dioxide laser dermabrasion, is presented. It has been 6 years since the first patient was treated in this way, and no cases of recurrence have been observed. This technique enables the removal of all or most of the pigmented lesion, with minimal scarring and without the need for disfiguring skin grafts. It has been well proved that there is an increased risk of malignant changes among patients with these lesions, although the amount of increased risk for the patient is not clear. Evidence from a review of the currently available literature is presented to indicate why this management method, at best, should decrease this risk and, at worst, should make no difference to the overall risk for individual patients.     

Trends in mortality from primary tumours of skin in Canada

In Canada the death rate from all primary malignant neoplasms of skin has remained approximately stable since the early 1950s. The rates have fallen in those over age 65 and risen in those aged 45 to 64 and 15 to 44. We have no reason to believe that this is due to a change in certification or to a deterioration in treatment. The rise has been wholly due to a doubling in the death rate from malignant melanoma. This is in agreement with the trend of Canadian incidence data and with the experience of other developed countries. We are of the opinion that this change is largely due to a genuine increase in the incidence of malignant melanoma.     

Congenital nevocellular nevus: a review of the treatment controversy and a report of 46 cases

Because congenital nevocellular nevi can be distinguished clinically and histologically from acquired nevi, and because of their apparent increased potential for malignant degeneration, we favor complete one-stage excision of these nevi, regardless of the size of the lesion or the age of the patient, at the earliest opportunity, whenever such surgery is feasible and practical. If there is a question about the clinical diagnosis, a cutaneous punch biopsy can help determine the true nature of the lesion. Significantly, Walton et al. and Rhodes and coworkers found discrepancies in the literature concerning the level of nevus cells in neonates. They concluded that until these differences are reconciled, nevus cells in the deep reticular dermal collagen may be a sufficient, but not a necessary criterion for the diagnosis of congenital melanocytic nevus. We currently favor complete one-stage excision of congenital nevocellular nevi and feel that treatment by tangential excision or dermabrasion require further study. Finally, we present this paper as "advice" not only to the three authors who, in a recent issue of the British Journal of Plastic Surgery, requested it, but also to all clinicians. Hopefully, with time and further study, better criteria will be determined and a more definitive approach to this problem will be established.     

Pulmonary Artery Stenosis—A Frequent Part of the Congenital Rubella Syndrome

The case records, cardiac catheterization and angiographic findings in 32 patients were reviewed to illustrate the high incidence of pulmonary artery stenosis in congenital rubella syndrome. These interesting lesions often are of equivocal or unknown clinical significance compared with the other malformations that beset these patients, but to know of them explains certain physical findings and to study their incidence is an unusual opportunity to pinpoint a definite cause for a congenital cardiac lesion.     

Second primary malignant tumors in patients with ocular melanoma

Multiple primary malignant tumors have been documented with increased frequency over the last two decades. Continuously increasing success of modern oncotherapy has led to long-term remissions in many cases, but this success rate poses a growing risk for the development of second primary malignancies. The incidence of those involving an intraocular tumor is relatively rare. In the present study we report five ocular melanoma patients with second primary malignant tumors diagnosed during a fourteen-year period in our department. We wish to emphasize that an intraocular mass lesion in a patient with a history of a previous malignancy should not be dismissed as a metastatic lesion. The diagnosis of an intraocular lesion as a separate primary tumor drastically changes the prognosis and the therapeutic approach.     

Childhood liver tumor in Hungary: two interesting clinical cases

Hepatic tumors account for 0.5-2% of all childhood tumors in Hungary, based of the data last ten years. More than half of the cases were histologically malignant. The worldwide incidence of malignant hepatic tumors is 1.6 / 1 million. Here we present two patients with hepatoblastoma. In the first case the size of the initially inoperable tumor diminished following the chemotherapy and total surgical resection became possible. No sign of relapse occurred so far. The second case included a congenital hepatic tumor which was remarkable because of its unusual clinical presentation and histology.     

Racial differences in nasopharyngeal carcinoma in the United States

Background: Nasopharyngeal carcinoma (NPC) is a malignant neoplasm arising from the mucosal epithelium of the nasopharynx. Different races can have different etiology, presentation, and progression patterns. Methods: Data were analyzed on NPC patients in the United States reported to the SEER (Surveillance, Epidemiology, and End Results) database between 1973 and 2009. Racial groups studied included non-Hispanic whites, Hispanic whites, blacks, Asians, and others. Patient characteristics, age-adjusted incidence and mortality rates, treatment, and five-year relative survival rates were compared across races. Stratification by stage at diagnosis and histologic type was considered. Multivariate regression was conducted to evaluate the significance of racial differences. Results: Patient characteristics that were significantly different across races included age at diagnosis, histologic type, in situ/malignant tumors in lifetime, stage, grade, and regional nodes positive. Incidence and mortality rates were significantly different across races, with Asians having the highest rates overall and stratified by age and/or histologic type. Asians also had the highest rate of receiving radiation only. The racial differences in treatment were significant in the multivariate stratified analysis. When stratified by stage and histologic type, Asians had the best five-year survival rates. The survival experience of other races depended on stage and type. In the multivariate analysis, the racial differences were significant. Conclusions: Analysis of the SEER data shows that racial differences exist among NPC patients in the U.S. This result can be informative to cancer epidemiologists and clinicians.     

Gastric polyposis

In a series of 48 cases of gastric polyps, 40 patients had benign lesions while the polyps in the remaining eight cases were malignant. Although the symptomatology in this series was not uniform, only one patient was entirely asymptomatic. Of the eight patients with malignant lesions, three had polyps which could well be described as small, suggesting that size may not be a reliable criterion of benignity even in a single lesion. Laboratory studies indicated that anemia, achlorhydria and occult blood in the stools are frequently associated with gastric polyps. There was no apparent correlation, however, between these phenomena and the benign or malignant nature of the lesions.X-ray examination, performed in every instance, was completely negative in six cases and inconclusive in an additional five. Gastroscopy did not reveal the presence of polyps in three of 15 cases. Malignant change was detected by cytologic examination in one case in which both roentgen and gastroscopic examination were negative. The author believes that in most cases of gastric polyps operative treatment affords the greatest degree of safety. An operative procedure of considerably less extent than total gastric resection often is feasible. However, if true polyps occur where removal can be effected only by total gastric resection, the incidence of malignant change in these lesions would seem to indicate the advisability of such a procedure unless positive contraindicating conditions are present.     

Primary thyroid angiosarcoma: an unusual localization

ABSTRACT: The finding of thyroid nodules is a very common occurrence in routine clinical practice. Approximately 5% to 7% of the entire population have thyroid nodules. Vascular lesions are one of the most controversial issues in thyroid pathology. These include benign lesions such as hemangiomas and, rarely, malignant tumors such as angiosarcomas or undifferentiated angiosarcomatoid carcinomas. In particular, angiosarcoma of the thyroid gland is a rare, highly aggressive malignant vascular tumor and in Italy the greatest geographical incidence of this lesion is witnessed near the Alps. Here, a case of thyroid angiosarcoma in a 71-year-old man with a history of goiter for about 20 years is described. The unusual localization of this lesion, the difficulties in reaching a definitive diagnosis for this particular histological type of primary tumor and a history of long-standing multinodular goiter in thyroid of an older man from outside the Alpine region prompted us to report this case of thyroid angiosarcoma mainly to discuss surgical, histopathological and immunohistochemical features.     

Congenital anterior abdominal wall defects in England and Wales 1987-93: retrospective analysis of OPCS data.

OBJECTIVES: Analysis of incidence and characteristics of congenital abdominal wall defects, with special reference to the differences between the incidence of gastroschisis and exomphalos (omphalocele). DESIGN: Retrospective analysis using data from the Office of Population Censuses and Surveys (recoded to differentiate exomphalos and gastroschisis) and the National Congenital Malformation Notification Scheme. SETTING: England and Wales, 1987 to 1993. RESULTS: 1043 congenital anterior abdominal wall defects were notified within the seven year study period. Of these, 539 were classified as gastroschisis, 448 as exomphalos, 19 as "prune belly syndrome," and 37 as "unclassified." Gastroschisis doubled in incidence from 0.65 in 1987 to 1.35 per 10,000 total births in 1991, with little further change; the incidence of exomphalos decreased from 1.13 to 0.77 per 10000 births. The overall incidence of notified congenital abdominal wall defects was 2.15 per 10000 total births. Gastroschisis was associated with a lower overall maternal age than exomphalos and with a significantly lower proportion of additional reported congenital malformations (5.0%) than in the cohort with exomphalos (27.4%) (odds ratio 0.14, 95% confidence interval 0.09 to 0.22; P < 0.001). The sex ratio of the two cohorts was the same. The incidence of gastroschisis and exomphalos was higher in the northern regions of England than in the south east of the country. CONCLUSIONS: The national congenital malformation notification system showed an increasing trend in the incidence of fetuses born with gastroschisis and a progressive decreasing incidence of exomphalos in England and Wales between 1987 and 1993. Although the reasons for this are likely to be multifactorial, a true differential change seems likely. The observed increase in incidence of gastroschisis relative to exomphalos and the differentiation in maternal age have implications for resource management within the NHS and warrant further epidemiological monitoring. Regional differences may be due to a dietary or environmental factor, which requires further study.     

Genetic characteristics of recessive sensorineural hearing loss

Genetic characteristics of recessive sensorineural hearing impairment mediated by 5 recessive genes were studied. One of these is responsible for early progressive hearing loss, others causing congenital deafness. The incidence of early progressive recessive hearing loss in a population is 1:20,000, gene frequency being 0.007; the incidence of heterozygotes for this gene is 1.4%. The incidence of each of 4 forms of recessive congenital hearing loss in a population is 1.125:10,000, the frequency of these genes being 0.0106; the incidence of heterozygotes for each of these genes is 2.1%. The total frequency of all recessive genes for sensorineural hearing impairment is 0.0494 and the incidence of heterozygotes for all genes is 9.9%. The frequency of different genotypes for recessive genes specifying sensorineural hearing loss was established, based on the data obtained.     

Application of the Macrophage Migration Inhibition Test to Screen Patients with Early Cancer and Obtain Prognostic Determinations of Cancer Treatment

A modified direct method of the macrophage migration inhibition test (MMIT) was attempted on a large number of patients with malignant or benign tumors. Results of the MMIT in almost all patients with benign tumors were negative except for those with hydatidiform moles, dermoid cysts and viral benign tumors such as verruca plana which were positive. The number of cases determined as false positives were exceptionally few. Conversely, about the half of the patients with malignant tumors were positive. The majority of negative cancer patients were confirmed pathologically to be advanced cases and, therefore, were postulated to have been immunologically unresponsive. The remaining false negative patients were diagnosed to be very early cases with their malignant foci too small to be effective antigenic stimuli. The MMIT was also performed postoperatively on some of the patients using autologous antigens, which had been preserved by freezing, for examination of changes in the per cent migration index. The results led the authors to conclude that postoperative repetitions of the test permitted them to tell that cancer cells had been completely eradicated or that a relapse might occur in the near future. Examinations of cross reactivity between tumor antigens revealed that such reactivity exists between cancer antigens and antigens originating in hydatidiform moles and that there is also a very strong cross reactivity between allogeneic cancer antigens regardless of differences in the organs of origin. This fact suggests that the present test is effective for the screening of preoperative patients with early cancer.     

Isolation,fine mapping and expression profiling of a lesion mimic genotype, <Emphasis Type="Italic">spl</Emphasis><Superscript><Emphasis Type="Italic">NF4050</Emphasis>-<Emphasis Type="Italic">8</Emphasis></Superscript> that confers blast resistance in rice

We evaluated a large collection of Tos17 mutant panel lines for their reaction to three different races of Magnaporthe oryzae and identified a lesion mimic mutant, NF4050-8, that showed lesions similar to naturally occurring spl5 mutant and enhanced resistance to all the three blast races tested. Nested modified-AFLP using Tos17-specific primers and southern hybridization experiments of segregating individuals indicated that the lesion mimic phenotype in NF4050-8 is most likely due to a nucleotide change acquired during the culturing process and not due to Tos17 insertion per se. Inheritance and genetic analyses in two japonica × indica populations identified an overlapping genomic region of 13 cM on short arm of chromosome 7 that was linked with the lesion mimic phenotype. High-resolution genetic mapping using 950 F₃ and 3,821 F₄ plants of NF4050-8 × CO39 delimited a 35 kb region flanked by NBARC1 (5.262 Mb) and RM8262 (5.297 Mb), which contained 6 ORFs; 3 of them were ‘resistance gene related’ with typical NBS–LRR signatures. One of them harbored a NB–ARC domain, which had been previously demonstrated to be associated with cell death in animals. Microarray analysis of NF4050-8 revealed significant up-regulation of numerous defense/pathogenesis-related genes and down-regulation of heme peroxidase genes. Real-time PCR analysis of WRKY45 and PR1b genes suggested possible constitutive activation of a defense signaling pathway downstream of salicylic acid but independent of NH1 in these mutant lines of rice.     

Primary carcinoma of the gallbladder; review of 173 cases

One hundred seventy-three cases of primary carcinoma of the gallbladder were analyzed. In the group studied they made 2.11 per cent of all malignant tumors found at autopsy and were found in 1.89 per cent of all cases in which operation was done on the biliary tract. There was no appreciable change in the incidence of this tumor at autopsy during the period studied (1918-1948) at the Los Angeles County Hospital. Sixty-eight per cent of the cases were in females. A particularly high incidence was noted in Mexican females. Upper abdominal pain, loss of weight, nausea and vomiting, jaundice, and palpable mass or enlarged liver were the most common clinical features. Approximately one-third of the patients in whom the lesion was found at operation and one-fifth of all the patients whose records were studied had a history of chronic gallbladder disease. All but two of the 38 patients operated on were dead or had clinical recurrence within two years. One was alive and well 12 years after cholecystectomy. The most common gross appearance, particularly at autopsy, was a large tumor mass replacing the gallbladder and radiating to nearby organs, particularly the liver. In about one-third of the cases the tumor was grossly limited to the gallbladder. Polypoid tumors occurred in only about 10 per cent of the cases and most of the tumors were diffusely growing adenocarcinoma. Perforation appeared in nine cases, usually with fistula to the gastrointestinal tract. All of the tumors were histologically adenocarcinoma, usually of simple glandular structure. No purely squamous cell growth occurred. Gallstones were found in 79.8 per cent of the cases.     

PRIMARY CARCINOMA OF THE GALLBLADDER—Review of 173 Cases

One hundred seventy-three cases of primary carcinoma of the gallbladder were analyzed. In the group studied they made 2.11 per cent of all malignant tumors found at autopsy and were found in 1.89 per cent of all cases in which operation was done on the biliary tract. There was no appreciable change in the incidence of this tumor at autopsy during the period studied (1918-1948) at the Los Angeles County Hospital. Sixty-eight per cent of the cases were in females. A particularly high incidence was noted in Mexican females.Upper abdominal pain, loss of weight, nausea and vomiting, jaundice, and palpable mass or enlarged liver were the most common clinical features. Approximately one-third of the patients in whom the lesion was found at operation and one-fifth of all the patients whose records were studied had a history of chronic gallbladder disease.All but two of the 38 patients operated on were dead or had clinical recurrence within two years. One was alive and well 12 years after cholecystectomy.The most common gross appearance, particularly at autopsy, was a large tumor mass replacing the gallbladder and radiating to nearby organs, particularly the liver. In about one-third of the cases the tumor was grossly limited to the gallbladder. Polypoid tumors occurred in only about 10 per cent of the cases and most of the tumors were diffusely growing adenocarcinoma. Perforation appeared in nine cases, usually with fistula to the gastrointestinal tract. All of the tumors were histologically adenocarcinoma, usually of simple glandular structure. No purely squamous cell growth occurred.Gallstones were found in 79.8 per cent of the cases.