Hemihypertrophy and Wilms's Tumour

Björklund, Stig-Ivar

doi:10.1111/j.1651-2227.1955.tb04141.x

Cited by 22 publications

(2 citation statements)

References 6 publications

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“…While numerous reports on genetic abnormalities in IH cases were published, additional assessment of other family members to rule out any kind of body asymmetry may be highly informative because hereditary basis of the disorder, the instability of the 11p15.5 chromosomal region, is very well established 1–5,8–10. A close connection between IH and Wilms tumor (nephroblastoma, a common pediatric neoplasm of the kidney) was postulated already in the 1950s and was confirmed in more recent publications 13,14. While the overall median age for Wilms tumor is 3.5 years, adolescents and even adults also can be affected 15,16.…”

Section: Resultsmentioning

confidence: 89%

Screening Procedure for Hemihypertrophy: Preliminary Results of International Multicenter Prospective Study

Vaiman

Shilco²,

Roitblat

et al. 2019

cajgh

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Introduction: Isolated or congenital hemihypertrophy is a rare disorder characterized by asymmetric overgrowth of one side of the body. This article describes the protocol and preliminary results of a lateral body asymmetry (hemihypertrophy) screening procedure performed in healthy adolescents in a multicenter study. The reported incidence of hemihypertrophy varies between different publications and standardized protocols are needed to improve research in this area.Methods: Our screening program is taking place in Australia, Israel, Mexico, Ukraine and USA. Procedure includes two steps: (1) “three measurements – three questions” screening, or assessment of face, palms, and shins; (2) in-depth assessment of selected cases in order to exclude localized, lesional, and syndrome-related cases as well as body asymmetry within normative range and to select suspected cases of isolated hemihypertrophy. This step includes measurements of various anatomical regions and a detailed questionnaire.Results: At this stage, the screening procedure is completed and the selected participants are advised to refer to medical institutions for further clinical and genetic follow up to exclude possible tumors and other accompanying disorders.Conclusion: We present an easy-to-use selection tool to identify children with suspected IH, which results in the selection of the risk group that may benefit from referral to a pediatrician and a clinical geneticist.

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Section: Resultsmentioning

confidence: 89%

Screening Procedure for Hemihypertrophy: Preliminary Results of International Multicenter Prospective Study

Vaiman

Shilco²,

Roitblat

et al. 2019

cajgh

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“…Careful review of all titles and abstracts of these references yielded 35 original case reports and/or case series of subjects with Wilms tumor and hemihypertrophy or original case series with a minimum of three subjects with hemihypertrophy which met inclusion criteria. After eliminating subjects meeting the exclusion criteria (Table 2), 21 original case reports or case series representing 58 appropriate subjects remained for systematic analysis (Table 3) (2,13–32).…”

Section: Resultsmentioning

confidence: 99%

Presence of Vascular Anomalies with Congenital Hemihypertrophy and Wilms Tumor: An Evidence‐Based Evaluation

Kundu

Frieden

2003

Pediatric Dermatology

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Congenital hemihypertrophy is an uncommon condition of unknown etiology characterized by unilateral overgrowth of part or all of one side of the body. Hemihypertrophy is known to be associated with certain childhood tumors, most notably Wilms tumor (or nephroblastoma), and for this reason infants with hemihypertrophy are often followed with serial abdominal ultrasounds. Klippel-Trénaunay syndrome (KTS) is the triad of port-wine stain, venous varicosities, and soft tissue and/or bony hypertrophy. Children with KTS typically have localized rather than generalized hemihypertrophy, but occasionally the hypertrophy is more extensive than the vascular anomaly itself. Information is lacking about whether hemihypertrophy in this setting can also be a risk factor for Wilms tumor. We systematically reviewed the medical literature to determine whether well-documented cases of Wilms tumor in the setting of both hemihypertrophy and vascular anomalies have been described, and if found, whether the association was sufficiently frequent that routine screening for Wilms tumor in this setting should be recommended. A review of case reports and case series in the pediatric population was undertaken using specific inclusion and exclusion criteria. We found 4 of 58 subjects with hemihypertrophy and Wilms tumor had a reported vascular anomaly, but in only one case was a clear-cut diagnosis of KTS confirmed. The relationship of the other three vascular anomalies reported was of uncertain significance. In conclusion, our review suggests that the risk of Wilms tumor in the setting of localized soft-tissue hypertrophy in conjunction with a vascular malformation is quite low. More extensive hemihypertrophy extending to body sites remote from the vascular malformation itself could have a higher risk of Wilms tumor, although the magnitude of this risk is uncertain. Our findings suggest that routine serial abdominal ultrasounds in patients with vascular malformations in association with localized soft-tissue hypertrophy are unwarranted.

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Syndrome of Congenital Hemihypertrophy and Elevated Urinary Gonadotropins

Silver¹,

Gruskay²

1957

AMA Am J Dis Child

138

134

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Minor differences in size between the two sides of the body are common and usually have no significance. However, severe disproportion is relatively rare and there are less than 130 cases reported in the literature.In 1953 Silver and co-workers1 described a syndrome of congenital hemihypertrophy and elevated excretion of urinary gonadotropins in two children who failed to show any other evidence of apparent endocrine disturbance. The purpose of this paper is to present the third case of this syndrome in a 7-year-old boy. Report of CaseA boy was first seen in the New Haven Hospital Pediatric Out-Patient Clinic at the age of 10 weeks because of a difference in size of his legs. He had been born at term following an entirely uneventful pregnancy and had been delivered by Caesarian section. Birth weight was 3100 gm. (6 lb. 12\m=1/2\oz.). Because he was cyanotic at birth, he had been given supplemental oxygen and after 24 hours appeared entirely normal.When he was about 2 months old his mother first noted a difference in the size of his legs and brought him to the clinic. On physical examination the only positive findings were a diffuse enlargement of the left buttock, leg, and foot, the presence of a small café-au-lait spot on the left lower back and two small capillary hemangiomas on the right upper lid and the glabella. Both arms appeared to be equal in size.At the age of 10 months he was admitted to the New Haven Hospital for diagnostic studies to determine the cause of the enlargement of the left leg. At that time the left leg measured 2 cm. longer than the right, the left foot 1 cm. longer than the right, and the circum¬ ference of the left thigh 2.5 cm. greater than the right. He did not use the enlarged limb as actively as the other but muscular power seemed to be equal in both. No difference in temperature or color of the legs was noted. Femoral pulsations were strong and equal, and no bruit was heard. Roentgenograms of the lower extremities revealed diffuse enlarge¬ ment of the bones and soft tissues on the left, but these structures were otherwise normal.At operation oxygen saturation was found to be the same in blood obtained from both femoral veins. Exploration of the subcutaneous tissue of the left leg revealed apparently nor¬ mal blood vessels and lymphatics. No arterio¬ venous aneurysm was demonstrated. A biopsy of skin and subcutaneous tissue was interpreted as showing normal skin with slight edema of the subcutaneous tissue.The patient was next seen in the Out-Patient Clinic at the age of 7 10/12 years. His mother reported that he had grown and developed normally. He had full use of his legs and was able to walk, run, and hop in a normal manner even when wearing regular shoes. Review of systems was not contributory. He weighed 24.5 kg. (54 lb.) and was 122 cm. (48 in.) tall. The capillary hemangiomas on the right upper lid and the brow were no longer visible. The café-au-lait spot on the left lower back measured 2.7 by 1.4 cm. It had an irregular border, and four 1 mm. darker brown areas Downloaded F...

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Hemihypertrophy and Wilms's Tumour

Cited by 22 publications

References 6 publications

Screening Procedure for Hemihypertrophy: Preliminary Results of International Multicenter Prospective Study

Screening Procedure for Hemihypertrophy: Preliminary Results of International Multicenter Prospective Study

Presence of Vascular Anomalies with Congenital Hemihypertrophy and Wilms Tumor: An Evidence‐Based Evaluation

Syndrome of Congenital Hemihypertrophy and Elevated Urinary Gonadotropins

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