360
Letters to the Editor
The Journal of Pediatrics February 1976
clustering. A ease-control study should be made to determine the clinical significance of the nevus sebaceous of Jadassohn in relationship to malignancy in children.
Philip Lanzkowsky, M.D., F.R.C.P., D.C.H. Ashok Shende, M.B., B.S. Division of Pediatric Hematology Oncology Department of Pediatrics Long Island Jewish-Hillside Medical Center New Hyde Park, N. Y. 11040 Department of Pediatrics of the School of Medicine of the Health Sciences Center of the State University of New York at Stony Brook REFERENCES
1.
Fig. 1. Typical scalp lesion in nevus sebaceous of Jadassohn. children were born with a circumscribed, rounded, slightly elevated plaque on the scalp with overlying alopecia. Excisional biopsy of the scalp lesion in each case confirmed the diagnosis of nevus sebaceous of Jadassohn. Fig. 1 shows the typical scalp lesion. Nevus sebaceous o f Jadassohn, most commonly found on the scalp or face, is a hamartoma of epithelial germ cells of ectodermal origin that possess potential to develop into sebaceous or apocrine glands. The natural history of the nevus consists of three phases of evolution.' During infancy and childhood it consists of a circumscribed, slightly raised, waxy, hairless plaque; histopathologically there are underdeveloped or abortive sebaceous glands and hair follicles. At puberty the lesion becomes verrucous and nodular, and the histopathologic change is that of papillomatous epidermal hyperplasia; there are also numerous mature sebaceous and apocrine glands with absence of hair follicles. During the postpuberty period the lesion is complicated by transformation into benign or malignant nevoid tumors, which include basal cell epithelioma, syringoadenoma papilliferum, solid hidradenoma, infundibuloma, sebaceous epithelioma, apocrine cystadenoma, keratocanthoma, sebaceous carcinoma, bilateral salivary gland adenocarcinoma, and rarely squamous cell carcinoma. Because of these complications the recommended mode of therapy is excision of the lesion through full thickness of the skin. The local oncogenic potential of nevus sebaceous of J adassohn has been well documented in the literature, but its association, if any, with other childhood malignancies has not hitherto been described. Since the frequency of nevus sebaceous of Jadassohn in the general population is not known, the association o f this nevus with other malignancies in children might be due to chance
Miller RW: Relation between cancer and congenital defects: An epidemiologic evaluation, J Natl Cancer Inst 40:1079, 1968. 2. Heath CW Jr, Manning MD, and Zelkowitz L: Case clusters in the occurrence of leukemia and congenital malformations, Lancet 2:136, 1964. 3. Lanzkowsky P: Absence of pectoralis major muscle in association with acute leukemia, J PEDIATR 86:817, 1975. 4. Mehregan AM and Pinkus M: Life history of organoid nevus, Arch Dermatol 91:574, 1965.
Diagnostic value of scintigraphy of liver in hydatid disease in children To the Editor: We present a summary of our experience in the diagnosis of liver hydatid disease with colloid liver scintigraphy and blood pool liver scintigraphy. MATERIALS
AND METHODS
From 1966 a study was carried out on 143 children 14 years of age or less in whom hydatid disease of the liver was suspected. All patients had colloid liver scintigraphy. For most of them the radioactive pharmaceutical was indium 113m colloid. In a few patients techneticum 99m sulfur colloid and gold 198 colloid were used. From 1972 every patient with a space-occupying lesion in the liver had blood pool scintigraphy of the liver in order to establish the degree of vascularization of the cold area disclosed earlier by the colloid scan. In this paper we included the first 13 cases studied in this way. The radiopharmaceutical used was indium 113m in hydrochloride solution. All patients with a positive diagnosis of liver hydatid cyst were treated surgically. The remaining children have been followed to date. RESULTS In 65 patients the colloid liver scintigraphy detected the spaceoccupying lesion and in 78 patients the possibility of a spaceoccupying lesion was eliminated.
Volume 88 Number 2
In 52 patients who had only colloid scintigraphy, hydatid cyst was diagnosed on the basis of one or more cold areas, spherical in shape, in children with a clinical history of possible hepatic infestation and positive immunologic tests. The smallest cyst diagnosed measured 3 cm. The liver hydatid disease was in all instances confirmed by surgery. In 13 patients who had blood pool liver scintigraphy, the images of blood pool were interpreted by comparing the radioactivity in the cold area, identified earlier by the colloid scan, with the radioactivity on a similar area of normal liver parenchyma. O f the 13 patients studied, ten children had one cyst, two had two cysts, and one had five cysts. In total we studied the blood pool of 19 cysts. Eighteen hydatid cysts failed to concentrate radioactivity, an indication of absence of vascularization. One hydatid cyst in a 7-year-old girl exhibited radioactivity, but less than that in the surrounding normal parenchyma; a few hours earlier she had suffered abdominal trauma. The colloid scan identified two cold areas, whereas the blood pool scan detected absence o f radioactivity in one and some radioactivity in the other one, but less than that in a similar area of normal parenchyma. By surgical exploration it was found that the latter corresponded to a hydatid cyst with intracystic hemorrhage. COMMENT In our experience, colloid scan was 100% effective in confirming or eliminating the existence of a space-occupying lesion in the liver of children suspected of hydatid disease. Blood pool liver scintigraphy showed that the cold area produced by hydatid cyst in the colloid scan failed to exhibit blood pool radioactivity as being clearly different from areas occupied by primary or metastatic neoplasms. Exceptionaiiy, the traumatic rupture of the cyst led to a false negative result. The activity from the radiopharmaceutical agent which accumulated within the blood of the cystic cavity and in the surrounding inflammatory tissues was interpreted as the blood pool in a vascularized lesion.
Letters to the Editor
36 1
Conventional colloid liver scintigraphy and blood pool liver scintigraphy, owing to their effectiveness and harmlessness, have proved to be procedures of choice for the diagnosis of liver hydatid disease in children. 1-5 AIvaro Osorio, M.D. Pediatric Department School of Medicine Montevideo, Uruguay Radioisotopist M.D. Centro de Medicina Nuclear Antonio Pitez, M.D. Rodolfo Ferando, M.D. Eduardo Touya, M.D. Radioisotopist M.D. Centro de Medieina Nuelear REFERENCES
1. Osorio A: La gammagrafia hepfitica en pediatria. Su utilidad, indicaciones e interpretacidn, Tesis de Doctorado, 1967, Fac Med, Montevideo, Uruguay. 2. Osorio A, and Touya (h) JJ: La gammagrafia en la hidatidosis hepfitica del nino, Arch Pediatr Uruguay 39:189, 1968. 3. Touya (h) JJ, Osorio A, Touya E, Bekerman C, Paez A, and Ferrari M: Scintigraphy of the liver, lungs, spleen, kidneys, brain, heart and bones in the diagnosis of hydatid cysts, in Medical radioisotope scintigraphy. A symposium, vol 2, Ed. I.A.E.A., Vienna, 1969, p 459. 4. Lubin E, and Lewitus Z: Blood pool scanning in investigating hepatic mass lesions, Sem Nucl Med 2:128, 1972. 5. Webster EW, Alpert NM, and Brownell GL: Radiation doses in pediatric nuclear medicine and diagnostic X-ray procedures, in James AE, Wagner HN, and Cooke RE, editors: Pediatric nuclear medicine, Philadelphia, 1974, WB Saunders Company, chapter 1, p 34.
Letters to the Editor
The Journal of Pediatrics February 1976
clustering. A ease-control study should be made to determine the clinical significance of the nevus sebaceous of Jadassohn in relationship to malignancy in children.
Philip Lanzkowsky, M.D., F.R.C.P., D.C.H. Ashok Shende, M.B., B.S. Division of Pediatric Hematology Oncology Department of Pediatrics Long Island Jewish-Hillside Medical Center New Hyde Park, N. Y. 11040 Department of Pediatrics of the School of Medicine of the Health Sciences Center of the State University of New York at Stony Brook REFERENCES
1.
Fig. 1. Typical scalp lesion in nevus sebaceous of Jadassohn. children were born with a circumscribed, rounded, slightly elevated plaque on the scalp with overlying alopecia. Excisional biopsy of the scalp lesion in each case confirmed the diagnosis of nevus sebaceous of Jadassohn. Fig. 1 shows the typical scalp lesion. Nevus sebaceous o f Jadassohn, most commonly found on the scalp or face, is a hamartoma of epithelial germ cells of ectodermal origin that possess potential to develop into sebaceous or apocrine glands. The natural history of the nevus consists of three phases of evolution.' During infancy and childhood it consists of a circumscribed, slightly raised, waxy, hairless plaque; histopathologically there are underdeveloped or abortive sebaceous glands and hair follicles. At puberty the lesion becomes verrucous and nodular, and the histopathologic change is that of papillomatous epidermal hyperplasia; there are also numerous mature sebaceous and apocrine glands with absence of hair follicles. During the postpuberty period the lesion is complicated by transformation into benign or malignant nevoid tumors, which include basal cell epithelioma, syringoadenoma papilliferum, solid hidradenoma, infundibuloma, sebaceous epithelioma, apocrine cystadenoma, keratocanthoma, sebaceous carcinoma, bilateral salivary gland adenocarcinoma, and rarely squamous cell carcinoma. Because of these complications the recommended mode of therapy is excision of the lesion through full thickness of the skin. The local oncogenic potential of nevus sebaceous of J adassohn has been well documented in the literature, but its association, if any, with other childhood malignancies has not hitherto been described. Since the frequency of nevus sebaceous of Jadassohn in the general population is not known, the association o f this nevus with other malignancies in children might be due to chance
Miller RW: Relation between cancer and congenital defects: An epidemiologic evaluation, J Natl Cancer Inst 40:1079, 1968. 2. Heath CW Jr, Manning MD, and Zelkowitz L: Case clusters in the occurrence of leukemia and congenital malformations, Lancet 2:136, 1964. 3. Lanzkowsky P: Absence of pectoralis major muscle in association with acute leukemia, J PEDIATR 86:817, 1975. 4. Mehregan AM and Pinkus M: Life history of organoid nevus, Arch Dermatol 91:574, 1965.
Diagnostic value of scintigraphy of liver in hydatid disease in children To the Editor: We present a summary of our experience in the diagnosis of liver hydatid disease with colloid liver scintigraphy and blood pool liver scintigraphy. MATERIALS
AND METHODS
From 1966 a study was carried out on 143 children 14 years of age or less in whom hydatid disease of the liver was suspected. All patients had colloid liver scintigraphy. For most of them the radioactive pharmaceutical was indium 113m colloid. In a few patients techneticum 99m sulfur colloid and gold 198 colloid were used. From 1972 every patient with a space-occupying lesion in the liver had blood pool scintigraphy of the liver in order to establish the degree of vascularization of the cold area disclosed earlier by the colloid scan. In this paper we included the first 13 cases studied in this way. The radiopharmaceutical used was indium 113m in hydrochloride solution. All patients with a positive diagnosis of liver hydatid cyst were treated surgically. The remaining children have been followed to date. RESULTS In 65 patients the colloid liver scintigraphy detected the spaceoccupying lesion and in 78 patients the possibility of a spaceoccupying lesion was eliminated.
Volume 88 Number 2
In 52 patients who had only colloid scintigraphy, hydatid cyst was diagnosed on the basis of one or more cold areas, spherical in shape, in children with a clinical history of possible hepatic infestation and positive immunologic tests. The smallest cyst diagnosed measured 3 cm. The liver hydatid disease was in all instances confirmed by surgery. In 13 patients who had blood pool liver scintigraphy, the images of blood pool were interpreted by comparing the radioactivity in the cold area, identified earlier by the colloid scan, with the radioactivity on a similar area of normal liver parenchyma. O f the 13 patients studied, ten children had one cyst, two had two cysts, and one had five cysts. In total we studied the blood pool of 19 cysts. Eighteen hydatid cysts failed to concentrate radioactivity, an indication of absence of vascularization. One hydatid cyst in a 7-year-old girl exhibited radioactivity, but less than that in the surrounding normal parenchyma; a few hours earlier she had suffered abdominal trauma. The colloid scan identified two cold areas, whereas the blood pool scan detected absence o f radioactivity in one and some radioactivity in the other one, but less than that in a similar area of normal parenchyma. By surgical exploration it was found that the latter corresponded to a hydatid cyst with intracystic hemorrhage. COMMENT In our experience, colloid scan was 100% effective in confirming or eliminating the existence of a space-occupying lesion in the liver of children suspected of hydatid disease. Blood pool liver scintigraphy showed that the cold area produced by hydatid cyst in the colloid scan failed to exhibit blood pool radioactivity as being clearly different from areas occupied by primary or metastatic neoplasms. Exceptionaiiy, the traumatic rupture of the cyst led to a false negative result. The activity from the radiopharmaceutical agent which accumulated within the blood of the cystic cavity and in the surrounding inflammatory tissues was interpreted as the blood pool in a vascularized lesion.
Letters to the Editor
36 1
Conventional colloid liver scintigraphy and blood pool liver scintigraphy, owing to their effectiveness and harmlessness, have proved to be procedures of choice for the diagnosis of liver hydatid disease in children. 1-5 AIvaro Osorio, M.D. Pediatric Department School of Medicine Montevideo, Uruguay Radioisotopist M.D. Centro de Medicina Nuclear Antonio Pitez, M.D. Rodolfo Ferando, M.D. Eduardo Touya, M.D. Radioisotopist M.D. Centro de Medieina Nuelear REFERENCES
1. Osorio A: La gammagrafia hepfitica en pediatria. Su utilidad, indicaciones e interpretacidn, Tesis de Doctorado, 1967, Fac Med, Montevideo, Uruguay. 2. Osorio A, and Touya (h) JJ: La gammagrafia en la hidatidosis hepfitica del nino, Arch Pediatr Uruguay 39:189, 1968. 3. Touya (h) JJ, Osorio A, Touya E, Bekerman C, Paez A, and Ferrari M: Scintigraphy of the liver, lungs, spleen, kidneys, brain, heart and bones in the diagnosis of hydatid cysts, in Medical radioisotope scintigraphy. A symposium, vol 2, Ed. I.A.E.A., Vienna, 1969, p 459. 4. Lubin E, and Lewitus Z: Blood pool scanning in investigating hepatic mass lesions, Sem Nucl Med 2:128, 1972. 5. Webster EW, Alpert NM, and Brownell GL: Radiation doses in pediatric nuclear medicine and diagnostic X-ray procedures, in James AE, Wagner HN, and Cooke RE, editors: Pediatric nuclear medicine, Philadelphia, 1974, WB Saunders Company, chapter 1, p 34.
