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Cervical tumors of thymic origin in children
INTERNATIONAL ABSTRACTS
m a g n a (the banana sign). In a further eight cases the cerebellum was not displayed. None of these changes was seen in 100 patients presenting consecutively for routine ultrasound examination in the second trimester.--Prem Purl Cerebrospinal Fluid Hydrodynamic Studies in Children. K. Haus, S. Blomquist, S. Sundin, et al. J Neurol Neurosurg Psych 49:536-548, (May), 1986.
Children with suspected disturbances of C S F hydrodynamics were studied with the constant pressure incision method of Ekstedt. The test requires that the child lie still in the supine position for 34 hours. Three to five measurements were performed in 232 children. The authors conclude that the testing C S F pressure is 1.3 KPa, sagittal sinus pressure is 1.1 KPa, pressure difference across the arachnoid villi is 0.5 KPa, and C S F production rate is 6.8 mm3s -1. Meningitis developed in four children.--R.J. Brereton
469
were white. Eighteen patients had Dukes' stage C disease, with median survival of 7 months and one patient alive 12 months after diagnosis. Three patients had Dukes' stage B disease, with two patients surviving 16 months each and one presently alive 7 months after diagnosis. Only one patient had Dukes' stage A disease and is alive 121 months after diagnosis. Large bowel carcinoma in children is predominantly mucinous adenocarcinoma and occurs in the adolescent age group. Tumor distribution is fairly even throughout the large bowel, and all subtypes of the tumor are positive for carcinoembryonic antigen (CEA) on immunohistologic study. Active tumor and tumor regrowth are always accompanied by high serum levels of CEA. The tumor has no predilection for sex, but is significantly more frequent in blacks (P < .05). Discovery of the tumor in an early stage improves the prognosis.--George Holcomb, Jr Pancreoblastoma in e Neonate Associated W i t h Beckwith-Wiedemann-Syndrome. S.R. Potts, S. Brown, and M.D. O'Hara. Z
Kinderchir 41:56-57, (February), 1986. NEOPLASMS Gastrointestinal Malignancies in Infancy, Childhood, and Adolescence. J.F. Goldthorn and P.C. Canizaro. Surg Clin North A m
66:845-861, (August), 1986. The authors provide a review of cancers that occur in the gastrointestinal tract and in the hepatobiliary system. Less than 5% of benign and malignant tumors in the pediatric age group arise from the gastrointestinal tract. Adenocarcinoma of the colon and rectum is the most common gastrointestinal carcinoma in childhood, although only eight persons per million will develop it in the second decade of life and even fewer during the first decade. Up to 50% of juvenile colorectal malignancies are mucin-secreting adenocarcinomas, which are rapidly advancing and have a poor prognosis. Malignancies in the remaining gastrointestinal tract, including the esophagus, stomach, and small bowel are extremely rare. NonHodgkin's lymphoma is the most common malignancy of the small intestine in children, and the distal ileum is the most common site of involvement. Malignancies of the gallbladder, common and hepatic ducts, and pancreas are exceedingly rare in the pediatric age group. Embryonal rhabdomyosarcoma or sarcoma botryoides of the biliary tract is a unique tumor of the biliary tract in children that carries a very poor prognosis. The best treatment is aggressive surgical extirpation, which involves complete incision of the extrahepatic biliary tree and subsequent reconstruction with a Roux-en-Y portoenterostomy. The two most commonly diagnosed hepatic malignancies in children are hepatoblastomas and hepatocellular carcinomas. Hepatoblastomas occur primarily in infancy and are rarely seen after 3 years of age. Hepatocellular carcinomas occur in children below 4 years of age or in early adolescence. The principle treatment of primary liver malignancies is surgical resection. The use of multi-drug chemotherapy has been shown to convert large tumors into ones that are more easily resectable. There are no data to indicate that adjunctive therapy will result in cure if a total excision cannot be obtained.--Richard R. Ricketts Cancer of the Large Bowel in Children. S.G. Koh and W.W. Johnson. South Med J 79:931-935, (August), 1986.
The authors present the morphologic and clinical features of large bowel carcinoma in 22 patients seen over 18 years at St Jude Children's Research Hospital. Eighteen cases of mucinous adenocarcinoma, three of well-differentiated adenocarcinoma, and one case of poorly differentiated adenocarcinoma were seen. The patients ranged in age from 9 to 19 years with a median age of 15. There were 12 female and ten male patients; 12 were black and ten
A cystic abdominal mass was removed from a male child born at 32 weeks gestation. Histologic examination revealed a pancreoblastoma. Following ventilatory support for six days, the postoperative course was uneventful. Biochemistry, including blood glucose levels, were at all times normal. Both clinical and histologic features of Beckwith-Wiedemann syndrome developed during the first 12 weeks of life. The child remained well and thriving with no tumor recurrence at 10 months of age. The association between pancreoblastoma and Beckwith-Wiedemann syndrome has not been reported previously. Unlike many pancreatic tumors, prognosis for pancreoblastoma is usually good and complete surgical excision can be expected to be curative.--Thomas A. Angerpointner Testieular Tumors W i t h Peutz-Jeghers Syndrome. D.M. Wilson,
W.C. Pitts, R.L. Hintz, et al. Cancer 57:2238-2240, (June), 1986. Peutz-Jeghers syndrome consists of mucocutaneous pigmentation and intestinal polyposis. Females with this syndrome are known to have an increased incidence of gonadal tumors, notably an unusual ovarian tumor described as a "sex cord tumor" with annular tubules (Scully). These tumors may be bilateral and multifocal. This report describes the third male with Peutz-Jeghers syndrome who has been found to have testicular tumors. The 6-year-old patient described had bilateral multifocal testicular tumors of a Sertoli cell type. These tumors were believed to be benign, and the testicles were not removed. Estradiol levels have been intermittently elevated. D.M. Hays Cervical Tumors of Thymic Origin in Children. G. Vanneuville, J.M.
Mondie, D. Marouby, et al. Chir P6diatr 26:104-106, (March), 1985. The authors report two new cases of right cervical tumors without any specific clinical symptoms. Pathologic examination showed aberrant cervical thymus. Surgical removal was performed and follow-up was uneventful. There was neither myasthenia nor immunologic deficit. The literature was reviewed and 70 cases found. The condition occurs more often in boys and on the left side. A complete excision must be performed.--J.M. Guys Urolithiasis in Childhood Acute Leukemia and Nonhodgkin's Lym-
phoma. C. Pui, S. Roy, III, and H.N. Noe. J Urol 136:1052-1054, (November), 1986.
Ureteral calculi were found in five children who were receiving or had recently completed remission induction therapy for acute leukemia or lymphoma. All five patients had abdominal or back pain and
m a g n a (the banana sign). In a further eight cases the cerebellum was not displayed. None of these changes was seen in 100 patients presenting consecutively for routine ultrasound examination in the second trimester.--Prem Purl Cerebrospinal Fluid Hydrodynamic Studies in Children. K. Haus, S. Blomquist, S. Sundin, et al. J Neurol Neurosurg Psych 49:536-548, (May), 1986.
Children with suspected disturbances of C S F hydrodynamics were studied with the constant pressure incision method of Ekstedt. The test requires that the child lie still in the supine position for 34 hours. Three to five measurements were performed in 232 children. The authors conclude that the testing C S F pressure is 1.3 KPa, sagittal sinus pressure is 1.1 KPa, pressure difference across the arachnoid villi is 0.5 KPa, and C S F production rate is 6.8 mm3s -1. Meningitis developed in four children.--R.J. Brereton
469
were white. Eighteen patients had Dukes' stage C disease, with median survival of 7 months and one patient alive 12 months after diagnosis. Three patients had Dukes' stage B disease, with two patients surviving 16 months each and one presently alive 7 months after diagnosis. Only one patient had Dukes' stage A disease and is alive 121 months after diagnosis. Large bowel carcinoma in children is predominantly mucinous adenocarcinoma and occurs in the adolescent age group. Tumor distribution is fairly even throughout the large bowel, and all subtypes of the tumor are positive for carcinoembryonic antigen (CEA) on immunohistologic study. Active tumor and tumor regrowth are always accompanied by high serum levels of CEA. The tumor has no predilection for sex, but is significantly more frequent in blacks (P < .05). Discovery of the tumor in an early stage improves the prognosis.--George Holcomb, Jr Pancreoblastoma in e Neonate Associated W i t h Beckwith-Wiedemann-Syndrome. S.R. Potts, S. Brown, and M.D. O'Hara. Z
Kinderchir 41:56-57, (February), 1986. NEOPLASMS Gastrointestinal Malignancies in Infancy, Childhood, and Adolescence. J.F. Goldthorn and P.C. Canizaro. Surg Clin North A m
66:845-861, (August), 1986. The authors provide a review of cancers that occur in the gastrointestinal tract and in the hepatobiliary system. Less than 5% of benign and malignant tumors in the pediatric age group arise from the gastrointestinal tract. Adenocarcinoma of the colon and rectum is the most common gastrointestinal carcinoma in childhood, although only eight persons per million will develop it in the second decade of life and even fewer during the first decade. Up to 50% of juvenile colorectal malignancies are mucin-secreting adenocarcinomas, which are rapidly advancing and have a poor prognosis. Malignancies in the remaining gastrointestinal tract, including the esophagus, stomach, and small bowel are extremely rare. NonHodgkin's lymphoma is the most common malignancy of the small intestine in children, and the distal ileum is the most common site of involvement. Malignancies of the gallbladder, common and hepatic ducts, and pancreas are exceedingly rare in the pediatric age group. Embryonal rhabdomyosarcoma or sarcoma botryoides of the biliary tract is a unique tumor of the biliary tract in children that carries a very poor prognosis. The best treatment is aggressive surgical extirpation, which involves complete incision of the extrahepatic biliary tree and subsequent reconstruction with a Roux-en-Y portoenterostomy. The two most commonly diagnosed hepatic malignancies in children are hepatoblastomas and hepatocellular carcinomas. Hepatoblastomas occur primarily in infancy and are rarely seen after 3 years of age. Hepatocellular carcinomas occur in children below 4 years of age or in early adolescence. The principle treatment of primary liver malignancies is surgical resection. The use of multi-drug chemotherapy has been shown to convert large tumors into ones that are more easily resectable. There are no data to indicate that adjunctive therapy will result in cure if a total excision cannot be obtained.--Richard R. Ricketts Cancer of the Large Bowel in Children. S.G. Koh and W.W. Johnson. South Med J 79:931-935, (August), 1986.
The authors present the morphologic and clinical features of large bowel carcinoma in 22 patients seen over 18 years at St Jude Children's Research Hospital. Eighteen cases of mucinous adenocarcinoma, three of well-differentiated adenocarcinoma, and one case of poorly differentiated adenocarcinoma were seen. The patients ranged in age from 9 to 19 years with a median age of 15. There were 12 female and ten male patients; 12 were black and ten
A cystic abdominal mass was removed from a male child born at 32 weeks gestation. Histologic examination revealed a pancreoblastoma. Following ventilatory support for six days, the postoperative course was uneventful. Biochemistry, including blood glucose levels, were at all times normal. Both clinical and histologic features of Beckwith-Wiedemann syndrome developed during the first 12 weeks of life. The child remained well and thriving with no tumor recurrence at 10 months of age. The association between pancreoblastoma and Beckwith-Wiedemann syndrome has not been reported previously. Unlike many pancreatic tumors, prognosis for pancreoblastoma is usually good and complete surgical excision can be expected to be curative.--Thomas A. Angerpointner Testieular Tumors W i t h Peutz-Jeghers Syndrome. D.M. Wilson,
W.C. Pitts, R.L. Hintz, et al. Cancer 57:2238-2240, (June), 1986. Peutz-Jeghers syndrome consists of mucocutaneous pigmentation and intestinal polyposis. Females with this syndrome are known to have an increased incidence of gonadal tumors, notably an unusual ovarian tumor described as a "sex cord tumor" with annular tubules (Scully). These tumors may be bilateral and multifocal. This report describes the third male with Peutz-Jeghers syndrome who has been found to have testicular tumors. The 6-year-old patient described had bilateral multifocal testicular tumors of a Sertoli cell type. These tumors were believed to be benign, and the testicles were not removed. Estradiol levels have been intermittently elevated. D.M. Hays Cervical Tumors of Thymic Origin in Children. G. Vanneuville, J.M.
Mondie, D. Marouby, et al. Chir P6diatr 26:104-106, (March), 1985. The authors report two new cases of right cervical tumors without any specific clinical symptoms. Pathologic examination showed aberrant cervical thymus. Surgical removal was performed and follow-up was uneventful. There was neither myasthenia nor immunologic deficit. The literature was reviewed and 70 cases found. The condition occurs more often in boys and on the left side. A complete excision must be performed.--J.M. Guys Urolithiasis in Childhood Acute Leukemia and Nonhodgkin's Lym-
phoma. C. Pui, S. Roy, III, and H.N. Noe. J Urol 136:1052-1054, (November), 1986.
Ureteral calculi were found in five children who were receiving or had recently completed remission induction therapy for acute leukemia or lymphoma. All five patients had abdominal or back pain and