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Bile duct carcinoma in an adolescent
INTERNATIONAL ABSTRACTS
gallbladder wall. At laparotomy, which was performed with the tentative diagnosis of chronic cholecystitits and cholelithiasis, a peanut-sized nodule was found at the neck of the gallbladder. Since frozen sections revealed adenocarcinoma, excision of the liver bed and regional lymph nodes was performed. Postoperatively, chemotherapy was given. The girl is well 24 months following surgery. Carcinoma of the gallbladder is an extremely rare tumor in childhood. The authors could find only two other reported c a s e s . Thomas A. Angerpointner
Bile Duct Carcinoma in an Adolescent. M.J. Czaja, J.P. Golfarb,
K.C. Cho, et al. Am J Gastroenterol 80:486-89, (June), 1985. A 17-year-old black female was found to have a well-differentiated papillary adenocarcinoma of the bile duct with involvement of the liver. She was treated with excision of the carcinoma from within the bile duct and placement of an internal stent. She then received a course of 5,500 rads of external radiation. Twelve months after surgery, she was doing well. This patient had none of the conditions proposed to be etiological factors of bile duct carcinoma, such as gallstones, congenital malformations (such as choledochal cyst), ulcerative colitis, parasitic infections, or exposure to toxins. Of the two previous cases reported in the literature, who presented at an age less than 20 years, one had a ten-year history of ulcerative colitis, and the second had a choledochal cyst.--Richard R. Ricketts
Fibromatosis of Infancy and Childhood. Histology, Ultrastructure and Clinicopathologic Correlation. D. Schmidt and D. Harms. Z
Kinderchir 40:40-46, (February), 1985. Fibromatoses are rare nonmetastasizing tumors of unknown etiology that tend to invade surrounding tissues, are subject to frequent recurrencies after surgical excision, regress sometimes spontaneously, and may be solitary or multiple. Clinicopathologic data of 59 cases of fibromatosis in children are presented. The most frequent type was infantile (desmoid-type) fibromatosis. More than 50% of the tumors developed during the first 5 years of life. There was no significant sex difference. The most common sites of involvement were the lower extremities, followed by the trunk, and the upper extremities. Vimentin could be demonstrated in the tumor cells by immunohistochemical methods. Positive staining for dipeptidylaminopeptidase (DAP) IV suggested a myofibroblastic nature of the neoplastic cells. It is emphasized that clinical behavior depends on
471
number, location, and histologic appearance of the lesions as well as age of the patient.-- Thomas A. Angerpointner Synovial Sarcoma in Childhood. S.J. Israels, H.S.L. Chan, A.
Daneman, et al. Am J Roentgenol 142:803-806, (April), 1984. Over a 64-year period, seven children presented with synovial sarcomas. There were five males and two females ranging in age from birth to 12 years (mean 4.4 yr). All presented with an extremity mass. Five patients survived 11 months to 27 years after diagnosis. This soft-tissue sarcoma occurs near joints but originates from primitive mesenchymal cells, which resemble synovial cells. These seven patients represented 2.6% of soft tissue sarcomas seen during this period (268 tumors), and the mean age was lower than other reported series. Radiographic findings are nonspecific and may not reveal the full extent of the tumor. The authors recommend CT to accurately determine the extent of local involvement and to evaluate the lungs for metastatic disease. The present management of this tumor includes limb salvage surgical procedures, local irradiation, and chemotherapy.--Randall W. Powell Turcot Syndrome: A Case with Spinal Cord and Colonic Neoplasms. D.R. Radin, K.C. Fortgang, C.S. Zee, et al. Am J Roentge-
nol 142:475-476, (March), 1984. Two sisters, aged 12 and 13 at presentation, form the basis for this succinct review of this rare syndrome, which consists of primary malignancy of the CNS in association with colonic polyposis. The 13-year-old girl presented with a spinal cord tumor, which resulted in quadriparesis and 4 months later developed rectal bleeding and a constricting lesion in the descending colon (adenocarcinoma). Two months after her colectomy she succumbed to severe respiratory problems. An older sister presented at 12 years of age with rectal bleeding and adenocarcinomas of the transverse and sigmoid colon resulting in total colectomy. Eight months later she developed symptoms of increased intracranial pressure and CT revealed two intracerebral masses while a chest radiograph revealed multiple nodules. She died one month later and autopsy was refused. Both patients had multiple large caf&au-lait spots and axillary freckling. As depicted in the case reports, half of the patients present with CNS symptoms, the other half with GI tract symptoms. The number of polyps usually is less than 100 but are larger at an earlier age than those in familial polyposis. Glioblastoma multiforme is the most common type of CNS tumor with most lesions being located in the cerebrum.--Randall W. Powell
gallbladder wall. At laparotomy, which was performed with the tentative diagnosis of chronic cholecystitits and cholelithiasis, a peanut-sized nodule was found at the neck of the gallbladder. Since frozen sections revealed adenocarcinoma, excision of the liver bed and regional lymph nodes was performed. Postoperatively, chemotherapy was given. The girl is well 24 months following surgery. Carcinoma of the gallbladder is an extremely rare tumor in childhood. The authors could find only two other reported c a s e s . Thomas A. Angerpointner
Bile Duct Carcinoma in an Adolescent. M.J. Czaja, J.P. Golfarb,
K.C. Cho, et al. Am J Gastroenterol 80:486-89, (June), 1985. A 17-year-old black female was found to have a well-differentiated papillary adenocarcinoma of the bile duct with involvement of the liver. She was treated with excision of the carcinoma from within the bile duct and placement of an internal stent. She then received a course of 5,500 rads of external radiation. Twelve months after surgery, she was doing well. This patient had none of the conditions proposed to be etiological factors of bile duct carcinoma, such as gallstones, congenital malformations (such as choledochal cyst), ulcerative colitis, parasitic infections, or exposure to toxins. Of the two previous cases reported in the literature, who presented at an age less than 20 years, one had a ten-year history of ulcerative colitis, and the second had a choledochal cyst.--Richard R. Ricketts
Fibromatosis of Infancy and Childhood. Histology, Ultrastructure and Clinicopathologic Correlation. D. Schmidt and D. Harms. Z
Kinderchir 40:40-46, (February), 1985. Fibromatoses are rare nonmetastasizing tumors of unknown etiology that tend to invade surrounding tissues, are subject to frequent recurrencies after surgical excision, regress sometimes spontaneously, and may be solitary or multiple. Clinicopathologic data of 59 cases of fibromatosis in children are presented. The most frequent type was infantile (desmoid-type) fibromatosis. More than 50% of the tumors developed during the first 5 years of life. There was no significant sex difference. The most common sites of involvement were the lower extremities, followed by the trunk, and the upper extremities. Vimentin could be demonstrated in the tumor cells by immunohistochemical methods. Positive staining for dipeptidylaminopeptidase (DAP) IV suggested a myofibroblastic nature of the neoplastic cells. It is emphasized that clinical behavior depends on
471
number, location, and histologic appearance of the lesions as well as age of the patient.-- Thomas A. Angerpointner Synovial Sarcoma in Childhood. S.J. Israels, H.S.L. Chan, A.
Daneman, et al. Am J Roentgenol 142:803-806, (April), 1984. Over a 64-year period, seven children presented with synovial sarcomas. There were five males and two females ranging in age from birth to 12 years (mean 4.4 yr). All presented with an extremity mass. Five patients survived 11 months to 27 years after diagnosis. This soft-tissue sarcoma occurs near joints but originates from primitive mesenchymal cells, which resemble synovial cells. These seven patients represented 2.6% of soft tissue sarcomas seen during this period (268 tumors), and the mean age was lower than other reported series. Radiographic findings are nonspecific and may not reveal the full extent of the tumor. The authors recommend CT to accurately determine the extent of local involvement and to evaluate the lungs for metastatic disease. The present management of this tumor includes limb salvage surgical procedures, local irradiation, and chemotherapy.--Randall W. Powell Turcot Syndrome: A Case with Spinal Cord and Colonic Neoplasms. D.R. Radin, K.C. Fortgang, C.S. Zee, et al. Am J Roentge-
nol 142:475-476, (March), 1984. Two sisters, aged 12 and 13 at presentation, form the basis for this succinct review of this rare syndrome, which consists of primary malignancy of the CNS in association with colonic polyposis. The 13-year-old girl presented with a spinal cord tumor, which resulted in quadriparesis and 4 months later developed rectal bleeding and a constricting lesion in the descending colon (adenocarcinoma). Two months after her colectomy she succumbed to severe respiratory problems. An older sister presented at 12 years of age with rectal bleeding and adenocarcinomas of the transverse and sigmoid colon resulting in total colectomy. Eight months later she developed symptoms of increased intracranial pressure and CT revealed two intracerebral masses while a chest radiograph revealed multiple nodules. She died one month later and autopsy was refused. Both patients had multiple large caf&au-lait spots and axillary freckling. As depicted in the case reports, half of the patients present with CNS symptoms, the other half with GI tract symptoms. The number of polyps usually is less than 100 but are larger at an earlier age than those in familial polyposis. Glioblastoma multiforme is the most common type of CNS tumor with most lesions being located in the cerebrum.--Randall W. Powell