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Breast malignancy in children
ABSTRACTS
FROM POSTER SESSIONS
mass screening belonged to stage III. All showed no amplification of N-myc. All 14 are surviving after total or subtotal resection, and chemotherapy. It could be concluded that mass screening at 6 months detects a unique population of infants with neuroblastoma whose prognosis is good, even in advanced stages. Some tumors so discovered could represent subclinical tumors destined for spontaneous regression, which previously might have gone undiagnosed. Chemotherapy may be unecessaty in stage III patients detected by mass screening. Primary Malignancy of the Salivary Gland in Children David A. Rogers, Bhaskar N. Rao, Laura Bowman, Neyssa Marina, Irvin D. Fleming, Kurt P. Schropp, Thorn E. Lobe, Mahesh Kumar, St Jude Children s Research Hospital, Memphis, TN
We have treated 17 patients with a malignancy arising from a major salivary gland (16 parotid, 1 submaxillary). Nine patients had rhabdomyosarcoma (RMS). The age of the patients ranged from 5 months to 14 years (median, 11 years). The usual presentation was a mass at the angle of the mandible. One patient presented with a facial nerve palsy. Two patients presented with concomitant cervical adenopathy. In eight patients the mass was biopsied and the patient was then treated according to an existing prospective protocol. One patient underwent a superficial parotidectomy initially. Seven patients received radiation therapy. In two patients rapid progression of the disease precluded this treatment. Seven patients died with disease 3 months to 4 years (median, 6 months) after presentation, All of these patients had progressive local and distant disease. Two patients are alive without evidence of disease. both 6 years after presentation. Eight patients presented with carcinoma of the salivary gland. The age of these patients ranged from 4 to 17 years (median, 9 years). The usual presentation of these patients was a mass over the affected gland. These patients were treated initially with complete excision of the malignancy. This was accomplished by either a subtotal parotidectomy with a regional lymphadenectomy or resection of the submaxillary gland with a suprahyoid lymph node dissection. In only one patient was disease found in the regional lymph nodes. In five patients local control was obtained with surgery alone. Four of these patients are alive without evidence of disease. One patient died of disseminated disease. Three patients had a local recurrence and received radiation therapy. All of these patients achieved local control but one died of disseminated disease. We conclude that RMS of the salivary gland should be biopsied and then treated with chemotherapy and radiation therapy. The other lesions should be managed with complete excision. Recurrences may be managed with radiation therapy. Breast Malignancy in Children David A. Rogers, Thorn E. Lobe. Irvin R. Fleming, Kurt P. Schropp, Bhaskar N. Rae, Albert0 Pappo, The University of Tennessee, MemphislLeBonheur Children s Medical Center/St Jude Children’s Research Hospital, Memphis, TN
In 25 years at a cancer center, we have treated 15 children with breast malignancy including 14 females and 1 male. Patients present with either: primary malignancy (l), metastatic disease (12) or secondary malignancy (2). Our female patient with primary breast malignancy had an alveolar rhabdomyosarcoma. was treated with wide excision, and is currently receiving chemotherapy. Twelve patients (80%) presented with metastatic disease and, in only three were breast biopsies performed. The primary lesion was rhabdomyosarcoma in eight patients, non-Hodgkin’s lymphoma in one patient, Hodgkin’s lymphoma in one patient, neuroblastoma in
577
one patient, and signet cell adenocarcinoma in one patient. All died of their disease. Two female patients presented with invasive ductal carcinoma of the breast after treatment for Hodgkin’s disease. Both patients underwent mastectomy. One died of the second malignancy, one is alive without evidence of disease for two years. We conclude that: (1) breast malignancies have three decidedly distinct presentations in pediatrics; (2) the breasts of pediatric oncology patients should be carefully and routinely examined for metastatic disease: and (3) metastatic disease to the breast in children is associated with an extremely poor prognosis. Diagnosis and Treatment of a Rare Case of Congenital Arterioportal Fistula Steven S. Rothenbetg, Man, L. Brandt, Bany Toombs. William J. Pokomy, Cora and Webb Mading Department of Surgery. Baylor College of Medicine and Department of Radiology, St Luke’s Episcopal Hospital, Houston. TX
Congenital arterioportal fistula in children is an extremely rare and usually fatal lesion. These patients most often present with the usual stigmata of portal hypertension, but may also present with abdominal distention, functional bowel obstruction or malabsorption. This report describes the eighth documented case of hepatic artery-portal vein fistula in the literature. This patient was 2% months old at the time he presented with abdominal distention, failure to thrive, and massive splenomegaly. Color-flow Doppler and standard arteriography documented a large intrahepatic arterioportovenous fistula being fed by an aberrant left hepatic artery originating from the left gastric. The area of the fistula was located in the medial segment of the left lobe with extension into the right lobe, making resection unfeasible. Because of his progressive symptoms. surgical ligation of the left gastric artery was performed which resulted in temporary control. The fistula recurred after 1 month with progressive abdominal distention, failure to thrive, and malabsorption. Repeat arteriography with placement of thrombogenie coils directly into the saccular portion of the fistula resulted in successful closure of the fistula. Follow-up at 1 year showed no recurrence with resolution of the symptoms as well as normal growth and development. This report exemplifies the usefulness of advanced noninvasive ultrasound imaging techniques in the diagnosis of vascular anomalies in children, as well as the importance of combined surgical and radiologic treatment of these rare. potentially fatal anomalies. Hepatocyte Transplantation in Swine T. Takeda, S. Murphy, S. Uyama, G.M. Otgan, J. P. Vacanti, The Children s Hospital. Boston, MA
Hepatocyte transplantation is a potential therapy for end-stage liver disease and various metabolic disorders. Previously, many have reported hepatocyte implantation in rats. but studies in larger models more analogous to human application are few. We developed a pig model for hepatocyte transplantation. Polyvinyl alcohol sponges measuring 2 x 3 x .8 cm were implanted in the small bowel mesentery, preperitoneal space, and subcutaneous tissue of pigs to allow prevascularization. After 1 week, donor liver was treated with collagenase and hepatocytes were isolated. The donor hepatocytes were injected into the sponges in the recipient swine. On days 0, 1, 4, and 8 after hepatocyte transplantation, the sponges were extirpated and examined histologically using hematoxylin and eosin staining. Hepatocytes engrafted and reorganized over time into a pattern with features of liver tissue. Nodules of hepatocytes showed evidence of vascularization similar to sinusoid formation. Specialized portions of hepatocyte membranes appeared similar to biliary
FROM POSTER SESSIONS
mass screening belonged to stage III. All showed no amplification of N-myc. All 14 are surviving after total or subtotal resection, and chemotherapy. It could be concluded that mass screening at 6 months detects a unique population of infants with neuroblastoma whose prognosis is good, even in advanced stages. Some tumors so discovered could represent subclinical tumors destined for spontaneous regression, which previously might have gone undiagnosed. Chemotherapy may be unecessaty in stage III patients detected by mass screening. Primary Malignancy of the Salivary Gland in Children David A. Rogers, Bhaskar N. Rao, Laura Bowman, Neyssa Marina, Irvin D. Fleming, Kurt P. Schropp, Thorn E. Lobe, Mahesh Kumar, St Jude Children s Research Hospital, Memphis, TN
We have treated 17 patients with a malignancy arising from a major salivary gland (16 parotid, 1 submaxillary). Nine patients had rhabdomyosarcoma (RMS). The age of the patients ranged from 5 months to 14 years (median, 11 years). The usual presentation was a mass at the angle of the mandible. One patient presented with a facial nerve palsy. Two patients presented with concomitant cervical adenopathy. In eight patients the mass was biopsied and the patient was then treated according to an existing prospective protocol. One patient underwent a superficial parotidectomy initially. Seven patients received radiation therapy. In two patients rapid progression of the disease precluded this treatment. Seven patients died with disease 3 months to 4 years (median, 6 months) after presentation, All of these patients had progressive local and distant disease. Two patients are alive without evidence of disease. both 6 years after presentation. Eight patients presented with carcinoma of the salivary gland. The age of these patients ranged from 4 to 17 years (median, 9 years). The usual presentation of these patients was a mass over the affected gland. These patients were treated initially with complete excision of the malignancy. This was accomplished by either a subtotal parotidectomy with a regional lymphadenectomy or resection of the submaxillary gland with a suprahyoid lymph node dissection. In only one patient was disease found in the regional lymph nodes. In five patients local control was obtained with surgery alone. Four of these patients are alive without evidence of disease. One patient died of disseminated disease. Three patients had a local recurrence and received radiation therapy. All of these patients achieved local control but one died of disseminated disease. We conclude that RMS of the salivary gland should be biopsied and then treated with chemotherapy and radiation therapy. The other lesions should be managed with complete excision. Recurrences may be managed with radiation therapy. Breast Malignancy in Children David A. Rogers, Thorn E. Lobe. Irvin R. Fleming, Kurt P. Schropp, Bhaskar N. Rae, Albert0 Pappo, The University of Tennessee, MemphislLeBonheur Children s Medical Center/St Jude Children’s Research Hospital, Memphis, TN
In 25 years at a cancer center, we have treated 15 children with breast malignancy including 14 females and 1 male. Patients present with either: primary malignancy (l), metastatic disease (12) or secondary malignancy (2). Our female patient with primary breast malignancy had an alveolar rhabdomyosarcoma. was treated with wide excision, and is currently receiving chemotherapy. Twelve patients (80%) presented with metastatic disease and, in only three were breast biopsies performed. The primary lesion was rhabdomyosarcoma in eight patients, non-Hodgkin’s lymphoma in one patient, Hodgkin’s lymphoma in one patient, neuroblastoma in
577
one patient, and signet cell adenocarcinoma in one patient. All died of their disease. Two female patients presented with invasive ductal carcinoma of the breast after treatment for Hodgkin’s disease. Both patients underwent mastectomy. One died of the second malignancy, one is alive without evidence of disease for two years. We conclude that: (1) breast malignancies have three decidedly distinct presentations in pediatrics; (2) the breasts of pediatric oncology patients should be carefully and routinely examined for metastatic disease: and (3) metastatic disease to the breast in children is associated with an extremely poor prognosis. Diagnosis and Treatment of a Rare Case of Congenital Arterioportal Fistula Steven S. Rothenbetg, Man, L. Brandt, Bany Toombs. William J. Pokomy, Cora and Webb Mading Department of Surgery. Baylor College of Medicine and Department of Radiology, St Luke’s Episcopal Hospital, Houston. TX
Congenital arterioportal fistula in children is an extremely rare and usually fatal lesion. These patients most often present with the usual stigmata of portal hypertension, but may also present with abdominal distention, functional bowel obstruction or malabsorption. This report describes the eighth documented case of hepatic artery-portal vein fistula in the literature. This patient was 2% months old at the time he presented with abdominal distention, failure to thrive, and massive splenomegaly. Color-flow Doppler and standard arteriography documented a large intrahepatic arterioportovenous fistula being fed by an aberrant left hepatic artery originating from the left gastric. The area of the fistula was located in the medial segment of the left lobe with extension into the right lobe, making resection unfeasible. Because of his progressive symptoms. surgical ligation of the left gastric artery was performed which resulted in temporary control. The fistula recurred after 1 month with progressive abdominal distention, failure to thrive, and malabsorption. Repeat arteriography with placement of thrombogenie coils directly into the saccular portion of the fistula resulted in successful closure of the fistula. Follow-up at 1 year showed no recurrence with resolution of the symptoms as well as normal growth and development. This report exemplifies the usefulness of advanced noninvasive ultrasound imaging techniques in the diagnosis of vascular anomalies in children, as well as the importance of combined surgical and radiologic treatment of these rare. potentially fatal anomalies. Hepatocyte Transplantation in Swine T. Takeda, S. Murphy, S. Uyama, G.M. Otgan, J. P. Vacanti, The Children s Hospital. Boston, MA
Hepatocyte transplantation is a potential therapy for end-stage liver disease and various metabolic disorders. Previously, many have reported hepatocyte implantation in rats. but studies in larger models more analogous to human application are few. We developed a pig model for hepatocyte transplantation. Polyvinyl alcohol sponges measuring 2 x 3 x .8 cm were implanted in the small bowel mesentery, preperitoneal space, and subcutaneous tissue of pigs to allow prevascularization. After 1 week, donor liver was treated with collagenase and hepatocytes were isolated. The donor hepatocytes were injected into the sponges in the recipient swine. On days 0, 1, 4, and 8 after hepatocyte transplantation, the sponges were extirpated and examined histologically using hematoxylin and eosin staining. Hepatocytes engrafted and reorganized over time into a pattern with features of liver tissue. Nodules of hepatocytes showed evidence of vascularization similar to sinusoid formation. Specialized portions of hepatocyte membranes appeared similar to biliary