Alimentary tract duplications are unusual anomalies that may require surgical intervention in the neonate, infant, and occasionally in the older child. The clinical presentation of patients with alimentary tract duplications includes bleeding, abdominal pain, intussusception, and respiratory distress, or it may be an incidental finding on either abdominal examination or chest x-ray. A review of 96 patients with 101 duplications seen over the last 37 years is reported herein. Twenty-one duplications were confined to the thorax; three were thoracoabdominal, and 77 were abdominal. Seventy-four patients presented as infants less than 2 years of age, and 22 patients were older. Ectopic gastric mucosa was found in 21 duplications, and pancreatic tissue was found in five. Seventy-five duplications were cystic and 26 were tubular. Ultrasonography, computed tomography (CT), and myelography are helpful diagnostic tools. Ninety-four of the 96 patients underwent surgical management for their duplications. One duplication was found at necropsy, and one patient was asymptomatic and did not undergo operation. A single death occurred in a 2-day-old infant who had intrauterine volvulus and meconium peritonitis. Management was based on the age and condition of the patient, the location of the lesion, whether it was cystic or tubular and communicating with the true intestinal lumen, and whether it involved one or more anatomic locations. Generally, total excision was preferred, but staged approaches were sometimes necessary.
BACKGROUNDThe authors investigated the incidence and relative risk of secondary malignant neoplasms in long‐term survivors of osteosarcoma.METHODSA comprehensive list of 509 patients with primary osteosarcoma treated at our institution between February 1973 and March 2000 was identified. All study patients received chemotherapy and/or surgery on one of six different protocols (T4, 5, 7, 10, 12, and CCG‐7921/POG‐9351). Chemotherapy was scheduled for up to 40 weeks with some variations in the actual treatment period and consisted of various combinations of the following agents: high‐dose methotrexate, doxorubicin, bleomycin, cyclophosphamide, dactinomycin, vincristine, cisplatin, and ifosfamide.RESULTSSecondary malignant neoplasms (SMN) occurred in 14 of 509 patients. Only one had pulmonary metastasis at diagnosis and subsequent multiple recurrences that required thoracotomies and further modification of the chemotherapy regimen. The median age at diagnosis for osteosarcoma was 16.6 years (range, 3.1–74.4 years). The median follow‐up was 5.2 years (range, 0.1–25.0 years). The time interval from diagnosis of the primary osteosarcoma to the development of SMN was 1.3–13.1 years (median, 5.5; 95% confidence interval [CI], 3.6–9.6). The most common SMN occurred in the central nervous system (n = 4): anaplastic glioma, meningioma, high‐grade glioma, and maxillary astrocytoma. There were two cases of acute myeloid leukemia and one case each of myelodysplastic syndrome, non‐Hodgkin lymphoma, high‐grade pleomorphic sarcoma, leiomyosarcoma, fibrosarcoma, breast carcinoma, and mucoepidermoid carcinoma. The overall 5 and 10‐year cumulative incidences of SMNs were 1.4% ± 1.1% and 3.1% ± 1.8%. The standardized incidence ratio was 4.6 (95% CI, 2.53–7.78, P = 0.00001) for the cohort and 3.64 (95% CI, 1.82–6.52, P = 0.0007) when patients with a history of retinoblastoma or Rothmund‐Thomson syndrome were excluded.CONCLUSIONSThe overall incidence of secondary malignancies in long‐term survivors of osteosarcoma was significantly higher than the expected incidence of cancer in the general population. However, the standardized incidence ratios were much lower than those reported for Hodgkin disease and retinoblastoma. Although additional follow‐up is warranted, the successes of current treatment regimens consisting of intensive, high‐dose chemotherapy in combination with topoisomerase II inhibitors outweigh the risks. Cancer 2002;95:1728–34. © 2002 American Cancer Society.DOI 10.1002/cncr.10861
In a small subset of patients who developed late metachronous OS, combined-modality therapy with surgery and aggressive chemotherapy may result in long-term postmetachronous survival. This implies that principles used in treatment of primary OS may be applied to patients with late metachronous skeletal OS.
When children and adolescents present with primary epithelial lung malignancy a majority will have advanced disease and experience a delay in diagnosis. The histologic types of tumors encountered are similar to lung tumors occurring in adults, although the frequency of the various types differs. Carcinoid tumors are more frequent, and less common subtypes of bronchogenic carcinoma are also more prevalent in the pediatric age group. Similar to the adult population, the prognosis of these tumors is dependent on histology and stage. Patients with carcinoid tumors seem to have the best prognosis, followed by adenocarcinoma. The highly aggressive basaloid carcinoma has the worst.
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