ABSTRACT
BACKGROUND/PURPOSE: Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature. METHODS: During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included. RESULTS: There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma. CONCLUSIONS: Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors.
Subject(s)
Gastrointestinal Stromal Tumors/pathology , Gastrointestinal Stromal Tumors/therapy , Stomach Neoplasms/pathology , Stomach Neoplasms/therapy , Adolescent , Anastomosis, Roux-en-Y , Biopsy, Needle , Chemotherapy, Adjuvant , Child , Child, Preschool , Cohort Studies , Combined Modality Therapy , Female , Gastrectomy/methods , Gastrointestinal Stromal Tumors/epidemiology , Humans , Immunohistochemistry , Infant , Infant, Newborn , Male , Neoplasm Staging , Prognosis , Radiotherapy, Adjuvant , Retrospective Studies , Risk Assessment , Stomach Neoplasms/epidemiology , Survival Analysis , United States/epidemiologyABSTRACT
PURPOSE: Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms. METHODS: We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005. We conducted a comprehensive literature review of lipoblastoma and summarized the results of the largest series published. RESULTS: Most patients presented with an enlarging, palpable, firm, nontender, mobile mass. The male-to-female ratio was 1.9:1. The anatomical distribution was trunk (n = 12), extremity (n = 12), groin (n = 5), and neck (n = 3). Average age at resection was 2.8 years (range, 2.6 months to 12 years). Thirty-one cases were completely excised, although 1 patient underwent staged partial excision to preserve nerve function. Chromosomal analysis performed in selected patients revealed characteristic aberrations in chromosome 8. Complications included keloid formation (n = 3), wound infection/dehiscence (n = 2), wound seroma (n = 1), and transient brachial plexus neurapraxia (n = 1). Average follow-up was 7.4 months (range, 1 day to 6.5 years); 2 patients were lost to follow-up. There were no recurrences. CONCLUSIONS: A staged approach with meticulous sparing of the neurovascular bundle provides excellent functional outcome for patients with large tumors. Nonmutilating surgical excision is the treatment of choice.
Subject(s)
Neoplasms, Adipose Tissue/surgery , Soft Tissue Neoplasms/surgery , Child , Child, Preschool , Female , Humans , Male , Retrospective StudiesABSTRACT
Recent advances in the treatment of pelvic malignancies in children has resulted in an increased life expectancy. In the past, treatment of soft tissue sarcomas with simple surgical excision resulted in a recurrence rate of approximately 75%. Combination of chemotherapy, radiotherapy, and surgery have significantly altered the outcome of advanced pelvic soft tissue sarcomas. With the improved survival rate of patients treated with combination therapy, sterility secondary to radiation therapy has become an issue. As little as 600 rads to the gonads may result in sterility later in life. To alleviate this problem, relocating the gonads from the field of radiation has been suggested. In this report, we present a novel approach in which the testes are wrapped in SILASTIC (Dow Corning, Midland, MI) sheaths and are relocated temporarily into the anterior abdominal wall away from the radiation field. This alternative surgical approach should be considered in boys who are afflicted with a pelvic/perineal malignancy requiring radiation therapy.
