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Metastasis from atrial myxoma is uncommon. We report on a patient who developed metastasis of the left atrial myxoma to the skin at 15 years after surgical resection. The primary lesion had not recurred. The myxoma has the potential for metastatic growth.
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AIM: To evaluate role of chest computed tomography (CTC) and chest radiography (CXR) in management of patients with testicular germ cell tumours (GCT). PATIENTS AND METHODS: An analysis was undertaken of staging and re-assessment CTC and CXR examinations performed on patients with GCT over a 4.5-year period. Data were obtained on clinical presentation, tumour histology, tumour marker levels and clinical course. Consensus review interpretation was combined with these data to obtain a 'standard of reference'. Sensitivity, specificity and predictive values were derived by comparison of original imaging reports to 'standard of reference'. RESULTS: Six hundred and twenty-three CTC examinations on 207 patients with GCT were included. Intrathoracic metastases were identified in 1% of seminoma patients compared with 20% of non-seminoma (NSGCT) patients. CTC was more accurate than CXR in the detection of intrathoracic metastases at 0.97, 0.96-0.98 (95% CI) compared with 0.91, 0.89-0.93. The agreement between imaging techniques and the standard of reference (determined by Kappa statistic) was respectively 0.96 for CTC and 0.65 for CXR. In GCT patients undergoing re-assessment with both CXR and CTC, CXR never detected unknown intrathoracic metastatic disease. Abdominopelvic lymphadenopathy was associated with intrathoracic metastases (P < 0.001), however re-assessment CTC did identify intrathoracic metastases in 27 cases without concurrent abdominopelvic disease. CXR was negative in 19 of these. CONCLUSION: Routine interval CXRs are unnecessary in NSGCT patients undergoing regular re-assessment CTC due to the low additional yield and limited effect on management. Re-assessment should still include CTC. In low risk, pure seminoma patients (abdominal CT and marker negative) re-assessment CTC can be safely avoided. Baseline CTC is advocated with CXR alone for re-assessment.
A child with mediastinal and supraclavicular ganglioneuroma, heterochromia iridis, and Horner's syndrome is described. The authors postulate that the thoracic neoplasm originated as a congenital neuroblastoma with metastasis to the supraclavicular lymph nodes and subsequent benign transformation, and that the ocular abnormalities resulted from sympathetic ganglion injury by the tumor in infancy. This seems to be the first published report of such ocular abnormalities associated with mediastinal ganglioneuroma.
Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.
We describe the clinical courses of four infants with infantile myofibromatosis (IM). This entity is a mesenchymal disorder of early infancy characterized by the formation of tumors in skin, muscle, viscera, bone, and subcutaneous tissues. Previously known as congenital generalized fibromatosis, IM was formerly thought to be a rare condition that was frequently fatal. The majority of the 170 affected patients we describe have been diagnosed since 1980. Furthermore, the mortality rate for these patients is less than 15%. Our review includes the clinical manifestations, as well as histopathologic features, and discusses the prognosis in affected infants. We found that infants with solitary lesions or multiple lesions without visceral involvement generally have a benign course. However, in patients with the multicentric form of the disorder and visceral involvement, 73% have died. Because the lesions may not be easily discernible and most spontaneously resolve, the condition is underdiagnosed and underreported. IM is the most common fibrous tumor of infancy and must be considered when evaluating children who present with either solitary or multiple tumors, particularly during the neonatal period.
Cystic hygromas are relatively uncommon benign tumors of the lymphatic system. The lesions frequently are apparent at birth, and more than 90% are detected before the end of the second year of life. Most commonly, cystic hygroma presents as a soft tissue mass in the posterior triangle of the neck, and only rarely does it extend into the mediastinum. Isolated intrathoracic hygromas are exceedingly rare and have been reported infrequently among children. Herein the authors review three cases of intrathoracic cystic hygroma, spanning a period of 30 years at their institution.
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OBJECTIVE: The purpose of this report is to evaluate our results in patients who underwent prosthetic bony reconstruction after chest wall resection. METHODS: We retrospectively reviewed all patients who underwent chest wall resection and reconstruction with prosthetic material at the Mayo Clinic. RESULTS: From January 1, 1977, to December 31, 1992, 197 patients (109 male patients and 88 female patients) underwent chest wall resection and reconstruction with prosthetic material. Median age was 59 years (range, 11-86 years). The indication for resection was recurrent chest wall malignancy in 65 patients (33.0%), primary chest wall malignancy in 62 patients (31.5%), contiguous lung or breast carcinoma in 58 patients (29.4%), and other reasons in 12 patients (6.1%). Three patients (1.5%) each had an open draining wound. This review covers 2 time periods. Sixty-four patients (32.5%) underwent reconstruction with polypropylene mesh during the period from 1977 to 1986. One hundred thirty-three patients (67.5%) underwent reconstruction with polytetrafluoroethylene from 1984 to 1992. Soft tissue coverage was achieved with transposed muscle in 116 patients (58.9%), local tissue in 81 patients (41.1%), and omentum in 3 patients (1.5%). There were 8 deaths (operative mortality rate, 4.1%). Ninety-one patients (46.2%) experienced complications. Seromas occurred in 14 patients (7.1%). Wound infections occurred in 9 patients (4.6%; 5 patients with polypropylene mesh and 4 patients with polytetrafluoroethylene). The prosthesis was removed in all 5 patients with polypropylene mesh and in none of the patients with polytetrafluoroethylene. Follow-up was complete in 179 operative survivors (94.7%) and ranged from 1 to 204 months (median, 26 months). A well-healed asymptomatic wound was present in 127 patients (70.9%). CONCLUSIONS: Chest wall resection and reconstruction with prosthetic material will yield satisfactory results in most patients. Little difference exists between polypropylene mesh and polytetrafluoroethylene.
A total of 102 men treated for germ cell tumor with chemotherapy containing cisplatin was referred for a secondary operation with signs of tumor in the retroperitoneum or chest. Of the patients 85 underwent laparotomy, 14 underwent thoracotomy and 3 had both operations. Residual tumors were completely resected in 66 patients and incompletely resected in 30, while no tumor was found in 6. The resected specimen was malignant in 18 patients, of whom 11 had complete removal of all malignant tissue. All patients with malignancy in the resected specimen received further chemotherapy. Long-term disease-free status was obtained in 75% of those patients who had a complete resection, compared with 14% in the group with incomplete resection. There was no evidence of malignant disease at operation in 78 patients but 5 of them later died of the disease. Malignant tissue was present in the residual tumor in only 1 of 15 patients whose primary tumor was seminoma alone. Resection was attempted in 14 patients despite abnormal tumor markers preoperatively. Only 5 of these patients achieved a disease-free status and 2 of them died later of malignant disease. Over-all 79 of the 102 patients are without evidence of disease (medium postoperative observation 23 1/2 months). We conclude that a secondary operation constitutes an important part of the treatment of patients with germ cell cancer.
An occult Leydig cell tumor is described in a patient who presented with gynecomastia. Diagnosis was established by means of testicular ultrasonography and selective testicular venous sampling. The pathophysiology and management of this rare disease entity are discussed.
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This article describes the use of computed tomography for the evaluation of pediatric patients with a variety of chest diseases. Indications for chest CT, constraints of technical factors on the study, and analysis of the benefits of CT examination versus risks will also be discussed.
A wide spectrum of malignant and benign diseases can present as tumors involving the chest in children. The imaging evaluation of these children is initially directed by clinical presentation and usually begins with a chest radiograph. Further imaging with CT, MRI, or rarely ultrasonography may be performed to better detect, localize, or characterize the lesion(s). The differential diagnosis of these tumors is guided not only by tumor location and characterization, but also by patient age and clinical presentation. Familiarity with the clinical and radiographic aspects of these diseases will help the radiologist to continue to play a crucial role in the diagnosis and management of these children.
Preoperative radiographic studies fail to uncover a significant number of patients with unresectable malignancies. Small peritoneal studs of tumor, lymph node involvement, and small liver metastases are common causes of understaging by imaging studies. Videoscopic staging offers a higher degree of accuracy among these patients, with minimal complications and a shorter recovery time than with exploratory laparotomy. The addition of laparoscopic sonography and peritoneal lavage may further increase sensitivity to unresectable disease. Staging videoscopy has been applied to hepatobiliary and gastrointestinal malignancies, pleural and pulmonary tumors, and gynecologic malignancies. The author believes that videoscopic staging will become increasingly common as it is further studied and the best applications are delineated.
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