Case report: renal osteodystrophy in association with spinal stenosis in achondroplasia.

A 54 year old woman with achondroplasia presented with symptoms of spinal stenosis. Subsequent studies revealed end-stage renal disease with severe renal osteodystrophy that was characterised by radiographs and new bone biomarkers. We speculate that renal osteodystrophy accentuated spinal stenosis, which is a common feature of achondroplasia in adults.

The prevalence and location of metastases from ocular melanoma: imaging study in 110 patients.

Ocular melanoma is characterized by an unpredictable clinical course, during which fulminant metastatic disease may occur after a prolonged disease-free interval. The purpose of this study was to determine the pattern of metastatic involvement in this disease. The clinical and radiologic findings in 110 patients with metastatic ocular melanoma were reviewed. The 54 men and 56 women were 24-79 years old (mean, 50 years) when the primary tumor was first diagnosed. Metastases were present in three patients at the time of first diagnosis and occurred in 107 patients 2 months to 36 years later (mean, 52 months). One hundred five patients died between 1 and 38 months after the onset of metastatic disease. Hepatic metastases developed in 101 patients (92%), and in 60 (55%) of these, the liver was the only organ involved initially. Pulmonary parenchymal metastases developed in 34 patients (31%), but in only four of them were metastases confined to the lungs. Twenty-five patients (23%) had bone involvement, mostly affecting the spine. Nineteen patients (17%) had skin or subcutaneous metastases, but in only two of them was this the initial finding. Nodal involvement was shown in 15 patients (14%), almost always associated with extensive hepatic metastases. Brain and adrenal metastases were seen in five and three patients, respectively. Hepatic involvement occurs in almost all patients who develop metastatic ocular melanoma, and the liver is the most common initial site of metastatic involvement. Metastases may develop after a long disease-free interval.

Thoracic lymphoma.

Mediastinal adenopathy is a common manifestation of HD in a usually predictable pattern involving anterior and middle mediastinal nodes with or without disease in the hili. Hilar adenopathy is uncommon without detectable mediastinal disease and the lung is virtually never involved alone. In NHL the pattern of disease is more unpredictable. Lung manifestations include direct extension from involved nodes, nodules with or without cavitation, atelectasis secondary to endobronchial or nodal obstruction and rarely an interstitial pattern. Pleural effusions are not uncommon, but solid pleural masses are less frequent. Invasion of the pericardium is not often seen, although masses are commonly seen along the pericardium. Invasion of the chest wall occurs particularly with involvement of internal mammary nodes. Occasionally, posterior nodes will invade the adjacent vertebrae and spinal canal. The chest wall may be invaded by enlarged axillary nodes. Isolated chest wall soft-tissue masses are not common and are usually a manifestation of NHL, especially large cell lymphoma. Although the chest radiograph should detect most intrathoracic disease. CT has been found a necessary adjunct in patients with HD for accurate staging and therapy, especially if radiotherapy is planned. CT has been found helpful in NHL for staging, especially for the pleura and chest wall. MR imaging has not been found to contribute additional useful information except in selected cases.

Radiological features of extraskeletal Ewing sarcoma.

The radiological features of extraskeletal Ewing sarcoma were reviewed in 22 patients whose average age was 22 years. Tumours were located in the extremities (11 patients), abdomen or pelvis (six patients) and the chest (five patients). The tumours ranged in size from 2 cm to 20 cm, were mainly well circumscribed and showed no evidence of calcification prior to treatment. Most tumours (13 out of 14) were of low attenuation or contained areas of lower attenuation than muscle on computed tomographic examination, and in six out of seven patients studied by ultrasound the tumours were hypoechoic or partly anechoic. No distinctive post-contrast medium enhancement pattern on CT examination (11 patients) or angiographic features (three patients) were evident. Tumour haemorrhage was a frequent microscopic finding and changes consistent with this were present in one patient on magnetic resonance imaging examination. Distant metastases or local recurrence developed in 13 patients with lung being the most frequent metastatic site (eight patients). Although its radiological features are non-specific, extraskeletal Ewing sarcoma should be included in the differential diagnosis of noncalcified soft-tissue tumours especially in a young age group and where located in an extremity or paravertebral region of the chest.

Detection of hepatic metastases in breast cancer: the role of nonenhanced and enhanced CT scanning.

Nonenhanced and enhanced CT was compared in 88 patients with breast cancer and hepatic metastasis. Twenty-five patients had bolus, sequential dynamic CT, and 63 patients were scanned more slowly after a bolus or during drip infusion. Metastatic lesions were more conspicuous on nonenhanced CT and became isodense or nearly isodense after contrast medium administration in 28% of the patients scanned dynamically and in 29% of those scanned more slowly. Although breast cancer has not generally been considered a common origin of hypervascular metastases, we recommend that it be treated as such and that both enhanced and nonenhanced CT of the liver be obtained when patients are screened for metastasis.

The computed tomographic appearances and clinical significance of intussusception in adults with malignant neoplasms.

The computed tomography (CT) appearances of intussusception in 14 patients and the clinical follow-up of this condition in 17 adults with known primary malignant neoplasms were retrospectively reviewed. The ages of the 11 men and six women ranged from 25 to 83 years. Nine patients had been treated for malignant melanoma and the others for various primary neoplasms. Intussusception on CT was characterized by thickening of the affected bowel segment in all 14 patients and by the presence of intraluminal fat density material in 13, a concentric ring or "target" lesion in four, and an intra-luminal soft-tissue mass in nine. Five patients had intussusception, without other evidence of disease, caused by metastases in three patients, lipoma in one and idiopathic in one. Eleven of the other 12 patients had extensive disease, and one had small bowel cytomegalovirus infection. Five of these patients had more than one site of bowel involvement. Ten of the 12 patients had progression of disease on follow-up CT examinations and six died between 26 days and 7 months after diagnosis of intussusception. Intussusception may occur as the first indication of tumour recurrence or metastasis, but is more commonly a manifestation of widespread disease. However, even in patients with malignant neoplasms, it may be idiopathic or a result of benign neoplasm.

Preoperative chemotherapy for soft-tissue sarcomas of the extremities.

Forty-six patients with extremity soft-tissue sarcomas were treated with a mean of 4.4 cycles of preoperative adriamycin-based combination chemotherapy, followed by definitive local surgery and radiotherapy. All tumors were larger than 5 cm and of histologic type having significant risk of metastasis. Eighteen patients (40%) had an objective clinical response to the chemotherapy, while 27 patients (60%) did not respond. Patients with tumors responsive to chemotherapy had significantly improved overall survival (median 60 + months versus 32.7 months; p = 0.02), continuous disease-free survival (median 60 + months versus 15.1 months; p = 0.04), and distant metastasis-free survival (median 60+ months versus 28.5 months; p = 0.006) compared to the nonresponding patients. Tumor response to preoperative chemotherapy provides strong prognostic information and identifies a subgroup of patients most likely to benefit from chemotherapy.

Radiation-induced sarcoma of bone: CT findings in 19 cases.

We reviewed the CT findings in 19 cases of radiation-induced sarcoma of bone. The latent period before development of the sarcoma ranged from 5 to 50 years (mean, 17 years). In all 19 lesions, a soft-tissue extraosseous component was seen on CT, and 18 of them had associated bone destruction. Expansion of the affected bone and tumor-matrix mineralization each were present in 10 patients, but occurred together in only five patients. Periosteal reaction was seen in five patients, one of whom had an associated fracture. Radiation osteitis could not be identified on CT scans in the affected bone of any of the patients when tumor was present, but it was present in contiguous bone in two patients and had been shown 6 years before tumor became apparent in the affected bone in one other patient. Radiation-induced sarcoma of bone should be considered when bone destruction and an associated soft-tissue mass are shown on CT, or when changes occur in the appearance of previously stable irradiated bone.

Fibroadenoma of the breast: sonographic appearance.

The sonographic patterns of 100 fibroadenomas, including two giant fibroadenomas and two cystosarcoma phyllodes, were reviewed. The lesions were mostly hypoechoic (92%) and homogeneous in echotexture (72%). Twenty-seven percent of fibroadenomas showed irregular margins. Intratumoral calcifications were noted in 10% of lesions. A marked acoustic shadow without associated calcification was seen in six cases. The mean ratio of the length to the anteroposterior diameter of the tumors was 1.84 +/- 0.52 (standard deviation), indicating an elongation along the general orientation of the breast tissue planes. The ratios of length to anteroposterior diameter calculated in two matching groups of small (less than 1 cm3) fibroadenomas and carcinomas differed significantly. State-of-the-art high-frequency transducers, geometric analysis of tumors, and real-time ultrasound-guided fine-needle aspiration biopsy should help to distinguish between benign and malignant solid breast masses.

The radiologic manifestations of alveolar soft-part sarcoma.

Alveolar soft-part sarcoma is a rare soft-tissue tumor of unknown cellular origin that is characterized histologically by its organized "pseudoalveolar" pattern. The radiologic findings in 11 patients with this neoplasm were reviewed. The six men and five women were 16-48 years old (mean, 27 years). Nine patients had untreated primary tumors (thigh, four; forearm, two; and buttock, rectus abdominis muscle, and infratemporal fossa, one each) and two had locally recurrent masses (one each in the retroperitoneum and retrocrural space). All patients were evaluated by conventional radiography, two by sonography, eight by CT, five by angiography, and three by MR. Conventional radiographs showed the soft-tissue mass in only four patients; four lesions caused destruction of adjacent bone and two had soft-tissue calcification. Unenhanced CT showed low-attenuation lesions in four of five patients. The lesions were hypervascular on contrast-enhanced CT or angiography in each of nine patients studied. Prominent draining veins were shown by CT or angiography in five patients. Three lesions had a prolonged capillary stain on angiography. Alveolar soft-part sarcoma should be considered in the differential diagnosis of a hypervascular soft-tissue mass, particularly in the thigh of a young adult.

The clinical and radiologic manifestations of hemangiopericytoma.

The clinical and radiologic findings in nine patients with hemangiopericytoma were reviewed. There were eight women and one man with a mean age of 46 years. Seven of the neoplasms, including two locally recurrent tumors, were in the pelvis and two were in the thigh. Conventional radiographs were available for all patients. Five patients were evaluated by sonography, four by CT, three by angiography, and two by MR imaging. There was evidence of compression of adjacent viscera by six of the seven pelvic tumors with associated hydronephrosis in one patient. One thigh lesion had focal areas of speckled calcification. All five neoplasms evaluated by sonography showed a well-circumscribed hypoechoic lesion and three had significant sound through-transmission. Hypervascularity was documented by contrast-enhanced CT or angiography in each of three patients in whom these procedures were performed. Surgical resection of the pelvic neoplasms was complicated by marked hemorrhage. Hemangiopericytoma should be considered in the differential diagnosis of well-circumscribed hypervascular tumors in a middle-aged patient.

Sonographic detection and fine-needle aspiration biopsy of nonpalpable recurrent or metastatic melanoma in subcutaneous tissues.

Subcutaneous or lymph node metastases from melanoma may not be palpable because of their small size, their distance from the skin surface, or their location in an area of fibrosis due to previous surgery or irradiation. High-resolution sonography has been used to detect clinically occult foci of recurrent or metastatic melanoma, whereas real-time, ultrasound-guided, fine-needle aspiration biopsy provided cytologic confirmation. Following excision of cutaneous melanoma, sonographic follow-up of the areas of the surgical scar and lymphatic drainage is recommended whenever residual disease is suspected or when clinical evaluation is limited by postoperative or postradiation changes. Any nonpalpable hypoechoic mass detected in those areas should have a biopsy under real-time sonographic guidance.

MR imaging of malignant pleural mesothelioma.

The magnetic resonance (MR) findings in three patients with malignant pleural mesothelioma are described. All patients had a circumferential pleural mass surrounding the lung on the affected side. These tumors had a signal of intermediate intensity on T1-weighted images. The T2-weighted images showed a slight increase in signal intensity of the mass, with focal areas of very high signal intensity due to pleural fluid. Adenopathy was demonstrated by CT and MR in two patients. The extent of the tumor and its effects on adjacent structures were well appreciated on the coronal MR images.

Current status of myelography in the evaluation of the thoracic spinal cord.

The myelographic features in 35 patients with clinical thoracic spinal cord disease and normal plain radiographs were reviewed to re-evaluate the role of pre-operative myelography since the advent of computed tomography and magnetic resonance. The most frequent lesions were meningioma (9 cases), prolapsed intervertebral disc (9 cases), and metastases (8 cases). The extent and anatomical location of the lesion was demonstrated in all 19 patients without myelographic block. In 16 cases with complete block, the anatomical location was demonstrated in 14, and the extent of the lesion in four, using cervical puncture in two and CT myelography in one. Myelography remains a valuable method of assessment of the thoracic spinal cord and canal and is sufficient to allow the preoperative planning of surgical management in the absence of complete block. Additional investigation by computed tomography or magnetic resonance imaging is advised in the presence of complete block or an intramedullary lesion.

Computed tomography of hepatic oil embolism following lymphangiography.

Computed tomographic (CT) findings in two cases of hepatic oil embolism following lymphangiography are described. Both patients had lymphatic obstruction and opacification of the liver due to collateral flow of lymphangiographic contrast. Computed tomography of the liver showed a distinctive branching pattern due to the intrahepatic ethiodol.

Synovial sarcoma: MR imaging.

Ten patients with biopsy-proved synovial sarcoma were evaluated by magnetic resonance (MR) imaging on a 1.5-T unit. The lesions showed intermediate signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. Tumors were well-demarcated from normal tissues. Additional information included adjacent bone involvement (one case), femoral vein invasion by tumor (one case), and hemorrhage within the tumors (one case). Four patients underwent a repeat MR examination following chemotherapy. This showed a decrease in size and increase in the signal intensity of three tumors on T2-weighted images, proven to be due to necrosis in one. These changes correlated with clinical regression of disease. While MR in synovial sarcoma does not have any specific signal intensity, it proved to be useful in defining the extent of disease and in determining the response to chemotherapy.

Chemotherapy and embolization via the inferior epigastric artery for the treatment of primary and metastatic cancer.

We evaluated the results of arterial chemotherapy and embolization via the inferior epigastric artery and its branches in 10 patients with a variety of primary and metastatic neoplasms supplied by that vessel. A total of 15 infusions and five occlusions were performed. There were no complications related to arteriography, indwelling catheters, or arterial occlusion. The effects on tumor bulk ranged from complete necrosis in one patient to partial necrosis in three patients. Surgical resection was facilitated in four of six patients. Local recurrence of tumor occurred in two of these patients. Three of four patients with intractable pain had effective relief. Our experience suggests that transarterial therapy of tumors supplied by the inferior epigastric artery or its branches is a safe procedure and may be useful in tumor management.

CT and MR imaging of malignant germ cell tumor of the undescended testis.

Preoperative localization of the impalpable undescended testis is necessary to facilitate proper surgical planning. There is an increased incidence of malignant change in the undescended testis; demonstration of malignancy before surgery will significantly alter the treatment. We describe the computed tomographic (CT) and magnetic resonance (MR) findings in 2 patients with malignant change in an intraabdominal testis. The CT scan revealed lesions with areas of low density, 1 of which had focal calcifications; MR revealed lesions of predominantly low or intermediate signal intensity on both long and short TR/TE images, with some areas of very high signal on both sequences. After initial management with chemotherapy, the residual tumor was surgically resected. In neither instance was residual normal testis demonstrated. Both CT and MR are ideal methods of examining malignant transformation of the undescended testis, because of their ability to characterize the internal structure of the organ and, in the case of MR, its capacity for multiplanar imaging. They are almost of equal value except for the ability of CT to identify calcification and of MR to diagnose hemorrhage.