Greig's syndrome. Neonatal radiologic manifestations

A newborn infant with postaxial polydactyly of the hands, preaxial polydactyly of the feet, syndactyly and craniofacial dysmorphism (Greig's Syndrome) is reported. The radiographic manifestations in the neonatal period are described. The advanced bone age, the unusual shape of the iliac bones and the supernumerary prehallux toe are new radiological findings in Greig's Syndrome at this age. The diffuse distribution of skeletal anomalies suggests a more complex problem of the intrauterine process of ossification.     

Solitary rectal ulcer syndrome: radiologic manifestations

The solitary rectal ulcer syndrome is a distinct clinical entity occurring mainly in young patients who experience rectal bleeding. Solitary, and occasionally multiple, ulcers occur predominantly on the anterior or anterolateral aspects of the rectum. Current theories attribute this to pelvic muscle discoordination during defecation with partial rectal mucosal prolapse and traumatic ulceration. Classical histologic changes have been demonstrated that enable accurate diagnosis by the pathologist. Ten cases of biopsy-proven solitary rectal ulcer syndrome were reviewed. The radiographic abnormalities were: nodularity of the rectal mucosa (three cases), stricture formation (two cases), polypoid rectal masses (two cases), and ulceration (two cases). Radiologically this condition must be differentiated from other more serious entities such as carcinoma or inflammatory bowel disease.     

Pulmonary blastomycosis: radiologic manifestations

Blastomycosis, an airborne fungal disease with the lung the portal of entry, is endemic to the central and south central areas of the United States. The disease occurs in patients who range from asymptomatic to those with symptoms of acute pneumonia. Retrospective review of 27 cases from our institution revealed four well-defined radiographic patterns including air-space disease, nodular masses, interstitial disease, and cavitation. Some patients with air-space disease have symptoms of an acute pneumonia; more commonly they have no pulmonary symptoms. Air-space disease was the most frequent radiographic pattern in chronic blastomycosis with proved nonpulmonary disease; therefore, it cannot be regarded as indicative of early or acute blastomycosis. There was no relationship between the radiographic pattern and distribution, pulmonary symptomatology, or clinical stage of the disease. Our material does not support the previously suggested association of lower lobe air-space disease with early disease and upper lobe involvement with the chronic and often disseminated form. A more precise understanding of the variety of radiographic patterns and the spectrum of clinical presentations will facilitate diagnosis of pulmonary blastomycosis.     

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.     

Intraosseous lipomas: radiologic and pathologic manifestations

Sixty-one cases of surgically treated solitary intraosseous lipoma were staged into three categories depending on the degree of involution present histologically: stage 1, tumors of viable fat cells; stage 2, transitional cases composed partly of viable fat cells but also demonstrating fat necrosis and calcification; and stage 3, lesions demonstrating necrotic fat, calcification of necrotic fat, variable degrees of cyst formation, and reactive woven bone formation. Each of these stages had radiologic features that could be correlated with the histopathologic findings in the excised tissue. Examples of stage 3 lesions have frequently been misdiagnosed as unusual bone infarcts or other lesions. Intraosseous lipoma may be a less rare lesion than has previously been suggested.     

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.     

Pulmonary nontuberculous mycobacterial infection: radiologic manifestations.

The nontuberculous mycobacteria (NTMB) are a group of bacteria that can infect the cervical lymph nodes, skin, soft tissues, and lung. Pulmonary NTMB disease is increasing in prevalence and is most commonly caused by Mycobacterium avium-intracellulare or M kansasii. Occasionally, M xenopi, M fortuitum, or M chelonae also causes pulmonary disease. Diagnosis of pulmonary NTMB infection is often difficult because isolation of the organism from sputum or bronchoalveolar lavage fluid can represent airway colonization. The radiologic manifestations of pulmonary NTMB infection are protean and include consolidation, cavitation, fibrosis, nodules, bronchiectasis, and adenopathy. Pulmonary NTMB infection has five distinct clinicoradiologic manifestations: (a) classic infection, (b) nonclassic infection, (c) nodules in asymptomatic patients, (d) infection in patients with achalasia, and (e) infection in immunocompromised patients. Although classic NTMB infection may be indistinguishable from active tuberculosis, it is usually more indolent. The radiologic features of nonclassic NTMB infection are characteristic: bronchiectasis and centrilobular nodules isolated to or most severe in the lingula and middle lobe. In patients with acquired immunodeficiency syndrome, mediastinal or hilar adenopathy is the most common radiographic finding. Knowledge of the full spectrum of clinical and radiologic features of pulmonary NTMB infection is important to facilitate diagnosis and treatment.     

Epithelioid sarcoma:radiologic and pathologic manifestations.

The radiologic and pathologic manifestations of epithelioid sarcoma are presented based on an analysis of five cases, and the literature is reviewed. This rare entity tends to orginate in the extremities and metastasizes primarily via the lymphatics. Although the lesion grows slowly, it recurs with high frequency. Males are affected three times as often as females. The differential diagnosis includes both malignant and benign entities. The natural history and methods of treatment are reported.     

Yellow nail syndrome. Apropos of a case. A review of the main radiologic manifestations

The authors report a case of "yellow nail syndrome" and emphasize the radiological features of this recently described entity which physiopathology has not yet been established. A new feature to be described: a non symptomatic pericardial effusion in association with long standing bilateral pleural effusions.     

Posttransplantation cyclosporine-induced lymphoproliferative disorders: clinical and radiologic manifestations

Fifteen allograft transplant recipients acquired lymphoproliferative disorders after immunosuppressive therapy with cyclosporine and steroids. Many of these lymphoproliferative disorders regressed or disappeared completely after reduction of cyclosporine dose. This disease has several aspects that distinguish it from usual posttransplantation lymphomas that occur with regimens that do not contain cyclosporine. The time course from transplantation to onset of lymphoma is relatively short, with an average of approximately 8 months. Organs show a wide spectrum of abnormalities typical of other immunosuppression-associated lymphomas, but there is unique sparing of the central nervous system. The tumor is also unique in that it responds to a decrease in the cyclosporine dose.     

Pulmonary drug toxicity: radiologic and pathologic manifestations.

Pulmonary drug toxicity is increasingly being diagnosed as a cause of acute and chronic lung disease. Numerous agents including cytotoxic and noncytotoxic drugs have the potential to cause pulmonary toxicity. The clinical and radiologic manifestations of these drugs generally reflect the underlying histopathologic processes and include diffuse alveolar damage (DAD), nonspecific interstitial pneumonia (NSIP), bronchiolitis obliterans organizing pneumonia (BOOP), eosinophilic pneumonia, obliterative bronchiolitis, pulmonary hemorrhage, edema, hypertension, or veno-occlusive disease. DAD is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. It manifests radiographically as bilateral hetero- or homogeneous opacities usually in the mid and lower lungs and on high-resolution computed tomographic (CT) scans as scattered or diffuse areas of ground-glass opacity. NSIP occurs most commonly as a manifestation of carmustine toxicity or of toxicity from noncytotoxic drugs such as amidarone. At radiography, it appears as diffuse areas of heterogeneous opacity, whereas early CT scans show diffuse ground-glass opacity and late CT scans show fibrosis in a basal distribution. BOOP, which is commonly caused by bleomycin and cyclophosphamide (as well as gold salts and methotrexate), appears on radiographs as hetero- and homogeneous peripheral opacities in both upper and lower lobes and on CT scans as poorly defined nodular consolidation, centrilobular nodules, and bronchial dilatation. Knowledge of these manifestations and of the drugs most frequently involved can facilitate diagnosis and institution of appropriate treatment.