ABSTRACT
Mesoblastic nephroma (Bolande's tumour) is a rare renal tumour, but, overall, is the most frequent benign renal tumour in childhood and the most frequent renal tumour during the first year of life. It is increasingly diagnosed with US during the fetal period. We report the findings of two patients who showed the "ring" sign, an anechoic ring surrounding the tumour and typical of mesoblastic nephroma. We identified a Doppler signal in the ring in both cases, and pathology confirmed the vascular origin of this sign.
Subject(s)
Kidney Neoplasms/diagnosis , Nephroma, Mesoblastic/diagnosis , Adult , Diagnosis, Differential , Female , Fetus/abnormalities , Fetus/diagnostic imaging , Humans , Pregnancy , Pregnancy Complications, Neoplastic/diagnosis , Tomography, X-Ray Computed , Ultrasonography, Doppler , Ultrasonography, Prenatal , Wilms Tumor/diagnosisABSTRACT
Thoracic calcifications are found in the following locations: mediastinum, lung, pleura, cardiovascular system, and thoracic wall. The calcifications of the mediastinum include mostly inflammatory lesions and malignant neoplasms. Pulmonary calcifications are mainly inflammatory lesions and metastases of osteosarcomas. Where the cardiovascular system is concerned, the most common calcifications are those of the heart relating to tumors or surgery. Calcifications of the aortic wall and valve calcifications can be found. In the thoracic wall there are calcifications of the bone and of soft tissues. The knowledge of shape and location is very useful for the diagnosis of the underlying disease. Calcifications in the thorax are frequently manifestations of previous infectious processes. Less often, they may be due to neoplasms, metabolic disorders, or previous medical therapy.
Subject(s)
Bone Neoplasms/pathology , Calcinosis/diagnostic imaging , Cardiovascular Diseases/pathology , Respiratory Tract Diseases/pathology , Ribs/pathology , Spinal Diseases/pathology , Adolescent , Calcinosis/diagnosis , Calcinosis/pathology , Cardiovascular Diseases/diagnostic imaging , Child , Child, Preschool , Diagnosis, Differential , Humans , Radiography , Respiratory Tract Diseases/diagnostic imaging , Thorax/pathologyABSTRACT
Neu-Laxova Syndrome (NLS) is a severe disorder with intrauterine growth retardation, edema, and characteristic face (including microcephaly with receding forehead, protuberant eyes, a flattened nose, deformed ears, cleft palate, and micrognathia). Ichthyosis is often present. Limb anomalies include hypoplastic fingers and syndactyly of fingers and toes. Patients are usually stillborn or die shortly after birth. We report five unrelated patients--four with atypical NLS and one with typical NLS. All five patients were stillbirths. Clinically, the atypical NLS patients showed a large skull; rhizo-, meso-, and acromelia; and hypoplasia of the metacarpals and phalanges. The feet were similarly affected. Radiographically, the atypical patients showed interpediculate narrowing and hypoplastic vertebral bodies. The long bones were stick-like, showing diaphyseal widening that spared the metaphyses and was more pronounced in the lower extremities. The ilia had a half-moon configuration with widening of the sacrosciatic notches. The ischia were vertical and the pubic bone was absent. The typical NLS patient showed microcephaly, normal vertebral body, and long bone ossification, but a pelvic configuration similar to that of the atypical NLS patients. The common and distinguishing clinical and radiographic features are reviewed. Scott et al. [1981: Am J Med Genet 9:165-175] described two patients with NLS with radiographic and clinical findings similar to patients 1-4 reported here. Patients 1-4 of this report lack the typical findings of NLS and likely represent a distinct lethal skeletal dysplasia.
