Aids. Case for diagnosis.
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Acquired Immunodeficiency Syndrome↗
Biomedical subjects
J T Stocker
Publications and source records attributed to J T Stocker.
Sequestrations of the lung.
Sequestration of the lung is a well-recognized entity usually subclassified into one of two groups based on its location: intralobar sequestration (ILS), within the normal pleural investment, and extralobar sequestration (ELS), outside the normal pleural investment. ELS is clearly congenital in origin; it is seen most frequently in the neonatal period, is associated with other anomalies, and is supplied by systemic and/or pulmonary arteries. ILS, on the other hand, in the majority of cases, probably results from repeated episodes of chronic pneumonia producing, through the process of granulation tissue formation, parasitization of normally occurring pulmonary ligament arteries resulting in a systemic artery supply to the infected area of lung. Its location (almost exclusively in the lower lobes) is dependent on the availability of systemic arteries situated only in pulmonary ligaments between the lower lobes and the mediastinum. Further support for the acquired nature of this lesion is its almost complete absence in neonates and infants, the infrequency of associated anomalies, and the consistent features of chronic inflammation and fibrosis within resected specimens.
Abnormalities, Multiple↗
Congenital cytomegalovirus infection presenting as massive ascites with secondary pulmonary hypoplasia.
A unique presentation of congenital cytomegalovirus infection occurred in a premature infant who had massive ascites of undetermined etiology. The ascites, apparently present since early gestation, had compressed the thoracic cavity by elevating the diaphragm, resulting in severe pulmonary hypoplasia.
Ascites↗
Pulmonary manifestations of juvenile laryngotracheal papillomatosis.
Juvenile laryngotracheal papillomatosis spreads to involve the lungs in less than 1% of cases, and when this occurs, the prognosis is poor. In seven such cases, the lung lesions, which appeared either solid or cystic on radiographs, proved to be benign squamous cell proliferations or papillomas, with central cavities containing debris or air. They seemed to grow centrifugally, using the alveolar walls as scaffolding with central coalescence and lung destruction. Papillomas spread inferiorly from the larynx by direct extension as far as the major bronchi, but rarely beyond. However, the parenchymal lesions were widely scattered, and some were subpleural. This discrepancy suggests that fragments become detached, particularly during endoscopic resection, and are carried down the airways by airflow. Those that lodge proximal to the respiratory bronchioles may be removed by mucociliary action and cough. Those that travel more distally are poorly cleared and may grow. If enough lung parenchyma is destroyed, the patient can develop symptoms of restrictive lung disease in addition to signs of upper airway obstruction.
Adolescent↗
Malignant fibrous histiocytoma of the liver--a case report.
A case of primary malignant fibrous histiocytoma of the liver studied by light and electron microscopy and confirmed at autopsy is presented. Malignant fibrous histiocytoma, the most common adult soft tissue sarcoma, has been reported in most organs but to date has not been described as a primary liver tumor.
Histiocytoma, Benign Fibrous↗
A study of pulmonary ligament arteries. Relationship to intralobar pulmonary sequestration.
Intralobar pulmonary sequestration (ILS) has generally been considered a congenital malformation, mainly because of the presence of one or more systemic arteries to the sequestered portion of lung. We performed a study of the pulmonary ligaments in children without congenital pulmonary or vascular disease that demonstrated systemic arteries in ten of 11 cases, with as many as five arteries present in a single case. These arteries arose from the thoracic aorta, contributed to the esophageal plexus, and traversed the pulmonary ligament to ramify in the visceral pleura. A sequence of events including bronchial obstruction, pneumonia, pulmonary artery occlusion, pleuritis, and parasitization of pulmonary ligament (or diaphragmatic) arteries leading to the "creation" of an ILS is proposed.
Adolescent↗
Anomalies associated with pulmonary hypoplasia.
We reviewed the records of 756 consecutive newborns autopsied over a 10-yr period. Using published standards for normal lung weights, low lung weight for total body weight was determined to be a reasonable estimate of pulmonary hypoplasia. Seventy-seven infants with pulmonary hypoplasia were identified. Multiple congenital malformation syndromes were found in the majority of infants with pulmonary hypoplasia. These included major diaphragmatic anomalies, renal anomalies, chromosomal disorders, extralobar pulmonary sequestration, severe musculoskeletal disorders, and isolated right-sided cardiac lesions. Ten infants had no known associated anomalies. The various theories of pathogenesis of pulmonary hypoplasia are reviewed and the suggestion is made that the cause of pulmonary hypoplasia in newborns may well be multifactorial. Various theories of pathogenesis are discussed ranging from an actual lack of space for the lungs to grow, to the possible necessity for appropriate respiratory movement during intrauterine life, to the possibility, particularly in the case of Potter's syndrome, of a primary mesodermal defect affecting multiple organ systems.
Chromosome Aberrations↗
Focal nodular hyperplasia of the liver: a study of 21 pediatric cases.
Twenty-one cases of focal nodular hyperplasia of the liver in pediatric patients are presented and compared with 40 cases previously reported. In this series, the lesion was usually asymptomatic (95%) and presented as a nontender mass noted on routine physical examination or was discovered incidentally at autopsy. There was a distinct female predominance (81%). Radiographic examination demonstrated a vascular space-occupying hepatic mass. The lesions were noted bilaterally or in the left lobe in 62% of cases. They were large, nonencapsulated, firm masses with central stellate areas subdividing the lesions into multiple lobules. Microscopically, septa contained eccentrically thickened vessels, small bile ducts, and an acute and/or chronic inflammatory infiltrate. Hepatocytes resembled those of the normal liver but some contained increased glycogen and fat. The lesions had no malignant potential and, except in women taking oral contraceptives, could be treated conservatively.
Adolescent↗
Acquired aganglionosis.
A documented case of acquired aganglionosis is presented in which transrectal biopsies taken 4 mo apart first showed the presence then subsequently the absence of ganglion cells. Colostomy followed by a Soave procedure was curative.
Colostomy↗
Extralobar pulmonary sequestration: analysis of 15 cases.
Extralobar pulmonary sequestrations occurring in 15 patients were studied. Twelve of the lesions were discovered during the first day of life; all of these patients died. The lesions occurred more often in male patients (11 cases). Polyhydramnios was present in four infants, and these four plus an additional infant had localized or generalized edema. Associated congenital anomalies were present in ten patients, including three examples each of diaphragmatic hernia and bronchogenic cyst. In one case the extralobar pulmonary sequestration was composed entirely of a congenital cystic adenomatoid malformation. In one other case, congenital adenomatoid malformation was present in a nonsequestered portion of lung. Significant lymphatic dilatation was found in four cases, two of which resembled congenital lymphangiectasis. Hyaline membranes, present in the normal lungs of three preterm infants, were absent in extralobar pulmonary sequestrations.
Aorta, Thoracic↗
Undifferentiated (embryonal) sarcoma of the liver: report of 31 cases.
Thirty-one cases of undifferentiated (embryonal) sarcoma of the liver are presented. The tumor is found predominantly in the pediatric age group, the majority of patients (51.6%) being between 6 and 10 years of age. An abdominal mass and pain are the usual presenting symptoms. Radiographic examination is nonspecific except to demonstrate a space-occupying lesion of the liver. The tumors are large, single, usually globular and well demarcated, and have multiple cystic areas of hemorrhage, necrosis, and gelatinous degeneration. Histologic examination shows a pseudocapsule partially separating the normal liver from undifferentiated sarcomatous cells that, near the periphery of the tumor, surround entrapped hyperplastic or degenerating bile duct-like structures. Eosinophilic globules that are PAS positive are usually found within and adjacent to tumor cells. Areas of necrosis and hemorrhage are prominent. The prognosis is poor, with a median survival of less than 1 year following diagnosis.
Adolescent↗
Cystic and congenital lung disease in the newborn.
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Female↗
Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum.
Thirty-eight cases of congenital cystic adenomatoid malformation of the lung are described, and a classification based on clinical, gross, and microscopic criteria is proposed. The type I lesion is composed of single or multiple large cysts (more than 2 cm. in diameter), frequently producing mediastinal herniation. The cysts are lined by ciliated psuedostratified columnar epithelium. The walls of the cysts contain prominent smooth muscle and elastic tissue. Mucus producing cells are present in approximatley one-third of the cases, and cartilage in the wall is rarely seen. Relatively normal alveoli may be seen between the cysts. The prognosis is good. Radiographic analysis of the type I lesion can preoperatively suggest the diagnosis, especially with the typical multicystic pattern. The gross appearance of the lesion corresponds closely to the radiographic image and adds another dimension to the pathologist's evaluation of the disease. The type II lesion is composed of multiple small cysts (less than 1 cm. in diameter) lined by ciliated cuboidal to columnar epithelium. Structures resembling respiratory bronchioles and distended alveoli are present between the epithelium lined cysts. Mucous cells and cartilage are not present. Striated muscle fibers may be seen rarely. The type II lesion is associated with a high frequency of other congenital anomalies, and the prognosis is poor. The type III lesion is a large, bulky noncystic lesion producing mediastinal shift. Bronchiole-like structures are lined by ciliated cuboidal epithelium and separated by masses of alveolus-sized structures lined by nonciliated cuboidal epithelium. The prognosis is poor.
Abnormalities, Multiple↗
Persistent interstitial pulmonary emphysema: another complication of the respiratory distress syndrome.
Persistent interstitial pulmonary emphysema (PIPE) was observed in 22 infants during the neonatal period. All of the infants experienced respiratory distress during the first few days of life, and 21 of them were treated for the respiratory distress syndrome with artificial ventilation or oxygen or both. Ten infants developed a localized form of PIPE, with air-filled interstitial cysts measuring up to 3.0 cm in diameter confined to one or more lobes of lung. The involved segment of lung was resected in seven of these infants, all of whom survived. The remaining three died and autopsies were performed. A diffuse form of PIPE was observed in the other 12 infants. Numerous cysts that were predominantly small (less than 0.3 cm) were seen in all lobes of both lungs. These infants received high concentrations of oxygen for prolonged periods, resulting in bronchopulmonary dysplasia in 11 of the infants. All 12 infants died. PIPE is characterized histologically by air-filled interstitial cysts partially lined by multi-nucleated giant cells.
Cysts↗
Cavernous hemangioma of the liver.
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Adult↗
Cystic adenomatoid malformation of the lung. Morphologic analysis.
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Female↗
Post-infarction peripheral cysts of the lung in pediatric patients: a possible cause of idiopathic spontaneous pneumothorax.
Occlusion of the main pulmonary artery to the right lung in a newborn infant produced peripheral (subpleural) infarction of the lung and cyst formation. Two older infants were found at autopsy to have subpleural cysts of the upper lobes. One had had surgery for repair of an atrial septal defect and a patent ductus arteriosus eight months prior to death, while the second had died suddenly of carbon monoxide intoxication. The authors suggest that pulmonary arterial occlusion may have been responsible for cyst formation in all three cases and may produce cysts in other infants that lead to idiopathic spontaneous pneumothorax in older children and young adults. The presence of bronchopulmonary arteries in fetuses and newborn infants may provide the mechanism for the production of subpleural cysts following pulmonary arterial occlusion.
Child, Preschool↗
Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases.
Extralobar pulmonary sequestration (ELS) represents a mass of pulmonary parenchyma separate from the normal lung. The coexistence of congenital cystic adenomatoid malformation (CCAM) in ELS has been reported. To define this association, the clinical, gross, and histologic features of 50 ELS cases were analyzed. The age at diagnosis varied from birth to 65 years with 24% of cases diagnosed prenatally and 61% (23/38) diagnosed within the first 3 months of life. Fifty-two percent of cases were in females and 48% in males. Forty-eight percent of ELS(s) were located in the left hemithorax, 20% in the right hemithorax, 8% in the anterior mediastinum, 6% in the posterior mediastinum, and 18% beneath the diaphragm. The blood supply to the sequestration in 77% of cases was directly from the aorta. Grossly, the lung, though hypoplastic in some cases, was otherwise unremarkable. Fifty percent (23/46) of ELS cases were associated with a coexistent CCAM. In contrast to the series as a whole, 92% (11/12) of the ELS/CCAM cases, excluding those prenatally diagnosed, were diagnosed within the first 3 months and 57% occurred in females. ELS/CCAM lesions, while randomly distributed, were more frequently seen on the left side. Gross features of the ELS/CCAM cases were similar to those cases with ELS alone. All CCAM cases had a type 2 pattern on histologic examination with 48% of those cases also displaying rhabdomyomatous dysgenesis. Our findings indicate that the occurrence of CCAM in ELS is more frequent than reported in the literature and differs in presentation from ELS cases not associated with CCAM.
Adenoma↗