Pigmented cardiac paraganglioma.

A pigmented left atrial paraganglioma was found at autopsy in a 40-year-old black man who died unexpectedly. The cause of death was ascribed to coronary artery disease. The atrial mass was sharply demarcated and polypoid, measured 4 cm in greatest dimension, and had a cut surface that revealed dark red-brown soft tumor tissue. Histopathologically, the neoplasm exhibited a classic organoid clustering of cells (zellballen) with a prominent capillary network. The chief cells contained a brown-black pigment with histochemical characteristics of melanin. We report a case of pigmented cardiac paraganglioma because of its rarity. To our knowledge, no mention has been made of the presence of pigment in previously reported cases of cardiac paragangliomas.

IgA plasma cell infiltration of proximal respiratory tract, pancreas, kidney, and coronary artery in acute Kawasaki disease.

The etiology and pathogenesis of Kawasaki disease (KD) remain unknown. As previously reported, in US patients with acute KD, IgA plasma cells (PCs) infiltrate the vascular wall. To determine whether IgA PCs are increased at mucosal sites in KD and to determine whether other nonvascular KD tissues are infiltrated by IgA PCs, the cells were immunolocalized and quantitated in tissue sections taken from 18 US and Japanese patients who died of acute KD and from 10 age-matched controls. IgA PCs were significantly increased in the trachea of patients who died of acute KD, compared with controls (P<.01), a finding that was similar to findings in children with fatal respiratory viral infection. IgA PCs also infiltrated coronary artery, pancreas, and kidney in all KD patients. These findings strongly support entry of the KD etiologic agent through the upper respiratory tract, resulting in an IgA immune response, with systemic spread to vascular tissue, pancreas, and kidney.

Aggressive manifestations of inflammatory pulmonary pseudotumor in children.

We present three cases that illustrate the locally invasive radiographic appearance that inflammatory pulmonary pseudotumor can assume. Awareness and inclusion of inflammatory pseudotumor in the differential diagnosis of aggressive pleuropulmonary and mediastinal processes may have critical treatment implications.

Pharyngeal fibrovascular polyp in a child.

Fibrovascular polyp of the upper aerodigestive tract is an uncommon tumor that may present in pediatric patients with symptoms ranging from dysphagia to asphyxiation and death. We present a unique case of a pediatric patient with an asymptomatic fibrovascular polyp noted as an incidental finding on a cervical ultrasound evaluation. This lesion extended from the posterior tonsillar pillar and prolapsed freely into the nasopharynx and esophagus. The literature relevant to this case is reviewed, and the etiology, pathophysiology, and management principles are discussed.

Diffuse renal cystic disease in children: morphologic and genetic correlations.

During a 5-year period, we evaluated seven infants and two fetuses who presented with enlarged, hyperechoic kidneys. In each, the initial clinical diagnosis was autosomal recessive polycystic kidney disease (ARPKD). Among the seven unrelated infants were three Caucasian and four African-American infants. No syndromic stigmata were evident in any of these infants. At the time of the initial evaluation, the family data were incomplete for four infants. The two fetuses were presumed to be at-risk for ARPKD based on the diagnosis in previous siblings. Renal histopathology was evaluated in all nine cases and revealed a spectrum of cystic disease ranging from ARPKD to glomerulocystic kidney disease to autosomal dominant polycystic kidney disease to diffuse cystic dysplasia. In the eight cases for whom liver histopathology was available, varying degrees of biliary dysgenesis were evident. We present a detailed analysis of the key histopathological features in each case and discuss the histopathological findings in an embryological context. In addition, we address the current role of molecular genetics in the diagnostic evaluation.

Splenogonadal fusion with limb deficiency and micrognathia.

Splenogonadal fusion (SGF) is a rare abnormality with two known types. In the continuous type, the spleen is connected to the gonad, and there are often limb defects, micrognathia, or other congenital malformations such as ventricular septal defect, anal atresia, microgastria, spina bifida, craniosynostosis, thoracopagus, diaphragmatic hernia, hypoplastic lung and abnormal lung fissures, polymicrogyria, deficient coccyx, and bifid spine C6-T3. The discontinuous type is usually not associated with congenital defects, and the gonad that fused with an accessory spleen has no connection with the native spleen. The etiology of SGF is not known. Conceivably, a teratogenic insult occurring between 5 weeks' and 8 weeks' gestation could interfere with the normal development of the spleen, gonads, and limb buds. We describe a case of splenogonadal fusion in a stillborn black boy with associated micrognathia and limb deformities. Also, we review the possible teratogenic etiologies and embryonic basis of SGF.

Neuroblastoma invading the kidney.

Renal invasion by neuroblastoma is probably more common than is generally recognized. In this study, the incidence of renal parenchymal tumor invasion was 20.4% (10 patients) among 49 cases of abdominal neuroblastoma. Generally, the renoinfiltrative neuroblastomas were extensive and had unfavorable histological features as well as lymph node involvement; they were either stage III or IV. Tumor invasion occurred by direct penetration through the renal capsule and/or lymphatic perivascular spread. Imaging studies had 100% sensitivity and 94.9% specificity for detecting renal invasion of neuroblastoma. Surgery/pathology was the standard of reference. Two instances of misdiagnoses of renal invasion were attributed to inadequate resolution of older computed tomography films, partial volume effects, and renal distortion by tumor compression.

Adenoid cystic carcinoma of the lacrimal gland in a six-year-old girl.

A 6-year-old white female was found to have an adenoid cystic carcinoma originating in a lacrimal gland. Eighteen months following diagnosis, the tumor recurred. Conservative surgery has been the sole mode of therapy. To date, after four operations and quadrimenstral imaging surveillance, there is no sign of disease progression. Our purpose is to record the unusual occurrence of an adenoid cystic carcinoma of the lacrimal gland in a child. An interim report, 32 months after diagnosis, is presented.

Sonographic and computed tomography characteristics of liver ablation lesions induced by high-intensity focussed ultrasound.

RATIONALE AND OBJECTIVES: The authors have previously demonstrated the ability of high-intensity focused ultrasound (HIFU) to extracorporeally induce selective tissue destruction in the liver without causing damage to the intervening abdominal wall. The potential usefulness of HIFU as a noninvasive therapy for liver cancer has been suggested. This study observes sonographic and computed tomography (CT) characteristics of HIFU-ablated liver tissue in an attempt to assess the possibility of using these imaging methods to monitor the therapeutic results. METHODS: A sonoablated lesion was induced in the liver in each of 20 rabbits with a HIFU therapeutic system. Sequential imaging of the hepatic sonolesions with sonography and CT was performed up to 8 days after treatment, and the imaging patterns were correlated with the histopathology. RESULTS: Hepatic sonoablated tissue could be clearly visualized by sonography as a hypoechoic lesion. On contrast-enhanced CT, the sonolesions were depicted as nonenhanced low-density regions. There was good correlation among the sizes of sonography- and CT-depicted lesions and pathologic specimens. CONCLUSION: In this model, sonography and contrast-enhanced CT were useful imaging modalities for monitoring sonolesion evolution after HIFU treatment.

Changes of hepatoma echo patterns with tumor growth. A study of the microanatomic basis in a rat model.

OBJECTIVES: The echo patterns of human hepatoma vary considerably as the tumors grow. This sonographic study investigates the microanatomic basis of the echo-pattern changes in an animal model. METHODS: ACI rats (n = 35) with intrahepatically implanted Morris hepatoma 3924A were imaged with 10-MHz ultrasound from days 7 to 28 after tumor implantation. The sonograms were precisely correlated with the histologic sections and microangiograms obtained from the same section plane. RESULTS: Small hepatomas (size = 6.6 +/- 1.6 mm) without necrosis demonstrated a low-echo pattern. Medium-sized tumors (size = 11.7 +/- 3.3 mm) had a low-echo periphery and a high-echo center. The low-echo periphery corresponded to viable tumor and the high-echo core corresponded to central coagulation necrosis. Large tumors (size = 20.9 +/- 7.5 mm) with extensive necrosis revealed an irregular mixed-echo pattern. CONCLUSIONS: The trend of increasing echogenicity of the hepatomas with tumor growth is due to the progression of coagulation necrosis.

Multifocal primary neuroblastoma.

UNLABELLED: The purpose of this study is to present three patients with multifocal primary neuroblastoma, to review the literature, and describe the radiographic findings. SUBJECTS AND METHODS: Three children with multifocal neuroblastoma have been identified. The case histories and imaging findings in these patients are reviewed. RESULTS: Two children had synchronous and one child had metachronous multifocal primary neuroblastoma. The primary tumors were both in the abdomen in one patient, both in the chest in another patient, and in the chest and abdomen in the third patient. Evidence for multifocal origin of these tumors, rather than metastatic spread, is presented. CONCLUSION: Multifocal primary neuroblastomas can occur. The tumors maybe synchronous or metachronous. Awareness of this disorder may prevent errors in diagnosis and staging. Although not identified in our patients there is a strong familial incidence of neuroblastomas in those patients with multifocal tumors.

Laryngomalacia and intra-neural striated muscle in an infant with the Freeman-Sheldon syndrome.

Striated muscle cells within peripheral nerve trunks, a very rare dishistogenetic lesion, was found in the arytenoid submucosa of a ten and a half week old afroamerican male with the Freeman-Sheldon syndrome (craniocarpotarsal dysplasia) who presented with obstructive apnea. Laryngomalacia was confirmed endoscopically. Supraglottoplasty offered temporary relief of the obstruction. Our histopathological observation is puzzling and perhaps adds a new dimension to the appropriateness of 'dysplasia'. It remains to be seen whether the neuromuscular lesion is a coincidental finding, a mere curiosity or bears any clinical significance.

Extracorporeal liver ablation using sonography-guided high-intensity focused ultrasound.

RATIONALE AND OBJECTIVES: High-intensity focused ultrasound (HIFU) is the only radiation beam that can remotely destroy deep-seated tissue targets without causing damage to the intervening tissues. This study evaluates the ability of sonography-guided HIFU to extracorporeally induce liver ablation in a rabbit model. METHODS: Under sonographic guidance, the HIFU beam was transcutaneously focused at the target tissue in the liver through a subcostal approach. A computer controlled the HIFU exposure and transducer movement to destroy a preselected tissue volume. Simultaneous sonography monitored the tissue response. Ten insonated rabbits were killed from days 0 to 10, and the liver and intervening tissues were examined histologically. RESULTS: A sharply demarcated sonolesion of coagulation necrosis was produced in the liver in 9 of 10 animals. No damage was found in the intervening tissues (n = 6) when adequate acoustic coupling and proper beam path was applied. CONCLUSION: Sonography-guided HIFU might be a potential new modality for extracorporeal inducement of liver cancer ablation without resorting to laparatomy.

Myositis (fasciitis) ossificans in an infant.

Myositis (fasciitis) ossificans developed following minor trauma to the soft tissues of the posterior aspect of the right knee of a 5-month-old female infant. The clinical and pathological features of myositis ossificans are reviewed and the differential diagnosis of soft tissue ossification in early childhood is discussed.

Cavernous hemangioma of the lung in an infant.

A 10-week-old male was found to have a cavernous hemangioma in the right lower pulmonary lobe. Hemangiomas of the lung are infrequent and in pediatric patients exceptionally rare. This may explain why, among the catalogs of intrapulmonary cystic lesions in children, cavernous hemangioma is a conspicuously absent entity and is rarely imagined in the clinical differential diagnosis. We present one such case, establish its distinction from arteriovenous malformations, and briefly review the available literature.