Ureteropelvic junction obstruction due to inflammatory pseudotumor masquerading as hydronephrosis because of a neuropathic bladder in a child with myelomeningocele.

Inflammatory pseudotumors (IPTs), also known as inflammatory myofibroblastic tumors, are rare solid lesions, generally thought to be benign, which have been described in a variety of locations. We report a case of a child with a neuropathic bladder who developed a pseudotumor of the retroperitoneum that caused progressive ureteral obstruction distal to the ureteropelvic junction. Hydronephrosis as a complication of IPTs has been described with bladder and uterine IPTs; however, in our review of published reports, we found no reported cases of a retroperitoneal IPT causing proximal ureteral obstruction in a pediatric patient.

Experience with juvenile polyps in North American children: the need for pancolonoscopy.

We report a recent experience with juvenile polyps (JP) in a large cohort of North American children to determine if a pancolonoscopy (PC) is needed in all children with suspected polyps. We reviewed hospital charts of all patients with JP seen over a 9-yr period (January, 1990-October, 1998). A total of 331 JP were encountered during 195 procedures in 184 patients (64% males, 88% white, mean age 5.93 yr [range 0.42-15.5 yr], median age 4.84 yr). Painless rectal bleeding was the commonest symptom. PC was performed in 42% (82/195) of procedures, and 177 JP were encountered: 54% (97/177) were in the rectosigmoid colon, 14% (24/177) were in the descending colon, and 32% (56/177) were proximal to the splenic flexure (i.e., proximal polyps). Overall, proximal polyps were seen in 37% (31/82) of PC. Only proximal polyps were noted in 12% (10/82) of PC. Five patients were re-endoscoped after an initial limited examination because of continuing symptoms from proximal polyps. All but one of the polyps had typical features of a JP on histological examination. Though most JP are located in the left colon, a PC should be the initial procedure because: 1) 37% of PC revealed proximal polyps, 2) 32% of polyps were located proximal to splenic flexure, 3) persistence of symptoms from missed proximal polyp(s) necessitates a repeat study with attendant risks, and 4) there is a possibility of malignant transformation in an unidentified JP.

Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity.

Byler disease (ByD) is an autosomal recessive disorder in which cholestasis of onset in infancy leads to hepatic fibrosis and death. Children who have a clinically similar disorder, but are not members of the Amish kindred in which ByD was described, are said to have Byler syndrome (ByS). Controversy exists as to whether ByD and ByS (subtypes of progressive familial intrahepatic cholestasis [PFIC]) represent one clinicopathological entity. The gene for ByD has been mapped to a 19-cM region of 18q21-q22. PFIC caused by a lesion in this region, including ByD, can be designated PFIC-1. Examination of haplotypes in siblings with ByS in two unrelated non-Amish families showed that the gene(s) responsible for their disorder(s) did not lie in the PFIC-1 candidate region. On light microscopy and transmission electron microscopy (TEM), liver tissue differed between Amish children with PFIC-1, who had coarsely granular bile and at presentation had bland intracanalicular cholestasis, and the children with ByS in the two non-Amish families, who had amorphous or finely filamentous bile and at presentation had neonatal hepatitis. Bile acid composition of bile also differed: In the Amish children with PFIC-1 and in one ByS family, the proportional concentration of chenodeoxycholic acid (CDCA) in bile was low compared with normal bile; in the other ByS family, it was only slightly reduced. Genetic analysis and light microscopy and TEM of liver may help distinguish PFIC-1 from other forms of ByS.

Peritracheal fibrosis induced by sclerosing agents: a putative treatment for tracheomalacia.

Tracheomalacia is a congenital or acquired deficiency of the cartilages of the trachea. Treatment procedures include formation of external support by insertion of cartilage; bone for foreign material into the peritracheal tissue. A sclerosing agent, sodium morrhuate, was injected in the peritracheal tissue, blindly or after skin incision and subcutaneous tissue. In both groups peritracheal granulation tissue and inflammation evolved into peritracheal fibrosis surrounding the cartilage at the site of injection. The reaction was more pronounced following incision of the skin. No fibrosis was observed in a control group following hypertonic saline injection. The results suggest that injection of sclerosing agents might be considered as a conservative mode of treatment in tracheomalacia, by producing peritracheal fibrosis as external support.

Transjugular intrahepatic portosystemic shunting (TIPS) for treatment of severe hepatic veno-occlusive disease.

Severe veno-occlusive disease (VOD) of the liver is a frequent cause of morbidity and mortality in patients undergoing transplantation. While surgical portosystemic shunts have been reported to be useful in the treatment of severe hepatic VOD with intractable ascites, few of these patients are surgical candidates. We report a case of severe VOD after autologous peripheral blood progenitor cell transplantation treated with transjugular intrahepatic portosystemic shunting (TIPS). This procedure resulted in marked improvement in the patient's ascites, coagulation status and urinary output. The safety and efficacy of this non-surgical approach for the treatment of patients with severe VOD requires prospective studies.

Visual presentation of the staging of pediatric solid tumors.

Staging systems are used in staging most pediatric solid tumors outside the central nervous system. Common solid, nonneurologic pediatric tumors include liver tumors, Hodgkin disease, non-Hodgkin lymphoma, Wilms tumor, rhabdomyosarcoma, neuroblastoma, Ewing sarcoma, and osteosarcoma. Traditional staging of pediatric tumors depends on the anatomic distribution of the malignant disease. Almost all staging systems are based on the spread of the local primary tumor, metastasis to regional lymph nodes, and distant blood-borne metastatic spread. There is some variability as to how tumor spread is assessed. Such assessment may be performed before or after surgery. There are many potential problems with tumor staging systems. The systems vary in complexity and clinical usefulness, and there is some variation in the criteria used in the different systems. It is important for radiologists to have a sound working knowledge of staging systems to facilitate accurate staging. Imaging is an important aspect of every staging system.

Cystic angiomatosis of bone: MR findings.

Cystic angiomatosis of bone is a rare disorder characterized by widespread lytic bone lesions with or without associated soft tissue or visceral lymphangiomatosis. The disorder is often initially incorrectly diagnosed as Langerhans cell histiocytosis. Unfortunately, biopsy of long bone lesions will often yield only fluid, thereby making a histologic diagnosis difficult. This report describes the MR findings in a patient with cystic angiomatosis of bone. We believe that the MR findings are highly suggestive of the correct diagnosis and that MR should eliminate the need for excisional biopsy in future cases.

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.

A reproducible rat liver cancer model for experimental therapy: introducing a technique of intrahepatic tumor implantation.

To investigate therapeutic strategies for hepatoma, it is necessary to have a reproducible animal model with a tumor growth pattern allowing accurate assessment of results. Many techniques of intrahepatic tumor implantation (IHTI) have been devised for intrahepatic tumor models. Most of them, however, have the disadvantage of high rates of artificial tumor dissemination during tumor implantation, which interferes with the evaluation of therapy. To overcome this problem, we have developed a technique of IHTI in which a piece of Gelfoam is placed into a small incision in the liver for the purpose of both hemostasis and formation of a tension-free pocket to accept the tumor implant. In 583 ACI rats receiving IHTI with Morris hepatoma 3924A, the tumor take rate was 100%. Resembling the natural course of human hepatoma, the implanted tumor grows locally early in the course of disease and eventually invades the surrounding organs causing ascites and also metastasizes to the lung. Liver microangiography demonstrated that the tumor received blood supply mainly from the hepatic artery. This IHTI technique was also compared to two other methods of IHTI: insertion of fragments without using Gelfoam and implantation with a tumor cell suspension. A significantly lower rate of early lung metastases was achieved with our technique (0%) in comparison with other two techniques (41 and 80%). We conclude that this rat liver cancer model is reproducible and allows efficient evaluation of treatment modalities for liver cancer without interference from tumor at undesirable sites.

High-intensity focused ultrasound in the treatment of experimental liver cancer.

High-intensity focused ultrasound (HIFU) was used to treat Morris rat hepatoma 3924A implanted in the liver. Treatment was administered with a lens-focused 4-MHz transducer that created a focused beam of 550 W/cm2 at peak intensity. One hundred twelve rats with liver tumors were divided into two groups of 56 each. Group 1 received HIFU therapy while group 2 (the control group) did not. All rats were killed immediately or 1, 3, 7, 14, 21, or 28 days after treatment. Eight rats in each group were killed at each interval for pathologic and biochemical studies. Significant inhibition of the tumor growth was seen in the HIFU-treated group, with tumor growth inhibition rates of 65.4% to 93.1% from the third to the 28th day after treatment. Ultrasound-treated tumors showed direct thermal cytotoxic necrosis and fibrosis. An additional 56 ACl rats with liver tumors were divided into four groups of 14 each. Group 1 received doxorubicin hydrochloride intraperitoneally and HIFU therapy; group 2, HIFU therapy; group 3, doxorubicin hydrochloride; and group 4 (the control group), neither HIFU nor doxorubicin hydrochloride. Significantly improved survival rates were noted in HIFU-treated animals (groups 1 and 2) compared with those of groups 3 and 4. These data suggest that HIFU may be a useful method for local treatment of hepatic tumors.

The effect of chloroquine and hyperthermia on murine neuroblastoma.

A number of reports suggest that hyperthermia is an effective adjunctive treatment modality in management of neural crest tumors. Recent studies have demonstrated a synergistic effect of induced hyperthermia when coupled with chloroquine in an in vitro model. This study examines the effect of chloroquine and hyperthermia in an in vivo murine neuroblastoma model. Forty-seven Ajax white mice (weighing 20 to 30 g) received a subaxillary tumor burden (C-1300 murine neuroblastoma) per trochar (1.25 x 10(6) cells). The tumor was then incubated for 9 days. Mice were then divided into four groups: group 1, controls (n = 15); group 2, hyperthermia (n = 12); group 3, chloroquine (n = 10); and group 4, chloroquine with hyperthermia (n = 10). Hyperthermia was induced with 40 to 69 mW/cm2 at 2,450 MHz microwave radiation for 4 minutes to achieve a temperature of 41.5 degrees C for 10 of 14 treatment days. Chloroquine was administered intraperitoneally at a dose of 40 mg/kg body weight for 10 of 14 treatment days. Mice were weighed and tumor size was determined daily. Animals were killed on day 21 and postmortem examination was performed, with tumors graded histologically. Animal weight, tumor weight, and tumor size were similar for all groups (P greater than .05). Mortality was 6% in group 1, 25% in group 2, 50% in group 3, and 40% in group 4 (P less than .05). Rate of tumor metastases was also statistically different from controls: group 1, 0%; group 2, 60%; group 3, 90%; and group 4, 90% (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of omental, intercostal, and internal mammary artery pedicle wraps on bronchial healing.

Bronchial transection and devascularization is necessary in the course of sleeve resection or lung transplantation, leaving distal bronchial segments ischemic and subject to stricture or dehiscence. Thirty mongrel dogs underwent left lung autotransplantation. The bronchial anastomosis was wrapped with omentum (n = 9), intercostal muscle pedicle (n = 9), or internal mammary artery pedicle grafts (n = 6). Six control animals underwent bronchial anastomosis without an external wrap. Bronchial revascularization by capillary ingrowth from the pedicle to the bronchial submucosal plexus was demonstrated with all three types of vascular pedicle grafts; however, more consistent and confluent vascular ingrowth was provided by internal mammary artery pedicle grafts. Additionally, the bronchial anastomotic cross-sectional area was significantly better in the internal mammary artery group (84.5 +/- 3.3) as compared with that of the omental (68.4 +/- 8.3), intercostal muscle (66.9 +/- 10.9), or control groups (70.2 +/- 7.6). An internal mammary artery pedicle graft and the presence of dense confluent submucosal vascular ingrowth from any pedicle graft were independently predictive (p less than 0.05) of minimizing bronchial anastomotic narrowing. These data are consistent with previous findings suggesting that omental and intercostal muscle pedicle grafts promote early bronchial revascularization; moreover, the data demonstrate the superiority of an internal mammary artery pedicle graft to provide submucosal vascular ingrowth and to minimize anastomotic stenosis.

Cranial fasciitis of childhood.

Cranial fasciitis of childhood is a benign lesion occurring superficial to or involving the cranial bones of children at a median age of 18 months, with a male sex predilection of 2:1. It is histologically described as a well-circumscribed, loose proliferation of stellate to spindle-shaped fibroblasts in a myxoid background with foci of hemorrhage and hyalinization. Mitotic figures, increased cellularity, and cellular immaturity may be seen. The treatment of choice is excisional biopsy, since the lesion is not known to recur. It is important that the pathologist and surgeon alike be able to differentiate this benign condition from other lesions because of the clinically threatening presentation of cranial fasciitis.

Spindle cell sarcoma of the heart in childhood: light microscopic, ultrastructural, and immunohistochemical evidence for smooth muscle, endothelial, and fibroblastic differentiation.

We describe the clinical presentation and pathological features of a cardiac sarcoma that occurred within the right atrial cavity of a 14-year-old boy. The patient presented with a 2-month history of increasingly severe heart failure. Tumor tissue obtained by biopsy showed light microscopic features of leiomyosarcoma. Immunohistochemical reactions and ultrastructural studies provided evidence of tumor cell differentiation along three cell lines--smooth muscle, fibroblastic, and endothelial--suggestive of origin from a multipotent subendothelial reserve cell. Postmortem examination disclosed a large necrotic tumor filling the right atrial chamber. Implants were present on the pericardium, but there were no other metastatic lesions. The difficulty of making a diagnosis of these rare neoplasms when the tumor is small and potentially curable results in a grim prognosis.

Pancreatic cystadenoma in an infant: ultrastructural study.

A 10 x 6 x 4 cm multicystic cystadenoma arose in the pancreas of a 4-month-old male. Microscopically, the cysts were lined by short columnar or cuboidal cells that contained neither mucin nor glycogen. The lining cells had occasional nucleoli, contained electron dense vacuoles and apical aggregates of filaments, and were associated with a basal lamina. This is the youngest patient reported as having pancreatic cystadenoma, and the ultrastructure of the neoplasm was different from the 6 previously studied adult cases, suggesting that infantile and adult cystadenomas are different in nature.