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Biomedical subjects

S C Kaste

Publications and source records attributed to S C Kaste.

At least 73 records · Page 4Linked to original sources

Postoperative small-bowel intussusception in children with cancer.

The clinical and imaging features of small-bowel intussusception as a complication of abdominal surgery are not well known. Diagnosis may be delayed because symptoms mimic common postoperative complaints. In pediatric cancer patients, the side effects of chemotherapy and symptoms related to the primary malignancy may also obscure the diagnosis. We report the clinical and radiographic features of eight children undergoing evaluation and treatment for solid tumors who developed small-bowel intussusception following laparotomy. These children represent 2.2% of all children and adolescents who underwent laparotomy at a pediatric cancer research and treatment center during a 13-year period. With prompt diagnosis and intervention, morbidity and mortality from this rare but life-threatening postoperative complication can be avoided.

Child↗

Ewing's sarcoma as a second malignant neoplasm in a child previously treated for Wilms' tumor.

PURPOSE: Second malignant neoplasms (SMNs) are a rare occurrence after the successful treatment of childhood cancer. For survivors of Wilms' tumor, the 10-year cumulative risk of developing an SMN has been estimated to be 1%. Bone sarcomas arising within the radiation field are a common occurrence, yet Ewing's sarcoma has been rarely reported as an SMN. PATIENTS AND METHODS: A 10-year-old girl presented with dyspnea 5 years after the diagnosis of stage III favorable histology Wilms' tumor and was found to have a right intrathoracic mass arising from the eighth and ninth ribs. The mass was located at the edge of the previous radiation field. RESULTS: Tumor cytogenetics and immunocytochemistry were helpful in establishing the diagnosis of Ewing's sarcoma. Treatment included combination chemotherapy, with hematopoietic growth factor support, and excluded anthracyclines and radiotherapy. More than 1 year after the diagnosis of Ewing's sarcoma, the patient has completed therapy with no evidence of disease. CONCLUSIONS: This is the first report to our knowledge of Ewing's sarcoma arising in the irradiation field of a patient treated previously for Wilms' tumor.

Child↗

Use of Tl-201 to detect untreated pediatric Hodgkin disease.

PURPOSE: To evaluate the usefulness of thallium-201 scintigrams in depiction of supradiaphragmatic lymphadenopathy associated with newly diagnosed Hodgkin disease in pediatric patients. MATERIALS AND METHODS: Thallium-201 and gallium-67 scans of the chest and neck were obtained in 33 consecutive patients. Mediastinal, cervical, supraclavicular, and axillary lymph node activity was evaluated by independent raters, and results were compared in a blinded fashion with those on chest computed tomographic (CT) scans. RESULTS: Gallium and thallium scans demonstrated similar accuracy in depiction of CT-defined mediastinal masses. The kappa statistic indicated moderate to strong agreement with CT results. Similarly, there was no statistically significant difference between the accuracy of thallium and gallium scans in depiction of extramediastinal lymphadenopathy. CONCLUSION: Thallium scintigraphy is effective for depiction of lymphadenopathy in pediatric patients with untreated Hodgkin disease. Serial thallium studies may provide an alternative to gallium scans in monitoring response to treatment.

Adolescent↗

Increased confidence of diagnosis of Ewing sarcoma using T2-weighted MR images.

To assess whether magnetic resonance imaging could assist in diagnosing Ewing sarcoma, we retrospectively analyzed T1- and T2-weighted images of 24 consecutive solitary lytic bone lesions, including 18 cases of histologically proven Ewing sarcoma, and six aggressive-appearing benign lesions including eosinophilic granuloma and osteomyelitis (3 cases each). Images were obtained using a (1.0T) Siemens GBSII system. No signal intensity differences were noted among the various lesions on T1-weighted images. The observed differences in T2-weighted marrow signal intensities were scored on a 5-point scale (5 = water, 3 = fat, and 1 = muscle) and then histologically correlated. Sixteen of 18 Ewing sarcomas showed homogeneous T2-weighted signal isointense with fat and two were heterogeneous. Signals for all 6 benign lesions were homogeneous and more intense than fat on T2-weighted images. All Ewing sarcomas had associated soft tissue masses whereas, only one benign lesion, an eosinophilic granuloma had a small soft tissue component. In our patient population, a homogeneous intramedullary T2-weighted signal isointense with fat, and the presence of an associated soft tissue mass helped differentiate Ewing sarcoma from benign lytic lesions. The reproducibility of these results requires testing in larger series of patients using a variety of MR imaging equipment of differing field strengths.

Adolescent↗

Micrognathia after radiation therapy for childhood facial tumors. Report of two cases with long-term follow-up.

As the survival duration and cure rate of children with malignant conditions improve, awareness of long-term side effects is crucial. Two case reports demonstrate long-term changes in dentition and orofacial development in children who had been treated for facial malignant conditions. Changes induced mandibular and maxillary hypoplasia, partial anodontia, microdontia, altered eruption pattern, root stunting, and altered amelogenesis.

Child, Preschool↗

Persistent hyperplastic primary vitreous of the eye: imaging findings with pathologic correlation.

Persistent hyperplastic primary vitreous is a rare benign developmental disorder of the globe in which the embryonic hyaloid artery fails to regress normally, resulting in abnormal lenticular development and secondary changes of the retina and globe. This entity often presents a diagnostic challenge as it is the most common intraocular abnormality to be confused with retinoblastoma. Sonography, CT, and MR imaging have been used alone and in combination to diagnose this condition. This essay illustrates the imaging characteristics of persistent hyperplastic primary vitreous in two children with unilateral disease and in one child with bilateral disease as representative cases (Figs. 1-3). Available pathologic correlates also are presented.

Diagnosis, Differential↗

Malignant parotid tumors in patients previously treated for childhood cancer: clinical and imaging findings in eight cases.

OBJECTIVE: The purpose of this study was to describe the clinical and imaging characteristics of malignant neoplasms of the parotid gland in patients who had been successfully treated for childhood cancer. MATERIALS AND METHODS: Eight cases of parotid carcinoma were identified among survivors of primary childhood cancer treated at two centers. Medical records and imaging studies were reviewed to identify the frequency of second parotid tumors, to assess possible risk factors for their development, and to identify any specific MR or CT features of these tumors. RESULTS: Parotid tumors (mucoepidermoid carcinoma in seven cases and acinar cell carcinoma in one) were diagnosed a median of 9 years (range, 4-16 years) after diagnosis of the initial cancer. These tumors represented 6% of the second cancers diagnosed at one center, whereas salivary gland malignant neoplasms accounted for only 0.08% of primary cancers diagnosed during the same period. The patients ranged from 1 month to 14 years old at the time of initial diagnosis of acute leukemia (n = 7) or neuroblastoma (n = 1). All patients had received multiagent chemotherapy for their initial cancer, and six had received radiation to the head and/or neck. At diagnosis, the parotid tumors were painless, enlarging, periauricular masses without associated facial nerve palsy. Preoperative CT, performed in three cases, showed a solid enhancing parotid soft-tissue mass that was isodense with muscle. MR imaging in two cases showed inhomogeneously enhancing parotid masses. One was isointense with the contralateral parotid gland on T1- and T2-weighted images and hyperintense on contrast-enhanced T1-weighted images; the other was hypointense on T1-weighted images and showed inhomogeneous enhancement that was more intense than in the normal parotid gland. The borders were poorly defined in both these cases. CONCLUSION: Malignant parotid gland tumors are rare in the first two decades of life but may occur more often among patients who have previously received radiation therapy and/or chemotherapy. The CT and MR appearances of these lesions are variable.

Adolescent↗

Abnormal odontogenesis in children treated with radiation and chemotherapy: imaging findings.

Contemporary oncotherapy has improved survival of children with malignant diseases, but chemotherapy and radiotherapy also have deleterious effects. This essay illustrates the effects of chemotherapy and radiotherapy on dentition in children and adolescents. The illustrated abnormalities were seen on panoramic radiographs obtained before, during, and after treatment. Included are examples of hypodontia (partial anodontia), microdontia, altered eruption patterns, and root stunting. Comparison is made with normal dental development (Fig. 1).

Adolescent↗

Vaginal occlusion induced by cancer therapy.

Two adolescent girls with acquired vaginal occlusion and resultant hematometracolpos are presented. Both are long-term survivors of childhood malignancies, who prior to multiagent chemotherapy and pelvic irradiation had normal uteri by imaging. Both were amenorrheic post therapy. Routine follow-up computed tomography (CT) scans to monitor their malignancies demonstrated unexpected cystic pelvic masses, subsequently diagnosed as hematometracolpos.

Adolescent↗

Radiographic findings in 13q-syndrome.

Of 169 children with retinoblastoma treated at our institution between 1962 and 1993, 5 had concurrent severe mental retardation, hypotonia, and abnormalities of constitutional chromosome 13. The associated skeletal abnormalities of these 5 children are described and include delayed skeletal maturation, metaphyseal abnormalities, microcrania, facial bone abnormalities, and gracile long bones.

Abnormalities, Multiple↗

Typhlitis in children with cancer: a 30-year experience.

We identified 24 children treated for malignancies between 1962 and 1992 who had antemortem diagnoses of typhlitis that were confirmed on review. The study criteria specified the presence of fever, abdominal pain, and tenderness, with radiological evidence of right-sided colonic inflammation. Typhlitis was most frequent in patients treated for acute leukemias. Computed tomography and ultrasonography were more sensitive than plain radiography (false-negative rates, 15%, 23%, and 48%, respectively). The wider availability of these sensitive procedures and the increased intensity of chemotherapeutic regimens may account for a marked increase in the incidence of typhlitis over the past 5 years. Most patients responded to aggressive medical management, and typhlitis was fatal in only two cases (1 of 21 cases managed medically and 1 of 3 taken to surgery). Seven patients are alive > 1 year following the diagnosis. These findings contrast with prior descriptions of typhlitis as a preterminal event. Computed tomography and/or ultrasonography should be performed in all neutropenic patients with right-lower-quadrant signs to permit prompt diagnosis and treatment.

Adolescent↗

Multiple myeloma in an adolescent.

The radiographic findings of a 15-year-old Brazilian male with diagnosis of multiple myeloma are described. He presented with claudication and recent onset of tender painful swelling of the right mid leg. Radiographs showed wide spread "soap bubble" lesions of the skull, long bones, spine, pelvis, ribs, shoulders, and clavicles. The diagnosis was confirmed by the presence of a plasmacytoma on tissue biopsy (femur), serum IgG gammopathy and Bence-Jones proteinuria.

Adolescent↗

Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and perineal rhabdomyosarcoma.

Magnetic resonance (MR) imaging, ultrasound, and computed tomography (CT) were performed for diagnosis and follow-up of rhabdomyosarcoma (RMS) arising from the genitourinary tract in five children, pelvic musculature in one, and the perineum in three others. MR imaging performed at 1.0 T included the following: spin-density-, T1-, and T2-weighted images in all nine patients; gadopentatate dimeglumine (Gd-DTPA)-enhanced T1-weighted images in five; and short TI inversion recovery (STIR) images in two children. Longitudinal T1-weighted images were of additional help in localizing the primary tumor at diagnosis and detecting local spread. Residual or recurrent disease within the bladder was best detected by spin-density images. Gd-DTPA enhancement improved contrast in two studies, but paramagnetic artifacts obscured intravesical lesions in three other studies. STIR images disclosed pelvic and retroperitoneal lymphadenopathy. MR imaging is recommended as the key method of diagnosis and follow-up of pelvic RMS.

Adolescent↗

Remote congenital cerebral arteriovenous fistulae associated with aortic coarctation. Case report.

A neonate presented with anatomically discrete cerebral arteriovenous fistulae located in the right sylvian fissure and the cerebellar vermis that were initially detected by prenatal ultrasonography. Following delivery of the baby by caesarean section, both malformations were treated by surgical obliteration. These intracranial vascular lesions were associated with cardiac anomalies and a periductal coarctation of the aorta, which was treated with a left subclavian rotational arterial pedicle repair. Follow-up examination of the infant at age 13 months demonstrated an excellent clinical result with normalization of the circulation. The pathophysiology of this syndrome is discussed and the literature reviewed.

Abnormalities, Multiple↗

Multiple epiphyseal dysplasia misdiagnosed as bioinactive growth hormone disorder.

We have reported a case of multiple epiphyseal dysplasia (MED) that was misdiagnosed for many years as bioinactive growth hormone disorder. The condition was misdiagnosed because of its rarity and the physicians' lack of familiarity with it. MED should be considered in the differential diagnosis of a child with short stature and delayed growth. Close follow-up of a patient receiving growth hormone is necessary.

Child↗

Disseminated intrathoracic desmoplastic small round-cell tumor: a case report.

PURPOSE: Recently recognized as a distinct clinicopathologic entity, desmoplastic small round-cell tumors typically affect young men. These aggressive tumors usually arise in the abdomen; other sites of primary disease have been described only rarely. We report the case of an extraabdominal primary tumor with widespread dissemination, including the subcutaneous tissue, a previously unrecognized metastatic site. PATIENT AND METHODS: We describe the case of a 16-year-old boy with a primary extraabdominal metastatic desmoplastic small round-cell tumor. RESULTS: Our patient had a primary intrathoracic desmoplastic small round-cell tumor and widespread dissemination involving the subcutaneous tissue, kidney, liver, bone, and lymph nodes. Histopathologic analysis found intense desmoplasia and polyphenotypic expression of neural, muscle, and epithelial markers. Reverse transcriptase-polymerase chain reaction analysis of fresh tumor tissue confirmed the characteristic EWS-WT1 transcript. CONCLUSIONS: Broader than originally anticipated, the clinical spectrum of desmoplastic small round-cell tumors continues to evolve. Primary intrathoracic tumors with soft-tissue dissemination and polyphenotypic expression should prompt suspicion of this malignancy. Molecular analysis of fresh tumor tissue is an important adjunct to diagnosing this rare neoplasm.

Adolescent↗

A phase II trial of high-dose methotrexate in previously untreated children and adolescents with high-risk unresectable or metastatic rhabdomyosarcoma.

PURPOSE: The outcome for children with advanced-stage rhabdomyosarcoma remains poor with contemporary treatment regimens. Evaluation of new drugs is important to improve clinical outcome. Because methotrexate has shown promising activity in the treatment of patients with recurrent rhabdomyosarcoma, we conducted a phase II trial in untreated children with advanced-stage disease to evaluate the efficacy and safety of this agent. PATIENTS AND METHODS: Fifteen patients received 1 to 4 courses of high-dose methotrexate (HDMTX, 12 g/m2). Patients then received standard multiagent chemotherapy (vincristine, dactinomycin, cyclophosphamide, ifosfamide, mesna) with cytokine support and local radiotherapy. Patients who responded to HDMTX received four additional courses of this drug during continuation therapy. RESULTS: Twelve patients were evaluable for response after 2 or more courses of HDMTX; 4 achieved a partial response (33.3%). After administration of standard chemotherapy and radiation, the estimated 2-year progression-free survival for all patients was 56% (SD 15%). The drug was well-tolerated and the most common side effects included mucositis, transient elevation of transaminases, and neutropenia. The four patients who received additional courses of HDMTX during continuation therapy had limited toxicity which included mucositis, anemia, and thrombocytopenia. CONCLUSIONS: About one-third of children with previously untreated advanced-stage rhabdomyosarcoma responded to HDMTX. Its different mechanism of action and non-overlapping toxicity with other agents make HDMTX an attractive candidate for incorporation into front-line treatment regimens for rhabdomyosarcoma.

Adolescent↗