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A Cain

Publications and source records attributed to A Cain.

18 recordsLinked to original sources
PubMed2003

A comparative study of the optical characteristics of commonly used sinoscopes: do you know where you are looking?

Endoscopic ethmoidectomy is a delicate operation on the thin, honey-comb-like laminae of the ethmoidal sinuses. A high degree of precision is required to minimize disruption of the natural sinus mucociliary clearance system and to reduce the risk of orbital and skull base complications. It is therefore important that surgeons are aware of variation in the optical characteristics of the rod-lens telescopes produced by different manufacturers. A simple but precision endoscope goniometer was designed and manufactured. This was used to carry out a comparative study of the sinus telescopes in common use. The results showed significant variation in the view angle of the four brands of telescopes (Karl Storz, Olympus, Richards Smith & Nephew, and Richard Wolf) tested. Differences in the position of the horizon in the visual field, and different degrees of visuospatial distortion were evident. We also noted and measured the blind angle of telescopes, which, by virtue of a high degree of angulation, cannot view the horizon.

Endoscopy↗
PubMed2000

Endoscopic endonasal ligation of the sphenopalatine artery.

Internal maxillary artery ligation for persistent posterior epistaxis is traditionally performed via the transantral approach. Although usually effective in controlling the bleeding it carries significant risks, including damage to the infraorbital nerve, damage to dentition and oro-antral fistula formation. The alternative procedure of endoscopic ligation of the sphenopalatine artery at its exit from the sphenopalatine foramen avoids the morbidity associated with the transantral approach. The technique we describe involves the use of standard FESS instruments and a Ligge Clip applicator. This operation is well within the capability of the Otolaryngologist/Rhinologist experienced in endoscopic sinus surgery.

Arteries↗
PubMed1999

Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: the St. Jude Children's Research Hospital experience.

BACKGROUND: Because the natural history of pediatric patients with metastatic nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) had not been well described, we retrospectively reviewed our single-institution experience with these tumors. PROCEDURE: We identified 26 patients with metastatic NRSTS who were treated at St. Jude Children's Research Hospital from December 1971 through July 1995. We evaluated the characteristics of each patient, including age, sex, primary site, stage, type of therapy received, and outcome. Sites of metastatic disease at diagnosis and relapse were noted. RESULTS: The median age of the 26 study patients at diagnosis was 14. 8 years; 54% of patients were male and 69% were white. The most common histologies were synovial sarcoma, alveolar soft-part sarcoma, and malignant fibrous histiocytoma. Most primary tumors (73% of cases) occurred in the trunk or extremities. Most patients presented with large (>5 cm), high-grade lesions. All 26 patients received chemotherapy, and 8 responded to an ifosfamide- or cyclophosphamide-doxorubicin-based regimen. Radiotherapy was administered to 15 patients, and 13 had a partial or complete resection of the primary tumor. Six patients underwent thoracotomy. The estimated 2-year survival for the cohort was 34.6% +/- 8.9%; the 2-year progression-free survival was 15.4% +/- 6.3%. The lung was the most common site of failure. CONCLUSIONS: Children with metastatic NRSTS have a poor outcome, which is similar to that in adults. More effective systemic chemotherapy is needed to facilitate complete surgical resection of primary and metastatic sites. Aggressive pulmonary metastatectomy can increase disease control.

Adolescent↗
PubMed1999

Anticipating parental death in families with young children.

Findings from a community study of parentally bereaved children demonstrate that forewarning of death is not associated with more favorable mental health outcomes than is sudden death of a parent. The applicability of the of the general anticipatory grief literature to the circumstances of children facing the loss of a parent is called into question, and the need to assess more carefully the effect on children of a parent's protracted terminal illness is emphasized.

Adolescent↗
PubMed1999

Multifocal osteosarcoma following retinoblastoma.

Three survivors of retinoblastoma, one with hereditary bilateral and two with nonhereditary (spontaneous) unilateral disease, developed multifocal osteosarcoma. For one patient, unilateral retinoblastoma was followed by primitive neuroepithelioma at age 13 years. Multifocal chondroblastic osteosarcoma represented the patient's third malignant neoplasm. The course of multifocal osteosarcoma in these three patients compares to that of multifocal osteosarcoma which presents de novo in other patients without prior retinoblastoma.

Adult↗
PubMed1997

Comparison of primary osteosarcoma of flat bones with secondary osteosarcoma of any site.

BACKGROUND: The authors hypothesized that the outcomes for young patients who present with primary osteosarcoma of flat bones or secondary osteosarcoma after other primary malignancies are similar because the sites of these tumors rarely makes them amenable to complete surgical resection. METHODS: The authors reviewed their experience with 27 patients with primary osteosarcomas involving flat bones and 18 patients with secondary osteosarcomas of any site, evaluating treatment, metastatic patterns, and outcome. RESULTS: Eleven of the 27 primary osteosarcomas and 10 of the 18 secondary osteosarcomas were at least partially resectable at the time of initial diagnosis. At last follow-up, 10 of the 27 patients with primary flat bone osteosarcoma were alive, eight of whom were disease free. Three of 18 with the secondary osteosarcoma patients were alive, including one with locally recurrent disease after recent disease resections. Chemotherapy with single or combinations of agents rarely yielded responses for primary flat bone or secondary osteosarcomas. CONCLUSIONS: Outcome for both groups of patients was poor due to metastases and failure of local control. Sixteen of the 27 patients with primary osteosarcoma of flat bones died of their tumor; an additional patient died of doxorubicin-related cardiomyopathy and had microscopic disease in the primary site at autopsy. Thirteen of the 18 patients with secondary osteosarcoma died of their second metastatic cancer, 2 additional patients died of doxorubicin-related cardiomyopathy/coccidioidomycosis, and another of acute renal toxicity.

Adolescent↗
PubMed1997

Brain metastases in children with melanoma.

BACKGROUND: Brain metastases complicate the course of malignant melanoma in at least 20% of adult cases. These events are commonly preceded by metastases to other sites. Due to the rarity of malignant melanoma in children, little is known about the incidence, clinical features, and outcome of children with melanoma who develop brain metastases. METHODS: The authors reviewed the records of 44 children with malignant melanoma treated at St. Jude Children's Research Hospital over a 33-year period. Eight (18%) developed brain metastases during the course of their disease. The authors reviewed the clinical and radiologic features of six of these cases, for whom complete clinical information and imaging studies were available. RESULTS: The median age at diagnosis of malignant melanoma was 15 years (range, 11-21 years). Brain metastases developed a median of 20 months (range, 0-50 months) after diagnosis and were preceded by metastases to other organs in 5 patients. In most cases, lesions were supratentorial and multiple. Most showed radiologic signs of intralesional hemorrhage. All patients received whole brain radiotherapy, and one had surgical resection. Three patients received chemotherapy. Five patients died a median of 5 months (range, 2-10 months) after diagnosis of brain involvement. One patient, who had a single brain metastasis at diagnosis, is alive more than 34 months later. CONCLUSIONS: Brain metastases develop in a significant proportion of children with malignant melanoma and are associated with a poor outcome. The incidence, features, and outcome in children do not appear to differ from those in adults.

Adolescent↗
PubMed1996

Alveolar soft part sarcoma in children and adolescents: clinical features and outcome of 11 patients.

The clinical features and response to therapy of pediatric alveolar soft part sarcoma, a rare soft tissue sarcoma of uncertain histogenesis, have not been previously described in detail in the literature. We retrospectively reviewed the clinical characteristics of all patients with alveolar soft part sarcoma who were seen at our institution over a 32-year period. We found 11 patients with the diagnosis of alveolar soft part sarcoma. Their ages ranged from 2.8-16 years (median 9.8). Staging was determined using the Intergroup Rhabdomyosarcoma Study clinical grouping system and the UICC TNM system. Accordingly, there were six patients with grossly resected tumors (clinical groups I and II) and five with unresected or metastatic disease (clinical groups III and IV). Children with resected disease were more likely to have smaller noninvasive tumors. The main feature predictive of survival was tumor resectability, since chemotherapy in various combinations failed to produce significant tumor responses. Nine patients are disease-free with a median follow-up of 11.9 years. Surgical resection remains the mainstay of therapy for pediatric alveolar soft part sarcoma. Since active chemotherapy agents have not been identified, patients with unresected or metastatic disease may benefit from experimental agents. The survival rate of this cohort is superior to that seen in adults.

Adolescent↗
PubMedDate not supplied

Clear cell sarcoma of soft tissues in children and young adults: the St. Jude Children's Research Hospital experience.

Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.

Adolescent↗
PubMedDate not supplied

Brain metastases in pediatric Ewing sarcoma and rhabdomyosarcoma: the St. Jude Children's Research Hospital experience.

PURPOSE: Although brain metastases rarely occur in children with solid tumors, pediatric Ewing sarcoma (ES) and rhabdomyosarcoma (RMS) are among those most likely to metastasize to the brain. The authors review their institution's experience of brain metastases of ES and RMS. PATIENTS AND METHODS: The clinical characteristics, therapy, and outcome of all patients treated at St. Jude Children's Research Hospital over a 36-year period who had ES or RMS with brain metastases were reviewed. RESULTS: Of 419 patients with RMS, 10 (2.4%) had brain metastases. Of 335 patients with ES, 11 (3.3%) had brain metastases. The median age of the 21 patients was 10.4 years (range, 0.4-18.0 years) at the time of primary diagnosis. All had clinical signs of central nervous system (CNS) involvement. Outcome was dismal: The median duration of survival after diagnosis of brain metastasis was 2.7 months. The estimated survival 1 year after detection of brain involvement was 23.8%+/-8.5% (mean +/- standard error). One patient, who underwent chemotherapy, surgical resection, and radiotherapy, at the time of this writing is a long-term survivor. CONCLUSIONS: Brain metastases are rare in children with ES and RMS, but carry a grave prognosis. Because most brain metastases are accompanied by signs of neurologic involvement, routine imaging studies of asymptomatic children are not necessary. Combined-modality treatment offers the best chance of long-term survival.

Adolescent↗
PubMedDate not supplied

Hematologic abnormalities and acute myeloid leukemia in children and adolescents administered intensified chemotherapy for the Ewing sarcoma family of tumors.

PURPOSE: Current treatment of the Ewing sarcoma family of tumors (ESFT) includes intensive multiagent chemotherapy with topoisomerase II inhibitors, alkylating agents, and granulocyte colony-stimulating factor (G-CSF). This treatment approach has been associated with myelodysplasia and acute myeloid leukemia. Because macrocytosis and thrombocytopenia are distinctive features of myelodysplasia, the authors evaluated a cohort of patients treated for ESFT to determine the degree and duration of macrocytosis and thrombocytopenia and their relation with the development of therapy-related hematologic malignancies. PATIENTS AND METHODS: The study group consisted of 73 patients with ESFT treated on two consecutive protocols (EW92 and EW87). Both chemotherapy regimens incorporated the same agents but differed in cumulative drug dose, dose per course, and the use of G-CSF. Platelet counts and the mean corpuscular volume (MCV) of erythrocytes were determined at diagnosis and during follow-up visits after completion of treatment. RESULTS: Patients in the EW92 group had significantly greater MCVs after treatment than did the less intensively treated EW87 group. These changes persisted throughout the 40-month observation period. Patients in the EW92 group also had lesser mean platelet counts after treatment than those in the EW87 group. MCV differences (from baseline) were inversely related to platelet counts. The cumulative incidence of treatment-related acute myeloid leukemia was 7.8%+/-4.7% at 4 years in the EW92 group and zero in the EW87 group. CONCLUSION: Patients treated for ESFT with intensive chemotherapy that includes large doses of alkylators, topoisomerase II inhibitors, and G-CSF characteristically have persistently elevated MCVs and decreased platelet counts after completion of therapy. These hematologic abnormalities may represent stem cell damage, predisposing patients to myelodysplasia and acute myeloid leukemia, but further study is needed to establish this relation.

Adolescent↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children's Research Hospital.

The clinical characteristics and outcome of children with dermatofibrosarcoma protuberans (DFSP) treated at a single institution over a 34-year period were reviewed. A retrospective chart review identified five children with dermatofibrosarcoma protuberans. The diagnosis was confirmed in all cases by one of the authors. Age; sex; clinical group; tumor invasiveness and size, nodal status, and metastases (TNM stage); therapy; and outcome were retrieved from the medical records of these patients. The median age at diagnosis was 12.5 years. There were four boys, and all tumors arose in the extremities or trunk. All children underwent at least two surgical procedures to achieve negative or surgically microscopic positive margins. In addition, one patient received adjuvant radiotherapy for microscopically positive margins. All patients survive free of disease 1 month to 16.5 years after diagnosis. Dermatofibrosarcoma protuberans is rare among children and adolescents. Recognition of this pathologic entity is important for its proper treatment. Surgical resection (with 3-cm margins) is currently the standard therapy for DFSP.

Adolescent↗