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 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An unusual presentation of acute megakaryocytic leukemia (AMKL) is reported in two young children. The first child had a 10-day history of ptosis of the right eyelid as the initial manifestation of AMKL, a clinical picture not previously described in this variant of leukemia. Computed tomographic scanning showed multiple intracranial mass lesions, and the diagnosis of AMKL was confirmed by immunophenotyping of bone marrow blasts. The second child had Down syndrome and received alkylating agents and radiation therapy for treatment of metastatic rhabdomyosarcoma of the orbit. She had AMKL as second malignancy. Both patients had acquired chromosome 21 anomalies in their leukemic blasts. The first patient, constitutionally normal, had an i(21q) in his leukemic blasts; the patient with constitutional trisomy 21 had tetrasomy 21 and additional chromosomal changes. The clinical symptoms and the results of morphologic, immunologic, and cytogenetic studies are discussed.
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PMID:Acute megakaryocytic leukemia in children. Clinical, immunologic, and cytogenetic findings in two patients. 183 41

The correction of marked blepharoptosis in patients with severe or potential keratopathy will worsen the keratopathy and possibly lead to the complications of corneal ulceration and endophthalmitis. The conjunctival flap--cosmetic shell--ptosis procedure is well suited to this difficult management problem. Patients are initially treated with a conjunctival flap to protect their cornea. Subsequently they are fit with a cosmetic shell, and finally they undergo surgery to correct their ptosis. This three-stage procedure has produced excellent cosmetic and functional results in two patients, one of whom had ptosis and severe radiation-induced keratopathy following the treatment of a rhabdomyosarcoma; the other patient had severe ptosis associated with lack of corneal sensation and orbicularis function following removal of a cerebral meningioma.
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PMID:Conjunctival flap-cosmetic shell-ptosis procedure. Treatment of blepharoptosis in severe keratopathy. 259 74

A case of rhabdomyosarcoma of the left atrium and left ventricle demonstrated by echocardiography was reported. A 31-year-old man was admitted to our hospital for evaluation of recently developed exertional dyspnea. A holosystolic murmur and a protodiastolic sound were audible at the apex. A chest X-ray showed pulmonary congestion without cardiomegaly. The two-dimensional echocardiogram showed a dense stratified mass of echoes occupying the medial half of the left ventricular cavity, and a part of the abnormal mass of echoes was observed to move toward the left ventricular outflow tract during systole. Another small mass attached to the anterior mitral leaflet was also observed to prolapse partly into the left atrium during systole. The interatrial septum showed a thick and hard band of echo in the short-axis view. Right cardiac catheterization revealed pulmonary hypertension and the levogram of the pulmonary angiography showed left atrial and left ventricular filing defects. The repeated echocardiographic study showed the growth of the abnormal mass. The patient underwent operation, but he died of congestive heart failure thereafter. The necropsy diagnosis was rhabdomyosarcoma of the heart, involving the left atrium and left ventricle.
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PMID:[Rhabdomyosarcoma of the heart involving the left ventricle and left atrium]. 664 8

Between January 1985 and June 1995 a total of 12 patients (9 female, 3 male) underwent total reconstruction of the lower urinary tract using gastric tissue. Their mean age was 10 years (range, 5-25 years). Total gastric bladder substitution was performed in seven patients, whereas five other patients had composite continent reservoirs (stomach plus bowel) created. The diagnoses were cloacal exstrophy, classic bladder exstrophy, bladder rhabdomyosarcoma, bilateral ectopic ureters, and VACTERL association. The mean follow-up period was 4.5 years (range, 16 months to 9 years). The average bladder capacity was 309 ml, and the compliance averaged 12.9 ml/cmH2O. Continence was achieved in all patients, but the continence mechanism often required revision. Renal deterioration was noted only in one patient due to an obstruction at the site of a transureteroureterostomy. The complications included hyponatremic, hypochloremic metabolic alkalosis (two patients); prolapse of the neovagina (one patient); a ureterovesical junction obstruction (three patients); and revision of the neourethra or Mitrofanoff (six patients). Revision of the gastric tube used as the catheterizable channel was performed in three instances. Lengthening of the tube, including nippling in one instance, corrected persistent incontinence. Distal stenosis of a gastric tube required several plastic procedures for correction. A ureteral Mitrofanoff developed a distal stricture necessitating revision. Persistent leaking of an appendiceal Mitrofanoff was corrected with reimplantation. A tapered ileal neourethra required a tunneling procedure followed by Teflon injection to provide urinary continence. Complications seen most often involved the continence mechanism of these gastric neobladders or composite urinary reservoirs. Persistence, patience, and perseverance led to total urinary continence and adequate reservoirs for urine storage in all patients.
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PMID:Continent gastric pouch. 873 Nov 28

A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma. Despite continued chemotherapy and radiotherapy, he ultimately died of the disease. Alveolar rhabdomyosarcoma from distant sites rarely metastasizes to the extraocular muscles. However, our case shows that alveolar rhabdomyosarcoma may metastasize to the orbit and involve a single muscle.
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PMID:Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle. 1287 88

We report the case of a 31-month-old girl, treated by the ifosfamide-vincristine-actinomycin chemotherapy protocol for vaginal rhabdomyosarcoma, who developed a unilateral left ptosis on day 36 of chemotherapy, i.e. 7 days after the fifth vincristine dose (1.5mg/m(2) or 0.90 mg). The cumulative vincristine dose was thus 4.50mg. The remainder of the neurological and systemic examinations were unremarkable. Laboratory testing and thoracic-cervical-cranial CT were normal. Other causes of ptosis were excluded. The ptosis decreased a few days after the infusion of vincristine and re-increased just after a new dose. It finally resolved upon lowering the dose of vincristine. According to our observations, the role of vincristine in the pathogenesis of this ptosis appears "likely". In such cases, maintenance of treatment is possible with adjustment of vincristine doses, but requires regular follow-up.
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PMID:[Vincristine-induced unilateral ptosis: case report and review of the literature]. 2389 11

Intrasellar rhabdomyosarcoma is rare, and when arising in collusion with an unrelated entity, can present a diagnostic pitfall. We describe a case of a 56-year-old woman who presented with right eye ptosis and a pituitary mass, which was resected and diagnosed as atypical pituitary adenoma. Because of residual disease and atypical pathology, the patient received adjuvant radiation. Tumor recurrence was noted after 5 months, and a second resection was performed. Nests of atypical pituitary adenoma cells within an exuberant spindle cell stroma were noted, with areas of presumed radiation necrosis. A second recurrence prompted a third resection. After expert consultation and additional immunohistochemical stains for muscle markers, a diagnosis was rendered of primary rhabdomyosarcoma arising in association with a pituitary adenoma. Despite its rarity, a high index of suspicion for rhabdomyosarcoma arising within a pituitary adenoma should be maintained in cases of atypical pituitary adenoma with a stromal response.
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PMID:Primary Sellar Rhabdomyosarcoma Arising in Association With a Pituitary Adenoma. 2742 70