1/51. Trilateral retinoblastoma with an RB1 deletion inherited from a carrier mother: a case report.A presentation of intracranial tumor in bilateral and unilateral retinoblastoma with or without family history is termed as trilateral retinoblastoma (TRB). It always occurs either as a pineal tumor or supra/parasellar tumor, which differ in presentation and prognosis. We report here the first case of TRB with transmission of retinoblastoma gene (RB1) deletion from an unaffected mother (a carrier), presenting as concurrent intracranial neoplasm with bilateral retinoblastoma. The presence of RB1 mutation in both child and mother could be responsible for development of intracranial neoplasm which occurred simultaneously with bilateral RB in our patient. Our patient, who had a suprasellar mass, received radiation and intrathecal chemotherapy, and died 6 months after diagnosis. The occurrence of intracranial tumor in an asymptomatic stage can be avoided by routine computed tomography (CT) and magnetic resonance imaging (MRI) scan, and improved survival can be achieved by aggressive multimodality therapy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/51. 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.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
3/51. Nonocular cancer in retinoblastoma survivors.From a review of the records of 2,302 patients with retinoblastoma collated from CPMC and AFIP, it was found that retinoblastoma patients who survived their original eye cancer have a high incidence of second nonocular malignancies. The second neoplasms occur almost exclusively (97.5%) in patients who have had bilateral retinoblastoma, although retinoblastoma is much more commonly unilateral. The second neoplasms have appeared between 1 and 42 years after the successful treatment of retinoblastoma and have been fatal in approximately 85% of cases. Seventy-one percent of patients develop tumors in the field of the radiation beam; many of these were following treatment with low doses of radiation and after short latent periods. Nineteen percent of patients develop tumors clearly out of the field of radiation (eg, osteogenic sarcoma of the femur). retinoblastoma patients appear to be unusually radio-sensitive to low doses of radiation and develop tumors in the field of radiation following treatment of 3,500 rads. They have a high incidence of nonocular tumors clearly distant from the radiation beam develop nonocular malignancies even if no radiation has been given. The incidence of multiple malignancies in patients successfully treated for retinoblastoma appears to be higher than for any other primary malignancy whether they receive radiation or not.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/51. Second malignant neoplasms of the head and neck in survivors of retinoblastoma.retinoblastoma is a malignant tumor of the embryonic retina. Although it is rare, it is the most common primary eye tumor of childhood. life expectancy following treatment is now excellent, but survivors who have heritable retinoblastoma face an increased risk of a second malignant head or neck neoplasm. A second neoplasm, which often occurs in the irradiated field of the original tumor, has become the most significant threat to the survival of these patients. We report the case of a young girl who was cured of her retinoblastoma only to later develop a second nonocular tumor that metastasized to the superficial parotid gland. She underwent a superficial parotidectomy and neck dissection, but the malignancy eventually recurred and required further surgery and radiation therapy. In this article, we discuss the etiology, incidence, sites of occurrence, and management options for a second malignant neoplasm in retinoblastoma survivors. The head and neck surgeon must be vigilant in the diagnosis and management of second neoplasms in this patient population because they often occur in irradiated fields; surgical management is important to patient survival.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
5/51. Osteogenic sarcoma after retinoblastoma radiotherapy.Development of osteogenic sarcoma after retinoblastoma radiotherapy in three patients, two of whom were siblings, is reported. Pluridirectional tomography and plain skull radiography demonstrated soft tissue masses, sinus opacification, and bone destruction and sclerosis in all three patients. Computed tomography reliably indicated the presence or absence of intracranial tumor extension in the two patients in whom it was performed. Radionuclide bone scanning was a useful adjunct for osteosarcomatous detection.- - - - - - - - - - ranking = 0.011432807442469keywords = soft (Clic here for more details about this article) |
6/51. A high-grade primary leiomyosarcoma of the bladder in a survivor of retinoblastoma.Second nonocular malignancies develop with increased incidence in patients with hereditary retinoblastoma. osteosarcoma is by far the most common type with an incidence of up to 50%, followed by soft tissue sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases have been reported in the past 2 decades, one in the liver and another one in the urinary bladder, both of which developed after cyclophosphamide therapy. Here we report a case of vesical leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after the diagnosis of a hereditary retinoblastoma. The patient's retinoblastoma was treated with unilateral enucleation without adjuvant radiation or chemotherapy. We believe that this is the first report of vesical leiomyosarcoma occurring in a patient with retinoblastoma without a prior history of radiation or chemotherapy. This report is significant not only because of the rarity of vesical leiomyosarcoma as a second nonocular tumor in retinoblastoma patients, but also because of the infrequency of vesical leiomyosarcoma in general. We also investigated the potential molecular pathogenesis of the leiomyosarcoma.- - - - - - - - - - ranking = 0.011432807442469keywords = soft (Clic here for more details about this article) |
7/51. Trilateral retinoblastoma variant indicative of the relevance of the retinoblastoma tumor-suppressor pathway to medulloblastomas in humans.Results of recent studies have led investigators to suggest that the retinoblastoma tumor-suppressor (rb) gene plays an underappreciated role in the genesis of brain tumors. Such tumors cause significant rates of mortality in children suffering from hereditary retinoblastoma. It has been assumed that the pineal gland, which is ontogenetically related to the retina, accounts for the intracranial origin of these trilateral neoplasms. To address this issue, the authors describe an unusual trilateral retinoblastoma variant. The authors provide a detailed clinicopathological correlation by describing the case of a child with bilateral retinoblastoma who died of a medulloblastoma. The intraocular and intracranial neoplasms were characterized by performing detailed imaging, histopathological, and postmortem studies. karyotype analysis and fluorescence in situ hybridization were used to define the chromosomal defect carried by the patient and members of her family. An insertion of the q12.3q21.3 segment of chromosome 13 into chromosome 18 at band q23 was identified in members of the patient's family. This translocation was unbalanced in the proband. The intraocular and cerebellar neoplasms were found to be separate primary neoplasms. Furthermore, the pineal gland was normal and the cerebellar neoplasm arose within the vermis as a medulloblastoma. Finally, the two neoplasms had different and characteristically identifiable cytolological and immunohistochemical profiles. The findings of the present study, taken together with those of recent molecular and transgenic studies, support the emerging concept that rb inactivation is not restricted to central nervous system regions of photoreceptor lineage and that inactivation of this tumor suppressor pathway may be relevant to the determination of etiological factors leading to medulloblastoma in humans.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
8/51. Cystic retinoblastoma.A grossly cystic retinoblastoma, presumably a form of the diffuse infiltrating type, mimicked uveitis in an 81/2-year-old boy. A review of 44 cases indicated that grossly (clinically) detectably cysts are rare in this neoplasm, although microcysts are relatively common. Lesions of this nature, especially if presenting with a history of trauma, as in our patient, may lead the ophthalmologist to diagnose uveitis. The diagnosis of retinoblastoma may thus be unduly delayed.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
9/51. Soft tissue, pelvic, and urinary bladder leiomyosarcoma as second neoplasm following hereditary retinoblastoma.This report describes two patients who developed leiomyosarcomas, one involving the subcutaneous tissue of the thigh and the pelvic soft tissues and the other the urinary bladder, following hereditary retinoblastoma 36 and 38 years earlier, respectively. There is an increased risk of the development of sarcoma, most commonly osteosarcoma, as a second malignancy following hereditary retinoblastoma. leiomyosarcoma developing as a second malignancy has rarely been reported and most have occurred in the field of previous radiotherapy. The literature on leiomyosarcoma occurring as a second neoplasm following retinoblastoma is reviewed.- - - - - - - - - - ranking = 2.5114328074425keywords = neoplasm, soft (Clic here for more details about this article) |
10/51. leiomyosarcoma of urinary bladder following cyclophosphamide therapy: report of two cases.leiomyosarcoma of urinary bladder is rare, although it is the most common mesenchymal tumor in adults. We report two cases of this tumor following cyclophosphamide therapy. The first case is from a 53-year-old man with Wegener's granulomatosis treated for 6 years with cyclophosphamide. He presented with painless hematuria, and the initial biopsy of the bladder tumor revealed a malignant spindle cell neoplasm. A final diagnosis of leiomyosarcoma was made on radical cystoprostatectomy. The second example is from a 21-year-old man who had received cyclophosphamide in early infancy for a bilateral retinoblastoma. He also presented with painless hematuria, and a bladder tumor was resected transurethrally and diagnosed as leiomyosarcoma. He underwent partial cystectomy two months later. cyclophosphamide, when used for a neoplastic or non-neoplastic condition, is associated with an increased risk of developing bladder cancer. The distribution of histologic subtypes differs from that seen in spontaneous bladder tumors. A review of the literature shows an increased proportion of squamous cell carcinomas and sarcomas, especially leiomyosarcomas in cyclophosphamide exposed patients. acrolein, a cytotoxic metabolite of cyclophosphamide excreted in urine, is regarded as the most likely causative agent.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
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