1/14. 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 = 1keywords = gland (Clic here for more details about this article) |
2/14. 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 = 2keywords = gland (Clic here for more details about this article) |
3/14. retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/14. Intracranial malignancy in patients with bilateral retinoblastoma.Tumors from two patients with bilateral retinoblastoma and intracranial malignancies were studied from a clinical, histopathological, and an immunohistochemical standpoint. An antiserum derived against tissue cultured Y-79 retinoblastoma cells was utilized in order to examine the surface and cytoplasmic antigens of the two brain tumors, histopathologically diagnosed as an embryonal cell carcinoma (case 1), a pinealoblastoma (case 2), and one ocular retinoblastoma. The two brain tumors reacted with the antiserum to different degrees. In comparison, the ocular tumor reacted strongly. None of the tissues examined were found to contain retinal S- or P-antigen. The concept of retinoblastomas arising de novofrom the pineal gland and the association of intracranial malignancy occurring in retinoblastoma is stressed. The antigenic and immunologic relationships between normal retina and pineal gland and retinoblastoma, neuroblastoma, and pinealoblastomas are discussed.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
5/14. Pleomorphic adenoma of the lacrimal gland in an 18-year-old girl irradiated for bilateral retinoblastoma.PURPOSE: To report a case of pleomorphic adenoma of the lacrimal gland following irradiation for bilateral retinoblastoma. MATERIALS AND methods: Case report. A 4-month-old girl, with bilateral retinoblastoma, underwent enucleation of the right eye, systemic chemotherapy and bilateral external beam irradiation with a lateral field. The right anophthalmic socket (because of infiltration of the optic nerve) and the left eye (for relapse) were irradiated;. 17 years later, she developed a mass in the superotemporal quadrant of the left orbit. The mass was completely excised. RESULTS: Pleomorphic adenoma is rare in children and teenagers; it usually presents as a painless, slow growing mass in healthy adults. In this case, it developed as a second primary tumor after irradiation for retinoblastoma.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
6/14. Intrasellar primitive neuroectodermal tumor (PNET) in familial retinoblastoma: a variant of "trilateral retinoblastoma".An infant with bilateral familial retinoblastomas was found at age 6 months to have a large mass within the region of the sella turcica. The histology, immunoreactivity and ultrastructure of the tumor showed primitive neuroectodermal cells. A small number of cells showed positive immunostaining to retinal S antigen. This represents a rare form of so-called trilateral retinoblastoma. In most cases of trilateral retinoblastoma, the intracranial non-metastatic tumor is within the pineal gland. The occurrence of such a tumor in the region of sella turcica is very unusual and its possible significance is discussed in regard to our current understanding of the susceptibility to carcinogenesis conferred by inheritance of the mutant retinoblastoma gene, Rb-1.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/14. Second primary tumour following retinoblastoma.Reported is a child who at the age of 18 months had presented with a left retinoblastoma. The lesion had involved the optic nerve (Stage IV), and was successfully treated by enucleation and radiotherapy. The radiation field did not include the mandible or the submandibular triangle. He was followed up until he left the area aged 11 years. He represented aged 12 years with a progressive swelling of the left submandibular gland. histology of the surgically excised submandibular gland revealed a mucoepidermoid carcinoma of the submandibular gland. This case highlights the need for continual surveillance in survivors of retinoblastoma, as second primary tumours occur both within and outside of previous radiation fields. The clinical association between retinoblastoma and mucoepidermoid carcinoma of the submandibular gland has not been previously reported.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
8/14. Metastatic retinoblastoma of the mandible.Two cases of oral metastasis of retinoblastoma are reported. The first case was seen in iraq and the second case in england. review of the literautre revealed that when oral metastasis occurs, the prognosis must be considered to be grave; this was confirmed by Case 2. In each case there was metastasis in the symphyseal region, which followed enucleation of the affected orbit. Although the prognosis is poor, recent studies indicate that immunologic techniques may be useful to monitor the response to treatment of the primary lesion.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/14. Trilateral retinoblastoma: ocular and pineal retinoblastomas.Trilateral retinoblastomas, the syndrome of bilateral retinoblastoma associated with ectopic retinoblastoma in the pineal gland, is rare but well recognized. In contrast to bilateral retinoblastomas alone, the ocular retinoblastomas in trilateral retinoblastoma develop before the age of 6 months, and a family history positive for retinoblastoma is usually obtained. The retinal tumors are often quiescent at the time that the pineal tumor is discovered, and show no evidence of metastatic spread after enucleation of the globes. Pathologically, the pineal tumor is indistinguishable from the ocular retinoblastoma. The pathophysiology of this syndrome is not well understood, but a germinal mutation is thought to target photoreceptor tissue for further postzygotic mutation. Eventual expression depends on the inherited host resistance to the carcinogenic manifestation of these genes. The low host resistance of trilateral retinoblastoma is evident by the early age of presentation, the multicentric occurrence of the tumor, and the high early mortality rate despite aggressive management.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/14. Trilateral retinoblastoma. Report of two cases.Two children had bilateral retinoblastoma and a morphologically similar intracranial neoplasm localized to the region of the pineal gland as shown on computed tomography and at postmortem examination. The first child's intracranial malignant neoplasm produced symptoms that indicated its probable presence when the ocular tumors were first diagnosed. With the second child, there was a latent period of more than three years between the diagnosis and initial management of the ocular neoplasms and the onset of symptoms from the pineal tumor. We diagnosed both cases as trilateral retinoblastoma, which in our experience is a relatively frequent cause of mortality in patients with heritable retinoblastoma.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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