The prognosis of retinoblastoma in terms of survival. A computer assisted study. Part II

Sutter, E. De; Havers, W.; Höpping, W; Zeller, Gregory G.; Alberti, W.

doi:10.3109/13816818709028522

Cited by 32 publications

(17 citation statements)

References 9 publications

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“…This frequency appears to be higher than that observed in other recent studies 30,37,38 and might be attributed to the advanced stage at time of initial diagnosis in patients coming from developing countries. Most recurrences (7 of 11; 64%) occurred within the first year after initial diagnosis of retinoblastoma.…”

Section: Commentcontrasting

confidence: 54%

A Study of Pathologic Risk Factors in Postchemoreduced, Enucleated Specimens of Advanced Retinoblastomas in a Developing Country

Suryawanshi

Ramadwar²,

Dikshit³

et al. 2011

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs). Objectives.—To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death. Design.—This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded. Results.—Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (>60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (>60%) was a significant risk factor in univariate analysis, which is the unique feature in this study. Conclusions.—Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.

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Section: Commentcontrasting

confidence: 54%

A Study of Pathologic Risk Factors in Postchemoreduced, Enucleated Specimens of Advanced Retinoblastomas in a Developing Country

Suryawanshi

Ramadwar²,

Dikshit³

et al. 2011

Archives of Pathology &Amp; Laboratory Medicine

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“…Diagnosis of patients with bilateral retinoblastoma is made earlier than patients with unilateral retinoblastoma (11 and 22 months, respectively). Compared to other solid tumors, retinoblastomas are small at the time of the diagnosis and can be successfully treated in more than 95% of cases (de Sutter et al, 1987), although patients still require constant lifelong follow-up for early detection of recurrent tumors or detection of secondary tumors (for a review about diagnosis and treatment of retinoblastoma, see Balmer et al, in this issue).…”

Section: Retinoblastoma: the Pediatric Tumormentioning

confidence: 99%

pRb2/p130: a new candidate for retinoblastoma tumor formation

Falco

Giordano

2006

Oncogene

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Retinoblastoma is the most common primary intraocular tumor in childhood. Mutations in both the alleles of the RB1 gene represent the causative agent for the tumor to occur. It is becoming evident that, although these alterations represent key events in the genesis of retinoblastoma, they are not sufficient per se for the tumor to develop, and other additional genetic or epigenetic alterations must occur. A supportive role in the genesis of retinoblastoma has recently been proposed for the RB1-related gene RB2/ p130. Additionally, several other genetic alterations involving different chromosomes have been described as relevant in the tumorigenic process. In this review we will analyse current knowledge about the molecular mechanisms involved in retinoblastoma, paying particular attention to the mechanisms of inactivation of the biological function of the retinoblastoma family of proteins.

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“…There are reports that claim that choroidal invasion by tumor does not contribute to a poor outcome, while others claim that choroidal invasion can result in mortality in the range of 11%-81% [32,[87][88][89][90][91][92][93][94][95][96][97][98][99][100][101][102]. The combination of choroidal invasion along with any degree of optic nerve invasion has also been recognized as a risk factor with an adverse influence on outcome by some groups [48,103].…”

Section: High-risk Histopathologic Features In Enucleated Eyes Of Patmentioning

confidence: 99%

“…When tumor cells are present in the optic nerve posterior to the lamina cribrosa, the mortality rate is in the range of 13%-69% [32,38,[87][88][89][90][91][92][93][94][95][96][97][98]. It is generally accepted that tumor involvement anterior to the lamina cribrosa is not associated with greater mortality.…”

Section: High-risk Histopathologic Features In Enucleated Eyes Of Patmentioning

confidence: 99%

“…When there is evidence of active tumor at the surgical margin of the optic nerve at the time of enucleation, the associated mortality rate has been described to be as high as 50%-81% [32,[87][88][89][90][91][92][93][94]. When tumor cells are present in the optic nerve posterior to the lamina cribrosa, the mortality rate is in the range of 13%-69% [32, 38,[87][88][89][90][91][92][93][94][95][96][97][98].…”

Section: High-risk Histopathologic Features In Enucleated Eyes Of Patmentioning

confidence: 99%

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Retinoblastoma: Review of Current Management

Chintagumpala

Chévez-Barrios

Paysse³

et al. 2007

The Oncologist

172

141

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After completing this course, the reader will be able to:1. Discuss the need for a multidisciplinary approach to the management of children with retinoblastoma.2. Identify the patient factors that need to be considered when choosing the most appropriate initial and subsequent treatment for a child with retinoblastoma.3. Describe the role of genetics in the follow-up of retinoblastoma patients.Access and take the CME test online and receive 1 AMA PRA Category 1 Credit ™ at CME.TheOncologist.com CME CME ABSTRACTThe most common ocular cancer in children is retinoblastoma. It affects approximately 300 children in the U.S. every year. It can affect one or both eyes and the disease can be inherited. Altered discoloration of the pupil and strabismus are the usual symptoms that lead to medical attention. Subsequent appropriate diagnostic studies and care provided by a multidisciplinary team, including an ophthalmologist, a pediatric oncologist, a radiation oncologist, and a geneticist, among others, often result in optimal short-term and long-term care. The best initial and subsequent treatments are based on whether the child has unilateral or bilateral disease, the stage of the disease, and the age of the child. Enucleation, chemotherapy, and various forms of radiation therapy along with local ophthalmic therapies can be used in the treatment of retinoblastoma. Cure rates are high in children when the tumor is confined to the eye and has not spread systemically or into the orbit or brain. Children with the heritable form of retinoblastoma are at high risk for developing subsequent malignancies, most commonly sarcomas. This risk is greater for those children with the heritable form of the disease who were exposed to ionizing radiation at age <1 year. Exciting discoveries using animal models are providing new insights into the development of this disease and opening new avenues for targeted therapies that may lead to high cure rates with minimal toxicities. EPIDEMIOLOGYRetinoblastoma is the most common malignant ocular tumor in childhood and affects approximately 1 in 18,000 children Ͻ5 years of age in the U.S. [1]. The incidence is higher in developing countries, and in some countries in Central and South America retinoblastoma is one of the most common solid tumor malignancies in children [2]. The reason for this higher incidence is not clear. Lower socioeconomic status and the presence of human papilloma virus sequences in the retinoblastoma tissue have been implicated [3]. A higher risk for diseases like retinoblastoma in children born through in vitro fertilization was described previously, but a recent large study does not support this association [4]. Approximately 80% of children with retinoblastoma are diagnosed before 3 years of age. The diagnosis of retinoblastoma in children 6 years or older is extremely rare. Children with bilateral retinoblastoma constitute about 20%-30%. Patients with bilateral disease usually present at a younger age (14 -16 months) than patients with unilateral disease (29 -30 months) [...

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The prognosis of retinoblastoma in terms of survival. A computer assisted study. Part II

Cited by 32 publications

References 9 publications

A Study of Pathologic Risk Factors in Postchemoreduced, Enucleated Specimens of Advanced Retinoblastomas in a Developing Country

A Study of Pathologic Risk Factors in Postchemoreduced, Enucleated Specimens of Advanced Retinoblastomas in a Developing Country

pRb2/p130: a new candidate for retinoblastoma tumor formation

Retinoblastoma: Review of Current Management

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