Results of a stage-based protocol for the treatment of retinoblastoma.

Schvartzman, Enrique; Chantada, Guillermo; Fandiño, Adriana; Dávila, Maylin; Raslawski, Elsa; Manzitti, Julio

doi:10.1200/jco.1996.14.5.1532

Cited by 133 publications

(99 citation statements)

References 13 publications

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“…12,14,15,33 Massive choroidal invasion was not a significant risk factor in this study and was not correlated with the occurrence of an adverse event.…”

Section: Commentmentioning

confidence: 62%

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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“…12,14,15,33 Massive choroidal invasion was not a significant risk factor in this study and was not correlated with the occurrence of an adverse event.…”

Section: Commentmentioning

confidence: 62%

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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“…1 In contrast, systemic disease is difficult to cure, although it usually responds to chemotherapy. [5][6][7] As in other malignancies of similar prognosis, high dose intensification of chemotherapy has been tried for retinoblastoma. Two case reports of patients with bone marrow metastases who received HDC in complete remission and survived in continuous remission 13 and 17 months thereafter gave a first hint to the possiblility of cure in metastatic retinoblastoma.…”

Section: Discussionmentioning

confidence: 99%

“…RB remains a high-risk malignancy, however, under certain circumstances, such as involvement of the cut end of the optic nerve, 3,4 extrascleral spread into the orbita 5,6 or lymphatic, or hematogenous dissemination. 7 Central nervous system disease has a particularly bad prognosis. 1,8 Attempts to control or even cure disseminated retinoblastoma have followed the strategies used in neuroblastoma, the other and more frequent disseminated neuroectodermal malignancy of childhood.…”

mentioning

confidence: 99%

“…9,10 Responses of metastatic retinoblastoma have been shown to respond to cisplatin-etoposide and cyclophosphamide-doxorubicin, 5,11 and to etoposide and carboplatin. 12 Since it has been very difficult to convert such responses into cures, 7 the dose-response concept has been advocated and tried for retinoblastoma as for neuroblastoma, germ-cell tumors or brain tumors. Unlike these diseases, the paucity of patients has prevented a larger prospective trial to date.…”

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confidence: 99%

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High-dose chemotherapy with autologous stem cell rescue in children with retinoblastoma

Kremens

Wieland

Reinhard

et al. 2003

Bone Marrow Transplant

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Summary:Children with metastatic retinoblastoma are considered to have a poor prognosis after conventional chemotherapy. We used high-dose chemotherapy (HDC) with peripheral hematopoietic stem cell transplantation in such patients in an attempt to improve their survival. Four patients with bone marrow metastases and one child with extraorbital disease were treated with HDC after achieving complete remission by enucleation and conventional chemotherapy. The child with extraorbital tumor was the only one to receive local irradiation. The conditioning regimen included thiotepa (900 mg/m 2 ), etoposide (40 mg/kg) and carboplatin (1.5 g/m 2 ) in four patients, and BCNU (300 mg/m 2 ), cyclophosphamide (6.8 g/m 2 ) and etoposide (1.6 g/m 2 ) in one child. Hematologic recovery occurred without delay in all patients. The main toxicities were diarrhea, mucositis and infectious complications. No toxic deaths or any major late toxicities were observed. The child treated with the BCNU regimen developed a meningeal relapse 10 months after HDC, which was partially resected and treated with conventional chemotherapy, but not with radiotherapy. He is in complete remission (CR) 105 months off treatment. The other patients are in CCR for 107, 57, 9 and 8 months after HDC. HDC with thiotepa, etoposide and carboplatin may represent a curative option for children with extrabulbar or disseminated retinoblastoma responsive to chemotherapy. It may control occult CNS disease. The necessity to irradiate these children and the curative potential of this strategy for patients with bulky CNS disease remain to be determined.

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“…In spite of intensive conventional chemotherapy and irradiation such patients are rarely cured. 6 Irradiation of the orbit may be useful in patients with isolated metastases in the orbit or initial infiltration of the optic nerve. 4 High-dose chemotherapy with autologous stem cell rescue can improve the prognosis of retinoblastoma patients with bone metastases, but despite high-dose chemotherapy and additional irradiation, a high incidence of relapse in the central nervous system (CNS) is described in the literature in patients, where the bone relapse site was the skull bones or the bone marrow.…”

Section: Discussionmentioning

confidence: 99%

Recurrent disseminated retinoblastoma in a 7-year-old girl treated successfully by high-dose chemotherapy and CD34-selected autologous peripheral blood stem cell transplantation

Hertzberg

Kremens

Velten

et al. 2001

Bone Marrow Transplant

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Summary:A localized retinoblastoma of the left eye in a 7-yearold girl, was treated by enucleation. She received no additional therapy. Four months later, metastases of retinoblastoma in the lymph nodes, bone and bone marrow were diagnosed. Relapse chemotherapy consisting of three courses of vincristine, cyclophosphamide, etoposide and carboplatin led to a second complete remission. Subsequent high-dose chemotherapy with thiotepa, etoposide and carboplatin and autologous stem cell transplantation with CD34-selected stem cells were successful, with no adverse effects. No radiotherapy was given and the girl remains in continuous second remission with a follow-up of more than 4 years. Bone Marrow Transplantation (2001) 27, 653-655. Keywords: metastatic retinoblastoma; haematogenous metastasis; high-dose chemotherapy; autologous stem cell transplantationThe prognosis of retinoblastoma with haematogenous spread into bone or bone marrow is very poor. 1 There are only a few reports of successful therapy for disseminated retinoblastoma. [2][3][4] We describe the case of a 7-year-old girl suffering from relapse of retinoblastoma with metastases in lymph nodes, bone and bone marrow. After treatment, including high-dose chemotherapy, the girl remains in continuous second remission with a follow-up of more than 4 years. Case reportA seven-year-old girl suspected of having retinoblastoma presented with a 3-week history of leucokoria and divergent strabism of her left eye. The right eye was normal on exam- ination. No cases of retinoblastoma have occurred in her family to date. On tomography and magnetic resonance imaging (MRI), there was no evidence of extraocular extension, invasion of the optic nerve, or metastases. Enucleation of the left eye was performed. Histological examination revealed a poorly differentiated retinoblastoma with marked choroidal infiltration, but no signs of invasion of the optic nerve or involvement of the sclera. There was no evidence of recurrence in the left orbit or of development of a retinoblastoma in the right eye on subsequent examinations. The girl was given no further treatment. Four months later, a pre-auricular tumour on the left, 1.5 cm in diameter, was observed. The tumour was excised and histopathological examination showed metastatic infiltration of a lymph node with retinoblastoma cells. Bone marrow aspiration was also positive for retinoblastoma cells. Bone scintigraphy showed pathological enhancement in the area of the left temporal skull and in the third thoracic vertebra. MRI of the head and vertebral column showed evidence of metastatic lymphadenopathy in the left pre-auricular region and along both carotid arteries, together with small metastases in the left fronto-temporal, temporal and right parietal skull bones. In addition, there were metastatic lesions of the fourth, fifth and eighth throracic vertebrae as well as of the third lumbar vertebra ( Figure 1A). The spinal fluid was free of retinoblastoma cells. In summary, the girl suffered from a metastatic relapse of her ret...

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Results of a stage-based protocol for the treatment of retinoblastoma.

Abstract: Chemotherapy is not warranted to prevent systemic metastasis for intraocular disease. Patients with extraocular orbital disease and had a good outcome with this therapy. Patients with metastatic disease fared poorly, except for those with isolated malignant preauricular adenopathy.

Cited by 133 publications

References 13 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

High-dose chemotherapy with autologous stem cell rescue in children with retinoblastoma

Recurrent disseminated retinoblastoma in a 7-year-old girl treated successfully by high-dose chemotherapy and CD34-selected autologous peripheral blood stem cell transplantation

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