Is central nervous system prophylaxis necessary in ocular adnexal lymphoma?

Alvaro Restrepo, Luis E. Raez, Gerald E. Byrne, Thomas Johnson, Paul Ossi, Pasquale W Benedetto, Kara Hamilton, Clarence C. Whitcomb, Peter A. Cassileth

Research output: Contribution to journalArticle

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Abstract

Purpose: To examine the frequency of metastasis to the eye and central nervous system (CNS) from ocular adnexal lymphomas and to evaluate whether CNS prophylaxis is appropriate for these tumors. Patients and Methods: Seventy-one patients with biopsy-confirmed ocular adnexal lymphomas were evaluated between 1989 and 1995. The lymphomas were subclassified histopathologically according to the new Revised European-American Lymphoma (REAL) criteria. Molecular genetic analysis of tumor cell DNA was done by Southern blot. Patients had a complete ophthalmologic evaluation and metastatic work-up and were then routinely followed up by an ophthalmologist and a medical oncologist. Results: The 34 men and 37 women studied had a median age of 67 years (23 to 92). Ocular adnexal lymphomas were situated in the orbit in 54 patients, in the conjunctiva in 14 patients, and in the eyelid in 3 patients. Bilateral involvement occurred in 11 patients. The most common histologic diagnoses were (54 patients, 76%) extra-nodal marginal zone lymphomas and small lymphocytic lymphomas in 10 patients (14%). Molecular genetic analysis performed in all patients confirmed a monoclonal B-cell population in 55 patients (77%), including a single rearrangement of the immunoglobulin heavy chain gene in 14 patients, and more than two rearrangements in 41 patients. No patients had isolated T-cell gene rearrangements. Localized ocular adnexal lymphoma was diagnosed in 43 patients (61%), 17 patients (24%) were found to have concurrent extraocular lymphoma on metastatic work-up and 11 patients (15%) had a previous diagnosis of systemic lymphoma before the onset of their ocular tumor. The median duration of follow-up was 20 months. Overall, 32 patients (45%) had tumors, which remained localized to the orbit adnexa. Eleven patients (15%) relapsed, but none had eye or central nervous system involvement nor required CNS- directed therapy. Although eight patients died, only two died as a direct result of systemic lymphoma. No patient received CNS prophylaxis with either intrathecal chemotherapy and/or radiation therapy. Conclusion: Ocular adnexal lymphomas are rare non-Hodgkin's B-cell lymphomas. Metastatic involvement of the eye or central nervous system is rare and CNS prophylaxis with radiotherapy or chemotherapy is unnecessary.

Original languageEnglish
Pages (from-to)269-273
Number of pages5
JournalCritical Reviews in Oncogenesis
Volume9
Issue number3-4
StatePublished - Dec 1 1998

Fingerprint

Lymphoma
Central Nervous System
Orbit
Molecular Biology
Neoplasms
Radiotherapy
T-Lymphocyte Gene Rearrangement
Immunoglobulin Heavy Chain Genes
Drug Therapy
Conjunctiva
B-Cell Lymphoma
Eyelids
B-Cell Chronic Lymphocytic Leukemia
Southern Blotting
Non-Hodgkin's Lymphoma

Keywords

  • B-cell lymphoma
  • Central nervous system neoplasms
  • Non-Hodgkin's lymphoma
  • Ocular lymphoma

ASJC Scopus subject areas

  • Cancer Research

Cite this

Restrepo, A., Raez, L. E., Byrne, G. E., Johnson, T., Ossi, P., Benedetto, P. W., ... Cassileth, P. A. (1998). Is central nervous system prophylaxis necessary in ocular adnexal lymphoma? Critical Reviews in Oncogenesis, 9(3-4), 269-273.

Is central nervous system prophylaxis necessary in ocular adnexal lymphoma? / Restrepo, Alvaro; Raez, Luis E.; Byrne, Gerald E.; Johnson, Thomas; Ossi, Paul; Benedetto, Pasquale W; Hamilton, Kara; Whitcomb, Clarence C.; Cassileth, Peter A.

In: Critical Reviews in Oncogenesis, Vol. 9, No. 3-4, 01.12.1998, p. 269-273.

Research output: Contribution to journalArticle

Restrepo, A, Raez, LE, Byrne, GE, Johnson, T, Ossi, P, Benedetto, PW, Hamilton, K, Whitcomb, CC & Cassileth, PA 1998, 'Is central nervous system prophylaxis necessary in ocular adnexal lymphoma?', Critical Reviews in Oncogenesis, vol. 9, no. 3-4, pp. 269-273.
Restrepo, Alvaro ; Raez, Luis E. ; Byrne, Gerald E. ; Johnson, Thomas ; Ossi, Paul ; Benedetto, Pasquale W ; Hamilton, Kara ; Whitcomb, Clarence C. ; Cassileth, Peter A. / Is central nervous system prophylaxis necessary in ocular adnexal lymphoma?. In: Critical Reviews in Oncogenesis. 1998 ; Vol. 9, No. 3-4. pp. 269-273.
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abstract = "Purpose: To examine the frequency of metastasis to the eye and central nervous system (CNS) from ocular adnexal lymphomas and to evaluate whether CNS prophylaxis is appropriate for these tumors. Patients and Methods: Seventy-one patients with biopsy-confirmed ocular adnexal lymphomas were evaluated between 1989 and 1995. The lymphomas were subclassified histopathologically according to the new Revised European-American Lymphoma (REAL) criteria. Molecular genetic analysis of tumor cell DNA was done by Southern blot. Patients had a complete ophthalmologic evaluation and metastatic work-up and were then routinely followed up by an ophthalmologist and a medical oncologist. Results: The 34 men and 37 women studied had a median age of 67 years (23 to 92). Ocular adnexal lymphomas were situated in the orbit in 54 patients, in the conjunctiva in 14 patients, and in the eyelid in 3 patients. Bilateral involvement occurred in 11 patients. The most common histologic diagnoses were (54 patients, 76{\%}) extra-nodal marginal zone lymphomas and small lymphocytic lymphomas in 10 patients (14{\%}). Molecular genetic analysis performed in all patients confirmed a monoclonal B-cell population in 55 patients (77{\%}), including a single rearrangement of the immunoglobulin heavy chain gene in 14 patients, and more than two rearrangements in 41 patients. No patients had isolated T-cell gene rearrangements. Localized ocular adnexal lymphoma was diagnosed in 43 patients (61{\%}), 17 patients (24{\%}) were found to have concurrent extraocular lymphoma on metastatic work-up and 11 patients (15{\%}) had a previous diagnosis of systemic lymphoma before the onset of their ocular tumor. The median duration of follow-up was 20 months. Overall, 32 patients (45{\%}) had tumors, which remained localized to the orbit adnexa. Eleven patients (15{\%}) relapsed, but none had eye or central nervous system involvement nor required CNS- directed therapy. Although eight patients died, only two died as a direct result of systemic lymphoma. No patient received CNS prophylaxis with either intrathecal chemotherapy and/or radiation therapy. Conclusion: Ocular adnexal lymphomas are rare non-Hodgkin's B-cell lymphomas. Metastatic involvement of the eye or central nervous system is rare and CNS prophylaxis with radiotherapy or chemotherapy is unnecessary.",
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AU - Raez, Luis E.

AU - Byrne, Gerald E.

AU - Johnson, Thomas

AU - Ossi, Paul

AU - Benedetto, Pasquale W

AU - Hamilton, Kara

AU - Whitcomb, Clarence C.

AU - Cassileth, Peter A.

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N2 - Purpose: To examine the frequency of metastasis to the eye and central nervous system (CNS) from ocular adnexal lymphomas and to evaluate whether CNS prophylaxis is appropriate for these tumors. Patients and Methods: Seventy-one patients with biopsy-confirmed ocular adnexal lymphomas were evaluated between 1989 and 1995. The lymphomas were subclassified histopathologically according to the new Revised European-American Lymphoma (REAL) criteria. Molecular genetic analysis of tumor cell DNA was done by Southern blot. Patients had a complete ophthalmologic evaluation and metastatic work-up and were then routinely followed up by an ophthalmologist and a medical oncologist. Results: The 34 men and 37 women studied had a median age of 67 years (23 to 92). Ocular adnexal lymphomas were situated in the orbit in 54 patients, in the conjunctiva in 14 patients, and in the eyelid in 3 patients. Bilateral involvement occurred in 11 patients. The most common histologic diagnoses were (54 patients, 76%) extra-nodal marginal zone lymphomas and small lymphocytic lymphomas in 10 patients (14%). Molecular genetic analysis performed in all patients confirmed a monoclonal B-cell population in 55 patients (77%), including a single rearrangement of the immunoglobulin heavy chain gene in 14 patients, and more than two rearrangements in 41 patients. No patients had isolated T-cell gene rearrangements. Localized ocular adnexal lymphoma was diagnosed in 43 patients (61%), 17 patients (24%) were found to have concurrent extraocular lymphoma on metastatic work-up and 11 patients (15%) had a previous diagnosis of systemic lymphoma before the onset of their ocular tumor. The median duration of follow-up was 20 months. Overall, 32 patients (45%) had tumors, which remained localized to the orbit adnexa. Eleven patients (15%) relapsed, but none had eye or central nervous system involvement nor required CNS- directed therapy. Although eight patients died, only two died as a direct result of systemic lymphoma. No patient received CNS prophylaxis with either intrathecal chemotherapy and/or radiation therapy. Conclusion: Ocular adnexal lymphomas are rare non-Hodgkin's B-cell lymphomas. Metastatic involvement of the eye or central nervous system is rare and CNS prophylaxis with radiotherapy or chemotherapy is unnecessary.

AB - Purpose: To examine the frequency of metastasis to the eye and central nervous system (CNS) from ocular adnexal lymphomas and to evaluate whether CNS prophylaxis is appropriate for these tumors. Patients and Methods: Seventy-one patients with biopsy-confirmed ocular adnexal lymphomas were evaluated between 1989 and 1995. The lymphomas were subclassified histopathologically according to the new Revised European-American Lymphoma (REAL) criteria. Molecular genetic analysis of tumor cell DNA was done by Southern blot. Patients had a complete ophthalmologic evaluation and metastatic work-up and were then routinely followed up by an ophthalmologist and a medical oncologist. Results: The 34 men and 37 women studied had a median age of 67 years (23 to 92). Ocular adnexal lymphomas were situated in the orbit in 54 patients, in the conjunctiva in 14 patients, and in the eyelid in 3 patients. Bilateral involvement occurred in 11 patients. The most common histologic diagnoses were (54 patients, 76%) extra-nodal marginal zone lymphomas and small lymphocytic lymphomas in 10 patients (14%). Molecular genetic analysis performed in all patients confirmed a monoclonal B-cell population in 55 patients (77%), including a single rearrangement of the immunoglobulin heavy chain gene in 14 patients, and more than two rearrangements in 41 patients. No patients had isolated T-cell gene rearrangements. Localized ocular adnexal lymphoma was diagnosed in 43 patients (61%), 17 patients (24%) were found to have concurrent extraocular lymphoma on metastatic work-up and 11 patients (15%) had a previous diagnosis of systemic lymphoma before the onset of their ocular tumor. The median duration of follow-up was 20 months. Overall, 32 patients (45%) had tumors, which remained localized to the orbit adnexa. Eleven patients (15%) relapsed, but none had eye or central nervous system involvement nor required CNS- directed therapy. Although eight patients died, only two died as a direct result of systemic lymphoma. No patient received CNS prophylaxis with either intrathecal chemotherapy and/or radiation therapy. Conclusion: Ocular adnexal lymphomas are rare non-Hodgkin's B-cell lymphomas. Metastatic involvement of the eye or central nervous system is rare and CNS prophylaxis with radiotherapy or chemotherapy is unnecessary.

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