Mediastinal large-cell lymphoma with sclerosis (MLCLS) (original) (raw)

Abstract

In a retrospective analysis encompassing a 14 year period (1978-92), 22 patients (age range 19-71, median 30 years) were identified as having mediastinal large-cell lymphoma with sclerosis on the basis of clinical and pathological features. At presentation, 15/22 had 'bulky' disease and 11/22 had evidence of superior vena caval obstruction. Thirteen patients had stage II disease (6,II; 7,IIE), nine presented with stage IV disease. Complete remission (CR) was achieved in only 4/22 patients with the initial adriamycin-containing regimen. 'Good partial remission' (no clinical evidence of disease, minimal abnormalities of uncertain significance on radiological investigation) was achieved in a further seven patients and 'poor partial remission' (a reduction in measurable disease > 50%) in four, giving an overall response rate of 15/22 (68%). One patient died within 48 h of arrival at the hospital; 16 of the 17 remaining patients in whom anything less than CR was achieved subsequently received additional, alternative treatment (one chemotherapy, six mediastinal radiotherapy, nine both treatment modalities) but in only 2/16 did this result in any further degree of response. With a median follow-up of 5 1/2 years, 10/22 patients remain well without progression between 6 months and 14 years (5/6 in whom CR was eventually achieved and 5/11 in whom only partial remission was ever documented). The seven patients in whom the initial treatment demonstrably failed have all died. These results suggest that a proportion of patients with this rare subtype of high-grade B-cell lymphoma may be cured by chemotherapy alone and that the presence of a residual mediastinal mass after treatment does not necessarily imply treatment failure. However, patients in whom the initial chemotherapy fails have a very grave prognosis.

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Selected References

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  1. Addis B. J., Isaacson P. G. Large cell lymphoma of the mediastinum: a B-cell tumour of probable thymic origin. Histopathology. 1986 Apr;10(4):379–390. doi: 10.1111/j.1365-2559.1986.tb02491.x. [DOI] [PubMed] [Google Scholar]
  2. Bertini M., Orsucci L., Vitolo U., Levis A., Todeschini G., Meneghini V., Novero D., Tarella C., Gallo E., Luxi G. Stage II large B-cell lymphoma with sclerosis treated with MACOP-B. Ann Oncol. 1991 Nov-Dec;2(10):733–737. doi: 10.1093/oxfordjournals.annonc.a057853. [DOI] [PubMed] [Google Scholar]
  3. Chen J. L., Osborne B. M., Butler J. J. Residual fibrous masses in treated Hodgkin's disease. Cancer. 1987 Aug 1;60(3):407–413. doi: 10.1002/1097-0142(19870801)60:3<407::aid-cncr2820600321>3.0.co;2-n. [DOI] [PubMed] [Google Scholar]
  4. Dhaliwal H. S., Rohatiner A. Z., Gregory W., Richards M. A., Johnson P. W., Whelan J. S., Gallagher C. J., Matthews J., Ganesan T. S., Barnett M. J. Combination chemotherapy for intermediate and high grade non-Hodgkin's lymphoma. Br J Cancer. 1993 Oct;68(4):767–774. doi: 10.1038/bjc.1993.425. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Isaacson P. G., Norton A. J., Addis B. J. The human thymus contains a novel population of B lymphocytes. Lancet. 1987 Dec 26;2(8574):1488–1491. doi: 10.1016/s0140-6736(87)92622-5. [DOI] [PubMed] [Google Scholar]
  6. Jacobson J. O., Aisenberg A. C., Lamarre L., Willett C. G., Linggood R. M., Miketic L. M., Harris N. L. Mediastinal large cell lymphoma. An uncommon subset of adult lymphoma curable with combined modality therapy. Cancer. 1988 Nov 1;62(9):1893–1898. doi: 10.1002/1097-0142(19881101)62:9<1893::aid-cncr2820620904>3.0.co;2-x. [DOI] [PubMed] [Google Scholar]
  7. Jochelson M., Mauch P., Balikian J., Rosenthal D., Canellos G. The significance of the residual mediastinal mass in treated Hodgkin's disease. J Clin Oncol. 1985 May;3(5):637–640. doi: 10.1200/JCO.1985.3.5.637. [DOI] [PubMed] [Google Scholar]
  8. Klimo P., Connors J. M. MACOP-B chemotherapy for the treatment of diffuse large-cell lymphoma. Ann Intern Med. 1985 May;102(5):596–602. doi: 10.7326/0003-4819-102-5-596. [DOI] [PubMed] [Google Scholar]
  9. Lamarre L., Jacobson J. O., Aisenberg A. C., Harris N. L. Primary large cell lymphoma of the mediastinum. A histologic and immunophenotypic study of 29 cases. Am J Surg Pathol. 1989 Sep;13(9):730–739. doi: 10.1097/00000478-198909000-00002. [DOI] [PubMed] [Google Scholar]
  10. Lister T. A. The management of follicular lymphoma. Ann Oncol. 1991 Feb;2 (Suppl 2):131–135. doi: 10.1007/978-1-4899-7305-4_20. [DOI] [PubMed] [Google Scholar]
  11. Menestrina F., Chilosi M., Bonetti F., Lestani M., Scarpa A., Novelli P., Doglioni C., Todeschini G., Ambrosetti A., Fiore-Donati L. Mediastinal large-cell lymphoma of B-type, with sclerosis: histopathological and immunohistochemical study of eight cases. Histopathology. 1986 Jun;10(6):589–600. doi: 10.1111/j.1365-2559.1986.tb02512.x. [DOI] [PubMed] [Google Scholar]
  12. Miller J. B., Variakojis D., Bitran J. D., Sweet D. L., Kinzie J. J., Golomb H. M., Ultmann J. E. Diffuse histiocytic lymphoma with sclerosis: a clinicopathologic entity frequently causing superior venacaval obstruction. Cancer. 1981 Feb 15;47(4):748–756. doi: 10.1002/1097-0142(19810215)47:4<748::aid-cncr2820470420>3.0.co;2-8. [DOI] [PubMed] [Google Scholar]
  13. Perrone T., Frizzera G., Rosai J. Mediastinal diffuse large-cell lymphoma with sclerosis. A clinicopathologic study of 60 cases. Am J Surg Pathol. 1986 Mar;10(3):176–191. doi: 10.1097/00000478-198603000-00005. [DOI] [PubMed] [Google Scholar]
  14. Radford J. A., Cowan R. A., Flanagan M., Dunn G., Crowther D., Johnson R. J., Eddleston B. The significance of residual mediastinal abnormality on the chest radiograph following treatment for Hodgkin's disease. J Clin Oncol. 1988 Jun;6(6):940–946. doi: 10.1200/JCO.1988.6.6.940. [DOI] [PubMed] [Google Scholar]
  15. Scarpa A., Bonetti F., Menestrina F., Menegazzi M., Chilosi M., Lestani M., Bovolenta C., Zamboni G., Fiore-Donati L. Mediastinal large-cell lymphoma with sclerosis. Genotypic analysis establishes its B nature. Virchows Arch A Pathol Anat Histopathol. 1987;412(1):17–21. doi: 10.1007/BF00750725. [DOI] [PubMed] [Google Scholar]
  16. Scarpa A., Borgato L., Chilosi M., Capelli P., Menestrina F., Bonetti F., Zamboni G., Pizzolo G., Hirohashi S., Fiore-Donati L. Evidence of c-myc gene abnormalities in mediastinal large B-cell lymphoma of young adult age. Blood. 1991 Aug 1;78(3):780–788. [PubMed] [Google Scholar]
  17. Todeschini G., Ambrosetti A., Meneghini V., Pizzolo G., Menestrina F., Chilosi M., Benedetti F., Veneri D., Cetto G. L., Perona G. Mediastinal large-B-cell lymphoma with sclerosis: a clinical study of 21 patients. J Clin Oncol. 1990 May;8(5):804–808. doi: 10.1200/JCO.1990.8.5.804. [DOI] [PubMed] [Google Scholar]
  18. Trump D. L., Mann R. B. Diffuse large cell and undifferentiated lymphomas with prominent mediastinal involvement. Cancer. 1982 Jul 15;50(2):277–282. doi: 10.1002/1097-0142(19820715)50:2<277::aid-cncr2820500218>3.0.co;2-9. [DOI] [PubMed] [Google Scholar]
  19. Whelan J. S., Davis C. L., Rohatiner A. Z., Leahy M., MacCallum P. K., Gupta R. K., Matthews J., Norton A. J., Amess J. A., Lister T. A. Etoposide in combination with intermediate dose cytosine arabinoside (ID ARA C) given with the intention of further myeloablative therapy for the treatment of refractory or recurrent hematological malignancy. Hematol Oncol. 1992 Mar-Apr;10(2):87–94. doi: 10.1002/hon.2900100204. [DOI] [PubMed] [Google Scholar]
  20. Yousem S. A., Weiss L. M., Warnke R. A. Primary mediastinal non-Hodgkin's lymphomas: a morphologic and immunologic study of 19 cases. Am J Clin Pathol. 1985 Jun;83(6):676–680. doi: 10.1093/ajcp/83.6.676. [DOI] [PubMed] [Google Scholar]
  21. al-Sharabati M., Chittal S., Duga-Neulat I., Laurent G., Mazerolles C., al-Saati T., Brousset P., Delsol G. Primary anterior mediastinal B-cell lymphoma. A clinicopathologic and immunohistochemical study of 16 cases. Cancer. 1991 May 15;67(10):2579–2587. doi: 10.1002/1097-0142(19910515)67:10<2579::aid-cncr2820671030>3.0.co;2-h. [DOI] [PubMed] [Google Scholar]