title: Mediastinal large-cell lymphoma with sclerosis (MLCLS).
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.
authors.affiliation: ICRF Department of Medical Oncology, St Bartholomew's Hospital, West Smithfield, London, UK.
authors.forename: A Z
authors.initials: AZ
authors.lastname: Rohatiner
authors.forename: J S
authors.initials: JS
authors.lastname: Whelan
authors.forename: R K
authors.initials: RK
authors.lastname: Ganjoo
authors.forename: A J
authors.initials: AJ
authors.lastname: Norton
authors.forename: A
authors.initials: A
authors.lastname: Wilson
authors.forename: T A
authors.initials: TA
authors.lastname: Lister
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008479:Mediastinal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008875:Middle Aged; D009367:Neoplasm Staging; D012189:Retrospective Studies; D012598:Sclerosis / Q000150:complications* / Q000473:pathology; D016019:Survival Analysis; D013997:Time Factors; D017211:Treatment Failure; D016896:Treatment Outcome