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Lymphoma, Mediastinal B-Cell

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Treatment Strategy

Note-For the unusual patients with small mediastinal masses (<10 cm or <1/3 of the maximal chest diameter) & with stage I disease, treatment with 3 cycles of CHOP could be considered. If a complete remission is achieved after three cycles, this could be followed by involved field radiation.
REF:NEJM 339: 21-26, 1998

All other patients to receive 6 cycles of chemotherapy. If the gallium/PET scan remains positive or a significant residual mass is present (> 20% of the original volume), the risk for recurrence is high. Therefore, consolidation with XRT or HDT is recommended.

Most patients with PMBL will initially respond to treatment with shrinkage of the tumor, but rapid disease progression between treatment cycles is not uncommon.

A retrospective analysis of 35 patients with PMBL treated with high-dose cyclophosphamide, carmustine, and etoposide (CBV) + ABMT was done. Patients with primary refractory disease had 58% long-term DFS, & patients with relapsed disease had 27% long-term DFS. The strongest predictor of PFS was chemotherapy responsiveness immediately before transplant. But even in chemotherapy-refractory patients, 33% long-term survival was observed. Therefore, although HDC & ABMT are not usually recommended in refractory NHL, PMBL may be an exception.

Chemotherapy Regimen:
R+CHOP
R+ICE

Patients with PMBL present with a rapidly growing mass, originating from the anterior & superior mediastinum & surrounding structures.
The median age at presentation:30
Slight preponderance of females.
Diagnosed because of symptoms arising from compression of mediastinal structures.

PMBL is a B-cell tumor (rearrangement of the Ig gene).
CD20 positive
CD3 negative
Expression of surface or cytoplasmic Ig often absent.
This results in the inability to detect monoclonality by kappa/lambda staining.
CD21 expression and expression of class I and/or class II histocompatibility molecules is also typically absent.
CD 30 expression is sometimes present, but usually it is weak and inhomogeneous in contrast to the strong and uniform expression of CD30 in Hodgkins disease or anaplastic large-cell lymphoma

PRIMARY MEDIASTINAL B-cell lymphoma (PMBL) is a diffuse large B-cell lymphoma that arises in the thymus & was first described in 1980. It mainly affects young adults. PMBL was recognized as a specific entity in the REAL classification & accounts for approximately 5% of all patients with aggressive lymphomas (a frequency similar to that of Burkitts disease and lymphoblastic lymphoma).
Because of its skewed age-distribution, PMBL accounts for a much higher proportion of younger patients & of those undergoing autologous transplantation. There is uncertainty about the relevance & especially the management of this disorder

The management of PMBL patients should be guided by assessment of prognostic features in individual patients as well as by a weighing of long-term risks & benefits. The most widely accepted predictor of outcome is the response to initial treatment.