Site-specific extranodal MALT lymphoma treatment
| Site . | First-line, localized disease . | First relapse . | Advanced disease (bilateral or stage IV) . | Notes . | |
|---|---|---|---|---|---|
| First choice . | Second choice . | ||||
| Skin (single lesion) | Excision or punch; observe in case of negative margins | Radiotherapy (if margins are positive) | Rituximab | Rituximab or R-chemo | — |
| Skin (contiguous) | Radiotherapy | Rituximab | Rituximab or R-chemo | Rituximab or R-chemo | — |
| Skin (multiple) | Rituximab | None | R-chemo | Rituximab or R-chemo | — |
| Parotid | Parotidectomy; observe in case of negative margins | Rituximab (if residual tissue or positive margins) | R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | Limit radiotherapy to reduce xerostomia (especially with Sjögren syndrome) |
| Orbit, lacrimal gland | Radiotherapy | Rituximab | Alternative first-line choice or R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | — |
| Conjunctiva | Rituximab | Radiotherapy | Alternative first-line choice or R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | Published experiences with intralesional rituximab |
| Thyroid | Thyroidectomy (total or partial)+R-chemo | None | R-chemo or targeted agents | R-chemo | Radiotherapy to be avoided to preserve residual thyroid function |
| Lung | Lob(ul)ectomy+ rituximab or rituximab only | None | R-chemo | R-chemo (if bilateral or systemic) | Radiotherapy to be avoided to reduce lung fibrosis; avoid extensive surgery |
| Stomach | Antibiotics (if HP-positive) | Radiotherapy (if HP-negative) or rituximab | Alternative first-line second choice or R-chemo | R-chemo | — |
| Small bowel | Surgical resection+rituximab | None | R-chemo | R-chemo (if multiple lesions detected on CT scan or systemic) | Radiotherapy to be limited |
| Kidney | Nephrectomy (total or partial)+rituximab | None | R-chemo | R-chemo | Use of radiotherapy: to be discussed |
| Breast | Nodulectomy+rituximab or rituximab only | None | R-chemo | R-chemo (if bilateral or systemic) | Use of radiotherapy: to be discussed for unilateral disease |
| Site . | First-line, localized disease . | First relapse . | Advanced disease (bilateral or stage IV) . | Notes . | |
|---|---|---|---|---|---|
| First choice . | Second choice . | ||||
| Skin (single lesion) | Excision or punch; observe in case of negative margins | Radiotherapy (if margins are positive) | Rituximab | Rituximab or R-chemo | — |
| Skin (contiguous) | Radiotherapy | Rituximab | Rituximab or R-chemo | Rituximab or R-chemo | — |
| Skin (multiple) | Rituximab | None | R-chemo | Rituximab or R-chemo | — |
| Parotid | Parotidectomy; observe in case of negative margins | Rituximab (if residual tissue or positive margins) | R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | Limit radiotherapy to reduce xerostomia (especially with Sjögren syndrome) |
| Orbit, lacrimal gland | Radiotherapy | Rituximab | Alternative first-line choice or R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | — |
| Conjunctiva | Rituximab | Radiotherapy | Alternative first-line choice or R-chemo | Rituximab (if bilateral); R-chemo (if systemic) | Published experiences with intralesional rituximab |
| Thyroid | Thyroidectomy (total or partial)+R-chemo | None | R-chemo or targeted agents | R-chemo | Radiotherapy to be avoided to preserve residual thyroid function |
| Lung | Lob(ul)ectomy+ rituximab or rituximab only | None | R-chemo | R-chemo (if bilateral or systemic) | Radiotherapy to be avoided to reduce lung fibrosis; avoid extensive surgery |
| Stomach | Antibiotics (if HP-positive) | Radiotherapy (if HP-negative) or rituximab | Alternative first-line second choice or R-chemo | R-chemo | — |
| Small bowel | Surgical resection+rituximab | None | R-chemo | R-chemo (if multiple lesions detected on CT scan or systemic) | Radiotherapy to be limited |
| Kidney | Nephrectomy (total or partial)+rituximab | None | R-chemo | R-chemo | Use of radiotherapy: to be discussed |
| Breast | Nodulectomy+rituximab or rituximab only | None | R-chemo | R-chemo (if bilateral or systemic) | Use of radiotherapy: to be discussed for unilateral disease |
First-line treatment choices for localized disease are provided, when appropriate, according to our personal experience. Treatment of first relapse may be based on the approach not previously chosen for first-line treatment (alternative choice). Stage IV (according to Ann Arbor) indicates any dissemination of the disease to any nodal site and/or marrow and/or more than one extranodal site (apart from the initial extranodal site).
R-chemo, chemoimmunotherapy.