MALT lymphoma of the esophagus is a rare entity. In 2017, there were less than 20 reported cases of esophageal MALT lymphomas, most of which have been diagnosed in Japan (3). They are usually localized, but we found one case report of metastasis to the lungs and stomach (4) and one report of recurrence in the stomach and lung after initial radiotherapy (5). All treatment modalities have been used with generally good results, chemotherapy (CHOP-based) and resection being the most frequent (3, 4, 6–11). The main results of the available studies are summarized in Table 1 .

Despite the limited number of patients treated with radiotherapy in our review, extrapolation from other tumor sites would encourage the use of this modality, as MALT lymphomas are usually sensitive to radiation. In gastric lymphomas, radiotherapy can lead to 85-100% remission rates (4-5 years of disease-free survival), according to multiple small studies, especially in low-grade stages I and IIE (12–14). For this reason, since surgery—often indicated due to inconclusive biopsy results—and radiation therapy are effective treatment options with high remission rates, the safety profiles and tolerability of both modalities should be considered when treating patients with low-grade esophageal MALT lymphomas. Low doses of radiation therapy, compared to surgical resection, can be curative and associated with minimal side effects.

We found a single case of follicular lymphoma, described by Taal et al. in 1976. Based on the Working Formulation classification, it was diagnosed as a “small cleaved cell non-Hodgkin’s lymphoma” associated with extensive fistula formation, mainly to the left main bronchus. No manifestations of lymphoma were found outside of the esophagus. As dyspnea developed, the patient received a single dose of emergency chemotherapy but died three weeks later from sepsis (15).

DLBCL predominantly involves immunocompromised patients with HIV infection as a potential risk factor (16). All treatment modalities combined, outcomes were generally poor, and robust data is still lacking. While the results summarized in Table 2 show a 50% complete response rate, in most case reports, follow-up duration was either lacking or was very short, thus masking early disease progression, which would have been attributed to treatment failure. However, combined modalities, including radiation therapy, seem to yield better results, as all 5 patients who received either chemotherapy plus radiation therapy or surgery followed by chemotherapy and radiation therapy had a complete response, with the reports covering a follow-up period of up to 3 years.

Similarly to other types of esophageal lymphomas, T-cell lymphoma reports are scarce, and detailed treatment outcomes are missing in the literature. While two cases of primary NK/T-cell lymphoma were treated with chemotherapy only and achieved complete remission with a follow-up of up to 24 months (17, 18), a 40-year-old man in stage IE was also treated with CHOP but died due to complications (19).

Chemotherapy should be the mainstay of treatment, with a possible benefit when combined with radiation therapy. A review including Chinese case reports noted that systemic chemotherapy combined with concurrent radiotherapy might be an effective treatment for esophageal NK/T-cell lymphoma (17). Our literature search tends to validate this conclusion, as we noted a few cases of diffuse large T-cell Lymphomas treated with radiation therapy followed by either the HADAP regimen (patient survival of 14 months) or the CHOP regimen (2 patients, one with a survival of 7 months who died of sepsis despite evidence of early anti-tumor response, and one who survived ten months of follow-up) (20, 21). A fourth patient received two cycles of systemic chemotherapy with concurrent radiotherapy, but his general condition gradually worsened, and he passed away four months after admission (22).

Additionally, in 1999, 65 Gy irradiation was used to treat a primary esophageal T-cell lymphoma in clinical stage I(E)B with complete remission (23). The role of radiation therapy in T-cell lymphomas warrants further study, as radiotherapy has been considered of paramount importance for patients with clinically localized nasal disease, with approximately 70% of patients achieving complete remission after treatment (24).

Multifocal gut involvement has been described in mantle cell lymphoma (MCL) (25). However, esophageal involvement is uncommon. A retrospective study collecting data from 35 MCL patients with GI involvement found that the esophagus was affected in only 2 (5.7%) cases (26). All published cases were treated with chemotherapy, as lesions were usually seen in multiple locations in the GI tract. Of the few available cases, one died before receiving treatment (27); one was lost to follow-up, two had a good response to chemotherapy (28, 29), while one had no response to treatment (30). A “mantle cell lymphoma-like tumor” was treated with rituximab postoperatively and has been disease-free for more than 28 months after surgery (31).

ALCL was first described in 1985 as a large-cell neoplasm with an expression of CD30 in all neoplastic cells (32). It is an uncommon T-cell lymphoma, defined as a separate entity since the 2008 WHO lymphoma classification (33). ALCL can be ALK+ or ALK-. Radiotherapy in esophageal ALCL has not yet been reported. Only one case report in 1992 mentioned a treatment plan including chemotherapy followed by radiotherapy, but the patient was lost to follow-up (34).

The few other published cases were treated with chemotherapy (usually CHOP), sometimes followed by autologous peripheral blood hematopoietic stem cell transplantation, with partial (2 cases) or complete (2 cases) remission (2, 35–37). Also, one patient was diagnosed post-mortem (38). Usually, ALK+ lymphoma is very sensitive to chemotherapy; thus, if the diagnosis is made without the need for thoracotomy and resection, chemoradiotherapy may be sufficient.

In other types and locations of ALCL, such as limited-stage systemic ALCL or breast implant-associated ALCL, radiation therapy was used in association with chemotherapy (39): for example, following doxorubicin-based chemotherapy and radiotherapy, in early-stage primary systemic anaplastic large-cell lymphoma, the 5-year overall survival (OS), progression-free survival (PFS), and local control rates for all patients were 84.4%, 63.6%, and 90.8%, respectively (40).

No case of adult esophageal Burkitt lymphoma was found in our database search. However, although it does not match our initial inclusion criteria, we found a case report of a 17-year-old patient in India with stage II intermediate risk, Group-B disease by Murphy staging system managed with LMB-96 protocol for intermediate-risk BL. She had a complete response and no recurrence after nine months (41).

We found a single case of plasmablastic lymphoma treated in 2016 with resection followed by chemotherapy, without further details (42).