Linitis plastica (LP), a subset of gastric adenocarcinoma (GA), has been considered as a fatal disease with few management options. Little evidence has been reported regarding the role for surgical therapy in treating LP. A retrospective review of GA patients with LP from the surveillance, epidemiology, and end results database (2004–2009) was performed. 29,440 patients with GA were identified, of whom 948 (3.2%) had LP. After matching for American Joint Commission on Cancer (AJCC) stage, LP patients had significantly worse 5-year disease specific survival (DSS) compared with GA (6 vs 34%, P < 0.001). For potentially resectable LP patients (i.e., stage I–III), 5-year DSS was 0 per cent for no treatment and for radiation therapy alone, 18 per cent for both and surgery and radiation, and 20 per cent for surgery alone(P < 0.001). LP is a marker of poor survival in patients with GA. However, surgical resection provides the best oncologic outcomes in these patients with a 20 per cent 5-year DSS in patients with loco-regional disease.

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Microangiopathic hemolytic anemia (MAHA) can be an uncommon presentation of an underlying malignancy, most often due to signet-ring cell carcinoma (SRCC). Additionally, pure SRCC in a breast primary-tumor comprises <2% of all breast cancers (Shin SY, Park H, Chae SW, Woo HY. Microangiopathic hemolytic anemia as the first manifestation of metastatic signet-ring cell carcinoma of unknown origin: a case report and review of literature. Kor J Lab Med 2011;31:157–61). To the best of our knowledge, the combination of these two entities, pure breast primary SRCC along with MAHA, has not been reported. Here, we present such a rare case. We also evaluate the current literature regarding this and similar disease processes, of which evidence is scarce and further research is needed.

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