The significance of a nineteenth century definition in the era of genomics: linitis plastica

Abstract

Background: Linitis plastica due to gastric adenocarcinoma is a condition with a long history, but still lacks a standardized definition and is commonly confused with Borrmann type IV, Lauren diffuse, and signet-cell type gastric cancer. The absence of a clear definition is a problem when investigating its biological characteristics and role as a possible independent factor for prognosis. Nevertheless, the biological behavior for linitis plastica, which is unique, may be valuable in risk stratification and have implications for treatment. A definition of linitis plastica based on molecular or genomic criteria could represent a useful starting point for investigating new targeted therapies.

Main body: This literature review of linitis plastica will focus on the current classifications for gastric cancer, illustrating how the concept of linitis plastica relates to them in most cases and identifying a clear and reproducible definition. Moreover, the review will highlight the diagnostic challenges associated with linitis plastica, its prognostic implications, and the therapeutic options available. Future perspectives for its management are also addressed.

Conclusion: Linitis plastica is a carcinoma with a scirrhous stroma, involving the submucosal and muscular layers of the stomach even in the absence of mucosal alteration. In most cases, the primary cancer cells are signet-ring cells or scattered cells in the context of a poorly differentiated carcinoma. Diagnosis is challenging. Staging should be thorough, including diagnostic laparoscopy in all cases due to the high incidence of peritoneal involvement. The prognostic significance of linitis plastica is still controversial. Curative-intent surgery, when feasible, should be performed, with a multimodality treatment approach. Cancer-stroma interactions are important features of this disease, and represent attaining potential target for future therapies. Future pathologic assessments of gastric cancer should report the stromal reaction in order to allow better characterization of the tumor.

Keywords: Borrmann type IV; Diffuse; Gastric cancer; Linitis plastica; Scirrhous carcinoma; Signet ring.

Fig. 1

Borrmann types

Fig. 2

Venn diagrams depicting the relations between the current classifications and linitis plastica. a Western setting. b Eastern setting. c Molecular classifications

Fig. 3

Endoscopic aspect of two different types of linitis plastica: the waffle-like (c, d) and the flat type (e, f). a, b The aspect of a normal stomach

Fig. 4

Histological features of scirrhous tumors. Note the prominence of the stroma (especially on the left). Red arrow: a scattered signet-ring cell

Fig. 5

Different minimum exensions of the scirrhous reaction which may define a linitis plastica tumor (1/3 of the stomach surface)

Fig. 6

Upper gastrointestinal imaging (a–c) and computed tomography (d–f) diagnostic features of linitis plastica in three different patients (patient 1: a, d; patient 2: b, e; patient 3: c, f)