Gastric linitis plastica (GLP) is a very rare and aggressive subtype of diffuse gastric carcinoma with scirrhous stroma. It usually infiltrates the submucosal layer of the stomach wall, even without mucosal alteration, and forms a rigid, nondistensible thickening. It is characterized by poorly differentiated tumor cells and reactive fibrosis, which often form signet ring cells. It is associated with aggressive progression and poor prognosis, particularly in patients with advanced stage disease. It also has a high rate of peritoneal seeding, and recurrences are common, even after potentially curative surgery.
It can be a difficult differential diagnosis from other causes of a thickened gastric wall, such as ectopic varices or polypoid lesions. Endoscopic evaluation may be inconclusive, and a definitive diagnosis requires microscopic and immunohistochemical examination of the biopsy sample. It is essential to discuss the results of these tests with a pathologist.
The most important feature of linitis plastica is that it is associated with a metastatic origin, usually from the breast. In some cases, it is the first manifestation of occult primary breast cancer, and can occur many years after diagnosis and treatment of the original lesion.
In most patients, linitis plastica is diagnosed in an advanced or unresectable stage, with no chance of cure even after R0 resection. This is probably due to the very invasive nature of the tumor, which invades all layers of the stomach wall and metastasizes to the liver and peritoneum. The most frequent cause of linitis plastica is metastatic lobular carcinoma from the breast, but other primary neoplasms have also been reported.
Patients with linitis plastica have a high risk of death, mainly from peritoneal seeding and other complications related to the invasive nature of the tumor. Therefore, it is very important to perform a thorough staging, including diagnostic laparoscopy in all cases of this disease. The most effective therapy for linitis plastica is probably chemotherapy.
Patient education is very important, especially regarding the fact that the majority of patients with this disease are diagnosed in an advanced or non-curative stage, and that the chances for cure remain poor. They should be informed of the various options available for their management, and should understand that prophylactic surgery is an option. It is also important for them to understand the importance of a healthy diet, and that their genetic profile might determine their prognosis.
A multidisciplinary team is the best approach to the treatment of this rare and aggressive malignancy, and should include gastroenterologists, hepatologists, colorectal surgeons and radiation oncologists. Genetic testing should be performed for all patients with this disease, and counseling of the patient’s family members should be considered.
