One of the most serious forms of gastric cancer is linitis plastica (LP), a subtype of gastric adenocarcinoma. LP represents up 20% of gastric adenocarcinoma cases and is typically defined by its “signet ring” form seen on biopsy.1
In LP, there is diffuse involvement of the stomach and, frequently, evidence of metastases on clinical presentation. Patients with LP have a poor prognosis, with a 5-year survival of less than 10%.
Early diagnosis of LP is critical to the patient’s outcome, though that can be extremely challenging.
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Patients with gastric cancer will present with abdominal pain and weight loss more than 50% of the time.2 Additional symptoms include nausea, dysphagia, gastrointestinal bleeding (hematemesis, melena), and early satiety. Early satiety may be more prominent in patients in LP based on the diffuse involvement of the stomach, which causes limited distensibility.
As LP will frequently metastasize by the time the patient presents, there can be involvement of the liver and peritoneum leading to right upper quadrant pain, elevated liver function tests, and inability to tolerate adequate oral intake.
As many of the presenting symptoms can be non-specific, there may be a delay in diagnosis. LP can, however, be diagnosed in a multitude of ways.
Non-invasive testing includes upper gastrointestinal studies with barium contrast along with computed tomography (CT) of the abdomen and pelvis with oral contrast. Oral contrast allows visualization of the stomach’s ability to distend and will frequently show a “leather flask” shape due to the poor distensibility in patients with LP.3
The patient can also appear to have a functional gastric outlet obstruction based on the diffuse infiltration of the gastric walls. In addition to thickened gastric wall and possible gastric outlet obstruction, CT can also show peritoneal carcinomatosis if the LP spreads by the time of presentation.
Esophagoduodenoscopy (EGD) is a more invasive, albeit safe test that can allow for direct visualization of the gastric mucosa. Upon entering the stomach of a patient with LP, the stomach may not properly insufflate with air based on its diffuse involvement of multiple layers of the gastric wall. The mucosa can appear friable, with thickened, nodular folds. The mucosa can, however, also appear normal, which requires high clinical suspicion in further workup. The antrum and pylorus are most commonly involved in LP, while the fundus is the least involved.
In addition to EGD, endoscopic ultrasound (EUS) can also be used to visualize the different layers of the stomach and assess the depth of involvement of the malignancy.4 Suspicious mucosa should be biopsied multiple times to increase the pathological yield.
All gastric ulcers, regardless of their endoscopic appearance, should be biopsied. As LP involves the deep layers of the stomach, it is important to obtain an adequate amount of tissue. If there is a high clinical suspicion and traditional biopsies obtained from EGD are negative, EUS can be used for deeper biopsies or the patient may be referred to a surgeon for a full thickness biopsy.
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The diagnosis of LP can be difficult and uses a multifaceted approach between endoscopic and radiologic studies. As symptoms are non-specific, a clinician must have a strong clinical acumen when recommending the appropriate testing.
References
- Schauer M, Peiper M, Theisen J, Knoefel W. Prognostic factors in patients with diffuse type gastric cancer (linitis plastic) after operative treatment. Eur J Med Res. 2011;16(1):29-33.
- Wanebo HJ, Kennedy BJ, Chmiel J, Steele G Jr, Winchester D, Osteen R. Cancer of the stomach. A patient care study by the American College of Surgeons. Ann Surg. 1993;218(5):583-92.
- Mastoraki A, Papanikolaou IS, Sakorafas G, Safioleas M. Facing the challenge of managing linitis plastic-review of the literature. Hepatogastroenterology. 2009;56(96):1773-8.
- Yoshida S, Tanaka S, Kunihiro K, et al. Diagnostic ability of high-frequency ultrasound probe sonography in staging early gastric cancer, especially for submucosal invasion. Abdom Imaging. 2005;30(5):518-23.