What is a rare aggressive form of gastric cancer?

Linitis Plastica: A Rare and Aggressive Form of Gastric Cancer

Linitis plastica (Borrmann Type IV) is a rare, aggressive form of gastric cancer characterized by diffuse infiltration of the stomach wall, resulting in a rigid, thickened "leather bottle" appearance with poor prognosis and limited treatment options.

Clinical Characteristics

Linitis plastica represents a specific subtype of gastric adenocarcinoma with distinctive features:

• Pathological features:

  • Predominantly diffuse-type gastric adenocarcinoma according to Lauren classification 1
  • Often poorly differentiated with signet-ring cell morphology 1
  • Characterized by discohesive tumor cells diffusely infiltrating the gastric wall in a desmoplastic stroma 1

• Macroscopic appearance:

  • Classified as Borrmann Type IV in the advanced gastric cancer classification 1
  • Poorly demarcated, infiltrative, and diffuse tumor 1
  • Causes thickening and rigidity of the stomach wall, creating a "leather bottle" appearance 2
  • May present with characteristic "waffle-like" appearance of gastric folds on endoscopy 3

Epidemiology and Prognosis

• Incidence: Extremely rare form of gastric cancer 4

• Demographics:

  • In a National Cancer Database study, patients were predominantly white (78.5%) and female (51.9%) 5
  • More prevalent in low-risk areas compared to high-incidence regions 1

• Prognosis:

  • Extremely poor survival outcomes with mean overall survival of only 16.9 months 5
  • 1-year and 5-year overall survival rates of only 33% and 5%, respectively 5
  • Most patients (69%) present with advanced stage disease (stage IV) 5
  • Associated with higher mortality compared to other gastric cancer subtypes 6

Diagnostic Challenges

Linitis plastica is notoriously difficult to diagnose for several reasons:

• Late presentation: Often asymptomatic in early stages 1
• Submucosal spread: Tumor primarily infiltrates the submucosa rather than the mucosa 2
• Diagnostic limitations:
  • Standard endoscopic biopsies may miss the diagnosis due to submucosal location 2
  • Endoscopic ultrasound (EUS) with guided biopsy is particularly helpful for diagnosis 4, 2
  • EUS shows diffuse submucosal thickening of the gastric wall 4

Treatment Approaches

Treatment options are limited and outcomes remain poor:

• Multimodal approach: Combined treatment offers the best outcomes 5

  • Surgery with chemotherapy and/or radiation provides mean OS of 28.4 months
  • Surgery alone: 17.1 months
  • Chemotherapy and/or radiation alone: 12.3 months
  • No treatment: 8.1 months

• Surgical considerations:

  • Many patients are not candidates for curative surgery due to advanced disease 6
  • Total gastrectomy is often required when surgery is attempted 3

• Systemic therapy:

  • Chemotherapy regimens may include docetaxel in combination with cisplatin and fluorouracil for advanced gastric cancer 7
  • Neoadjuvant therapy should be considered before surgical resection 5

Clinical Implications

• Diagnostic vigilance: Clinicians should maintain high suspicion when encountering:

  • Diffuse gastric wall thickening on imaging
  • Rigid stomach with reduced distensibility
  • Abnormal gastric fold patterns on endoscopy

• Advanced diagnostic techniques:

  • EUS-guided fine-needle aspiration or bite-on-bite biopsy should be considered when standard biopsies are negative 4, 2
  • Comprehensive staging with CT, EUS, and laparoscopy with peritoneal washings is essential 1

• Treatment planning:

  • Multidisciplinary approach is mandatory 1
  • Assessment of HER2 status is recommended as HER2-positive tumors may benefit from targeted therapy 1
  • Palliative care should be integrated early in the disease course given the poor prognosis 6

Linitis plastica represents one of the most challenging forms of gastric cancer, with limited treatment options and poor outcomes. Early recognition through advanced diagnostic techniques and aggressive multimodal therapy offer the best chance for improved survival.