What are the other Names for this Condition? (Also known as/Synonyms)

• Gastroblastoma in Adulthood
• Pediatric Gastroblastoma

What is Gastroblastoma? (Definition/Background Information)

• Gastroblastoma is an extremely rare malignant tumor arising from muscularis propria of the stomach, especially in the antrum region
• It is a biphasic tumor

Incidence:

• Gastroblastomas are seen generally in boys and young men
• These tumors usually occur between ages 9 and 56 years; median age of occurrence is 27years
• Both males and females are affected
• It occurs worldwide and all races and ethnic groups may be affected

Risk factors and Cause:

• The cause of formation of Gastroblastoma is currently unknown
• An association with gene fusion MALAT1-GLI1 has been documented

Signs and symptoms:

• Many patients present with abdominal pain
• Fatigue and hematochezia (fresh blood in/with stools) can be occasional presentation

Diagnosis:

• Radiology - CT shows solid and cystic heterogenous mass in gastric wall or exophytic
• Tissue biopsy of the mass is essential for definitive diagnosis
  • Pathology microscopic features: Pathological examination of the tissue may reveal a biphasic histology comprising of spindle cells and epithelial cells arranged in nests
  • Immunohistochemistry shows epithelial component stains positive for various keratins like AE1/AE3, CAM5.2, Keratin 7,18.spindle cell component stains positive for vimentin, CD10,CD56
• MALAT1-GLI1 fusion gene demonstration in tumor cells is a requirement for establishing a  diagnosis

Complications:

• Metastases can occur commonly to the liver
• Gastric obstruction can occur if tumor is large
• Local recurrence following treatment

Treatment:

• Surgical resection of tumor is the mainstay of treatment
• Limited studies has shown chemotherapy to be ineffective

Prevention: Presently, it is not possible to prevent the formation of Gastroblastomas.

Prognosis:

• The prognosis depends on the stage of the Gastroblastoma
• Presently, due to the insufficient number of cases, the prognosis is not well-defined