AIRP Best Cases in Radiologic-Pathologic Correlation: Gastroblastoma: A Rare Biphasic Gastric Tumor

Abstract

A 19-year-old woman sought medical attention for diffuse abdominal pain that had been present for 5 months. The pain was worse at the inferior quadrants of the abdomen, radiated to the back, and was relieved with the use of oral analgesics. The patient reported that no diarrhea, constipation, anorexia, or weight loss was present. At physical examination, a mass was palpated in the right quadrants of the abdomen. Laboratory findings and medical history were unremarkable. Imaging FindingsAbdominal ultrasonography revealed a well-defined complex cystic lesion of indeterminate origin that was centered in the right abdominal quadrants with multiple cystic loculi of variable sizes and innumerable irregular internal septa. The contents of the loculi were anechoic, with increased through transmission.Computed tomography (CT) of the abdomen and pelvis was performed after administration of intravenous contrast material (iobitridol). CT images showed a lobulated complex cystic mass that was 13 cm in the largest dimension and appeared to protrude from the gastric antrum. The wall of the lesion was well defined with some foci of punctiform calcification. Multiple cystic loculi of variable sizes were also seen and were separated by internal septations with a maximal thickness of 10 mm (Fig 1). The wall and internal septa demonstrated moderate enhancement after administration of intravenous contrast material. No nodular enhancing components were detected. Contrast-enhancing adenopathy was seen along the greater curvature of the stomach, with short-axis measurements as much as 8 mm. The lesion was considered to be a protruding mass of the gastric antrum or a primary mesenteric tumor.Magnetic resonance (MR) imaging of the abdomen with intravenous contrast material (gadobenate dimeglumine) was performed to further evaluate the relationship of the mass with adjacent organs. Again, the lesion seemed to originate from the wall of the gastric antrum (Fig 2). MR images better displayed the cystic loculi compared with CT. The dimensions of the loculi varied from a few millimeters to 8 cm. The fluid content of the loculi was homogeneously hypointense on T1-weighted images and markedly hyperintense on T2-weighted images. The wall and internal septa were isointense relative to muscle both on T2-weighted images and unenhanced T1-weighted images and demonstrated moderate enhancement after administration of intravenous contrast material. Contrast-enhancing adenopathy along the great curvature of the stomach was observed. There were no signs of invasion of adjacent organs or mesenteric vessels, and no parenchymal lesions or peritoneal implants were detected.

Gastroblastoma described in adult patient

“…However, some differences had been reported, as one of the nine cases where Wey [5] mentioned existence of regional nodal and distant metastases at diagnoses, and other patient of this group described by Toumi [2] who developed loco-regional recurrence and local and distant lymph node metastases. Our case have brought some differences too, contrary to the postulate that gastroblastoma has been a disease only reported in young people, usually between the ages of 9-30 [6], mean age of 22 years [2], our patient is the first reported with 53 years. In addiction, is the fourth patient female in a predominantly male disease population.…”

Gastroblastoma harbors a recurrent somatic MALAT1–GLI1 fusion gene

“…To date, only eight cases have been reported, all in patients under 30 years of age. [1][2][3][4][5][6] Owing to its biphasic morphology, gastroblastoma may mimic other biphasic tumors of the stomach (eg, synovial sarcoma and sarcomatoid carcinoma). In smaller biopsies that do not demonstrate biphasic morphology, the differential diagnosis of gastroblastoma is broad, and includes a wide variety of other gastric tumors characterized by relatively bland epithelioid or spindled cells, including the recently described plexiform fibromyxoma, a rare gastric spindle cell neoplasm recently shown to harbor a MALAT1-GLI1 fusion gene in~15-20% of cases.…”

Gastroblastoma in old age

“…Sir : Gastroblastoma is a rare, distinctive gastric biphasic tumour affecting both sexes, with metastatic potential. To the best of our knowledge, 11 cases have been published so far, solidly supported by morphology, immunophenotype, and, in four instances, by identification of a characteristic MALAT – GLI1 fusion gene . A twelfth ‘gastroblastoma’, questionable because of the presence of cellular atypia significantly deviating from previous canonical examples, has been reported .…”

“…To the best of our knowledge, 11 cases have been published so far, solidly supported by morphology, immunophenotype, and, in four instances, by identification of a characteristic MALAT-GLI1 fusion gene. [1][2][3][4][5][6] A twelfth 'gastroblastoma', questionable because of the presence of cellular atypia significantly deviating from previous canonical examples, has been reported. 7 A gastroblastoma-like duodenal neoplasm has also been described.…”