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Gastroblastoma

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Medical condition
Gastroblastoma
SpecialtyGastroenterology/oncology

Gastroblastoma is a rare cancer that occurs in the stomach. Only six cases have been reported to date (2017).

A single case of a similar lesion has been reported in the duodenum. The term "duodenoblastoma" has been suggested for this lesion.

Signs and symptoms

These are non specific and include upper abdominal pain and fullness. Examination may reveal a mass.

The lesion may be investigated further by ultrasound, CT or MRI but diagnosis depends on endoscopic biopsy.

This lesion appears to have an indolent course but metastases have been reported in one case to date.

Genetics

In four cases described a mutation - a fusion between the MALAT1 and GLI1 genes - has been described. This fusion causes over expression of the GLI1 protein and activation of the Sonic hedgehog pathway.

Diagnosis

Histology

The tumour is biphasic and contains spindle and epithelial cells. The spindle cells form diffuse sheets. The epithelial cells occur in clusters which may form glandular structures.

Immunochemistry

The epithelial component is positive for keratin (keratin AE1/AE3, keratin 18, keratin 7) c-KIT and CD56.

The mesenchymal component is positive for CD10 and vimentin.

Electron microscopy

Desmosomes and microvilli are present.

Differential diagnosis

The histological differential diagnosis includes

Other rare stomach tumours

Several other rare tumours that may need to be considered in the differential diagnosis include

History

This tumour was first described in 2009. The authors described this lesion in two males and one female - ages 19, 27 and 30 years. Since its original description several further cases have been described.

References

  1. ^ Miettinen M, Dow N, Lasota J, Sobin LH (2009) A distinctive novel epitheliomesenchymal biphasic tumor of the stomach in young adults ("gastroblastoma"): a series of 3 cases. Am J Surg Pathol 33(9):1370-1377
  2. Wei J, Xu C, Tai Y (2016) Clinicopathologic features of gastroblastoma. Zhonghua Bing Li Xue Za Zhi 45(1):66-68
  3. Poizat F, de Chaisemartin C, Bories E, Delpero JR, Xerri L, Flejou JF, Monges G (2012) A distinctive epitheliomesenchymal biphasic tumor in the duodenum: the first case of duodenoblastoma? Virchows Arch 461(4):379-383
  4. Fernandes T, Silva R, Devesa V, Lopes JM, Carneiro F, Viamonte B (2014) AIRP best cases in radiologic-pathologic correlation: gastroblastoma: a rare biphasic gastric tumor. Radiographics 34(7):1929-1933
  5. Wey EA, Britton AJ, Sferra JJ, Kasunic T, Pepe LR, Appelman HD (2012) Gastroblastoma in a 28-year-old man with nodal metastasis: proof of the malignant potential. Arch Pathol Lab Med 136(8):961-964
  6. Graham RP, Nair AA, Davila JI, Jin L, Jen J, Sukov WR, Wu TT, Appelman HD, Torres-Mora J, Perry KD, Zhang L, Kloft-Mod Nelson SM, Knudson RA, Greipp PT, Folpe AL (2017) Gastroblastoma harbors a recurrent somatic MALAT1-GLI1 fusion gene. Mod Pathol doi: 10.1038/modpathol.2017.68
  7. Shin DH, Lee JH, Kang HJ, Choi KU, Kim JY, Park DY, Lee CH, Sol MY, Park JH, Kim HY, Montgomery E (2010) Novel epitheliomesenchymal biphasic stomach tumour (gastroblastoma) in a 9-year-old: morphological, ultrastructural and immunohistochemical findings. J Clin Pathol 63(3):270-274
  8. Wey EA, Britton AJ, Sferra JJ, Kasunic T, Pepe LR, Appelman HD (2012) Gastroblastoma in a 28-year-old man with nodal metastasis: proof of the malignant potential. Arch Pathol Lab Med 136(8):961-964
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