Peripheral primitive neuroectodermal tumor of the stomach in a 14-year-old boy

Abstract

Although PNETs are rare malignancies, they should be considered in the differential diagnosis of submucosal gastric tumours in adolescents with clinical alarm symptoms.NET was confirmed by detection of the characteristic EWS/FLI-1 fusion gene, resulting in a reciprocal translocation t(11;22)(q24;q12). Three distinct liver metastases were detected by CT, MRI, and PET. The tumour failed to respond to neoadjuvant polychemotherapy with vincristine, etoposide, doxorubicin, and ifosfamide. Subtotal gastrectomy was performed and, surprisingly, we found diffuse metastatic infiltration of the liver that had not been detected by preoperative staging. Due to the diffuse metastatic disease the young patient's prognosis has to be considered very poor. Because of the tumour's intense expression of CD117 (c-kit), the patient is now treated with the tyrosine kinase inhibitor imatinib (STI571).

Peripheral Primitive Neuro-Ectodermal Tumor - A Case Report

“…Among all these sites intraabdominal site is very rare. There are only five reported cases of gastric PNETs which were treated by surgery followed by chemotherapy [16,17,18,19,20]. The most important differential diagnosis is carcinoma (undifferentiated carcinoma or poorly differentiated neuroendocrine carcinoma).…”

Ewing's Sarcoma of the Stomach; Rare Case of Ewing's Sarcoma and Suggestion of New Treatment Strategy

“…Group A was defined as those whom had chemotherapy with mention of metastasis and vascular invasion to the adjacent bowel (No 1, 2, 3). (3,4,6) Group B was those who had chemotherapy without mention of metastasis (No 4, 5). (7,8) Group C, the third and last group, was those who had no chemotherapy and no metastasis (No 6, 7).…”

Ewing Sarcoma of the Jejunum: A Case Report and Literature Review.

“…The age range is from 9 years old to 68 years old. FISH break-apart EWSR1 was positive in 19 cases, negative in one case and was not carried out in 17 cases [9,18,22,23,.…”

“…Extra skeletal cases are rare, and these patients generally present at an older age and demonstrate a greater overall 5-year survival than skeletal Ewing sarcoma tumours [14,15]. Reports of primary liver involvement have been noted, as well as gastrointestinal sites of origin, including the stomach, small intestine, and colorectal [16][17][18][19]. Nevertheless, ES is extremely rare in the small bowel.…”