Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of the literature

Abstract

Primitive neuroectodermal tumor (PNET) is most commonly encountered in the soft tissue or bone in children and young adults, and its involvement in the intestines is exceedingly rare. To the best of our knowledge, eighteen cases have been reported to date. The present study reports three cases of PNET arising in the mesentery and ileocecum in 59- and 22-year-old males and a 36-year-old female. Computed tomography revealed a solid mass in the lower abdomen, with areas of cystic changes. Microscopically, the tumors were composed of small round cells arranged in sheets and rosettes with scant cytoplasm, hyperchromatic nuclei and a high mitotic rate. The tumor cells were immunopositive for CD99 and FLI1. EWS/FLI1 translocations were detected in all cases. Case 1 and case 2 underwent tumor resection without any preoperative radiotherapy, chemotherapy or biological therapy. Case 3 underwent tumor resection and received eight cycles of IAP chemotherapy (2.0 mg ifosfamide, 80 mg epirubicin, 30 mg cisplatin 30mg). Case 3 was followed up for 34 months until they succumbed to peritoneal recurrence, whereas the other cases were not followed up. The incidence of these small round-cell tumors in the intestinal system, their clinical and pathological features and differential diagnosis are discussed with a review of the literature.

Peripheral Primary Neuroectodermal Tumour of the Ileum: A Case Report and Review of the Literature

“…The treatment of these tumors is based on a multimodal treatment combining local surgery and/or radiotherapy followed by multidrug systemic chemotherapy [31] [32] [33] [34]. Current chemotherapy regimens include combinations of vincristine, cyclophosphamide and doxorubicin [32]. The addition of ifosfamide and etoposide to a standard regimen significantly improves the outcome for patients with non metastatic Ewing's sarcoma a [45].…”

<p>Extraskeletal Ewing’s sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation</p>

“…Surgery alone is associated with poor outcome. Therefore, enbloc resection with systemic adjuvant chemotherapy is primarily chosen for local and systemic control as for other ES/PNET 25. The longest reported survival was 204 months23 and the shortest was 3 months 26.…”

Mesenteric extraskeletal Ewing sarcoma with small bowel involvement

“…He observed that this highly aggressive, originally bone tumor was remarkably sensitive to radiation therapy. However extraskeletal cases are rare, several cases have been reported with different localisations, including -jejunum [1], chest wall [2], paravertebral region [3], stomach [4], lung parenchyma [5], breast tissue [6] and mesentery-ileocecum [7]. Soft tissue sarcomas encompass around 1% of all malignant tumors, but they are relatively more frequent in childhood and adolescent age [8], and extraskeletal Ewing sarcoma belongs to this group of tumors.…”