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Case Study
Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study
Joon Mee Kim, Young Chae Chu, Chang Hwan Choi, Lucia Kim, Suk Jin Choi, In Suh Park, Jee Young Han, Kyung Rae Kim, Yoon-La Choi, Taeeun Kim
Korean J Pathol. 2013;47(1):77-81.   Published online February 25, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.1.77
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  • 6 Crossref
AbstractAbstract PDF

A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with osseous component is reported. A 23-year-old man was admitted to our hospital because of acute severe abdominal pain. Abdominal computed tomography revealed a large solid and cystic, oval shaped mass, measuring 11.0×6.0 cm in the pelvic cavity. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming Homer-Wright rosettes and perivascular pseudorosettes, and showed areas of osteoid and bone formation. Immunohistochemical studies revealed that tumor cells expressed positivity against CD99 (MIC2), CD57, neuron-specific enolase, and vimentin. Fluorescence in situ hybridization study revealed Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement on chromosome 22q12. To the authors' knowledge this is the first documentation of a peripheral neuroectodermal tumor with osteoid and bone formation of the small bowel mesentery.

Citations

Citations to this article as recorded by  
  • Primary Ewing’s sarcoma in a small intestine – a case report and review of the literature
    Andrej Kolosov, Audrius Dulskas, Kastytis Pauza, Veslava Selichova, Dmitrij Seinin, Eugenijus Stratilatovas
    BMC Surgery.2020;[Epub]     CrossRef
  • Case report and literature review of Ewing's sarcoma in the gastrointestinal tract
    Christopher Bong, Iain Thomson, Guy Lampe
    Surgical Practice.2018; 22(2): 84.     CrossRef
  • Pediatric Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report
    You Sun Kim, Hye Min Moon, Kyu Sang Lee, Young Suk Park, Hyun-Young Kim, Ji Young Kim, Jin Min Cho, Hyoung Soo Choi
    Clinical Pediatric Hematology-Oncology.2017; 24(2): 162.     CrossRef
  • Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature
    Zhe Liu, Yuan-Hong Xu, Chun-Lin Ge, Jin Long, Rui-Xia Du, Ke-Jian Guo
    World Journal of Clinical Cases.2016; 4(9): 306.     CrossRef
  • Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of the literature
    LIBO PENG, LIMIN YANG, NAN WU, BO WU
    Experimental and Therapeutic Medicine.2015; 9(4): 1299.     CrossRef
  • Une curieuse tumeur digestive à cellules rondes
    Alia Zehani, Ines Chelly, Beya Chelly, Jean-Michel Coindre, Slim Haouet, Nidhameddine Kchir
    Annales de Pathologie.2014; 34(2): 104.     CrossRef
Original Article
DPC4 Expression in the Small Intestinal Adenocarcinomas
Sun Jae Lee, Eunsil Yu, Young Kyung Bae, Kee-Taek Jang, Joon Mee Kim, Han-Ik Bae, Seung-Mo Hong, Ghil Suk Yoon
Korean J Pathol. 2012;46(5):415-422.   Published online October 25, 2012
DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.415
  • 6,590 View
  • 45 Download
  • 1 Crossref
AbstractAbstract PDF
Background

Small intestinal adenocarcinomas (SACs) are rare malignancies of the alimentary tract with uncertain carcinogenesis.

Methods

We investigated the expression of deleted in pancreatic cancer 4 (DPC4) in 188 cases of surgically resected SACs, using tissue microarray technology.

Results

Twenty-four of the 188 tumors showed complete loss of Smad4/DPC4 expression in cytoplasm (score, 0; 12.8%). Eighty-four and 31 cases were moderately and strongly positive, respectively (score, 2 and 3; 44.7% and 16.5%, respectively) and 49 cases were focally or weakly stained (score, 1; 29.1%). Immunohistochemistry analysis showed that the expression of Smad4/DPC4 was related to an increased risk of lymphatic invasion but not to other clinicopathological features of the tumors (tumor location, differentiation, growth pattern, T stage, direct invasion, vascular invasion, and nodal metastasis). There was no significant association between Smad4/DPC4 expression and patient survival.

Conclusions

The present research is the first study to evaluate Smad4/DPC4 expression in a large sample of SACs with clinicopathologic correlation. Future studies should focus on the immunohistochemical and molecular characteristics of SACs to clarify their tumorigenesis.

Citations

Citations to this article as recorded by  
  • American Registry of Pathology Expert Opinions: Evaluation of poorly differentiated malignant neoplasms on limited samples - Gastrointestinal mucosal biopsies
    Andrew M. Bellizzi, Elizabeth A. Montgomery, Jason L. Hornick
    Annals of Diagnostic Pathology.2020; 44: 151419.     CrossRef

J Pathol Transl Med : Journal of Pathology and Translational Medicine