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- 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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Abstract
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
- Primary Ewing’s sarcoma in a small intestine – a case report and review of the literature
- Osteochondrodysplasia Pathologic study of 29 autopsy cases.
- Yi Kyeong Chun, Yee Jeong Kim, Sung Ran Hong, Min Suk Kim, Je G Chi
- Korean J Pathol. 1999;33(1):32-41.
- 1,589 View
- 15 Download
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Abstract
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- Osteochondrodysplasia is a heterogeneous group of disorders appearing short limbed dwarfism. Because many of these entities are lethal and hereditary, an accurate diagnosis is mandatory. The purpose of this study is to define the clinicopathologic features and radiologic findings of osteochondrodysplasia. We reviewed 29 autopsy cases of congenital short limbed dwarfism, consisting of thanatophoric dysplasia (TD) (12 cases), osteogenesis imperfecta (OI) (12 cases), asphyxiating thoracic dysplasia (ATD) (3 cases), short-rib-polydactyly syndrome (SRPS) (1 case) and hypochondrogenesis (1 case). The gestational age ranged from 16 to 41 weeks. Of 6 fetuses that were born alive, 3 were ATD, 2 were TD and 1 was hypochondrogenesis. TD was frequently complicated by hydramnios. Of 8 cases studied chromosomally, only 1 showed chromosomal abnormality -46XY, inv 9. Intrauterine growth retardation was frequently associated with OI. Pulmonary hypoplasia was present in 23 cases (79%), including all cases of ATD, SRPS and hypochondrogenesis, 11 in TD and 7 in OI. Other associated anomalies were present in 17 cases (59%).
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