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Non-gastrointestinal stromal tumor, mesenchymal neoplasms of the gastrointestinal tract: a review of tumor genetics, pathology, and cross-sectional imaging findings

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Abdominal Radiology Aims and scope

Abstract

There is a diverse group of non-gastrointestinal stromal tumor (GIST), mesenchymal neoplasms of the gastrointestinal (GI) tract that demonstrate characteristic pathology and histogenesis as well as variable imaging findings and biological behavior. Recent advancements in tumor genetics have unveiled specific abnormalities associated with certain tumors, influencing their molecular pathogenesis, biology, response to treatment, and prognosis. Notably, giant fibrovascular polyps of the esophagus, identified through MDM2 gene amplifications, are now classified as liposarcomas. Some tumors exhibit distinctive patterns of disease distribution. Glomus tumors and plexiform fibromyxomas exhibit a pronounced affinity for the gastric antrum. In contrast, smooth muscle tumors within the GI tract are predominantly found in the esophagus and colorectum, surpassing the incidence of GISTs in these locations. Surgical resection suffices for symptomatic benign tumors; multimodality treatment may be necessary for frank sarcomas. This article aims to elucidate the cross-sectional imaging findings associated with a wide spectrum of these tumors, providing insights that align with their histopathological features.

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Abbreviations

GCT:

Granular cell tumor

PFM:

Plexiform fibromyxoma

GT:

Glomus tumor

SFT:

Solitary fibrous tumor

IMT:

Inflammatory myofibroblastic tumor

IFP:

Inflammatory fibroid polyp

LMS:

Leiomyosarcoma

MGNET:

Malignant gastrointestinal neuroectodermal tumor

AS:

Angiosarcoma

PEComa:

Neoplasms of the perivascular epithelioid cells

SS:

Synovial sarcoma

KS:

Kaposi sarcoma

FDCS:

Follicular dendritic cell sarcoma

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Authors and Affiliations

  1. Departments of Radiology, New York University School of Medicine, New York, USA

    Krishna P. Shanbhogue

  2. University of Texas MD Anderson Cancer Center, Houston, USA

    Aditya S. Prasad

  3. Thomas Jefferson University Hospital, Philadelphia, USA

    Rashmi Balasubramanya

  4. Department of Pathology, Michael E. DeBakey VA Medical Center, Houston, USA

    Nisha S. Ramani

  5. Department of Abdominal Imaging, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1473, Houston, TX, 77030, USA

    Venkateswar R. Surabhi

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  1. Aditya S. Prasad
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  2. Krishna P. Shanbhogue
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  4. Rashmi Balasubramanya
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  5. Venkateswar R. Surabhi
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Correspondence to Venkateswar R. Surabhi.

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Prasad, A.S., Shanbhogue, K.P., Ramani, N.S. et al. Non-gastrointestinal stromal tumor, mesenchymal neoplasms of the gastrointestinal tract: a review of tumor genetics, pathology, and cross-sectional imaging findings. Abdom Radiol 49, 1716–1733 (2024). https://doi.org/10.1007/s00261-024-04329-1

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