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Omental mesenteric myxoid hamartoma, a subtype of inflammatory myofibroblastic tumor? Considerations based on the histopathological evaluation of four cases

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Abstract

Omental mesenteric myxoid hamartoma (OMH) is a distinctive myxoid lesion of infancy, characterized by a benign clinical behavior. In the current World Health Organization (WHO) classification of soft tissue tumors, it is considered as part of the morphologic spectrum of inflammatory myofibroblastic tumors (IMT), but this relationship with IMT is still subject to debate. Four lesions with histologic features of OMH occurring in newborns and toddlers are described and compared with classic, ALK-positive IMT. All OMH showed a peculiar dot-like immunostaining for ALK, which, in one of the cases, was cytogenetically found to be associated with an inversion of the ALK gene. While OMHs were positive for smooth muscle actin (SMA), desmin, WT1, podoplanin, and cytokeratins (CAM5.2 and AE1-3), IMT were consistently positive only for SMA (10 cases). ALK-1 displayed cytoplasmic staining in IMT and characteristic paranuclear dot-like staining in OMH.

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Acknowledgments

The authors wish to express their sincere thanks to the following pathologists for contribution of tissue, slides, and clinical information: Dr. Alejandra Henriquez V., Hospital Luis Calvo Mackenna, Santiago, Chile; Dr. Harry Kozakewich, Boston Children’s Hospital; Dr. William Fethiere, Good Samaritan Medical Center, Phoenix, AZ; and Dr. Herbert M. Reiman, Resurrection Medical Center, Chicago, IL.

Compliance with ethical standards

The Institutional Review Boards of all participating institutions granted approval for this study.

Funding

Financial support for this study was generously provided by the North Shore Cancer League of Chicago; however, the authors declare that they have no conflicts of interest.

Statement of informed consent

Informed consent was obtained from all patients at the time of surgical treatment.

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Author notes

    Authors and Affiliations

    1. Department of Medicine (DIMED), Surgical Pathology & Cytopathology Unit, University-Hospital of Padua, Via A. Gabelli 61, 35121, Padua, Italy

      K. Ludwig & R. Alaggio

    2. Department of Woman and Children’s Health, Pediatric Surgery Unit, University-Hospital of Padua, Via Giustiniani 3, 35121, Padua, Italy

      P. Dall’Igna

    3. Pathology Department, “San Bortolo” Hospital, Vicenza, Viale Rodolfi 37, 36100, Vicenza, Italy

      E. Lazzari & E. S. G. d’Amore

    4. Department of Pathology, Northwestern University, 303 East Chicago Avenue, Chicago, IL, 60611, USA

      P. M. Chou

    Authors
    1. K. Ludwig
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    2. R. Alaggio
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    3. P. Dall’Igna
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    4. E. Lazzari
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    5. E. S. G. d’Amore
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    6. P. M. Chou
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    Corresponding author

    Correspondence to R. Alaggio.

    Additional information

    K. Ludwig and R. Alaggio contributed equally to this work.

    Electronic supplementary material

    Below is the link to the electronic supplementary material.

    ESM 1

    OMH case with sparse (a) and prominent (b) number of mesenchymal cells in a myxoid stroma with rich vascular network (original magnifications 40×); larger vessels with perivascular cell condensation (c) and hyalinization of the wall (d) (original magnifications. 60×) (PDF 180401 kb)

    ESM 2

    OMH case with calretinin (a), podoplanin (b), CAM5.s (c), SMA (d), desmin (e) and WT-1 (f) staining pattern (original magnifications were ×60, ×160, ×50, ×50, ×50 and ×160 ,respectively) (JPEG 52863 kb)

    ESM 3

    Paranuclear dot-like ALK-1 staining (a) and FISH with LSI ALK break apart probe showing split signals in more than 80 % of nuclei (b) (JPEG 16286 kb)

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    Ludwig, K., Alaggio, R., Dall’Igna, P. et al. Omental mesenteric myxoid hamartoma, a subtype of inflammatory myofibroblastic tumor? Considerations based on the histopathological evaluation of four cases. Virchows Arch 467, 741–747 (2015). https://doi.org/10.1007/s00428-015-1842-4

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    • DOI: https://doi.org/10.1007/s00428-015-1842-4

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