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Placental Mesenchymal Dysplasia

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Benirschke's Pathology of the Human Placenta

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Abstract

Placental mesenchymal dysplasia (PMD) is a rare entity characterized by abnormalities in the vasculature in a subset of chorionic plate and stem villi. Gross features often include massive placentomegaly with tortuous, ectatic, and poorly ramified chorionic plate vessels which may be thrombosed. Stem villi typically contain multiple enlarged and thick-walled vessels surrounded by a “cuff” of abnormal mesenchyme, and myxomatous degeneration may form grossly identifiable cysts. The most constant clinical associations include intrauterine growth restriction, fetal hamartomas, and Beckwith-Wiedemann syndrome (BWS). The genetic basis of PMD is the selective expression of uniparental, paternally expressed genes in a subset of stromal cells. The most common mechanism is androgenetic-biparental mosaicism/chimerism. It is important to distinguish PMD from complete or partial hydatidiform mole, which may have considerable morphologic overlap, but differ greatly in clinical management. Immunohistochemistry for p57kip2 protein and a variety of molecular techniques are helpful in the differential diagnosis.

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Abbreviations

ABM/C:

Androgenetic-biparental mosaicism/chimerism

BWS:

Beckwith-Wiedemann syndrome

CHM:

Complete hydatidiform mole

FVM:

Fetal vascular malperfusion

HMH:

Hepatic mesenchymal hamartoma

IHC:

Immunohistochemistry

IUFD:

Intrauterine fetal demise

IUGR:

intrauterine growth restriction

PDP FISH:

Polymorphic deletion probe fluorescence in situ hybridization

PHM:

Partial hydatidiform mole

PMD:

Placenta mesenchymal dysplasia

SMA:

Smooth muscle actin

SNP:

Single nucleotide polymorphism

STR:

Short tandem repeat

UPD:

Uniparental disomy

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Authors and Affiliations

  1. University of Alabama at Birmingham, Department of Pathology, Birmingham, AL, USA

    Virginia E. Duncan & Ona Marie Faye-Petersen

  2. Seattle Children’s Hospital, Department of Pathology, Seattle, WA, USA

    Raj P. Kapur

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Editors and Affiliations

  1. Professor of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York Presbyterian Hospital, New York, NY, USA

    Rebecca N. Baergen

  2. Centre for Trophoblast Research, University of Cambridge, Cambridge, UK

    Graham J. Burton

  3. Professor Emeritus of Pathology, State University of New York, Stony Brook, NY, USA

    Cynthia G. Kaplan

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Duncan, V.E., Kapur, R.P., Faye-Petersen, O.M. (2022). Placental Mesenchymal Dysplasia. In: Baergen, R.N., Burton, G.J., Kaplan, C.G. (eds) Benirschke's Pathology of the Human Placenta. Springer, Cham. https://doi.org/10.1007/978-3-030-84725-8_27

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-84724-1

  • Online ISBN: 978-3-030-84725-8

  • eBook Packages: MedicineMedicine (R0)

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