Placenta

Nonneoplastic placental conditions and abnormalities

Noninfectious

Chorangiomatosis


Editorial Board Member: Ricardo R. Lastra, M.D.
Deputy Editor-in-Chief: Gulisa Turashvili, M.D., Ph.D.
Shahrazad Saab, M.D.

Last author update: 7 July 2023
Last staff update: 7 July 2023

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PubMed Search: Chorangiomatosis

Shahrazad Saab, M.D.
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Cite this page: Saab S. Chorangiomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/placentachorangiomatosis.html. Accessed March 29th, 2024.
Definition / general
  • Nonexpansile proliferation of anastomosing capillaries at the periphery of stem villi or immature intermediate villi
  • Closely related to chorangiosis and chorangioma and associated with advanced maternal age, extreme prematurity and multiple gestation; it is less common in those of African American ancestry (Hum Pathol 2000;31:945)
Essential features
  • Also called multifocal chorangiomatosis and multifocal diffuse chorangiomatosis
  • No gross findings; therefore, radiologic detection on antenatal evaluation is not possible
  • 2 subgroups: patchy (multiple foci, occupying < 10x microscopic field) and extensive (multiple foci with at least one > 4x microscopic field)
  • Fetal growth restriction, congenital malformation and adverse fetal outcomes associated with more extensive lesions
Terminology
  • Multifocal chorangiomatosis and multifocal diffuse chorangiomatosis
ICD coding
  • ICD-10: O43.813 - placental infarction, third trimester
Epidemiology
  • Maternal factors associated with multifocal chorangiomatosis include advanced maternal age and multiparity; it is less common in those of African American ancestry
  • Fetal outcomes can include growth restriction, congenital anomalies and stillbirth
Pathophysiology
  • Rare disorder without a clearly defined etiology or pathophysiology
  • Suspected to be due to fetal developmental anomalies or abnormal fetal blood flow
Diagnosis
  • Diagnosis is made on histologic evaluation of the placenta as there are no radiologic or grossly evident findings
Prognostic factors
  • Patchy multifocal chorangiomatosis has been associated with fetal growth restriction and prematurity
  • Extensive multifocal chorangiomatosis has been associated with macrosomia, congenital anomalies and stillbirth
Case reports
Microscopic (histologic) description
  • Anastomosing vascular proliferation along the edges of stem vessels and immature intermediate villi comprised of capillaries surrounded by pericytes and small, muscularized arterioles
  • Often with coexistent chorangiosis
  • 2 subtypes
    • Patchy: multiple foci occupying < 10x microscopic field
    • Extensive: multiple, more diffuse foci with at least one > 4x microscopic field
  • Reference: Hum Pathol 2000;31:945
Microscopic (histologic) images

Contributed by Shahrazad Saab, M.D.
Term placenta with intrauterine growth restriction

Term placenta with intrauterine growth restriction

Term placenta with delayed villous maturation Term placenta with delayed villous maturation

Term placenta with delayed villous maturation

Twin placentas (34 weeks gestational age) with fetal growth restriction of both fetuses Twin placentas (34 weeks gestational age) with fetal growth restriction of both fetuses

Twin placentas (34 weeks gestational age) with fetal growth restriction of both fetuses

Term placenta with severe fetal growth restriction

Term placenta with severe fetal growth restriction

Sample pathology report
  • Placenta:
    • Chorangiosis and multifocal chorangiomatosis
    • Immature (early third trimester) placenta, 256 g
Differential diagnosis
  • Chorangioma:
    • Benign, well circumscribed, nodular capillary proliferation
  • Localized chorangiomatosis:
    • Chorangioma that has spread to involve several contiguous primary stem villi (wandering chorangioma)
Board review style question #1

Which of the following describes the lesion shown above?

  1. A capillary proliferation localized to a group of contiguous primary stem villi
  2. A localized capillary proliferation which when > 3 cm can result in polyhydramnios and intrauterine growth restriction
  3. Enlarged villi with irregular contours, increased stroma, trophoblast inclusions and irregularly distributed arterioles, venules and capillaries
  4. Foci of excessive capillary proliferation present at the edges of stem and intermediate villi that can be associated with advanced maternal age, preterm birth and multiparty
  5. Multifocal or diffuse increase in villous diameter with increased stromal cellularity and the presence of nonperipheral villous capillaries
Board review style answer #1
D. Foci of excessive capillary proliferation present at the edges of stem and intermediate villi that can be associated with advanced maternal age, preterm birth and multiparty. Answer A is incorrect because it describes localized chorangiomatosis (wandering chorangioma). Answer B is incorrect since it describes a giant chorangioma, which has a high prevalence of poor fetal outcomes related to polyhydramnios, intrauterine growth restriction, cardiomegaly, high output heart failure and fetal hydrops. Answer C is incorrect because it describes dysmorphic villi. Answer E is incorrect because it describes immature villi.

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Reference: Chorangiomatosis
Board review style question #2
Which of the following is true about multifocal chorangiomatosis?

  1. Can be categorized as patchy or extensive
  2. Can be seen in placentas of mothers living at high altitudes
  3. Localized, nodular capillary proliferation
  4. Placentas are enlarged with segmentally abnormal proximal villi showing stromal overgrowth and vascular abnormalities affecting small and large villous vessels
Board review style answer #2
A. Can be categorized as patchy or extensive. Answer B is incorrect because it describes chorangiosis. Answer D is incorrect because it describes placental mesenchymal dysplasia. Answer C is incorrect because it describes chorangioma.

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Reference: Chorangiomatosis
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