Fetus-in-fetu in the cranium of a 4-month-old boy: histopathology and short tandem repeat polymorphism–based genotyping

Case report

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✓Fetus-in-fetu is a very rare condition in which one fetus is contained within another. About 100 cases have been reported, and in most of these the fetus was located in the retroperitoneum. The authors describe an extremely rare case of an intracranial fetus-in-fetu in an extraaxial location. This is the eighth intracranial fetus-in-fetu to be reported, the first intracranial extraaxial case, and involves the oldest documented patient with this condition.

Histopathological analysis of the mass revealed a degenerated amnionic membranelike tissue, well-differentiated extremities (including fingerlike structures), skin, matured lungs, well-formed intestines, cerebellar and cerebral tissue, and a notochord with ganglion cells. DNA analysis using short tandem repeat polymorphisms confirmed that the fetus-in-fetu mass and the host infant had heterozygous alleles and were of identical sex and genotype.

Abbreviations used in this paper: CT = computed tomography; MR = magnetic resonance; STR = short tandem repeat.

Article Information

Address correspondence to: Seung-Ki Kim, M.D., Ph.D., Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 103 Daehangno, Jongno-gu, Seoul 110-799, Republic of Korea. email: nsthomas@snu.ac.kr.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative CT scans and MR images of the well-demarcated large cystic and solid mass mixed with internal fat and calcified components, occupying half of the total intracranial space and severely compressing the brain parenchyma. A: Unenhanced CT scan. B: Axial T1-weighted image. C: Axial T1-weighted image with Gd enhancement. D: Axial T2-weighted image. E: Sagittal T1-weighted image with Gd enhancement. F: Coronal T1-weighted image with Gd enhancement.

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    Intraoperative photographs obtained before debulking the mass (left), and during the process of debulking (right). The mass was covered with a whitish-yellow membrane pathologically confirmed to be amniotic and showed sharp extra-axial demarcation. The mass was easily detached from the surrounding brain parenchyma. A deformed, armlike structure with fingers was located extraaxially.

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    Photomicrographs of the various tissue types found in the fetus-in-fetu. H & E stain. A: Amnionic membrane-like tissue, covered by double layers of cuboidal epithelium and basal cells and supported by loose fibrous tissue is shown (original magnification × 40). B: Notochord (original magnification × 40). C: Cerebellum with Purkinje cells (original magnification × 200). D: Well-formed larynx (original magnification × 40). E: Lung tissue (original magnification × 100). F: Intestinal tissue (original magnification × 1.25).

References

1

Afshar FKing TTBerry CL: Intraventricular fetus-in-fetu. Case report. J Neurosurg 56:8458491982

2

Bär WBrinkmann BBudowle BCarracedo AGill PLincoln P: DNA recommendations. Further report of the DNA Commission of the ISFH regarding the use of short tandem repeat systems International Society for Forensic Haemogenetics. Int J Legal Med 110:1751761997

3

Brand AAlves MCSaraiva CLoío PGoulão JMalta J: Fetus in fetu—diagnostic criteria and differential diagnosis—a case report and literature review. J Pediatr Surg 39:6166182004

4

Chua JHChui CHSai Prasad TRJabcobsen ASMeenakshi AHwang WS: Fetus-in-fetu in the pelvis: report of a case and literature review. Ann Acad Med Singapore 34:6466492005

5

Coolen JBradshaw BBhargava RLees GDemianczuk N: Fetus-in-fetu: confirmation of prenatal diagnosis with MRI. Prenat Diagn 27:73762007

6

Goldstein IJakobi PGroisman GItskovitz-Eldor J: Intracranial fetus-in-fetu. Am J Obstet Gynecol 175:138913901996

7

Hing ACorteville JFoglia RPBliss DP JrDonis-Keller HDowton SB: Fetus in fetu: molecular analysis of a fetiform mass. Am J Med Genet 47:3333411993

8

Hoeffel CCNguyen KQPhan HTTruong NHNguyen TSTran TT: Fetus in fetu: a case report and literature review. Pediatrics 105:133513442000

9

Hong SSGoo HWJung MRKim HJKim EAKim KS: Fetus in fetu: three-dimensional imaging using multidetector CT. AJR Am J Roentgenol 179:148114832002

10

Hopkins KLDickson PKBall TIRicketts RRO'Shea PAAbramowsky CR: Fetus-in-fetu with malignant recurrence. J Pediatr Surg 32:147614791997

11

Hung CFLam MS: Intracranial fetus in fetu: report of a case. J Formos Med Assoc 92:9209221993

12

Kimmel DLMoyer EKPeale ARWinborne LWGotwals JE: A cerebral tumor containing five human fetuses; a case of fetus in fetu. Anat Rec 106:1411651950

13

Kimpton CPGill PWalton AUrquhart AMillican ESAdams M: Automated DNA profiling employing multiplex amplification of short tandem repeat loci. PCR Methods Appl 3:13221993

14

Miura SMiura KYamamoto TYamanaka MSaito KHirabuki T: Origin and mechanisms of formation of fetus-in-fetu: two cases with genotype and methylation analyses. Am J Med Genet A 140:173717432006

15

Mutter GL: Teratoma genetics and stem cells: a review. Obstet Gynecol Surv 42:6616701987

16

Tsai CHLin JSTsai FJ: Intraventricular fetus in fetu: report of one case. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 34:1431501993

17

Wu JTBook LSudar K: Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr Res 15:50521981

18

Yang STLeow SW: Intracranial fetus-in-fetu: CT diagnosis. AJNR Am J Neuroradiol 13:132613291992

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