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hepatic mesenchymal hamartoma

Monday 24 January 2005

MHL, mesenchymal hamartoma of the liver

Definition: Mesenchymal hamartoma of the liver is the third most common liver tumor of infancy. The lesion is primarily mesenchymal in origin, believed to arise from a developmental abnormality in the formation of ductal plates during late embryogenesis.

Examples

- hepatic mesenchymal hamartoma

Synopsis

- hepatic tumor
- cut surfaces displaying multiple cysts with intervening loose fibrous tissue

Associations

- infantile hemangioendothelioma (15018455)
- hepatic undifferentiated embryonal sarcoma (11779051, 9331300)
- placental mesenchymal dysplasia (17378624)

Biology

- slightly elevated alpha fetoprotein (AFP) (15024683)

Cytogenetics

- recurrent translocations involving 19q13.4 (16544232, 1551077, 9688769, 15325096) as hepatic undifferentiated embryonal sarcoma

  • t(11;19)(q11;q13.4) translocation involving MALAT1 at 11q11 (17311249, 15325096)
  • interstitial deletion involving chromosome band 19q13 (16879027)

References

- Androgenetic/biparental mosaicism in an infant with hepatic mesenchymal hamartoma and placental mesenchymal dysplasia. Reed RC, Beischel L, Schoof J, Johnson J, Raff ML, Kapur RP. Pediatr Dev Pathol. 2008 Sep-Oct;11(5):377-83. PMID: 18260692

- Reed R, Kapur R. Hepatic mesenchymal hamartoma: A disorder of imprinting? Pediatr Dev Pathol. 2007 Jun 13;:1 PMID: 17990933

- Rajaram V, Knezevich S, Bove KE, Perry A, Pfeifer JD. DNA sequence of the translocation breakpoints in undifferentiated embryonal sarcoma arising in mesenchymal hamartoma of the liver harboring the t(11;19)(q11;q13.4) translocation. Genes Chromosomes Cancer. 2007 May;46(5):508-13. PMID: 17311249

- Francis B, Hallam L, Kecskes Z, Ellwood D, Croaker D, Kent A. Placental mesenchymal dysplasia associated with hepatic mesenchymal hamartoma in the newborn. Pediatr Dev Pathol. 2007 Jan-Feb;10(1):50-4. PMID: 17378624

- Talmon GA, Cohen SM. Mesenchymal hamartoma of the liver with an interstitial deletion involving chromosome band 19q13.4: a theory as to pathogenesis? Arch Pathol Lab Med. 2006 Aug;130(8):1216-8. PMID: 16879027

- Rakheja D, Margraf LR, Tomlinson GE, Schneider NR. Hepatic mesenchymal hamartoma with translocation involving chromosome band 19q13.4: a recurrent abnormality. Cancer Genet Cytogenet. 2004 Aug;153(1):60-3. Review. PMID: 15325096

- O’Sullivan MJ, Swanson PE, Knoll J, Taboada EM, Dehner LP. Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: report of a case and review of the literature. Pediatr Dev Pathol. 2001 Sep-Oct;4(5):482-9. PMID: 11779051

- Lauwers GY, Grant LD, Donnelly WH, Meloni AM, Foss RM, Sanberg AA, Langham MR Jr. Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Am J Surg Pathol. 1997 Oct;21(10):1248-54. PMID: 9331300

- Sarihan H, Yildiz K, Ozoran Y. An unusual case of ectopic mesenchymal hamartoma of the liver. S Afr J Surg. 1994 Dec;32(4):152-3; discussion 153-4. PMID: 7597513