2008, Number 2
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Acta Pediatr Mex 2008; 29 (2)
Hepatic hamartoma associated with diaphragmatic hernia in a new born
Alvarado-León U, Palacios-Acosta JM, Chávez-Enríquez EP, Vázquez-Gutiérrez VM, Hernández-Carmona C
Language: Spanish
References: 15
Page: 109-113
PDF size: 504.47 Kb.
ABSTRACT
Mesenchymal hamartoma occurs in about 15 to 30% of all benign hepatic tumors usually in patients under 5 years of age. It is less common in neonates. Its origin is related to structural developmental alterations of the liver. Recent genetic studies have shown that translocation involving a common breakpoint in chromosomes 11 and 19 (q13-q13.4) may be involved. We present the case of a mesenchymal hamartoma of the liver in a one day old boy referred to our hospital. He presented with an extrathoraxic pediculated tumor on the right side; respiratory distress and absence of right radio and ulnar bones. The tumor was excised and a diaphragmatic hernia on the same side was repaired. A histological study indicated a mesenchymal hamartoma. The patient had a successful recovery. Mesenchymal hamartoma of the liver is a rare tumor usually of the right lobe of the liver. It presents with respiratory distress, an enlarged abdomen and a palpable mass. This patient had multiple bone malformations. Surgical resection, when possible, is the treatment of choice. Malignant transformation and recurrences are rare. Patients with mesenchymal hamartomas should be followed closely since spontaneous remissions are possible.
REFERENCES
Martínez GM, Mondragón SR, Brunner CG, Jara AP, Gómez GE, González EY. Hamartoma mesenquimatoso de hígado. Informe de un caso. Cir Gen 2005;27(3):229-32.
Kaymakcalan H, Rao S. A 10 month old infant with progressive abdominal distension. Hosp Phys 2003;38:20-6.
Siddiqui M A, McKenna B J. Hepatic mesenchymal hamartoma:a short review. Arch Parh Lab Med, 2006;130(10):1567-9.
Kamata S, Nose K, Sawai T, Hasegawe T, Kuroda S, Sasaki T, Okada A, Tawara M. Fetal mesenchymal hamartoma of the liver: report of a case. J Ped Surg 2003;38(4):639-41.
Gangopadhyay AN, Sharma SP, Gopal SC, Yadava R, Arya NC.Mesenchymal hamartoma of liver. Ind Ped 1995;32:1109-11.
Barnhart DC, Hirschi RB, Garver KA, Geiger JD, Harmon CM, Coran AG. Conservative management of mesenchymal hamartoma of the liver. J Ped Surg 1997;32(10):1495-8.
Heyer C, Weitkaemper A, Teig N, Mueller A, Rieger C, Nicolas V. Hepatic mesenchymal hamartoma in a preterm newborn: demostration by low-dose multidetector CT. Acta Pediatr 2007;96(10):1538-42.
Jozwiak S, Michalowicz R, Pedich M, Rajszys. Hepatic hamartoma in tuberous sclerosis. Lancet 1992;339:180.
Rey MJ, Ordi J, Ribé A, García V J, Ayuso JR, Cardesa A.Hamartoma mesenquimatoso hepático de presentación en la edad adulta. Med Clin 1995;104:180-2.
Cheung YC, Tan CF, Wan YL, Lui KW, Tsai CC. MRI of multiple bliliary hamartomas. Brit J Radiol 1997;70:527-9.
Carta M, Maresi E, Guiffré M, Catalano G, Piro E, Siracusa F, Corsello G. Congenital hepatic mesenchymal hamartoma associated with mesenchymal stem villous hiperplasia of the placenta: case report. J Ped Surg 2005;40:37-9.
Lauwers GY, Grant LD, Donnelly WH, Meloni AM, Foss RM,Sanberg AA, Langham MR. Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Am J Surg Pathol 1997;21(10):1248-54.
Bin-Bin Y, Bing H, Li JW, Liu HS, Zou Y, Zhou YB, Zhuang K. Mesenchymal hamartoma of liver: magnetic resonance imaging and histopathologic correlation. World J Gastro 2005;11(37):5807-10.
Hart I. Tumors of the Fetus and Infants. Ed Springer, New York USA, 2002;pp303-20.
Puri P, Hollwarth ME. Pediatric Surgery. Ed Springer, Germany 2006;pp459-76.