Adult Mesenchymal Hamartoma of the Liver: Case Report and Literature Review

Bibliographic Details
Title: Adult Mesenchymal Hamartoma of the Liver: Case Report and Literature Review
Authors: Zachary Klaassen, Prakash R. Paragi, Ronald S. Chamberlain
Source: Case Reports in Gastroenterology, Vol 4, Iss 1, Pp 84-92 (2010)
Publisher Information: Karger Publishers, 2010.
Publication Year: 2010
Collection: LCC:Diseases of the digestive system. Gastroenterology
Subject Terms: Hepatic mass, Undifferentiated embryonal sarcoma, Mesenchymal hamartoma, Hepatobiliary surgery, Diseases of the digestive system. Gastroenterology, RC799-869
More Details: Mesenchymal hamartoma of the liver (MHL) is a rare benign lesion occurring primarily in the pediatric population. While the precise pathogenesis of the tumor is not certain, the most common theory relates to aberrant mesenchyme development in the portal tract likely related to the bile ducts. A 53-year-old female was evaluated for an incidental liver mass. Initial CT scan showed a cystic lesion in the right lobe of the liver and follow-up imaging revealed an increase in size and the percent solid component within the mass. In view of these changes, a nondiagnostic biopsy was obtained followed by extirpation of the lesion. Gross pathological review of the lesion identified a 9 × 9 × 7.5 cm, pink-yellow-tan, gelatinous mass, with a >1 cm clear surgical margin. Histologically, the mass consisted of benign dilated bile ducts, as well as myxoid stroma with spindle cells showing smooth muscle differentiation. The patient was discharged home on postoperative day five. A review of the literature for MHL in adults reports 30 previous cases, predominantly published as individual case reports describing the size, lobe(s) of the liver affected, and the cystic/solid nature of the tumor. MHL in adults may represent a potentially premalignant lesion, as the emerging literature supports a potential relationship between MHL and malignant undifferentiated embryonal sarcoma in regards to cytogenetic analysis. Aggressive surgical management of MHL in adults is mandated when feasible.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 1662-0631
00026018
52434249
Relation: http://www.karger.com/Article/FullText/260183; https://doaj.org/toc/1662-0631
DOI: 10.1159/000260183
Access URL: https://doaj.org/article/54656b52434249ca99f53f7213326c4b
Accession Number: edsdoj.54656b52434249ca99f53f7213326c4b
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  Data: Adult Mesenchymal Hamartoma of the Liver: Case Report and Literature Review
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  Data: Case Reports in Gastroenterology, Vol 4, Iss 1, Pp 84-92 (2010)
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  Data: Karger Publishers, 2010.
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  Data: <searchLink fieldCode="DE" term="%22Hepatic+mass%22">Hepatic mass</searchLink><br /><searchLink fieldCode="DE" term="%22Undifferentiated+embryonal+sarcoma%22">Undifferentiated embryonal sarcoma</searchLink><br /><searchLink fieldCode="DE" term="%22Mesenchymal+hamartoma%22">Mesenchymal hamartoma</searchLink><br /><searchLink fieldCode="DE" term="%22Hepatobiliary+surgery%22">Hepatobiliary surgery</searchLink><br /><searchLink fieldCode="DE" term="%22Diseases+of+the+digestive+system%2E+Gastroenterology%22">Diseases of the digestive system. Gastroenterology</searchLink><br /><searchLink fieldCode="DE" term="%22RC799-869%22">RC799-869</searchLink>
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  Data: Mesenchymal hamartoma of the liver (MHL) is a rare benign lesion occurring primarily in the pediatric population. While the precise pathogenesis of the tumor is not certain, the most common theory relates to aberrant mesenchyme development in the portal tract likely related to the bile ducts. A 53-year-old female was evaluated for an incidental liver mass. Initial CT scan showed a cystic lesion in the right lobe of the liver and follow-up imaging revealed an increase in size and the percent solid component within the mass. In view of these changes, a nondiagnostic biopsy was obtained followed by extirpation of the lesion. Gross pathological review of the lesion identified a 9 × 9 × 7.5 cm, pink-yellow-tan, gelatinous mass, with a >1 cm clear surgical margin. Histologically, the mass consisted of benign dilated bile ducts, as well as myxoid stroma with spindle cells showing smooth muscle differentiation. The patient was discharged home on postoperative day five. A review of the literature for MHL in adults reports 30 previous cases, predominantly published as individual case reports describing the size, lobe(s) of the liver affected, and the cystic/solid nature of the tumor. MHL in adults may represent a potentially premalignant lesion, as the emerging literature supports a potential relationship between MHL and malignant undifferentiated embryonal sarcoma in regards to cytogenetic analysis. Aggressive surgical management of MHL in adults is mandated when feasible.
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      – SubjectFull: Hepatic mass
        Type: general
      – SubjectFull: Undifferentiated embryonal sarcoma
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