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Review
. 2020 Apr 7;7(2):HEP19.
doi: 10.2217/hep-2020-0002.

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Sebastiao N Martins-Filho  1   2 , Juan Putra  2   3
Affiliations
Review

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Sebastiao N Martins-Filho et al. Hepat Oncol. .

Abstract

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

Keywords: C19MC; MALAT1; hepatic mesenchymal hamartoma; hepatic neoplasm; malignant transformation; pediatric liver tumors; placental mesenchymal dysplasia; undifferentiated embryonal sarcoma of the liver.

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Conflict of interest statement

Financial & competing interests disclosure The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript.

Figures

Figure 1.
Figure 1.. Pathologic findings of hepatic mesenchymal hamartoma.
Hepatic mesenchymal hamartoma is histologically characterized by (A) a multinodular growth of myxomatous mesenchymal stroma with intervening fibrous septa (hematoxylin & eosin, 4×); (B) each nodule consists of a bland spindle cell proliferation with scattered malformed bile ducts (hematoxylin & eosin, 10×). (C) Some of the nodules demonstrate a florid bile duct proliferation with scattered lymphocytic infiltrate (hematoxylin & eosin, 10×). (D) Entrapped island of hepatocytes (arrow) is occasionally identified in the periphery of the lesion (hematoxylin & eosin, 10×).
Figure 2.
Figure 2.. Pathologic findings of undifferentiated embryonal sarcoma of the liver.
Undifferentiated embryonal sarcoma of the liver generally shows (A) hypercellular sheets of tumor cells (hematoxylin & eosin, 4×); (B) the neoplastic cells are highly pleomorphic with an increased nuclear to cytoplasmic ratio and hyperchromatic nuclei; tumor giant cells (thick arrow) are frequently identified (hematoxylin & eosin, 10×). (C) The lesional cells are focally elongated/spindled with a loose, myxoid stroma (hematoxylin & eosin, 10×). (D) Necrosis (lower left) is occasionally seen (hematoxylin & eosin, 10×). (E) Mitotic figures (thick arrow) and apoptotic bodies (arrowheads) are readily identified (hematoxylin & eosin, 20×). (F) Eosinophilic hyaline globules (thin arrow), which are PAS-positive and diastase-resistant, may be observed in the neoplastic cell cytoplasm and extracellular matrix (hematoxylin & eosin, 20×).
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