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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 322-324
Hepatic angiosarcoma arising in an adult mesenchymal hamartoma

Department of Pathology, Govt. Medical College, Miraj- 416410, Maharashtra, India

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Date of Web Publication12-Jun-2010


Embryonal (undifferentiated) sarcomas arising in mesenchymal hamartoma are on record but cases of hepatic angiosarcoma (AS) arising in mesenchymal hamartoma (MH) of the liver are extremely rare. We report one such case in a 20-year-old male patient. He presented with a lump in the right hypochondriac region and pain of two years duration with rapid increase in size since two months. Ultrasonography (USG) revealed a well circumscribed mass on the undersurface of the right lobe of liver suggestive of hemangioma. The patient underwent resection of the mass. Histopathology revealed AS with areas of MH.

Keywords: Angiosarcoma, liver, mesenchymal hamartoma

How to cite this article:
Kulkarni MP, Agashe SR, Singh R, Sulhyan KR. Hepatic angiosarcoma arising in an adult mesenchymal hamartoma. Indian J Pathol Microbiol 2010;53:322-4

How to cite this URL:
Kulkarni MP, Agashe SR, Singh R, Sulhyan KR. Hepatic angiosarcoma arising in an adult mesenchymal hamartoma. Indian J Pathol Microbiol [serial online] 2010 [cited 2022 Aug 16];53:322-4. Available from: https://www.ijpmonline.org/text.asp?2010/53/2/322/64311

   Introduction Top

Mesenchymal hamartoma (MH) is a tumor malformation that develops in utero. It accounts for eight per cent of all liver tumors and pseudo tumors from birth to 21 years of age. [1] It usually manifests in the first two years of life but is also reported in adults. [2] The prognosis is excellent after surgical resection. Although malignant transformation to embryonal sarcoma is well known, [3],[4] angiosarcoma (AS) arising in MH is extremely rare. [5] We report a case of MH with angiosarcomatous transformation in a 20-year-old male.

   Case Report Top

A 20-year-old male patient presented with a lump in the right hypochondriac region of two years duration and intermittent dragging pain. There was no history of jaundice or vomiting. Initially the lump was slow growing, but since two months the patient noticed rapid increase in its size. Physical examination was unremarkable except for the presence of a huge palpable lump in the right hypochondrium. Routine hematological and biochemical investigations including liver function tests and serum alpha fetoprotein levels were within normal limits. He was negative for Hepatitis B surface antigen (HBsAg) and Hepatitis C viral antigen (HCV). USG revealed a large well circumscribed mass of 14 x 11cm size on the undersurface of right lobe of liver involving the caudate lobe. Peripheral solid component was seen with central cystic areas. The rest of the liver, intrahepatic biliary radicals and portal vein were unremarkable. A diagnosis of hemangioma was offered by the radiologist. Fine needle aspiration cytology (FNAC) was not done in view of this diagnosis. The mass was resected.

The specimen consisted of a well circumscribed mass measuring 16 x 10.5 x 4cm having a smooth contour. The entire cut surface was dark brown with scattered myxoid, cystic and necrotic areas [Figure 1].

Multiple sections from the center of the mass revealed a tumor composed of round to oval to spindle shaped cells with hyperchromatic nuclei, ill defined cytoplasmic borders and scanty cytoplasm lining anastomozing capillary sized vascular channels [Figure 2]. At places, these cells were seen lining variably sized cavernous spaces filled with blood. A few bizarre cells and tumor giant cells were noted. Focal myxoid change and extracellular eosinophilic hyaline globules were evident. Extensive areas of hemorrhage and necrosis were seen. Sections from the periphery of the mass showed areas resembling mesenchymal hamartoma composed of loose edematous connective tissue intermixed with bile ducts arranged in ductal plate malformation pattern [Figure 3]. Islands of native hepatocytes were also seen. With these findings a provisional diagnosis of angiosarcomatous transformation in mesenchymal hamartoma was made. Immunohistochemistry revealed positivity for CD 31 and CD 34 in the sarcomatous areas confirming the diagnosis of AS.

Our patient is currently on chemotherapy and is symptomatically better. Study of more cases will be needed to know whether there are any differences in the prognosis and behavior of AS arising de novo and AS arising in MH.

   Discussion Top

MH is usually a solitary, well circumscribed mass lesion with size ranging from 5 to 25 cms, occurring more frequently in the right lobe of liver. [6] Although it is multicystic in more than 90% of cases, solid mesenchymal hamartomas have also been reported. [7],[8] Fewer than half of the adult cases are predominantly solid. Certain characteristics of the tumors in adults differ from those found in childhood. Abdominal pain is the most frequent symptom in adults while occurrence of pain is rare in children. Also, in adults, the disease is more common in females whereas the sex distribution is equal in children. [8]

As far as the pathogenesis of MH is concerned, it is believed to be neoplastic. [6] Angiosarcomatous transformation in MH is an extremely rare event and to the best of our knowledge only one such case has been reported. [5] Our patient was a 20-year-old male who had an abdominal lump of two years duration with a history of rapid increase in size since two months. Histopathological study revealed typical AS along with areas resembling MH. The ductal plate malformation pattern of bile ducts and presence of loose edematous mesenchymal tissue gave a definite clue to the diagnosis of MH which was also supported by the fact that the lump was present for two years. Extramedullary haemopoiesis seen in more than 85% of MH was, however, not evident in our case. The diagnosis of AS was based on light microscopy as well as immunohistochemistry which revealed positivity for CD 31 and CD 34. CD 31 is believed to be the most sensitive immunostain for AS. [1] The eosinophilic globules seen in our case are also described by other workers in most cases of childhood AS. [9]

Our patient did not have exposure to any of the chemicals or drugs known to cause AS. Hence the etiology remains unknown.

Review of literature revealed only one case of AS arising in an adult MH. [5] The authors have reported a case of a 33-year-old Chinese woman who was diagnosed as hepatic hamartoma eight years ago on FNAC. She did not accept effective treatment, subsequently developed AS and succumbed to it.

   Acknowledgment Top

We are thankful to Department of Pathology, Tata Memorial Hospital, Mumbai, for providing us with immunohistochemical support.

   References Top

1.Ishak KG, Anthony PP, Niederau C, Nakanuma Y. Mesenchymal tumors of the liver In: Hamilton SR, Aaltonen LA.editors. World Health Organization classification of tumors: Pathology and genetics of tumors of the digestive system. Lyon: IARC Press; 2000. p. 191-8.  Back to cited text no. 1      
2.Rosai J. Liver-Tumors and tumourlike conditions. In: Juan Rosai, editor.Rosai and Ackerman′s Surgical Pathology. 9 th ed. Missouri: Elsevier; 2004. p. 992-1033.  Back to cited text no. 2      
3.de Chadarevian JP, Pawel BR, Faerber EN, Weintraub WH. Undifferentiated (embryonal) sarcoma arising in conjunction with mesenchymal hamartoma of the liver. Mod Pathol 1994;7:490-3.  Back to cited text no. 3      
4.Lauwers GY, Grant LD, Donnelly WH, Meloni AM, Foss RM, Sanberg AA, et al. Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Am J Surg Pathol 1997;21:1248-51.  Back to cited text no. 4      
5.Qiang Li, Jian W, Yan S, Long CY, Shan HX. Hepatic angiosarcoma arising in an adult mesenchymal hamartoma. Int Semin Surg Oncol 2007;4:3. Available from: www.issoonline.com/content/4/1/3 .  Back to cited text no. 5      
6.Washington K. Masses of the liver. In: Mills SE editor. Sternberg′s Diagnostic Surgical Pathology. 4 th ed. Philadelphia: Lippincott Williams and Wilkins; 2004. p. 1705-74.  Back to cited text no. 6      
7.Chung JH, Cho K J, Choi DW, Lee BH, Chi JG. Solid mesenchymal hamartoma of the liver in adult. J Korean Med Sci 1999;14:335-7.  Back to cited text no. 7      
8.Hernandez JC, Alfonso C, Gonzalez L, Samada M, Ramos L, Cepero-Valdez, et al. Solid mesenchymal hamartoma in an adult: A case report. J Clin Pathol 2006;59:542-5.  Back to cited text no. 8      
9.Stocker JT, Husain AN, Dehner LP, Chandra RS.The liver, gall bladder and biliary tract. In: Stocker JT, Dehner LP, editors. Pediatric Pathology. 2 nd ed. Philadelphia: Lippincott Williams and Wilkins; 2002. p. 705-95.  Back to cited text no. 9      

Correspondence Address:
Medha P Kulkarni
Parijat, Plot No.38, Rajput, Lane, Vidyanagar, Vishrambag, Sangli
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Source of Support: Department of Pathology, Tata Memorial Hospital, Mumbai, Conflict of Interest: None

DOI: 10.4103/0377-4929.64311

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  [Figure 1], [Figure 2], [Figure 3]

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