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ISSN: Print -2349-0977, Online - 2349-4387


 
 Table of Contents  
LETTER TO EDITOR
Year : 2014  |  Volume : 1  |  Issue : 3  |  Page : 246-249

Diffuse hepatic hemangiomatosis


1 Department of Radiology, Institute of Liver and Biliary Sciences, New Delhi, India
2 Department of HepatoBiliary Surgery, Institute of Liver and Biliary Sciences, New Delhi, India
3 Department of Pathology, Institute of Liver and Biliary Sciences, New Delhi, India

Date of Web Publication27-May-2015

Correspondence Address:
Binit Sureka
Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, New Delhi - 110 070
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2349-0977.157777

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How to cite this article:
Bansal K, Sureka B, Bharathy KG, Bihari C, Arora A. Diffuse hepatic hemangiomatosis. Astrocyte 2014;1:246-9

How to cite this URL:
Bansal K, Sureka B, Bharathy KG, Bihari C, Arora A. Diffuse hepatic hemangiomatosis. Astrocyte [serial online] 2014 [cited 2020 Sep 23];1:246-9. Available from: http://www.astrocyte.in/text.asp?2014/1/3/246/157777

Sir,

Diffuse hemangiomatosis of liver without extrahepatic involvement is a rare entity and association with giant cavernous hemangioma is even rarer. Moreover, its occurrence in an adult is described in the form of few case reports in the literature. In this report, we try to revisit this rare entity and briefly discuss the management and imaging differential diagnoses.

A 34-year-old female presented to the Out-patient Department with complaints of abdominal discomfort, mild epigastric pain on and off since 15-20 days. The pain was insidious in onset and gradually progressive in nature associated with a lump in the upper abdomen. The pain was confined to the epigastric region to start with, but later and gradually involved whole of the right upper abdomen. Pain was associated with early satiety and loss of weight. The patient also had nausea and one episode of vomiting since the onset of pain. The past history revealed on and off abdominal discomfort since 10 days which settled on its own. The lump in epigastric region was soft in consistency, tender, and continuous with liver dullness. The patient was afebrile, conscious, and oriented with stable vitals. Her pulse rate was 80/min, blood pressure of 120/70 mmHg, respiratory rate of 20/min. The laboratory findings indicated a hemoglobin level of 11.9 g/dL, a platelet count of 145 (10 9 /L), slightly elevated serum alanine aminotransferase (37 IU/L) and normal aspartate aminotransferase (36 IU/L). Hepatitis B virus antigen was negative, and alpha-fetoprotein was normal. Patient was evaluated outside with an ultrasound that revealed altered echo texture of liver and a large space-occupying lesion in the left lobe of the liver. No evidence of Rendu-Osler-Weber disease or hemangiomas in the extrahepatic region was seen on clinico-radiological evaluation.

At our Institution, the patient underwent contrast enhanced computed tomography of the abdomen. Dynamic computed tomography (CT) using hepatic protocol was performed which revealed a giant cavernous hemangioma in the left lobe of the liver. In addition to the large giant hemangioma in the left lobe, the entire liver was studded with typical and atypical hemangiomas [Figure 1]. Dynamic contrast-enhanced magnetic resonance imaging (MRI) was also done, which further confirmed the findings [Figure 2]. There was no evidence of capsular retraction or calcification. There was no evidence of intraabdominal metastasis or lymphadenopathy. Hence, a diagnosis of giant cavernous hemangioma with diffuse hepatic hemangiomatosis without extrahepatic involvement was given.
Figure 1: Dynamic computed tomography scan images showing (a-c) space occupying lesion in liver and large giant lesion in left lobe of liver which shows peripheral nodular enhancing lesions in liver in hepatic arterial phase (d-f) portal venous phase images showing progressive centripetal filling (white arrowhead) with additional nodular enhancing lesions (black arrow) (g-i) delayed phase images showing persistence of enhancement and gradual centripetal filling with nodular enhancing lesions (black arrow) adjacent to the giant hemangioma (white arrowhead).

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Figure 2: (a and b) Coronal reformatted T2-weighted images showing multiple typical as well as atypical hyperintense hemangiomas (arrows) in right lobe of liver (a) and giant cavernous hemangioma (arrowhead) (b) in left lobe of liver. (c and d) Coronal reformatted venous phase images showing enhancing lesions (arrows) in liver (c) and peripheral nodular enhancement (arrowhead) in giant hemangioma (d).

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The patient underwent left hepatectomy with middle hepatic vein with cholecystectomy. Left lobe of the liver weighed 850 g. A unilocular well-encapsulated cystic lesion is measuring 13 × 14 × 6 cm [Figure 3]. Outer surface congested by brownish discoloration. No breach in capsule identified. Cut-surface was spongy with multiple cystic spaces filled with blood. No solid mass lesion identified. Microscopic examination showed a tumor composed of cavernous sized blood filled spaces lined by single layer of benign looking plump endothelial cells with fibro-collagenous walls. The resection margin of the liver showed cavernous spaces interspersed within the liver parenchyma. Features were consistent with cavernous hemangioma [Figure 4].
Figure 3: (a) Intraoperative photographs. The left lobe of liver is completely replaced by the tumor (arrows) with additional nodular lesions surrounding the larger lesion. (b) Postoperative resected surgical specimen.

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Figure 4: (a) Gross and (b) microscopic (H and E, ×40) examination: Blood filled large spaces lined by benign endothelial cells (arrow).

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Most common benign liver tumors are hemangiomas. The estimated prevalence of liver hemangiomas is 5-20%. [1],[2] They usually present as a well-defined mass with classical imaging findings. Hepatic hemangiomatosis is a much rare condition, which is characterized by diffuse or nodular pattern of replacement of liver parenchyma with hemangiomatous lesions. Hepatic hemangiomas and hemangiomatosis are two separate entities with similar histological appearances. Co-existent of these both entities in liver in a single patient is much rare and is described in the form of case reports.

Contributing factors in the development of hepatic hemangiomatosis are not well-documented except for its association with rare systemic diseases, such as hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease), systemic hemangiomatosis and the possible role of drugs like use of metoclopramide and oral contraceptives. [2],[3]

Clinically, the patient may be asymptomatic or present with abdominal pain, abdominal discomfort, palpable mass, congestive heart failure due to high cardiac output states as a result of intra hepatic arteriovenous shunting. Rarely, patients may present with complications like rupture, thrombocytopenia and consumptive coagulopathy. [4]

On imaging, two types of hepatic hemangiomas have been described. These are typical hemangiomas and atypical hemangiomas. Atypical hemangiomas are further classified into giant hepatic hemangioma (>6 cm), flash filling hepatic hemangiomas, calcified hemangioma, hyalinized hemangioma, hemangiomas with capsular retraction, with surrounding regional nodular hyperplasia, with fatty infiltration, cystic hepatic hemangioma, pedunculated hepatic hemangioma and fluid-fluid level containing hepatic hemangioma. [5]

Hemangiomatosis is different from hepatic hemangioma. The boundary of the lesion is ill defined in hemangiomatosis whereas the periphery is well-defined in hemangioma. Two types of hepatic hemangiomatosis are seen diffuse and nodular form. Diffuse type shows involvement of the entire liver with multiple hemangiomas. Diffuse type is more common in infants. [6] In nodular type, focal involvement of the liver is seen with small discrete and coalescent nodules measuring <5 mm showing peripheral discontinuous enhancement during the arterial phase with filling in during portal venous and delayed phase is seen. [2] Rarely, hepatic hemangiomatosis is seen adjacent to a giant cavernous hemangioma in the liver parenchyma. The common hepatic artery may be prominent (>5 mm) in cases of hemangiomatosis and giant cavernous hemangiomas.

On the ultrasound, liver affected by hemangiomatosis appears as homogeneous hyperechoic areas with poorly defined margins. In nodular type, they may show a heterogeneous echo pattern with multiple discrete or coalescent small hypoechoic nodules on a hyperechoic background. CT and MRI may show typical as well as atypical imaging features depending on the pleomorphic histological appearance.

Differential diagnoses on imaging are hypervasular tumors of the liver like epithelioid hemangioendotheliomas, angiosarcomas, hypervascular metastasis, hepatocellular carcinoma, hepatic adenoma, focal nodular hyperplasia, mesenchymal neoplasms of the liver and primary hepatic carcinoid. Hemangiomas show hyperintense signal on T2-weighted imaging and dynamic contrast-enhanced scans show peripheral discontinuous nodular enhancement with centripetal progression. Focal nodular hyperplasia shows a bright T2 central scar which shows delayed enhancement. Hypervascular metastases are hyperintense on T2-weighted images but on dynamic scans show heterogeneous enhancement and peripheral washout (target lesions). Metastases treated with chemotherapy may mimic hemangioma, but careful clinical and previous imaging evaluation can clinch the diagnosis. Metastasis treated with chemotherapy show peripheral continuous nodular enhancement with centripetal progression.

The index case was unique in few aspects. Hemangiomatosis is rare in adults and co-existence of giant cavernous hemangioma and hemangiomatosis in liver in the same patient is also rare. Extrahepatic involvement was not seen. No cutaneous lesion was seen in this patient.

Recognition of co-existence of both hemangioma and hemangiomatosis in a single patient is important for surgical management. For symptomatic giant cavernous hemangioma with an acceptable surgical risk, operative resection in the form of enucleation, wedge excision, or anatomic lobectomy is sufficient. Enucleation is preferred as it is associated with less operative blood loss and reduced risk of biliary leaks. [7] Symptomatic patients with giant hemangiomas and associated extensive hemangiomatosis are difficult to manage by enucleation alone and require extensive lobectomy. Some of the cases may require hepatic transplantation or additional alternative management techniques such as irradiation therapy and arterial embolization. [2],[8]


  Conclusion Top


Diffuse hemangiomatosis of liver with coexisting giant hemangioma without extrahepatic involvement is rare in adult. Awareness of the existence of this uncommon entity and its imaging findings can help reach an accurate diagnosis and early management of these patients can reduce morbidity.

 
  References Top

1.
Weimann A, Ringe B, Klempnauer J, Lamesch P, Gratz KF, Prokop M, et al. Benign liver tumors : Differential diagnosis and indications for surgery. World J Surg 1997;21:983-90.  Back to cited text no. 1
    
2.
Jhaveri KS, Vlachou PA, Guindi M, Fischer S, Khalili K, Cleary SP, et al. Association of hepatic hemangiomatosis with giant cavernous hemangioma in the adult population : Prevalence, imaging appearance, and relevance. AJR Am J Roentgenol 2011;196:809-15.  Back to cited text no. 2
    
3.
Feurle GE. Arteriovenous shunting and cholestasis in hepatic hemangiomatosis associated with metoclopramide. Gastroenterology 1990;99:258-62.  Back to cited text no. 3
    
4.
Moon WS, Yu HC, Lee JM, Kang MJ. Diffuse hepatic hemangiomatosis in an adult. J Korean Med Sci 2000;15:471-4.  Back to cited text no. 4
    
5.
Vilgrain V, Boulos L, Vullierme MP, Denys A, Terris B, Menu Y. Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics 2000;20:379-97.  Back to cited text no. 5
    
6.
Kim EH, Park SY, Ihn YK, Hwang SS. Diffuse hepatic hemangiomatosis without extrahepatic involvement in an adult patient. Korean J Radiol 2008;9:559-62.  Back to cited text no. 6
    
7.
Singh RK, Kapoor S, Sahni P, Chattopadhyay TK. Giant haemangioma of the liver : Is enucleation better than resection? Ann R Coll Surg Engl 2007;89:490-3.  Back to cited text no. 7
    
8.
Maeda E, Akahane M, Watadani T, Yoshioka N, Goto A, Sugawara Y, et al . Isolated hepatic hemangiomatosis in adults : Report of two cases and review of the literature. Eur J Radiol Extra 2007;61:9-14.  Back to cited text no. 8
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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