Hepar Lobatum Carcinomatosum (HLC) is an acquired hepatic distorsion which generally refers to liver abnormalities in tertiary syphilis. It is an exceptional liver dysmorphy associated with liver metastases of carcinoma, most often breast carcinoma. It is a noncirrhotic hepatic disease. We report the case of a 58-year-old woman with HLC resulting from metastatic mammary carcinoma in the liver. We analyse through this observation the clinical, radiological and histological characteristics of this entity

Hepar lobatum; Breast; Cancer; Metastases

Hepar Lobatum Carcinomatosum (HLC) is an unusual clinical finding. It is an acquired liver dysmorphy with lobulated contour and linear depressions mostly known as the endstage of tertiary syphilis [1]. HLC have been described in association with liver metastases of carcinoma in many cases [2]. That is resulting from invasive metastatic breast cancer in most cases [3].

A 58-year-old woman was followed-up for carcinoma of the breast, which was staged T4bN1M1 with multiple liver mestastases .Breast Biopsy led to a diagnosis of an invasive ductal carcinoma grade III, Her2 positive, hormone-receptor-negative with a ki-67 index of 18% . She was initially treated by chemotherapy (5-Fluorouracil – Epirubicin-Cyclophosphamide). After 5 courses, the patient complained of hepatalgia and major asthenia. The physical examination showed hepatomegaly and a right upper quadrant abdominal pain. Last blood tests revealed thrombocytopenia and liver tests showed increased serum levels of aspartate and alanine aminotransferases which were respectively four and three times the upper normal limit. Low prothrombin ratio (58%) and increased glutamyl-transpeptidase which was fourteen times the upper normal limit were observed. The level of CA 15-3 was 584.6 UI/ml. There was no clinical or serological evidence for hepatitis B or C virus infection. Moreover, there was no history of alcohol or drug consumption. CT scan revealed a heterogeneous and dysmorphic liver with capsular retractions, bosselated contour and a major atrophy of the right liver lobe. Hepatic nodules were observed in the left lobe. Imaging revealed a tumor regression of metastases of the left liver lobe (Figure 1). Magnetic resonance imaging supported the diagnosis of Hepar Lobatum Carcinomatosum. A CT-guided liver biopsy was performed. Histological examination of the liver biopsy showed a proliferation with trabecular and glandular architecture invading the liver sinusoids and obstructing the biliary tract. No fibrosis was observed. There were no granulomas, giant cells or features indicating coexisting chronic hepatitis. The patient is still on second line chemotherapy by Capecitabine. A baseline CT scan was performed before starting Capecitabine showing dysmorphic liver with hypertrophy of segments VIII, VII, VI and IV with an extensive fibrosis supporting the diagnosis of Hepar Lobatum Carcinomatosum (Figure 2).

In this observation, we made the diagnosis of hepar lobatum by the association of hepatic dysmorphy, the evocative clinical context (liver metastases of breast cancer) and the physical examination (hepatomegaly and a right upper quadrant abdominal pain). The absence of risk factors for chronic liver disease (no alcohol consumption, no serological evidence for hepatitis B or C virus) dismisses the diagnosis of liver cirrhosis. This hypothesis was confirmed by the sequential data of the computed tomography and MRI that reported dysmorphic liver with capsular retractions and the histological examination. Hepar Lobatum (HL) is a non-cirrhotic hepatic dysmorphy. It is mostly known as the end-stage of tertiary syphilis [1]. Since 1924; HL was reported in association with liver metastases of carcinoma [2]. In most cases, that is resulting from invasive metastatic mammary carcinoma [3]. Liver metastases from the stomach, rectum has also been associated to that pathology [4-5].

The clinical manifestations of liver disease observed in patients who developed HLC have included jaundice, hepatomegaly, ascites, and esophageal varices [6]. The HL can be revealed by ruptured osophageal varices [7].

A variety of chemotherapy agents have been used in patients reported to have developed HLC including alkylating agents (Cyclophosphamide), cytotoxic antibiotics (Doxorubicin, Mitomycin, Mitoxanthrone) , antimetabolites (Methotrexate, 5-fluorouracil), and plant alkaloids (Vinblastine, Paclitaxel, Docetaxel) [7]. In our case, liver dysmorphy was detected in CT scan after 5 courses of Cyclophosphamide/Epirubicin/5-Fluorouracil (FEC regimen).

Liver imaging made the diagnosis in most cases showing liver with capsular retractions and lobulated pattern .A dysmorphic transformation of a previously smooth and normal sized liver is usually observed in the absence of etiologic factors of cirrhosis. Signs of portal hypertension are frequently observed in HLC. MRI may also be useful to support this diagnosis, showing large fibrous bands [8].

Histopathological examination usually shows fibrous septa containing scattered tumor cells running deep in the parenchyma [9]. Intrahepatic vessels, mostly sinusoidal and portal are invaded by metastatic cells causing an occlusion. Heterogeneous liver and atrophy of the invaded segments are a result of the vascular injury. The loss of of sinusoidal wall integrity can be associated with peri sinusoidal fibrosis [4,8].

HLC has a poor prognosis. The expected survival is usually less than 1 year [9].

Figure 1: CT scan of the liver showing a heterogeneous and dysmorphic liver with bosselated contour and a major atrophy of the right liver lobe

Figure 2: CT scan showing dysmorphic liver with hypertrophy of segments VIII, VII and IV

Hepar lobatum carcinomatosum resulting from metastatic carcinoma is a very rare phenomenon. This particular presentation could result from the combination between vascular injury caused by metastatic cells and chemotherapy agents. HLC has a poor prognosis.

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