Medical oncology department, Salah Azaiez Institute. Faculty of medecine of Tunis. El Manar University, Tunis, Tunisia
Corresponding author details:
Yosr Zenzri
Medical Oncology Resident Department of Medical oncology
Salah Azaiez Institute
Tunis,Tunisia
Copyright: © 2020 Yahyaoui Y, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 international License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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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