Clinical Case Summary

We present the case of a 75-year-old woman with in her medical history only medically controlled hypertension. 7 months prior to referral, she started experiencing fatigue, weight loss and anorexia. A CT scan showed multiple splenic and hepatic nodules, most suggestive of haemangiomata on MRI. A CT scan of the vertebral column 3 months later for back pain revealed new bone lesions; suspect for bone metastases or haemangiomata on subsequent MRI imaging. Haematological work-up for multiple myeloma and lymphoma was negative. PET-CT showed FDG-positive lesions in spleen, liver and bones. She was referred to our gastro-enterology service for a liver lesion biopsy. At admission, she had progressive hepatic failure with increased bilirubin and INR, low platelets and hyponatremia. Tumour markers CEA, CA 19.9, AFP were normal. Immunohistochemical (IHC) and histopathological results confirmed a metastatic lesion originating from a primary splenic angiosarcoma (AS). She was unfit for chemotherapy because of rapidly progressive hepatic failure. There was clinical deterioration with fatal outcome shortly after.
Splenic AS is rare with an annual incidence of 0.2/million and high metastatic risk (69-100%). There are no specific diagnostic clinical or biochemical signs. Differentiation on imaging is difficult due to overlap with (benign) lesions such as haemangiomata. Histopathological and IHC tissue analysis is needed for diagnosis. A splenic angiosarcoma has a poor prognosis with a median overall survival (OS) of 5-6 months. Only splenectomy improves OS in early stages. Chemotherapy is often based on regimens for other (angio)sarcomas, but prospective studies are lacking and no regimen has shown a significant effect on OS in this aggressive and rare disease. Based on imaging, the lesions in our patient were too quickly labelled as benign. She developed bone metastasis and hepatic failure during the diagnostic delay of 7 months.

References

We provide the images of the
- CT abdomen and initial MRI of the liver where both the liver and spleen lesions are visible.
- CT and MRI of vertebral column 3 and 4 months respectively after the initial imaging where the bone lesions are visible as well as an increase in size and amount of the lesions in liver and spleen
- PET-CT images with FDG-positive liver, spleen, bone lesions
- Histopathology slides: H&E staining showing sinusoidal growth patterns lined with atypical endothelial cells; IHC staining for ERG (vascular marker); a CD34 -, CD-8+ pattern typical for sinusoidal origin of the spleen.

Clinical Case Summary

Background: Hepatic artery aneurysm (HAA) is a rare and often asymptomatic pathology with a high risk of rupture [1]. HAA is mostly asymptomatic, and 60-80% of the patients are diagnosed when the aneurysm has complications [2]. HAA is a rare cause of jaundice, the most common causes are cholelithiasis, cholangitis, pancreatic head cancer and cholangiocarcinoma [3]. True aneurysms involve all layers of the wall, while pseudoaneurysms result from a tear in the vessel wall with subsequent periarterial hematoma formation.
Case Report: A 45-year-old male presented to the hospital with painless jaundice and fever. Patient is known to have chronic hepatitis B virus, with no treatment. Magnetic resonance cholangiopancreatography (MRCP) was done revealing dilated biliary radicles down to long common bile duct (CBD) stricture by mostly external compression.
Endoscopic retrograde cholangiopancreatography (ERCP) was done, opacification of biliary tree showed dilated biliary radicles on both sides down to tight long CBD stricture about 5 cm in length. Placement of plastic biliary stent was done with adequate biliary drainage.
Computed tomography (CT) done showed evidence of contrast extravasation of common hepatic artery (CHA), forming a large pseudoaneurysm 7x4 cm, compressing the CBD.
After discussion with the patient about treatment options, interventional radiology consultation was done, angiography with occlusion of aneurysm using mixture of lipiodol and histoacryl successfully. Follow-up CT showed reduction size of the aneurysm with no flow inside.
Discussion: Management of HAA is highly variable and depends on clinical presentation and anatomic location. Biliary stenting provides temporary relief for patients with obstructive jaundice. Definitive options include open aneurysmal repair versus endovascular therapy. Hepatic artery aneurysms represent a significant risk for hemorrhage and therefore must be addressed promptly once discovered [4].

References

1- Jana M, Gamanagatti S, Mukund A, Paul S, Gupta P, Garg P, Chattopadhyay TK, Sahni P. Endovascular management in abdominal visceral arterial aneurysms: A pictorial essay. World J Radiol 2011;3:182-7.
2- Sheldon DM, Crawford M, Mihrshahi S, Gallagher J, Storey D. Therapeutic considerations in obstructive jaundice due to hepatic artery aneurysm. HPB (Oxford) 2006;8:74.
3- Hama Y, Chakiri A, Azzaoui F, Dahmane H, Lafrouji S, et al. (2019) Hepatic Artery Aneurysm: A Rare Presentation as Painless Obstructive Jaundice. Med Rep Case Stud 4: 173.
4- Schick C, Ritter RG, Balzer JO, et al. Hepatic artery aneurysms: treatment options. Eur Radiol. 2004;14:157–159.

Clinical Case Summary

A 51-year-old gentleman presented with a four week history of weight loss and anaemia. He has no past medical history but previously, he drank alcohol excessively. Clinically, he was only found to have hepatomegaly. His ALT, ALP, GGT were 65, 267 and 726 respectively. His bilirubin, clotting profile, albumin, alpha-fetoprotein (AFP) and non-invasive liver screen were all normal. CT chest abdomen pelvis showed moderate hepatomegaly with homogenous echotexture and no evidence of malignancy. Four months later he represented with abdominal pain and transudative ascites. His liver profile became grossly deranged with ALT, ALP, GGT of 92, 1446 and 3200 respectively. His bilirubin became 21 and albumin 27. A repeat CT scan showed a large liver with marked focal fatty infiltration, new ascites and a suspicion of new poorly defined focal liver lesion in the left liver lobe. MRI liver showed hepatomegaly, moderate ascites, multiple linear subcapsular T1 low signal, T2 high signal non-enhancing foci mainly in segments 2 and 3 which were described as non-specific. His liver biopsy showed cords of epithelioid cells with hyperchromatic nuclei and cytoplasmic vacuoles on a background of myxohyaline stroma in keeping with hepatic haemangioendothelioma (HHE). He was discussed with our local tertiary liver unit and was deemed not for transplantation. He rapidly deteriorated over several days and re-presented with multi-organ failure and died within 6 months of index presentation. HHE is a rare malignant tumour of vascular endothelial lineage, which was first reported by Weiss et al. in 1982 [1]. The disease can be localised or multifocal within the liver but can also be present in other organs. It is a rare cause of hepatic decompensation which can be difficult to diagnose and treat. The tumour has been reported to be misdiagnosed in approximately up to 80% of cases, commonly as less rarer conditions such as angiosarcoma, cholangiocarcinoma, metastatic carcinoma and haepatoma [2,3].

References

1.Weiss, S.W. and Enzinger, F.M. (1982) “Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma,” Cancer, 50(5), pp. 970–981. Available at: https://doi.org/10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z.
2. Torres LR, Timbo LS, Ribeiro CM, Galvao Filho MM, Verrastro CG, et al. Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review. Radiol Bras. 2014;47:194–6.
3. Virarkar, M. et al. (2020) “Hepatic Hemangioendothelioma: An update,” World Journal of Gastrointestinal Oncology, 12(3), pp. 248–266. Available at: https://doi.org/10.4251/wjgo.v12.i3.248.

Clinical Case Summary

Introduction: Sarcoidosis is a multi-systemic disorder of unknown etiology that primarily involves the pulmonary system. Nevertheless, a diagnosis can be challenging for clinicians because of the potential for almost any organ to be affected. Hepatic granulomas occur in 50-65% of patients with systemic sarcoidosis, while isolated hepatic sarcoidosis is rare.
Aims & Methods: A 50-year-old Montenegrin female patient was consulted by a gastroenterologist due to multiple focal lesions open etiology in the liver and spleen detected on routine ultrasound examination.
Results: Laboratory results showed normal complete blood count, total bilirubin 11.1 µmol/L, aspartate-aminotransferase 35 U/L, alanine-aminotransferase 25 U/L, gamma-glutamyl transferase 247 U/L, pancreatic amylase 40 U/L, and lipase 23 U/L. Tumor markers were in range: carcinoembryonic antigen (CEA), cancer antigen (CA) 19-9, alpha fetoprotein (AFP), CA 72-4, except CA 15-3, which was slightly elevated. Increased activity of serum angiotensin-converting enzyme was shown. Viral and serological tests for hepatotropic viruses and autoimmune liver diseases were negative. Imaging methods performed were: Multi-Slice Computer Tomography (MSCT) of the lungs, abdomen and pelvis, Nuclear Magnetic Resonance of the abdomen with Magnetic Resonance Cholangiopancreatography (MRCP), mammography, upper endoscopy and colonoscopy, but these did not provide an answer to the primary cause of the focal lesion. In the end, a liver biopsy was performed, where non–caseous granulomas were described and a diagnosis of sarcoidosis was established.
Conclusion: The patient had isolated sarcoidosis of the liver and spleen, as no granulomatous abnormalities were identified in other organs. The patient showed a remarkable clinical response to corticosteroid therapy. Isolated hepatic and splenic sarcoidosis should be considered in asymptomatic patients with hepatosplenomegaly and suspected metastatic disease.

References

1. Grunewald J, Grutters JC, Arkema EV, Saketkoo LA, Moller DR, Müller-Quernheim J. Sarcoidosis. Nat Rev Dis Primers. 2019 Jul 4;5(1):45. doi: 10.1038/s41572-019-0096-x. Erratum in: Nat Rev Dis Primers. 2019;5(1):49
2. Franzen DP, Brutsche M, Nilsson J, Böni C, Daccord C, Distler O, Elsener D, Funke-Chambour M, Gruner C, Hayward-Könnecke H, Hostettler KE, Kündig T, Ribi C, Seebach JD, Seeger H, Vrugt B, Kolios AGA. Sarcoidosis - a multisystem disease. Swiss Med Wkly. 2022;152:w30049

Disclosure

This abstract was presented at the 11^th Congress of North Macedonian Society of Gastroenterohepatology with international participation held from June 9-11, 2023 in Ohrid, North Macedonia.

Clinical Case Summary

Post-procedural haemorrhage following percutaneous liver biopsy is a rare yet potentially life-threatening complication, with fistulisation of a hepatic haematoma into the gastrointestinal tract being an exceedingly rare event.
A 75-year-old lady with suspected autoimmune liver disease underwent an ultrasound-guided percutaneous liver biopsy with a 16G needle, performed in a single pass and no immediate complications. Two hours later, the patient developed haemorrhagic shock and was transferred to the ICU, where a contrast-enhanced CT scan revealed a 10 × 9 cm hepatic haematoma in the right lobe with active arterial extravasation and haemoperitoneum (Figure 1). Angiography confirmed active bleeding from a segmental branch of the right hepatic artery (Figure 2), which was successfully embolised with microcoils (Figure 3).
Follow-up CT scan 45 days later showed persistent encapsulated haematoma with fistulisation to duodenal bulb (Figure 4). Gastroscopy showed no haemorrhagic content through the fistula (Figure 5). Given the patient's stable condition and reviewed by a multidisciplinary team, surgery was not required. Radiological control after three months, shows near-complete haematoma resolution and fistula closure (Figure 6). Autoimmune hepatitis was confirmed, and immunosuppressive therapy deferred until resolution of the acute complication.
Percutaneous liver biopsy is safe, with ultrasound guidance reducing complication risk. The most common adverse event is pain, in up to 25% of cases. Haemorrhagic complications, including haemoperitoneum, hepatic haematoma, or haemobilia, occur in 0.01–0.5% of cases. Haemobilia, the rarest complication (~0.006%), presents with abdominal pain, jaundice, and melaena. In cases of active bleeding, selective arterial embolisation is preferred. Fistulisation into the gastrointestinal tract is rare and usually associated with large haematomas. Early diagnosis and intervention were key to preventing a fatal outcome in this case.

References

Tian G, Kong D, Jiang T, Li L. Complications After Percutaneous Ultrasound‐Guided Liver Biopsy. Journal of Ultrasound in Medicine. 2020 Jan 30;39(7):1355–65.

Clinical Case Summary

Background: Syphilis is a chronic systemic infection caused by the spirochete Treponema pallidum, often referred to as "the great imitator" due to its ability to affect nearly any organ. Syphilitic hepatitis, or "luetic jaundice," is a rare manifestation, indicating spirochete dissemination to the liver.
Case Report: A 26-year-old female was admitted to the hospital with fever (39.5 °C), fatigue, sweating, tremor, dark urine, pale stools, and significant weight loss (15 kg over two months). She also reported lower extremity muscle and joint pain, cough and slowing of speech. On examination, she had icteric skin, a generalized maculopapular rash, and enlarged cervical lymph nodes. No history of alcohol or drug use was noted.
Laboratory tests revealed elevated levels of both cytolytic and cholestatic liver enzymes, along with increased bilirubin an elevated C-reactive protein.
Abdominal ultrasound and contrast CT showed hepatomegaly, hilar and retroperitoneal lymphadenopathy and splenomegaly.
Viral hepatitis markers (HBsAg, HBV PCR, HCVAb, HCV PCR, anti-HAV IgM, anti-HEV IgM) and antibodies for CMV, EBV, HIV, HSV-1,2 were negative. Wright-Hedelson test for brucellosis and serological markers for autoimmune hepatitis were negative while IgG and cerulopasmin levels were within normal range. Given systemic findings and history, syphilis was considered. Serologic tests were positive for Treponema pallidum (ELISA, TPHA, MRP). Liver biopsy revealed mild active hepatitis. The diagnosis of ^secondary syphilitic hepatitis was made. The patient was started on antimicrobial therapy. During follow-up, liver function tests normalized, and systemic symptoms resolved.
Conclusion: This case underscores the importance of considering syphilis in the differential diagnosis of unexplained hepatitis. Although rare, syphilitic hepatitis should be considered in patients with liver enzyme elevations and nonspecific symptoms, particularly when no clear alternative diagnosis is found.

References

1) Malvar G, Cardona D, Pezhouh MK, Adeyi OA, Chatterjee D, Deisch JK, Lamps LW, Misdraji J, Stueck AE, Voltaggio L, Gonzalez RS. Hepatic Secondary Syphilis Can Cause a Variety of Histologic Patterns and May Be Negative for Treponeme Immunohistochemistry. Am J Surg Pathol. 2022 Apr 1;46(4):567-575. doi: 10.1097/PAS.0000000000001848. PMID: 34864775.
2)Supronowicz Ł, Rogalska M. Syphilitic hepatitis. Clin Exp Hepatol. 2024 Mar;10(1):9-13. doi: 10.5114/ceh.2024.136235. Epub 2024 Mar 12. PMID: 38765910; PMCID: PMC11100338.
3)Al Dallal HA, Narayanan S, Alley HF, Eiswerth MJ, Arnold FW, Martin BA, Shandiz AE. Case Report: Syphilitic Hepatitis-A Rare and Underrecognized Etiology of Liver Disease With Potential for Misdiagnosis. Front Med (Lausanne). 2021 Nov 29;8:789250. doi: 10.3389/fmed.2021.789250. PMID: 34912834; PMCID: PMC8666961.
Figure 1: Histological images of the liver biopsy
Figure 2: Abdominal CT images
Figure 3: Images of maculopapular rash

Introduction

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymfohistiocytosis (HLH) is poorly described life-threatening condition. It is caused by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan involvement. Clinically, MAS is characterized by fever, cytopenia, hepatosplenomegaly and elevated circulating ferritin. MAS can develop into acute liver failure (ALF) with poor prognosis.

Aims & Methods

We retrospectively analysed patients referred to our transplantation centre for ALF in whom the diagnosis of MAS was confirmed by histology. We focused on diagnosis leading to MAS, survival, transplantation outcome and clinical, laboratory and histological findings.

Results

From January 2005 to November 2022, we found eight patients with MAS referred to our centre for ALF. The underlying condition was EBV infection in five cases and adult Still disease in three cases. All eight patients had strongly elevated circulating ferritin (in average 19321 μg/l), leukocytosis and thrombocytopenia. All patients except one had at least one negative bone marrow or lymph node examination prior to definitive diagnosis of MAS. Seven patients were treated with high-dose corticosteroids. Four patients met King's College criteria for urgent liver transplantation. Two of them were transplanted. Despite intensive therapy based on mechanical ventilation, continuous renal replacement therapy and circulation support, their clinical condition further deteriorated and all four patients died on average 34 days after symptom onset of multiorgan failure. At the autopsy, hemophagocytic macrophages have been described in multiple organs. Four patients who responded to corticosteroid therapy are in long-term remission after specialized haematological treatment with etoposide.

Conclusion

Macrophage activation syndrome represents rare cause of acute liver failure with high mortality, it is difficult to diagnose and even harder to treat. Our case series suggests that when the patients meet King's College criteria, they die despite the successful liver transplant. Therefore, it is important to carefully consider liver transplantation in these patients.