Diffuse Hepatomegaly Research Articles

P224 A challenging case of pyrexia of unknown origin of adrenal mass

Poster session 2, September 22, 2022, 12:30 PM - 1:30 PMA familiar dictum in tropical countries is to consider the diagnosis of tuberculosis (TB) in a patient with fever of unknown origin until proven otherwise. Often, in resource-limited settings, a response to a trial of empiric anti-tuberculous therapy (ATT) is needed to prove TB. However, chronic granulomatous diseases such as invasive fungal infections and sarcoidosis have clinical features resembling extrapulmonary TB. Thus, the workup for a definitive diagnosis is necessary. A 53-year-old male presented with low grade, intermittent fever, loss of weight and appetite for a duration of 4 months and generalized abdominal pain for the last 2 months. He has lost about 16 kg in the past 6 months. He also reported a mild cough with scanty white mucoid sputum. There was no contact with a case of TB. He developed small oval/circular copper Color patchy lesions in the trunk for the last 1 month (Fig. 1). The general examination of the patient was grossly normal except for hepatomegaly. Routine blood examination, renal and liver function test, and chest X-ray were normal and HIV ELISA was non-reactive. With the strong suspicion of TB, the patient underwent CT scanning of the chest and abdomen was done which showed bilateral heterogeneous adrenal mass with few hypodense areas within measuring right side 3.1 × 1.8 cm and left side 3.1 × 2.5 cm in suprarenal location and liver is enlarged size 19.3 cm with normal attenuation (Fig. 2). There were no clinical features to suggest pheochromocytoma or Cushing's syndrome. The PET-CT revealed metabolically active disease involving bilateral adrenal gland, abdominal and retrosternal lymph node with diffuse hepatomegaly. Transabdominal ultrasound-guided FNA was done from adrenal lesions. The tests for tuberculosis were negative and the cytology reported necrotizing granuloma with intracytoplasmic yeast cells suggestive of histoplasmosis. The adrenal endocrine profile was normal. From skin lesions, a biopsy was done. Direct KOH from skin lesion revealed budding yeast cells while the cultures are awaited. The patient was treated with 3 mg/kg liposomal amphotericin B for 2 weeks followed by daily itraconazole for maintenance. The fever was resolved after 8 days of liposomal amphotericin B and skin lesions softened after 3 weeks of therapy.

Spontaneous outbreak of Yersinia enterocolitica infection and co-infection with Escherichia coli in black-tufted marmosets (Callithrix penicillata)

Yersiniosis is a zoonotic bacterial disease that affects humans and animals, including primates. The aim of the study was to report one case of fatal Yersinia enterocolitica infection and two cases of co-infection with Escherichia coli in captive black tufted marmosets (Callithrix penicillata) in Apucarana, Paraná, south Brazil. The affected animals presented severe diarrhea and progressed to death. Gross examination showed multifocal to coalescing necrosis in the liver, mild diffuse hepatomegaly, mild diffuse necrotizing enteritis, moderate splenomegaly and focally extensive hyperemia of the leptomeninges. Microscopically, the liver lesions comprised multifocal areas of lytic necrosis with intralesional colonies of gram-negative coccobacilli, characterizing a severe, random, multifocal to coalescing necrotizing hepatitis. A moderate multifocal lymphocytic cholangiohepatitis, severe focally extensive mucosal necrosis in the small intestine, and mild multifocal lymphoplasmacytic leptomeningitis in the brain were observed. Two cases of co-infection by Y. enterocolitica and E. coli, and one case by Y. enterocolitica were confirmed through bacterial culture, biochemical characteristics and 16S rRNA. To the best of the author’s knowledge, it is the first report of an infection of Y. enterocolitica and co-infection with E. coli in black-tufted marmosets resulting in diarrhea, septicemia and death.

Ultrasound diagnosis and differential diagnosis of primary hepatic lymphoma

Objective To investigate the diagnostic value of ultrasound in primary hepatic lymphoma (PHL).Methods The ultrasonic and clinical features in 40 cases of PHL were analyzed retrospectively.All cases were pathologically confirmed by surgical operation or ultrasound-guided biopsy.Results The most common presentation of PHL was a solitary lesion which occurred in 57.5％ (23/40) of patients,followed by multiple lesions in 30.0％ (12/40),and diffuse infiltration patterns in 12.5 ％ (5/40).The first two types were usually shown on ultrasound as hypoechoic lesions with surrounding normal parenchyma in 88.6％ (31/35) of cases.The diffuse pattern presented as diffuse hepatomegaly without nodules in the parenchyma.There were 52.5％ (21/40) of the lesions which could not be diagnosed either as benign or malignant by ultrasound.Conclusions The clinical presentations and imaging features of PHL are non-specific.PHL should be considered as a differential diagnosis in cases of metastases when no primary tumour is apparent,or when sonographic findings show atypical features of primary carcinoma or hemangioma.When patients with unexplained fever and diffuse hepatomegaly,PHL should be considered and precaution should be taken to prevent to misdiagnose these patients as having fatty liver or chronic disease. Key words: Ultrasound; Liver; Lymphoma; Diagnosis

Integrative role of MRI in the evaluation of primary biliary cirrhosis

To evaluate magnetic resonance imaging (MRI) findings in patients with primary biliary cirrhosis (PBC) and to determine the value of diffusion-weighted imaging (DWI) in the assessment of liver fibrosis. The following MRI findings were reviewed in 44 patients: periportal T2-weighted hyperintensity, periportal halo sign (T1- and T2-weighted periportal hypointensity), lymphadenopathy, signs of portal hypertension and morphological liver changes. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for b = 800 s/mm(2). Periportal hyperintensity and periportal halo sign were observed in 72.7% and 66.7% of patients, respectively. Lymphadenopathy was noted in 28 patients (63.6%) and diffuse hepatomegaly in 18 (40.9%). Significant positive correlation was observed between histological stage and periportal halo sign (p = 0.613), hepatomegaly (p = 0.443), and portosystemic collaterals (p = 0.391). The mean ADCs (×10(-3) mm(2)/s) were significantly different at stage I versus III and IV, and stage II versus IV. No significant difference was found between stages II and III. For prediction of stage ≥ II and stage ≥ III areas under receiver operating characteristic curves were 0.879 and 0.906, respectively. MRI with DWI could be used as a part of diagnostic protocol in the further evaluation of PBC patients providing noninvasive assessment of liver fibrosis progression. • MRI provides insight into the morphological liver changes in primary biliary cirrhosis (PBC) • The periportal "halo" sign is a highly specific finding in PBC • Diffusion-weighted MR imaging allows noninvasive assessment of liver fibrosis grade.

Primitive Neuroectodermal Tumor of the Liver: A Case Report

Ewing sarcoma/primitive neuroectodermal tumor is a rare tumor of soft tissues of thoraco-pulmonary regions, pelvis and lower extremities. Involvement of visceral organs by primitive neuroectodermal tumor is even rarer, with the kidney being the most commonly involved organ. Involvement of the liver has been reported in the form of metastasis from other primary sources presenting as liver abscess. We report a 20-year-old lady presenting with massive hepatomegaly, with computed tomography scan evidence of diffuse hepatomegaly and a normal porta and intrahepatic biliary radicles. She subsequently underwent ultrasonography-guided true-cut needle biopsy of the liver. Histopathology of the liver revealed nests of small round blue tumor cells in the background of hepatocytes infiltrating the liver, which expressed Mic-2 and Fli-1, and were negative for cytokeratin, desmin, hepatocyte-specific antigen (OCHIE5), synaptophysin, chromogranin A and CD-20. Immunohistochemistry revealed CD-99-positive. Extensive search regarding any possible different site of involvement by the tumor was negative. The patient responded to a combination therapy of vincristine, adriamycin and cyclophosphamide alternating with ifosfamide and etoposide 3 weekly over 43 weeks and has been doing well even after 1 year of diagnosis. The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy.

Tuberculosis congénita: Presentación infrecuente de una enfermedad habitual

Congenital tuberculosis is a severe rapidly progressive disease which differs from neonatal tuberculosis because patients present tuberculous lesions during their first weeks of life, primary hepatic complex or hepatic caseous granulomas, confirmation of tuberculosis in placenta or in maternal genital tract (uterus or adnexa), and exclusion of birth channel or postnatal exposure. We report a case of a 20 days newborn baby admitted to the neonatal unit with fever, hepatoesplenomegaly, abdominal distension and respiratory symptoms. Abdominal echography showed ascitis, diffuse hepatomegaly and splenomegaly, focal hipoecoical spleen images and portal lymphadenopathy. Thorax X-ray with micronodular infiltrates. Maternal pneumonia with pleural effusion was reported during pregnancy. Empirical treatment was initiated with 4 antituberculous drugs: isoniazid, rifampicin, pyrazinamide and ethambutol suspecting tuberculosis. She died the next day. M. tuberculosis was obtained in a gastric lavage culture, blood cultures and post mortem spleen puncture.

Clinical significance of multislice spiral CT scans in hepatic veins occlusion in Budd-Chiari syndrome

Budd-Chiari syndrome with hepatic vein occlusion (HVBCS) can induce severe portal hypertension and liver damage. We retrospectively analyzed hepatic CT features of HVBCS and evaluated the usefulness of triphasic enhancement of CT examinations and CT angiography (CTA) in its diagnosis. Twenty-five cases with HVBCS, confirmed by digital subtraction angiography (DSA), received a triphasic enhancement CT scan within one week before DSA. The CTA images of the relevant blood vessels were reconstructed with maximum intensity projection, volume rendering and oblique reformat techniques. Compared with DSA, the detection rate of transverse CT and CTA images for abnormal hepatic vein were 81.7% (58/71) and 95.8% (68/71) (chi(2) = 7.044, P = 0.008), for membranous obstruction were 47.4% (9/19) and 84.2% (16/19) respectively (chi(2) = 5.729, P = 0.017), for segmental obstruction were 88.0% (22/25) and 100% (25/25) respectively (chi(2) = 1.418, P = 0.234). The detection rates for hepatic vein stenosis were 100% with each method. Diffuse hepatomegaly was found in all 6 cases in acute phase and 3 of 19 cases in chronic phase who had severe obstruction of three hepatic veins without patent intrahepatic collaterals. The other 16 cases in chronic phase had hepatatrophia to different extents related to the obstructed hepatic vein. All in acute phase and 15 in chronic phase presented typical patchy enhancement initially in caudate lobe and perihilar areas and enlarged with time delay. In all cases, parenchyma areas with atrophy, necrosis and congestion demonstrated lower and later enhancement. In all the parts, which had normal enhancement at least one patent outflow hepatic vein, accessory hepatic vein or collateral vessel was detected. Dynamic enhancement CT examination by multislice spiral CT not only could improve the diagnosis of HVBCS by CTA technique, but also could noninvasively provide anatomical information and reveal damage to the hepatic parenchyma.

Primary lymphoma of the liver: clinical and pathological features of 10 patients.

Nine out of 10 patients with primary lymphoma of the liver presented in a manner that did not suggest a tumour. The initial diagnoses were chronic active hepatitis in three cases and "granulomatous cholangitis", inflammatory pseudotumour, and anaplastic carcinoma in one case each. Moreover, extensive haemorrhagic necrosis in three cases initially suggested the Budd-Chiari syndrome. All the tumours were diffuse non-Hodgkin's lymphomas like the 50 cases reported previously, but they differed from most of these in that nine were of T cell phenotype. Five were pleomorphic small T cell, two T zone, and two T lymphoblastic lymphomas: only one was centrocytic and of B cell lineage. This report extends the range of clinical manifestations (diffuse hepatomegaly without a tumour), histological appearances (resemblance to chronic inflammatory or vascular liver diseases) and phenotype (of T cell lineage) of primary lymphoma of the liver: these features seemed to be related in this series. Recognition is important as prognosis remains favourable in appropriately treated cases. Although the appearances of the liver biopsy specimens may be difficult to interpret, the destructiveness of the infiltrate is an important clue to the diagnosis.