Laparoscopic cholecystectomy: Histopathological analysis of metabolic associated fatty liver disease and fibrosis.

INTRODUCTION: Metabolic (dysfunction) associated fatty liver disease (MAFLD) and cholelithiasis are highly prevalent and are associated with common risk factors such as obesity, hypertriglyceridemia, and fasting glucose levels; however, it is not clear whether cholelithiasis is associated with MAFLD or fibrosis. OBJECTIVE: To determine MAFLD severity and associated risk factors in patients diagnosed with cholelithiasis. MATERIALS AND METHODS: Observational, cross-sectional and prolective study (from October 2018 to March 2020) of patients undergoing elective laparoscopic cholecystectomy with liver biopsy, excluding other causes of hepatic disease or significant alcohol consumption. MAFLD detection was based on histology using the Kleiner score and one of the following criteria: overweight/obesity, T2DM, or evidence of metabolic dysregulation. The AST to Platelet Ratio Index, the NAFLD Fibrosis Score, the fibrosis-4 index and the hepatic steatosis index were performed to assess the relationship of non-invasive hepatic scores with histopathology. RESULTS: 80 patients median age (interquartile range) was 42 (18) years, with a BMI of 27.9 (6.11) Kg/m2. Of all patients, 58.8% had MAFLD, 78.7% were women, and 13.8% had the severe form (formerly named NASH). No substantial correlation between biochemical parameters and histopathological analysis of MAFLD and fibrosis was observed. CONCLUSION: Because cholelithiasis and MAFLD are highly prevalent diseases, it is essential to conduct studies on the relationship between both pathologies. Currently, liver biopsy is the best diagnostic method since the predictive biochemical models did not show a substantial correlation to classify MAFLD. Its early detection is relevant since a considerable percentage of advanced fibrosis (8.7%) was found.

Acute Cardiac Tamponade Secondary to Cardiac Vascular-Type Pleomorphic Leiomyosarcoma: Case Report.

Soft tissue sarcomas represent <1% of all neoplasms. Leiomyosarcomas comprise only 5-7% of cases, and only 2% of these are vascular. Vascular leiomyosarcomas are extremely rare and represent only 0.001% of all neoplasms, the venous type being up to 5 times more frequent. Arterial leiomyosarcomas most frequently affect the great vessels, being fatal in most cases. In the reported cases of arterial leiomyosarcomas, the most frequently affected site is the pulmonary artery. We present the clinical case of 2 patients (a 42-year-old woman and a 36-year-old man) with a diagnosis of arterial pleomorphic leiomyosarcoma that conditioned cardiac tamponade as the initial manifestation. As it is an exceptionally rare neoplasm and with few cases reported in the literature, it is important to identify and describe this pathology which, due to the impossibility of offering surgical treatment, represents a therapeutic challenge.

Rapid on-site cytologic evaluation during endoscopic ultrasound-guided biopsies of pancreatic solid lesions.

BACKGROUND: Endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) is safe and effective for the diagnosis of pancreatic adenocarcinoma. Although rapid on-site evaluation (ROSE) can improve tissue collection and increase diagnostic yield, its utility has been recently questioned. OBJECTIVE: Determine the diagnostic efficacy of EUS-FNB with ROSE of pancreatic masses in a new echoendoscopy unit. METHOD: Cross-sectional and comparative study of patients who underwent EUS-FNB of pancreatic masses between January and July 2017. Patient demographics, ultrasonographic details and pathology reports were examined. RESULTS: A total of 23 procedures were analyzed. Median age was 59 years (range: 46-77). The group with ROSE had 13 patients (56.5%) and the group without ROSE 10 (43.5%). The final pathology report showed enough and adequate sample in 100% of the group with ROSE (13/13 vs. 5/10; p = 0.007). Diagnosis of malignancy was established in 84.6% of the biopsies (11/13 vs. 2/10; p = 0.003) in the group with ROSE. CONCLUSIONS: ROSE is useful to improve the diagnostic efficacy of EUS-FNB of pancreatic masses, especially in new EUS centers or in centers with a low diagnostic yield.

ANTECEDENTES: La biopsia con aguja fina guiada por ultrasonido endoscópico (BAF-USE) es segura y eficaz para el diagnóstico del adenocarcinoma pancreático. La revisión citológica rápida en sala (ROSE, rapid on-site evaluation) puede mejorar la calidad de la muestra y el rendimiento diagnóstico; en años recientes se ha cuestionado su utilidad. OBJETIVO: Determinar la eficacia diagnóstica de la ROSE durante las BAF-USE de lesiones pancreáticas sólidas en un nuevo centro de ecoendoscopia. MÉTODO: Estudio transversal y comparativo en el que se incluyeron todos los pacientes a quienes se realizó BAF-USE de lesiones pancreáticas sólidas entre enero y julio de 2017. Se evaluaron datos demográficos, ecográficos y de patología de las BAF-USE. RESULTADOS: Se analizaron 23 procedimientos. La mediana de edad fue de 59 años (rango: 46-77). En el grupo con ROSE hubo 13 pacientes (56.5%) y en el grupo sin ROSE hubo 10 (43.5%). En el grupo con ROSE, el reporte de patología mostró muestra adecuada en el 100% (13/13 vs. 5/10; p = 0.007), así como diagnóstico de malignidad en el 84.6% (11/13 vs. 2/10; p = 0.003). CONCLUSIONES: La ROSE es una herramienta útil para mejorar el diagnóstico de BAF-USE de lesiones pancreáticas sólidas, principalmente cuando hay bajo rendimiento diagnóstico y en nuevas unidades de ecoendoscopia.

Prevalence, Risk Factors, and Survival of Patients with Intrahepatic Cholangiocarcinoma.

PURPOSE: To investigate the prevalence, related risk factors, and survival of intrahepatic cholangiocarcinoma in a Mexican population. MATERIAL AND METHODS: We conducted a cross-sectional study at Medica Sur Hospital in Mexico City with approval of the local research ethics committee. We found cases by reviewing all clinical records of in-patients between October 2005 and January 2016 who had been diagnosed with malignant liver tumors. Clinical characteristics and comorbidities were obtained to evaluate the probable risk factors and the Charlson index. The cases were staged based on the TNM staging system for bile duct tumors used by the American Joint Committee on Cancer and median patient survival rates were calculated using the Kaplan-Meier method. RESULTS: We reviewed 233 cases of hepatic cancer. Amongst these, hepatocellular carcinomas represented 19.3% (n = 45), followed by intrahepatic cholangiocarcinomas, which accounted for 7.7% (n = 18). The median age of patients with intrahepatic cholangiocarcinoma was 63 years, and most of them presented with cholestasis and intrahepatic biliary ductal dilation. Unfortunately, 89% (n = 16) of them were in an advanced stage and 80% had multicentric tumors. Median survival was 286 days among patients with advanced stage tumors (25th-75th interquartile range, 174-645 days). No correlation was found between the presence of comorbidities defined by the Charlson index, and survival. We evaluated the presence of definite and probable risk factors for the development of intrahepatic cholangiocarcinoma, that is, smoking, alcohol consumption, and primary sclerosing cholangitis. DISCUSSION: We found an overall prevalence of intrahepatic cholangiocarcinoma of 7.7%; unfortunately, these patients were diagnosed at advanced stages. Smoking and primary sclerosing cholangitis were the positive risk factors for its development in this population.

Cystic angiosarcoma of the liver. A previously undescribed neoplasm.

 We report an example of a cystic hepatic angiosarcoma that to our knowledge has not been previously described. The patient was a 70 year old woman who was admitted to the emergency room because of hypovolemic shock. A computed tomography showed four heterogeneous hepatic cystic masses varying from 2.5 to 11.2 cm; one of these with rupture and formation of a subcapsular hematoma. The cyst wall was lined by several layers of neoplastic epithelioid and spindle shaped endothelial cells that in some areas extended to the underlying stroma. They expressed CD31 and CD34, and were negative for cytokeratin. The patient is alive with residual hepatic cystic angiosarcoma. However, follow up is too short to be significant.

Phosphaturic mesenchymal tumors. Survey of 8 cases from a single Mexican medical institution.

Phosphaturic mesenchymal tumor (PMT) is a morphologically heterogeneous soft tissue and bone neoplasm, producing a paraneoplastic syndrome due to phosphate wasting. These tumors produce fibroblast growth factor 23, which is implicated in renal tubule phosphate loss. Medical records of patients seen from 1999 to 2013 with osteomalacia associated or not with a tumor were reviewed. Clinical and laboratory data, radiographic studies, and follow-up of 8 patients were tabulated. Histologic features and the immunoprofile of the tumors were analyzed. There were 208 patients with osteomalacia, but only 8 (3.84%) had osteomalacia associated with a tumor. The median age of the patients was 40 years. The tumor size ranged from 1.5 to 4 cm. Five were located in soft tissues and skin; and 3, in bones. Osteomalacia symptoms lasted from 2 to 14 years with a median of 6 years. Laboratory data showed hypophosphatemia and phosphaturia in all patients. All tumors were histologically benign. Histologically, the salient features were a hemangiopericytoid pattern, chronic hemorrhage, and microcystic areas. All neoplasms were diffusely positive for vimentin and focally positive for epithelial membrane antigen, CD34, and S-100 protein. Ki-67 was positive in approximately 10% of neoplastic cells in 2 cases and less than 1% in the remainder. We report 8 cases of PMTs producing osteomalacia, from a single third-level Mexican medical institution. These tumors occurred in soft tissues, skin, and bones. All tumors were benign, small, not easily detected by physical examination and diagnosed due to the metabolic abnormalities.

The nuclear receptor FXR, but not LXR, up-regulates bile acid transporter expression in non-alcoholic fatty liver disease.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease. Patients with non-alcoholic steatohepatitis (NASH) have increased plasmatic and hepatic concentrations of bile acids (BA), suggesting that they can be associated with the progression of the disease. Hepatic nuclear receptors are known to modulate genes controlling BA metabolism; thus, in this work we aimed to compare the expression of liver nuclear receptors -farnesoid X (FXR), small heterodimer partner (SHP) and liver X alpha (LXRα) receptors- and BA transporters -sodium+/taurocholate cotransporting polypeptide (NTCP) and bile salt export pump (BSEP)- in liver biopsy samples of patients with simple steatosis (SS) and NASH. MATERIAL AND METHODS: Forty patients with biopsy-proven NALFD were enrolled between 2009 and 2012; liver biopsies were classified as SS (N = 20) or NASH (N = 20) according to the NAFLD activity score. Gene expression of nuclear FXR, LXRα, SHP, NTCP and BSEP was analyzed by real-time reverse transcription polymerase chain reaction and protein level was quantified by western blot. RESULTS: Gene expression of FXR, SHP, NTCP and BSEP was significantly up-regulated in the NASH group in comparison with SS patients (P < 0.05). In contrast, protein level for FXR, SHP and NTCP was decreased in the NASH patients vs. the SS group (P < 0.05). Gene and protein profile of LXRα did not show differences between groups. CONCLUSIONS: The results suggest that liver nuclear receptors (FXR and SHP) and BA transporters (NTCP and BSEP) are associated with the progression of NAFLD.

Papillary in situ and intramucosal adenocarcinoma of the lower third of common bile duct. A report and review of the literature.

We report the case of a 37-year-old woman with no relevant medical history. She was admitted to the hospital for epigastric pain related with food intake for 4 days; the pain did not improve with symptomatic management. A laparoscopic cholecystectomy due to acute lithiasic cholecystitis was performed. However, after 4 days, postoperative painless jaundice was evident; thus, endoscopic retrograde cholangiopancreatography was performed, which revealed an amputation of intrapancreatic common bile duct, as well as secondary intra- and extrahepatic bile duct dilatation. Brushing of the distal portion of the common bile duct revealed a well-differentiated adenocarcinoma. Therefore, a Whipple procedure with pylorus preservation was performed. Pathologic diagnosis of a papillary in situ adenocarcinoma with two microscopic foci of microinvasion was established. The pathologic Tumor-Node-Metastasis (TNM) stage was pT1, pN0, pM0, R0. The patient is asymptomatic and disease-free 24 months after surgery. In general, adenocarcinomas of the extrahepatic bile ducts are uncommon and have a poor prognosis. However, symptomatic patients with early disease stages are even rarer and can be cured surgically.

Cystadenomas of the liver and extrahepatic bile ducts: Morphologic and immunohistochemical characterization of the biliary and intestinal variants.

Cystadenomas of the liver and extrahepatic bile ducts (EHBD) are uncommon but distinctive neoplasms whose terminology and epithelial phenotype have been a source of controversy. We reviewed 20 cases, 16 arising in the liver and 4 in the EHBD. Eighteen patients were women, with a mean age of 36.5 years. Eighteen tumors were multiloculated and 2 were unilocular. The tumor size ranged from 4 to 29 cm (average, 11 cm). The cyst fluid in 13 tumors was described as serous, in 2 as clear, in 2 others as hemorrhagic, and in 1 as serous and mucinous. Only in 2 tumors was the fluid described as mucinous. In 18 cystadenomas, the predominant epithelial lining consisted of a single layer of cuboidal or low-columnar nondysplastic cells similar to those of the gallbladder or bile ducts. This epithelial lining was strongly positive for cytokeratins 7 and 19, and focally positive for MUC1. Only 2 cystadenomas showed predominant intestinal differentiation characterized by mature goblet cells and columnar absorptive cells. These cells expressed CDX2, MUC2, and cytokeratin 20. Admixed with the goblet and columnar cells, there were serotonin-containing cells and Paneth cells. These 2 tumors showed extensive areas of high-grade dysplasia and invasive adenocarcinoma with intestinal phenotype. A subepithelial ovarian-like stroma was present in all tumors. None of the patients died of the tumors. We believe that the term mucinous cystic tumor recommended by the World Health Organization for all cystadenomas of the liver and EHBD is a misnomer.

Bone metastases as the initial presentation of hepatocellular carcinoma. Two case reports and a literature review.

Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver and is the fifth most common cancer in the world; its incidence has been increasing in recent years. Extrahepatic spread is present at the time of diagnosis in only about 5 to 15% of patients. Skeletal metastasis of HCC occurs less frequently compared with other cancers and is considered a rare primary form of presentation. We report two cases of unsuspected HCC presenting with multiple bone lesions as the initial presentation. The first patient was a 76-year-old man with symptoms of fatigue and back pain. The PET-CT revealed the hypercaptant bone lesions and a liver lesion. The pathology report showed that the metastases were positive for the hepatic marker HEPAR-1, indicating that they had originated from the HCC. The second patient was a 56-year-old man. He presented to the emergency department for right shoulder pain and weakness of the entire right arm with no history of trauma. During hospitalization, the patient became quadriplegic. MRI revealed osseous blastic lesions in the cervical vertebrae and right shoulder. A CT-guided biopsy was performed in the cervical lesion and showed poorly differentiated carcinoma. Immunohistochemistry staining was positive for HEPAR-1. In conclusion, this cases show an unusual presentation of HCC with skeletal metastasis.

Association of urothelial carcinoma of the renal pelvis with papillary and medullary thyroid carcinomas. A new sporadic neoplastic syndrome?

We describe 2 adult women (72 and 54 years), 1 with a low-grade noninvasive papillary urothelial carcinoma of the renal pelvis, who 14 years later developed a papillary carcinoma in 1 thyroid lobe and a medullary carcinoma in the contralateral lobe. Both neoplasms were similar in size and appeared symmetrical. Despite its small size, the medullary carcinoma metastasized in multiple cervical lymph nodes. The second patient had a high-grade invasive papillary urothelial carcinoma of the renal pelvis that infiltrated the renal parenchyma and metastasized in one of the lungs. Five months later, a papillary carcinoma was discovered in the thyroid gland. The 2 papillary thyroid carcinomas were of the follicular variant. Adjacent to 1 papillary carcinoma, there was a dominant nodule of a colloid and adenomatous goiter. The medullary carcinoma contained stromal amyloid and was immunoreactive for calcitonin and carcinoembryonic antigen. There was no C-cell hyperplasia (medullary carcinoma in situ). The 2 patients are alive, 1 is living with pulmonary metastasis from the high-grade urothelial carcinoma. Twelve cases of this neoplastic association were registered in the Survey, Epidemiology, and End Results Program from 1980 to 2009. We believe that the combination of these unusual neoplasms in the same patient may represent a new sporadic neoplastic syndrome.

Endometrial stromal sarcomas: immunoprofile with emphasis on HMB45 reactivity.

OBJECTIVES: We describe the morphologic and immunohistochemical features of 17 endometrial stromal neoplasms, 16 sarcomas, and one stromal nodule. METHODS: We reviewed 35 cases interpreted as endometrial stromal neoplasms, but 17 high-grade endometrial stromal sarcomas (ESS) and one case of mixed endometrial sarcoma and leiomyosarcoma were excluded from the study. Data from the Surveillance Epidemiology and End Results program on low- and high-grade ESS for 1973 through 2003 were obtained. RESULTS: One uterine primary ESS had collections of clear cells (20%), while a metastatic ESS contained predominantly clear cells (90%). CD10 (88.2%) and smooth muscle actin (70.5%) were the most common positive immunohistochemical markers. The latter marker was located in the cytoplasm in 47% of the ESS and in the nucleus in 23.5%, a previously unreported feature. HMB45 was detected in 23.5% of the ESS, which contrasts with the 2% reported by other authors. CONCLUSIONS: The presence of clear cells and HMB45 reactivity does not justify the term perivascular epithelioid cell tumors for these neoplasms. Two of 17 patients with ESS died of metastatic disease. However, among 274 cases of ESS (all stages included) collected by the Surveillance Epidemiology and End Results Program of the National Cancer Institute during a 30-year period, the 10-year survival rate was 94%.

IgG4-Related Autoimmune Prostatitis: Is It an Unusual or Underdiagnosed Manifestation of IgG4-Related Disease?

IgG4-related disease (IgG4-RD) encompasses a wide range of extrapancreatic manifestations. Albeit some are relatively well known, others such as autoimmune prostatitis remain poorly described. We present a 61-year-old Latin-American male with autoimmune pancreatitis (AIP) who presented with lower urinary tract symptoms (LUTS), normal prostate specific antigen (PSA) test, and prostate enlargement attributed to benign prostatic hyperplasia (BPH). He underwent a transurethral resection of the prostate (TURP) after which symptoms were resolved. On histopathology, prostatic stroma had a dense inflammatory infiltrate rich in plasma cells and lymphocytes; immunohistochemical morphometric assessment showed >10 IgG4-positive plasma cells/high power field (HPF). The diagnosis of IgG4-related prostatitis was postoperatively. We compared the patient characteristics with those of previous reports on Asian patients. Shared findings included prostate enlargement, LUTS (symptoms that can be confused with BPH), and PSA within normal limits or mild elevations. IgG4-related prostatitis is rarely considered as a preprocedural diagnosis, even in patients with evidence of IgG4-RD. Involved prostate zones include mainly central and transitional zones and less frequently the peripheral. Currently, there is insufficient data about the natural history and outcome. Whether steroids, transurethral resection, or both are the treatment of choice needs to be elucidated.

Hepatolithiasis with secondary cholangitis and supernumerary left hepatic lobe.

We report the case of a 31-year old woman with recurrent cholangitis secondary to hepatolithiasis. The stones were composed of calcium bilirubinate. The patient also had a supernumerary hepatic lobe connected to the inferior aspect of the segment III of the liver. The role of the supernumerary hepatic lobe in the development of hepatolithiasis is unclear and may be coincidental.

Rokitansky-Aschoff sinuses mimicking adenocarcinoma of the gallbladder: a study of 8 cases.

We describe 8 cases of cholecystectomy specimens (6 laparoscopic and 2 open cholecystectomies) with Rokitansky-Aschoff (R-A) sinuses that were misinterpreted as adenocarcinomas. They were compared with 8 examples of classical R-A sinuses and 6 cases of R-A sinuses containing foci of adenocarcinoma. Five cases misinterpreted as adenocarcinomas consisted of densely packed, closely opposed R-A sinuses with little intervening stroma or surrounded by a desmoplastic stroma. They were lined by a single layer of cuboidal or columnar cells. There were also pseudostratified columnar cells with mucin-containing cytoplasm and hyperchromatic or vesicular nuclei but without mitotic figures. In 2 cases, the columnar cells had subnuclear vacuoles. Small papillary projections into R-A sinuses were seen in 4 cases, and in 3 others collections of metaplastic pyloric glands, some connected to the epithelium of the sinuses, were recognized. There was focal reactive atypia in both the epithelium of the surface and that of the sinuses. The R-A sinuses resembling gland-like structures had a laminar distribution rather than a disorderly haphazard distribution seen in well-differentiated adenocarcinoma. The remaining 3 cases misinterpreted as adenocarcinomas consisted of numerous deeply penetrating long and short R-A sinuses that branched in different directions and which reach the subserosal or perimuscular connective tissue mimicking invasion. The sinuses were surrounded by hyperplastic smooth muscle bundles and lined by pseudostratified columnar cells mixed with a few goblet cells showing reactive atypia and no mitotic figures. There was focal reactive atypia in both the epithelium of the surface and that of the sinuses. The 2 types of R-A sinuses did not label with carcinoembryonic antigen or p53 and had very low proliferative activity as measured by the MIB1-labeling index. All patients are alive and disease free from 8 months to 17 years (mean follow-up 7 y). In contrast, the foci of invasive adenocarcinoma that arose in R-A sinuses consisted of glands lined by atypical cuboidal or columnar cells with loss of polarity, large hyperchromatic or vesicular nuclei, prominent nucleoli, and mitotic figures, quite different from the cells lining the R-A sinuses. Because of increasing number of laparoscopic cholecystectomies performed annually in the United States, pathologists should become familiar with these gallbladder lesions that are usually incidental findings but can simulate malignant epithelial neoplasms.

Nonmucinous cystadenomas of the pancreas with pancreatobiliary phenotype and ovarian-like stroma.

Among 31 benign cystic neoplasms of the pancreas diagnosed as mucinous cystadenomas, we identified 9 (29%) cases of nonmucinous cystadenomas with a pancreatobiliary phenotype and an ovarian-like stroma. Although both cystic tumors belong to the same family, they should be separated because their epithelial lining and cyst fluid are different. The lining cells of the nonmucinous cystadenomas consisted of a single layer of cuboidal cells, similar to the epithelial cells of the normal pancreatic ducts, and were not dysplastic (90%-100% of the lining cells). The cyst fluid was described as serous or clear. The remaining 22 classical mucinous cystadenomas, lined predominantly by mucinous and foveolar epithelium, revealed focal pancreatobiliary epithelium in 86% of the cases, and 6 pancreatic invasive mucinous cystadenocarcinomas failed to show pancreatobiliary differentiation. We believe that these nonmucinous cystadenomas of the pancreas represent a distinctive subset of cystic neoplasms of the pancreas that probably have no malignant potential.

Mucinous cystadenoma of the pyelocaliceal system: a report of 3 examples and an analysis of 17 previously published cases.

We report 3 patients all men between 45 and 64 years of age with unilocular or multilocular mucinous cystadenomas of the kidney. One tumor arose from the renal pelvis, and 2 involved the entire pyelocaliceal system. The tumors measured between 2.4 and 37 cm in greatest dimension. Two patients were asymptomatic, and 1 had recurrent attack of acute pyelonephritis. Microscopically, the morphology and immunophenotype (CK20, MUC2, and CDX2 positive) of the tumors were similar to the colonic adenomas. Two patients were asymptomatic 24 and 64 months after surgery, including the patient with mucinous cystadenoma and intramucosal carcinoma. One patient died of acute myocardial infarction, and his tumor was an autopsy finding. Only 17 cases of mucinous cystadenomas and 5 cases of mucinous cystadenocarcinomas have been reported. Of the 17 mucinous cystadenomas, 2 arose in horseshoe kidneys. The mean size of these neoplasms was 15 cm (2.4-37 cm). Despite their large size, some patients with mucinous cystadenomas were asymptomatic. Sixty percent were associated with renal lithiasis. Thirty percent progressed to mucinous adenocarcinomas, and only 2 cases showed areas of intramucosal carcinomas. Two cases were associated with carcinoid tumors, similar to those reported in the appendix. Most patients were asymptomatic after surgery, and only 1 patient died by abdominal sepsis related to adenomucinosis. The 3 examples of mucinous cystadenomas of the pyelocaliceal system reported here, and those previously published indicate that they are very uncommon neoplasms with morphology and intestinal immunophenotype similar to the colonic adenomas.

Granulomatous hepatitis caused by Q fever: a differential diagnosis of fever of unknown origin.

The differential diagnosis of fever of unknown origin (FUO) includes infectious, neoplastic, rheumaticinflammatory and miscellaneous diseases. We report the case of a 35-year-old man with FUO caused by Q fever. A liver biopsy showed the characteristic fibrin-ring lipogranulomas compatible with Q fever. The serologic tests confirmed the diagnosis of acute infection by Coxiella burnetii. The therapeutic response was excellent. In conclusion, we described a patient with acute Q fever and granulomatous hepatitis.