Biliary Cystadenoma in a Child: A Rare Entity.

Biliary cystadenoma, a rare potentially malignant hepatic cystic lesion, is characterized by multiloculations and septations. It is common in middle-aged females (about 5% of nonparasitic liver cysts); only 12 cases are described in children. We report a rare case of hepatic biliary cystadenoma in a 3-year-old girl, with a gradually increasing lump in the right upper abdomen. Complete excision with a healthy liver margin was done.

De novo hepatocellular carcinoma 18 years after liver and small bowel transplantation in a one-year-old pediatric patient.

De novo HCC following transplantation in a child is a rare occurrence. Even within the adult liver transplantation population, there are a limited number of published cases. In this report, we present a case of de novo HCC found in a child, post-multivisceral transplantation. A 19-year-old boy, at the age of one, received liver and small bowel transplantation due to short gut syndrome secondary to midgut volvulus and total parenteral nutrition-associated liver disease. Eighteen years later, he was found to have a large mass involving the right hepatic dome consistent with HCC. To the best of our knowledge, this is the second reported case after gut transplantation and the third case post-liver transplantation in the pediatric population.

Non-invasive evaluation of a liver mass in a patient post splenectomy.

INTRODUCTION: Numerous conditions may cause liver lesions, solitary or multiple, benign or malignant. It can be crucial to establish the correct diagnosis. Splenosis is a rare condition that may result from the spillage of cells from the splenic pulp following abdominal trauma, accidental lesions to the spleen during operation or elective splenectomy. These splenic 'implants', which are often multiple, can be located anywhere in the peritoneal cavity, although they are most often found in the left upper quadrant of the abdomen. They may be confused with neoplasms or endometriosis, and may rarely be the cause of small bowel obstruction. CASE PRESENTATION: A 35-year-old man presented with a hepatic mass, and malignancy was suspected. After extensive investigation, it was diagnosed as splenosis using Tc-99m-labelled heat-denaturated red blood cells scintigraphy, without the need for liver biopsy. We consider this the most effective method for diagnosing splenosis. CONCLUSION: When splenosis is suspected, Tc-99m-labelled heat-denaturated red blood cells scintigraphy can be used to confirm the diagnosis, and may avoid invasive investigation.

Impact of image guided fine needle aspiration cytology in diagnosis of pediatric hepatic mass and cytohistologic concordance.

BACKGROUND: Pediatric hepatic mass is a heterogeneous group of benign and malignant lesions. Percutaneous fine needle aspiration cytology (FNAC) can be utilized as a diagnostic modality to assess the nature of radiologically demonstrated hepatic lesions and thus facilitate individualized treatment. AIMS AND OBJECTIVE: The objective of the present study was to determine the diagnostic accuracy of percutaneous FNAC of pediatric liver masses, a procedure that is less invasive than open biopsy. MATERIALS AND METHODS: A prospective, observational study was carried out in the Department of Pathology in collaboration with Department of Pediatric surgery and Radio-diagnosis including 31 pediatric patients presenting over last two years (June 2011 to May 2013) with focal hepatic lesion on ultrasound and computed tomography (CT) scan. FNAC was carried out under image guidance and cytodiagnosis was reached after appropriate staining. By comparing with histopathology reports, diagnostic accuracy of cytology was evaluated. RESULT: Among 31 cases included in the study, 51.6% cases were cytologically benign and hemangioma was the most common benign lesion. Hepatoblastoma was the most accounted malignant tumour (12.9%). FNAC provided 94% sensitivity and 92% specificity in diagnosing benign and malignant tumours. Overall diagnostic accuracy was 93.10%. No significant complication was noted. CONCLUSION: Percutaneous FNAC under image guidance is an effective diagnostic tool for diagnosis of primary and metastatic tumours of liver in pediatric patients.

Augmented environments for the targeting of hepatic lesions during image-guided robotic liver surgery.

BACKGROUND: Stereotactic navigation technology can enhance guidance during surgery and enable the precise reproduction of planned surgical strategies. Currently, specific systems (such as the CAS-One system) are available for instrument guidance in open liver surgery. This study aims to evaluate the implementation of such a system for the targeting of hepatic tumors during robotic liver surgery. MATERIAL AND METHODS: Optical tracking references were attached to one of the robotic instruments and to the robotic endoscopic camera. After instrument and video calibration and patient-to-image registration, a virtual model of the tracked instrument and the available three-dimensional images of the liver were displayed directly within the robotic console, superimposed onto the endoscopic video image. An additional superimposed targeting viewer allowed for the visualization of the target tumor, relative to the tip of the instrument, for an assessment of the distance between the tumor and the tool for the realization of safe resection margins. RESULTS: Two cirrhotic patients underwent robotic navigated atypical hepatic resections for hepatocellular carcinoma. The augmented endoscopic view allowed for the definition of an accurate resection margin around the tumor. The overlay of reconstructed three-dimensional models was also used during parenchymal transection for the identification of vascular and biliary structures. Operative times were 240 min in the first case and 300 min in the second. There were no intraoperative complications. CONCLUSIONS: The da Vinci Surgical System provided an excellent platform for image-guided liver surgery with a stable optic and instrumentation. Robotic image guidance might improve the surgeon's orientation during the operation and increase accuracy in tumor resection. Further developments of this technological combination are needed to deal with organ deformation during surgery.

A case of hepatic reactive lymphoid hyperplasia: the review of 23 cases from the literatures.

A 70-year-old woman was referred to our hospital because of slight elevation of soluble interleukin-2 receptor (sIL-2R) and accumulation of 18F-fluorodeoxyglucose (FDG) in S8 of the liver on positron emission tomography. The mass was strongly suspected to be malignant because of contrast enhancement and enlargement in size of the mass, and suspicion of portal vein invasion. Hepatic S8 subsegmentectomy was performed for diagnostic and therapeutic purposes. Hematoxylin and eosin staining of the resected specimen showed small lymphocytes with no atypia and no formation of lymphoid follicles. Immunostaining showed CD3-positive cells in the interfollicular region and CD20-positive cells in the lymphoid follicles. Both CD10 and BCL-2 were negative in the follicular germinal center. CD138-positive plasma cells were observed and there was no light chain restriction. Based on polyclonal growth pattern of lymphocytes in the lymphoid follicles and interfollicular region, she was diagnosed with hepatic reactive lymphoid hyperplasia (RLH).Review of the English literature of hepatic RLH which referred to imaging findings yielded 23 cases, including this case. As a result, we suggest that liver biopsy should be performed for definitive diagnosis, when hepatic RLH is suspected by imaging findings and backgrounds.

Pheochromocytoma in an Ectopic Adrenal Gland.

A pheochromocytoma is an uncommon tumor that originates from the chromaffin cells of the adrenal medulla. Also, adrenal tissue not located in its typical position is referred to as ectopic adrenal tissue. It is relatively uncommon in adults and is usually asymptomatic. Therefore, a pheochromocytoma arising from ectopic adrenal tissue is even a rarer finding and presents as a unique diagnostic challenge. A 20-year-old man presented with vague abdominal pain, and upon imaging, a mass located behind the liver was initially discovered. Subsequently, it was identified as a mass growing in an ectopic adrenal gland. He underwent exploratory laparotomy and resection of the mass. A pheochromocytoma in an ectopic adrenal gland was confirmed by histopathology.

Spontaneous Biloma With a Hepatic Mass.

We report a case of an 86-year-old Hispanic male who presented with generalized itching and jaundice. Computed tomography (CT) imaging revealed a hepatic mass and an extensive spontaneous biloma, a condition rarely associated with malignancy. Subsequent biopsy of the mass confirmed moderately differentiated adenocarcinoma of the pancreaticobiliary tract. The patient underwent successful percutaneous drainage of the biloma and was discharged with a plan for further outpatient management. This case study highlights a rare manifestation of spontaneous biloma related to malignancy, broadening the clinical understanding of its association with malignancy, diagnosis, and management.

A Primary Extracutaneous Presentation of Merkel Cell Carcinoma.

Merkel cell carcinoma (MCC) is a rare aggressive cutaneous neuroendocrine malignancy with a mortality rate of around 33%. The presence of advanced disease at the time of diagnosis is associated with poor prognosis. Twofold etiologies have been described in the pathogenesis of Merkel cell carcinoma: chronic exposure to ultraviolet (UV) light and Merkel cell polyomavirus (MCPvY). MCC usually affects sun-exposed skin areas, and the presence of cutaneous nodules is the hallmark of the disease. However, there have been case reports in the literature where the diagnosis of MCC was made in the absence of any cutaneous findings. We present a case report of Merkel cell carcinoma that is unique in its presentation because of the presence of pulmonary and hepatic nodules and the absence of cutaneous lesions.

Ovarian growing teratoma syndrome with multiple metastases in the abdominal cavity and liver: A case report.

BACKGROUND: Growing teratoma syndrome (GTS) is an unusual presentation of an amazing transformation of teratoma from malignant to benign on pathology during or after systemic or intraperitoneal chemotherapy. The definitive pathogenesis is still not fully understood due to the lack of large-sample studies. CASE SUMMARY: A 53-year-old woman underwent radical surgery and postoperative intraperitoneal chemotherapy due to immature teratoma of the right ovary at the age of 28. She remained well during a 25-year follow-up period after surgery. Multiple asymptomatic solid masses were found in the liver on ultrasonography a month ago. Enhanced computed tomography (CT) of the abdomen revealed multiple masses in the abdominal cavity. The largest one was located in the posterior peritoneum next to the sixth segment of the right liver, about 7.9 cm × 7.5 cm in size. Three masses were present inside the liver, and one mass was in the right pelvic floor. Multiple lumps in the abdominal cavity were completely removed by surgery. During the operation, multiple space-occupying lesions were seen, ranging in size from 0.5 to 3 cm, and grayish white in color and hard in texture. Ovarian GTS was finally diagnosed based on postoperative pathology. After surgery, she recovered uneventfully. During a 3-year follow-up, the patient remained free of the disease without any recurrence on CT scan. CONCLUSION: GTS is a rare phenomenon characterized by conversion of immature teratoma to mature one during or after chemotherapy and presents as growing and metastasizing masses. The pathogenesis of GTS is unclear, and the prognosis is good after surgical resection.

Lymphoplasmacytic lymphoma with IgG-κ paraproteinemia presenting as a hepatic bulky mass.

Lymphoplasmacytic lymphoma (LPL) usually involves bone marrow (BM) and sometimes lymph nodes and spleen. LPL presenting as a hepatic mass lesion is extremely rare, with only one case reported in the English literature. A 70-year-old Japanese female presented to us with a right hypochondriac mass with tenderness. Computed tomography (CT) revealed a 14 cm-sized bulky hepatic mass. Laboratory findings showed a normal white blood cell count of 4.1×109/L with 4% plasmacytoid lymphocytes; normocytic anemia, Hb 9.4 g/dL; high soluble IL-2 receptor level, 2,290 U/mL; and elevated IgG, 10,306 mg/dL. Furthermore, IgG-κ monoclonal protein was detected. 18F-fluorodeoxyglucose-positron emission tomography/CT revealed abnormal uptake in the liver mass; left supraclavicular, parasternal, abdominal, and left inguinal lymph nodes; and bilateral lung bases. Magnetic resonance imaging showed no bone lesions. BM aspiration and liver biopsy showed predominant infiltration of small lymphocytes admixed with plasmacytoid lymphocytes and plasma cells. In the liver specimen, lymphoepithelial lesions were not observed. The small lymphocytes were positive for CD20, CD79a, and bcl-2, and negative for CD5, CD10, cyclin D1, and IRTA1; plasma cells in BM were positive for CD19, CD45, IgG, and κ-chain, and negative for CD20, and CD56. MYD88 L265P mutation, reported in approximately 40% of non-IgM LPL cases, was not detected in the liver specimen and BM cells. The frequency is lower than that of typical IgM LPL. These findings led us to a diagnosis of LPL with IgG-κ paraproteinemia. The patient underwent four courses of R-CHOP and two courses of Bendamustine-R. Partial remission was achieved.

Primary Hepatic Neuroendocrine Tumor: A Case Report and Literature Review.

Primary hepatic neuroendocrine tumors (PHNETs) are an utterly rare subtype of neuroendocrine tumors (NETs) that arise from cells of the neuroendocrine system. Due to the rarity and lack of distinctive radiological features, diagnosis and management of this tumor are challenging. Herein, we report a case of PHNET in a 19-year-old previously healthy female patient whose diagnosis was confirmed by histopathology and immunohistochemistry. This case emphasizes the importance of considering PHNETs in the differential diagnosis of a hepatic mass, management of patients with this disease, and post-operative follow-up.

Case Report: Transcatheter Arterial Embolization for the Initial Management of Intra-Abdominal Hemorrhage From a Hepatic Tumor in a Cat.

An 8-year-old Ragdoll cat was admitted to our hospital after its owner noticed sudden lethargy. Abdominal ultrasonography showed a large amount of blood in the abdominal cavity, and the cat was diagnosed as having hemorrhagic shock caused by the rupture of an intra-abdominal mass. Blood transfusion was performed on the 1st day of hospitalization. On the 2nd day, contrast-enhanced computed tomography (CT) was performed, and hemorrhage from a mass originating in the caudate lobe of the liver was noted. Transcatheter arterial embolization (TAE) was performed to stop the bleeding from the mass using Gelpart to embolize the feeding artery. The following day, fever and elevation of liver enzyme levels were observed, but these subsided within a few days. At discharge 5 days after TAE, no fluid was found in the peritoneal cavity, and no further intra-abdominal bleeding occurred. Sixty-six days after TAE, we were able to perform resection surgery with the cat in good condition. A partial response was observed on CT performed before surgery. Histopathology revealed cholangiocellular adenoma. The cat was doing well as of postoperative day 549. This case indicates that TAE may be effective for initial hemostasis and stabilization of conditions in animals with tumor-induced hemorrhage.

Hepatic Hodgkin Lymphoma Presenting as Solitary Hepatic Mass Following Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorder in a Patient With Rheumatoid Arthritis.

Lymphoproliferative disorders (LPDs) occur frequently in patients with rheumatoid arthritis (RA) under methotrexate treatment. Some LPDs spontaneously regressed after methotrexate discontinuation, but classic Hodgkin lymphoma (CHL)-type LPDs frequently relapse, and chemotherapy is usually required for the treatment. CHL usually spreads in contiguous lymph nodes and then infiltrates in organs at an advanced stage. Thus, hepatic Hodgkin lymphoma (HHL) without lymphadenopathy is extremely rare at diagnosis. We present a case of methotrexate-associated LPDs associated with systemic lymphadenopathy and hepatosplenic mass in a 71-year-old woman with RA under methotrexate treatment over 10 years. Although spontaneous remission occurred after methotrexate discontinuation, she developed HHL presenting as a solitary hepatic mass without lymphadenopathy 3 years after spontaneous regression. She received brentuximab vedotin (BV) combination chemotherapy without bleomycin to avoid pulmonary toxicity. Complete metabolic response was achieved after four courses of BV combination chemotherapy, and the activity of RA was kept to be in remission. Our case suggested that the recurrence lesions of LPDs may present at unexpected site, which is not coincide with the primary site, and BV combination chemotherapy is a promising regimen for limited-stage CHL-type LPDs in patients with RA owing to its anti-lymphoma effect on CHL-type LPDs and a possible targeted therapy for RA.

Differential Diagnosis of Hepatic Mass with Central Scar: Focal Nodular Hyperplasia Mimicking Fibrolamellar Hepatocellular Carcinoma.

Fibrolamellar hepatocellular carcinoma is a primary hepatic tumor that usually appears in young adults. Radical surgery is considered curative for this kind of tumor, so early diagnosis becomes essential for the prognosis of the patients. The main characteristic of this entity is the central scar, which is the center of differential diagnosis. We report the case of a 30-year-old man who was diagnosed with fibrolamellar hepatocellular carcinoma by ultrasonography. Contrast-enhanced CT confirmed this diagnosis, and the patient underwent a [18F] fluorocholine PET/CT. Hypermetabolism and the morphology in the nuclear medicine exploration suggest neoplastic nature of the lesion. Radical surgery was performed, and histopathologic analysis was performed, which resulted in focal nodular hyperplasia. Hepatic masses with central scar could have a difficult differential diagnosis, and focal nodular hyperplasia could mimic fibrolamellar hepatocellular carcinoma imaging patterns. These morphofunctional characteristics have not been described in [18F] Fluorocholine PET/CT, so there is a need to find out the potential role PET/CT in the differential diagnosis of hepatic mass with central scar.

A Leiomyosarcoma of Inferior Vena Cava Presenting as a Liver Metastasis Mass in a Patient with History of Transitional Cell Carcinoma.

The most probable diagnosis for a newly detected mass in the cancer patients is secondary metastasis. However, the multiple primary tumors should not be off the table of diagnoses. In this study, a 70-year-old man with the history of transitional cell carcinoma (TCC) was reported who had been referred due to a newly detected mass in the hepatic segment one which adhered to the inferior vena cava (IVC). Although the most probable diagnosis according to the patient's medical history was secondary metastasis, the biopsy revealed a leiomyosarcoma (LMS) tumor. Therefore, a mass biopsy can be determinative for confirming the diagnosis and further management of cancer patients with a newly detected mass.