Right hepatectomy for giant cavernous hemangioma with diffuse hemangiomatosis around Glisson’s capsule

Diffuse liver hemangiomatosis with giant cavernous hemangioma in adult is extremely rare. A 35 year-old woman presented to hospital with main complaint of epigastric pain and abdominal fullness. An enhanced computed tomography scan revealed a massive liver tumor in right lobe about 150 mm in size. There was contrast enhancement at the periphery of the mass consistent with a cavernous hemangioma. She underwent right hepatectomy. Histologically, it was diagnosed as a cavernous hemangioma. And also, hemangiomatous lesions were scattered around the Glisson’s capsule on the back ground liver. These hemangiomatous lesions were not recognized preoperatively. Even if we couldn’t diagnose hemangiomatosis around the main giant hemangioma preoperatively, we need to take enough surgical margins because the giant hemangioma has the potential to have small hemangiomatous lesions around the tumor. We reported right hepatectomy for giant cavernous hemangioma with diffuse hepatic hemangiomatosis without an extrahepatic lesion in an adult.     

Arteriovenous shunting and cholestasis in hepatic hemangiomatosis associated with metoclopramide

Diffuse hemangiomatosis of the liver became apparent in a 22-year-old woman while she was receiving medication with metoclopramide and experiencing the well-known adverse effect of the drug, hyperprolactinemia with secondary amenorrhea and galactorrhea. The hemangiomatosis was demonstrated by ultrasonography, computerized tomography, arteriography, and laparotomy with biopsy. When arteriovenous shunting became life-threatening and severe abdominal pain and cholestasis developed, the patient's name was placed on the waiting list for liver transplantation. However, after stopping the medication with metoclopramide, abdominal pain disappeared, cholestasis decreased, and the arteriovenous shunts in the liver closed completely. This course of disease represents either a spontaneous or a drug-induced activation and regression of hepatic hemangiomatosis. However, the long-term metoclopramide medication indicates a potential role of this drug in the promotion of hepatic angiogenesis. Hepatic angiomatosis in the adult seems to be neither a static nor a steadily progressive disorder but a process with active and regressive phases probably induced by a transient imbalance of angiogenic and angiostatic factors. Such a course should be kept in mind when major surgery or liver transplantation for hepatic hemangiomatosis is planned. It seems prudent to obtain a thorough drug history of all patients with hepatic hemangiomatosis. Whether hepatic hemangiomatosis can be drug induced or not, further investigation of the factors involved in hepatic angiogenesis is warranted.     

A case of an elderly female with diffuse hepatic hemangiomatosis complicated with multiple organic dysfunction and Kasabach–Merritt syndrome

Since diffuse hepatic hemangiomatosis (DHH) is an extremely rare disease especially in adults, the etiology and natural course of adult-onset DHH has not been well understood. We report a case of DHH complicated with multiple organic dysfunction and Kasabach–Merritt syndrome (KMS) in an 83-year-old female. She presented with mild abdominal distension and laboratory findings revealed thrombocytopenia and abnormal coagulation, indicating disseminated intravascular coagulation (DIC). Enhanced computed tomography revealed diffuse, hypodense hepatic nodules with delayed enhancement involving the whole liver, and multiple hypodense splenic legions. To obtain a definitive diagnosis, laparoscopic-guided biopsy was performed. Histological findings revealed irregularly dilated non-anastomotic vascular spaces, which were lined with flat endothelial cells without cellular atypia. We diagnosed this as DHH complicated with splenic lesions and KMS. Although the patient was treated with symptomatic treatment, such as anti-coagulation therapy, hemangiomatous lesions, especially in the spleen, progressed rapidly, leading to worsening of DIC. Finally, the patient died of multiple organ failure at 12 months after diagnosis. A postmortem examination demonstrated diffuse hemangiomatosis of not only the liver and spleen, but also the adrenal glands and bone marrow. Despite no malignant histologically, DHH can be fatal if it progresses rapidly within a short period of time.     

Diffuse hepatocellular calcification developing in a patient on chronic hemodialysis after ischemic hepatitis

Diffuse hepatic calcification is a rare condition. Previous reports have described patients with end-stage renal disease who developed diffuse hepatic calcification after ischemic hepatitis caused by shock. We herein present a similar case. A 41-year-old man on chronic hemodialysis developed ischemic hepatitis due to shock induced by ventricular tachycardia, followed by progressive hepatic failure. Necropsy of the liver revealed diffuse hepatocellular calcification. Given the similarity by which our case and previously reported cases developed this rare condition, we postulate that chronic renal failure is involved in the pathogenesis of diffuse hepatic calcification.     

Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anemia, and thrombocytopenia

In an elderly patient with oligosymptomatic giant splenomegaly, clinical and laboratory data were nondiagnostic, while nonhomogeneous splenic enlargement was the only finding detected by imaging procedures. Splenectomy was performed and diffuse hemangiomatosis of predominantly capillary-type found. The failure of imaging techniques to even hint at the nature of the underlying disorder is comprehensible in view of the organ being essentially replaced in toto by the abnormal vascular channels. Diffuse splenic hemangiomatosis, a rare condition, may cause hypersplenism, and its diagnosis may be elusive because of misleading patterns on imaging.     

Primary hepatic carcinoid tumor: one Egyptian center experience

BACKGROUND/AIMS: Carcinoid tumors of the liver are rare and pose both a diagnostic and therapeutic dilemma. Our aim was to study the diagnosis and treatment of primary hepatic carcinoid and to highlight its incidence in relation to hepatocellular carcinoma in our series and review of literature. METHODOLOGY: Between March 1992 and May 2005, we managed 5 patients (1 male, 4 females) with primary hepatic carcinoid in our center. RESULTS: The main presentation was upper abdominal pain with palpable mass, while in one patient tumor was discovered accidentally, none of them had carcinoid syndrome. The tumors were located in the left lobe in one patient, caudate lobe in two patients and right lobe in two patients. The diagnosis was confirmed histologically with light microscopy and immunohistochemistry. Four patients remain alive and disease free after follow-up of 72, 18, 16, and 4 months. One patient died after 11 years of follow-up with recurrence after 10 years, with mean follow-up of 45.2 +/- 53.1 months in May 2005. CONCLUSIONS: Primary hepatic carcinoid tumor is rare. It occurs on top of non-cirrhotic liver. Hepatic resection even in large-sized tumor is the treatment of choice.     

Portal thrombosis due to intrahepatic cholangiocarcinoma following successful treatment for hepatocellular carcinoma

A 57-year-old man, who had undergone hepatic arterial infusion chemotherapy with right portal occlusion for hepatocellular carcinoma was admitted to our hospital because of severe abdominal pain. Contrast-enhanced computed tomograms revealed that most areas of the liver were not enhanced, a finding suspicious for perfusion disturbance in the liver. Angiography revealed an interrupted right hepatic artery. Arterial portograms revealed complete obstruction of the right portal vein and a small left branch of the portal vein. Despite anticoagulant therapy with urokinase for portal vein thrombosis, the patient died from hepatorenal failure. Autopsy revealed that cholangiocarcinoma occupied almost the entire parenchyma of the right lobe, although the treated hepatocellular carcinoma lesion was completely necrotic. The right hepatic artery was obstructed due to direct invasion of tumor. There were diffuse thrombi in the left portal branches surrounded by tumor infiltrating along Glisson's sheath to the peripheral portion of the left lobe.     

Primary pulmonary rhabdomyosarcoma as a rare differential diagnosis of small cell lung cancer

Primary pulmonary rhabdomyosarcoma is a rare entity and the histological differential diagnosis can be difficult. We report on a 43-year old female patient, smoker (25 pack-years), in whom a large solitary brain metastasis was diagnosed and enucleated. Histological examination revealed a typical small cell carcinoma and histological examination of biopsies obtained from a tumor in the left upper lobe of the lung was compatible with a small cell carcinoma. Despite chemotherapy there was a progressive tumor growth. Bronchial biopsies again showed a small cell tumor, although immunohistochemistry proved it to be a pleomorphic rhabdomyosarcoma. Due to the progressive tumor growth with necrosis and superinfection and a lack of further metastases lobectomy of the left upper lobe was performed, complicated by postoperative pleural empyema, limiting the possibilities of adjuvant therapy. Early relapse occurred with pleural, pulmonary, chest wall and spinal metastases. Laminectomy and extirpation of the spinal metastases, local radiotherapy and chemotherapy with iphosphamide and doxorubicine led to partial remission and clinical improvement for few months only. The patient died from metastatic primary rhabdomyosarcoma of the lung. This rare tumor mimicked small cell lung cancer. Appraisal of the atypical clinical course and a close dialogue between pathologists and clinicians enabled the correct diagnosis.     

Primary Hepatic Low-Grade B-Cell Lymphoma of the Mucosa-Associated Lymphoid Tissue Type: A Case Report and Review of the Literature

Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis A presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin D1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.     

Clinical features and imaging diagnosis of biliary cystadenocarcinoma of the liver

Biliary cystadenocarcinoma of the liver is a relatively rare disease. Herein, we reported a case of biliary cystadenocarcinoma with a review of the literature. A 71-year-old female was admitted with the chief complaint of epigastralgia. The imaging studies revealed a biliary cystadenocarcinoma in the left hepatic lobe with suspicion of direct invasion to the left and middle hepatic veins and inferior vena cava. However, there was no direct invasion of the tumor to these veins in operation findings, and an extended left hepatic resection was performed without resection of inferior vena cava. The tumor was histologically diagnosed as biliary cystadenocarcinoma of the liver. Diagnosis of biliary cystadenocarcinoma is usually difficult preoperatively, however, a diagnosis was possible with the use of imaging studies. It was suggested that this tumor originated from a benign cystadenoma because of the existence of a transitional zone between normal cells and atypical cells in the cystic wall. Systematic hepatectomy was recommended as the initial treatment in consideration of the features of cystadenocarcinoma.     

Primary hepatic lymphoma in a patient with chronic hepatitis B

Primary hepatic lymphoma is a rare disorder and the clinical behavior remains unknown. We report a patient with primary hepatic lymphoma who had chronic hepatitis B. She was asymptomatic; however, a solitary tumor in the left lobe was incidentally detected. After left hepatic lobectomy was performed, a diagnosis of non-Hodgkin's lymphoma was made. No tumor was found except in the liver. Immunohistochemical stains for hepatitis B surface and core antigens were positive in hepatocytes; however, both were negative in the tumor tissue. The patient received no chemotherapy and the tumor relapsed. After chemotherapy, the tumor disappeared. However, exacerbation of hepatitis occurred after the fourth chemotherapy. The patient was followed up without chemotherapy, and she remains in apparent remission. Chemotherapy is effective against primary hepatic lymphoma and, if possible, patients with this disorder should be treated with chemotherapy postoperatively.     

Primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type: case report and review of the literature

A primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) is very rare. We found a solitary mass 27 mm in size in the left lobe of the liver of a 58-year-old Japanese man with a history of hepatitis-C infection. Based on the results of imaging studies, the tumor was diagnosed as a hepatocellular carcinoma (HCC). The left lobe of the liver was lobectomized and microscopic findings showed that the tumor was a hepatic MALT lymphoma, while immunohistochemistry showed it to be positive for CD20 and CD79a. In a fluorodeoxyglucose-positron emission tomography examination integrated with computed tomography scanning (FDG-PET CT) before surgery, the tumor was revealed to have a high standardized uptake value (SUV) for FDG. The patient received chemotherapy after surgery. To the best of our knowledge, 45 cases had been reported with a mean age for all patients of 61.4 years. The pathogenesis remains unclear, although half of the patients had a past history of chronic inflammatory liver disease. Surgical resection was performed in most cases and some patients received postoperative chemotherapy or radiotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.     

Collagenous nodule mixed simple cyst and hemangioma coexistence in the liver

A 20-year-old female patient presented with two masses located in the left liver.In this patient, a computed tomography(CT) scan revealed a hypodense mass and a second well-defined mass with a calcified nodule in the left hepatic lobe.No enhancements were apparent in or around the masses.A laparotomy was performed due to the patient's symptoms, namely, the atypical CT findings and a risk of rupture of the subcapsular lesion.The operation revealed two masses in the left hepatic lobe and a left liver resection was subsequently performed.One of the masses involved segment Ⅲ and the other mass was located in segment Ⅳ.The histopathologic findings supported a diagnosis of collagenous nodule mixed simple cyst and hemangioma.A diagnosis of collagenous nodule mixed simple hepatic cyst is extremely rare and radiologically mimics a teratoma, hepatolithiasis, parasitic cyst, or hemangioma.Although hepatic hemangiomas are the most common benign tumors found in the liver, the present case showed atypical radiographic features.     

Subacute liver failure by pseudocirrhotic metastatic breast cancer infiltration

Hepatic metastases are common in the clinical course of breast cancer and typically appear as mass lesions. This report describes the case of a 70-year-old woman with a history of breast cancer and no previously known liver disease presenting with the first episode of variceal bleeding and subacute hepatic failure. Imaging studies indicated liver cirrhosis without signs of malignant focal lesions. Comprehensive diagnostic work-up was negative for specific causes of liver disease and provided no evidence for tumor recurrence. Finally transjugular liver biopsy revealed a marked diffuse desmoplastic infiltration by breast cancer cells. Malignant pseudocirrhosis is an unusual pattern of metastatic tumor, spread representing a rare but important differential diagnosis of progressive liver failure. Liver biopsy is the key procedure to establish the diagnosis as imaging studies may mimic cirrhosis.     

Epithelioid hemangioendothelioma of the liver

Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. We present two cases of this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Both our cases are highly illustrative of the two ways in which hepatic EH can present (nodular or diffuse) and of its diagnostic and therapeutic management. Neoplastic cells expressed the factor VIII-related antigen, CD31 or CD34. Treatment was surgical resection in one patient and liver transplant in the other. Although EH of the liver has a better prognosis than other hepatic neoplasms, conservative treatment is not recommended. Our cases highlight the importance of a histological diagnosis to avoid it being mistaken for another entity.     

Synchronous occurrence of a hepatic myelolipoma and two hepatocellular carcinomas

Myelolipoma is a rare tumor composed of fat and bone marrow components, most of which are located in the adrenal gland. Myelolipoma in the liver is extremely rare. To date, only 10 cases have been reported in the English-language medical literature. In one of these cases, the hepatic myelolipoma was found within a hepatocellular carcinoma(HCC). In the present study, we report the first case of the synchronous occurrence of hepatic myelolipoma and HCCs in different liver sections of one patient, a 26-year-old female who was admitted to our hospital because of a 4-d history of upper abdominal pain. The unenhanced computed tomography(CT) images showed a well-defined lowdensity mass with adipose components in the right liver lobe, 4.2 cm × 4.1 cm in size. Two inhomogeneous low-density masses were found in the left liver lobe, 8.6 cm × 7.7 cm and 2.6 cm × 2.6 cm in size. The masses in both the right and left liver lobes were heterogeneously enhanced in the contrast-enhanced CT images. Based on the results of the imaging examination, the mass in the right liver lobe was preliminarily considered to be a hamartoma, and the two masses in the left liver were preliminarily considered to be HCCs. We performed a right hepatectomy, a left hepatic lobectomy, and a cholecystectomy. Microscopic and immunohistochemical results revealed that the tumor in the right liver lobe was a hepatic myelolipoma, and that the two tumors in the left liver lobe were HCCs.     

Hepatic tumor rupture following effectual treatment with irinotecan in a patient with highly refractory malignant lymphoma

We describe a patient with highly refractory malignant lymphoma who died of hepatic tumor rupture following treatment with irinotecan (CPT-11). This 60-year-old man with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma) demonstrated disease recurrence in the liver and the vertebrae following high-dose chemotherapy and autologous hematopoietic stem cell transfusion. He was treated with CPT-11 at a dose of one third of the conventional dose used for non-Hodgkin's lymphoma in Japan. The tumor in the liver markedly decreased in size but then ruptured. Although pathologic hepatic tumor rupture is a rare complication in patients with malignant lymphoma of the liver, this case demonstrates that hepatic tumor rupture may occur in refractory malignant lymphomas that reveal extensive degradation by this new, effective salvage therapy.     

Solitary fibrous tumor of the liver expressing CD34 and vimentin： A case report

A case of a successfully treated solitary fibrous tumor （SFT） of the liver is reported. An 82-year-old female presented with left upper abdominal discomfort, a firm mass on palpation, and imaging studies revealed a large tumor, 15 cm in diameter, arising from the left lobe of the liver. A formal left hepatectomy was performed. Microscopic evaluation showed spindle and fibroblast-like cells within the collagenous stroma. Immunohistochemistry disclosed diffuse CD34 and positive vimentin, supporting the diagnosis of a benign SFT. The patient remained well 21 months after surgery. SFT of the liver is a very rare neoplasm of mesenchymal origin. In most cases it is a benign lesion, although some may have malignant histological features and recur locally or metastasize. With less than 30 reported cases in the literature, little can be said regarding its natural history or the benefitsof adjuvant radiochemotherapy. Complete surgical resection remains the cornerstone of its treatment.     

Inflammatory myofibroblastic tumor of the lung: recurrence after steroid treatment

We report a case of a patient with inflammatory myofibroblastic tumor of the lung, which recurred after steroid treatment. The diagnosis of the tumor was confirmed by a core needle lung biopsy and pathology. The patient was initiated on steroid therapy, which on complete response was slowly tapered and discontinued. Few months later, the patient presented with dyspnea again. Chest radiography and computed tomography scans depicted recurrence at the tumor resection site in the right lobe of lung and a new tumor in the left lower lobe. Restarting the steroid therapy, led to regression of the tumors at both sites. This is a rare case of inflammatory myofibroblastic tumor recurring poststeroid therapy and adds to the current clinical knowledge about this peculiar manifestation of this disease entity.     

Resection of an icteric type hepatoma with tumor thrombi filling the right posterior bile duct

A 67-year-old male with jaundice was found to have hepatocellular carcinoma in the right hepatic lobe and tumor thrombi in the common hepatic duct. Physicians initially considered the tumor unresectable, and treated the patient with transcatheter arterial infusion chemotherapy and biliary endoprosthesis. The patient developed a liver abscess after the second transcatheter arterial infusion, and the physicians consulted our department for another form of therapy. Percutaneous transhepatic biliary drainage was performed to relieve revived obstructive jaundice. Cholangiography revealed tumor thrombi extending through the right posterior segmental bile duct into the common hepatic duct. Most biliary branches of the caudate lobe joined with the left lateral posterior segmental branch. Arterial and portal venous branches of the caudate lobe were not involved. Right hepatic lobectomy and extrahepatic bile duct resection were performed 1 year after initial diagnosis. On histologic examination, the epithelium of the right posterior segmental bile duct, which was filled with the tumor thrombi, was not detected. The patient is alive without recurrence 24 months after surgery. Careful investigation of biliary branches of the caudate lobe on cholangiography is essential to determine the necessity of caudate lobectomy in patients with hepatocellular carcinoma and tumor thrombi filling the right posterior segmental bile duct.