Tuberous sclerosis associated with multiple hepatic lipomatous tumors and hemorrhagic renal angiomyolipoma.

We report a case of tuberous sclerosis associated with hepatic lipomatous tumors and renal angiomyolipomas. Abdominal ultrasonography revealed a high echoic large tumor in the left kidney. A provisional diagnosis of angiomyolipomas of the kidney was made based on computed tomography. Subsequent laparotomy revealed that the extracted tumor was renal angiomyolipoma. It was also revealed that there was an association with hepatic lipomatous tumors thought to be lipomas or angiomyolipomas by liver biopsy. Nearly half of all cases of angiomyolipoma in the kidney are reported as occasional association with tuberous sclerosis complex, but lipomatous tumors in the liver are rare.     

Basket pattern blood flow signals discovered in a case of splenic hamartoma by power Doppler ultrasonography

We present the gray-scale ultrasonography (GSUS), power Doppler ultrasonography (PDUS), abdominal computed tomography (CT), and magnetic resonance imaging (MRI) findings for a case of splenic hamartoma in a 27-year-old man, showing a φ 50 mm homogeneous, iso- and hypo-echoic splenic mass with evidence of a small plural cystic lesion. This splenic hamartoma showed increased vascularity on power Doppler sonograms. PDUS showed multiple circular blood flow signals inside the mass (i.e. a basket pattern), which was consistent with the small plural cystic lesion shown by GSUS. Spectral analysis also confirmed arterial and venous flow. CT scans showed that the mass had low-density relative to the normal spleen and MRI showed that the mass was isodense, relative to the normal spleen. Therefore, CT and MRI are not useful for the diagnosis of splenic hamartoma. Ultrasonography can be used to diagnose splenic hamartoma without administration of a contrast material and therefore is an indispensable method for the diagnosis of splenic hamartoma.     

Congenital absence of the portal vein complicating hepatic tumors

Congenital absence of the portal vein (CAPV) is a rare malformation that is often accompanied by other anomalies such as cardiac and skeletal malformations and/or hepatic tumors. We describe here a case of CAPV complicating hepatic tumors in a 16-year-old Japanese girl. Abdominal ultrasonography revealed a hyperechoic tumor in the liver and dilatation of the portal vein that appeared to be connected directly with the inferior vena cava. Subsequent abdominal computed tomography (CT) revealed tumors and magnetic resonance angiography confirmed that the portal vein entered directly into the inferior vena cava just above the liver. In addition, there was absence of the right portal vein and the left intrahepatic branch except for the presence of left portal vein only within the porta hepatis. These findings led to a diagnosis of CAPV complicated hepatic tumors. Careful monitoring of these hepatic tumors is ongoing due to the possibility of malignant transformation.     

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.     

Spontaneous rupture of splenic hamartoma in a patient with hepatitis C virus-related cirrhosis and portal hypertension： A case report and review of the literature

Spontaneous rupture is a rare complication of splenic hamartoma. A review of the literature revealed only four such cases. To the best of our knowledge, this is the first report of spontaneous rupture of splenic hamartoma associated with liver cirrhosis and portal hypertension. A 53-year-old woman, who was followed up for aortic dissection and hepatitis C virus (HCV)-related liver cirrhosis, was referred with sudden left chest and shoulder pain. An abdominal ultrasound showed intraabdominal bleeding, and computed tomography indicated rupture of a splenic tumor. Emergent splenectomy was carried out. The postoperative course was uneventful, and the patient was discharged on the 13th postoperative day. Pathology revealed the tumor to be a ruptured splenic hamartoma. The non-tumorous splenic parenchyma revealed congestive changes. We consider that the presence of liver cirrhosis and portal hypertension are risk factors for spontaneous rupture of the splenic hamartoma.     

Spontaneous rupture of a hepatic angiomyolipoma: Report of a case

A 70-year-old female experienced sudden onset of back pain on the right side and was admitted to our hospital in December 2010. Abdominal computed tomography revealed an S7 hepatic mass measuring 7 cm in diameter accompanied by a subcapsular hematoma. Emergency angiography confirmed the diagnosis of a ruptured hepatic mass, and hemostasis was carried out by embolization of A8 and A7 of the liver. A right hepatic lobectomy was carried out 39 days following transarterial embolization. Although almost all aspects of the tumor were necrotic, residual tumor cells stained positive for HMB-45, and negative for α-SMA, S-100, CD 34, c-kit, CAM 5.2, and hepatocytes. The MIB-1 index was 2 %. Pathological diagnosis was hepatic angiomyolipoma (HAML). The patient has shown no signs of recurrence at 42 months following surgery. Here, we report on this case of spontaneous HAML rupture and discuss therapeutic strategies for HAML and ruptured hepatic tumors.     

Unresectable hilar cholangiocarcinoma completely reduced by external radiation therapy

A 68-year-old woman was referred to our hospital with liver dysfunction. Abdominal ultrasonography showed an isoechoic mass in the hepatic hilum. Abdominal computed tomography (CT) disclosed a homogenous mass adjacent to the portal bifurcation, and endoscopic retrograde choledocho-pancreatography (ERCP) revealed complete obstruction from the common hepatic duct to the hepatic duct confluence. After insertion of the endoscopic nasobiliary drainage (ENBD) tube, laparotomy was performed, revealing an approximately 5x6-cm elastic hard mass at the hepatic hilum, mainly spreading to the left hepatic duct, and involving surrounding vascular structures such as the portal vein and right and left hepatic arteries. Therefore, she was treated by external radiation therapy (ERT) of 60 Gy in 30 sessions after insertion of endoscopic retrograde biliary drainage (ERBD) tubes to bilateral hepatic ducts to replace the ENBD tube. After the completion of ERT, she was discharged from the hospital in good health. Three months later, she was readmitted for cholangitis and received antibiotic treatment. Surprisingly, abdominal CT disclosed complete remission of the tumor. ERCP revealed patency of the common hepatic duct and hepatic duct confluence. Therefore, the ERBD tubes were removed. Specimens from the hepatic duct confluence, and bilateral hepatic ducts showed no malignancy histologically. She is doing well without recurrence more than 4 years after surgery.     

Granulomatous reaction in hepatic inflammatory angiomyolipoma after chemoembolization and spontaneous rupture

A 77-year-old Japanese woman was transported to a nearby hospital due to sudden abdominal pain and transient loss of consciousness. Abdominal computed tomography (CT) suggested hemoperitoneum and hepatic nodule. She was conservatively treated. Contrast-enhanced CT two months later revealed an increased mass size, and the enhancement pattern suggested the possibility of hepatocellular carcinoma (HCC). Under a clinical diagnosis of HCC, transcatheter arterial chemoembolization (TACE) was performed. A subsequent imaging study revealed that most of the lipiodol used for the embolization was washed out. Therefore, surgical resection was performed. Histologically, the nodule contained numerous inflammatory cells including small lymphocytes, plasma cells and macrophages. Notably, epithelioid granulomatous features with multinucleated giant cells were observed in both the nodule and background liver. Some of the multinucleated giant cells contained oil lipid. Among the infiltrating inflammatory cells, spindle-shaped, histiocytoid or myoid tumor cells with eosinophilic cytoplasm were found. The tumor cells were positive for Melan A and HMB45. The nodule contained many IgG4-positive plasma cells; these were counted and found to number 72.6 cells/HPF (range: 61-80). The calculated IgG4:IgG ratio was 33.2%. The nodule was finally diagnosed as previously ruptured inflammatory angiomyolipoma modified by granulomatous reaction after TACE.     

Mesenchymal hamartoma of the liver: report of an adult case and review of the literature.

A case of mesenchymal hamartoma of the liver incidentally found to be a hyperechoic mass by abdominal ultrasonography in a 62-year-old male is described. Computed tomography and magnetic resonance imaging of the liver confirmed the presence of a well-demarcated inhomogeneous mass in the left medial segment. Hepatic arteriography revealed the mass to be generally hypovascular. Histopathologic diagnosis of the resected specimen was a solid form of benign mesenchymal hamartoma, a rather rare lesion for the patient's age. Fifteen adult cases of hepatic mesenchymal hamartoma in the literature including the present case are reviewed.     

A case report of abdominal splenosis - a practical mini-review for a gastroenterologist

Splenosis is a benign condition caused by ectopic autotransplantation of splenic tissues after splenic trauma or surgery. It is usually diagnosed accidentally. However, occasionally splenosis poses a significant diagnostic dilemma, especially when this condition presents as a metastatic malignant disease on abdominal imaging. This is the case report of a 54-year-old woman, who underwent post-traumatic splenectomy at the age of 12 years. The physical examination of this patient complaining of epigastric and low back pain, except for postoperative abdominal scar, as well as laboratory tests were normal. Esophagogastroduodenoscopy showed chronic gastritis. Abdominal ultrasound revealed no spleen, enlarged lymph nodes in the right retroperitoneum and a focal lesion in the uterus. In addition, the CT scan disclosed enlarged left hepatic lobe, numerous oval tumors between intestinal loops, in the caecal region, under the diaphragm and abdominal wall, periaortic enlarged lymph nodes and an osteolytic lesion in the first lumbar vertebra, suggesting disseminated malignant disease. The exploratory laparotomy showed multiple abdominal nodules, a tumor of the ileum, the greater omentum and the uterus. Except for the last one (leiomyoma), all excised tumors proved to have histological structure typical for the spleen and, finally, abdominal splenosis was diagnosed. Splenosis should be considered when a differential diagnosis of tumor-like lesions disclosed on abdominal imaging occurs in a patient with a splenic injury in the past.     

A case of primary splenic angiosarcoma with intraperitoneal hemorrhage treated by transcatheter arterial embolization

Splenic tumors are very rare. In Japan only 42 cases of splenic angiosarcoma have been reported. We encountered a case of spontaneous rupture of a splenic angiosarcoma and liver metastasis. A 60-year-old woman who suddenly went into hemorrhagic shock presented at our hospital. Then acute spontaneously ruptured spleen and hepatic tumors were diagnosed by abdominal CT. After emergency TAE, the patient was hemodynamically stable, but died of liver failure 13 days after admission. The pathological diagnosis was primary splenic angiosarcoma with multiple organ metastasis on autopsy. Splenic angiosarcoma should be kept in mind in the differential diagnosis of splenomegaly or splenic tumor. TAE can be effective in primary hemostasis for angiosarcoma with intraperitoneal hemorrhage from multiple tumors.     

Common and epithelioid variants of hepatic angiomyolipoma exhibit clonal growth and share a distinctive immunophenotype

Angiomyolipoma represents a rare liver tumor of uncertain histogenesis that is commonly considered a hamartoma. A series of 12 hepatic angiomyolipomas, including 3 samples of the epithelioid subtype, was analyzed for clonality using the human androgen receptor gene locus (HUMARA). Four of 6 informative cases revealed monoclonality. The polyclonal pattern in the 2 remaining cases was most probably caused by excessive infiltration of inflammatory cells. Monoclonality with an identical X-chromosomal inactivation pattern in all nodules was found in a multifocal recurrent tumor indicating a metastatic process. Despite the morphologic heterogeneity, all tumors displayed an identical immunohistochemical labeling pattern. It is concluded that different subtypes of hepatic angiomyolipoma exhibit a monoclonal and hence probably neoplastic growth and share an identical immunophenotype suitable for their identification even in small biopsy specimens. The epithelioid subtype may give rise to intrahepatic metastasis.     

Asymptomatic lymphangioma involving the spleen and retroperitoneum in adults

Lymphangioma, a benign neoplasm of the lymphatic system, is common in children but rare in adults. Its clinical manifestations include abdominal pain, nausea,vomiting and a palpable mass. However, abdominal sonography or abdominal computed tomography (CT) scan can also incidentally reveal lymphangioma. A larger or symptomatic lymphangioma is treated with total resection to prevent recurrence, infection, torsionand enlargement. Although lymphangioma rarely becomes malignant, its prognosis is generally good.We report a cystic lymphangioma of the spleen and retroperitoneum, which was incidentally found in a 56-year-old man who was hospitalized due to a colon mass. Physical examination showed no specific findings.Abdominal CT revealed a 5.7 cm, non-enhanced multilobulated cystic mass with multiple septa in the spleen and a 10 cm lobulated cystic mass in the paraaorticarea. Splenectomy and retroperitoneal resectionof the cystic mass were conducted. The endothelium of splenic and retroperitoneal cyst was immunohistochemically stained with D2-40 antibody. The patient was finally diagnosed with splenic cystic and retroperitoneal cavernous lymphangioma.     

A case of primary neuroendocrine carcinoma of liver presenting with liver abscess]

Primary hepatic neuroendocrine cell carcinoma is a very rare tumor. We experienced a 75-year-old woman with primary hepatic neuroendocrine carcinoma presenting with pyogenic liver abscess. Abdominal CT scan revealed a multiseptated liver abscess and an enlarged lymph node in portocaval portion. We performed percutaneous drainage of the liver abscess, but the amount of drained pus did not decrease after 20 days. The follow-up abdominal CT scan showed that the cystic portion of liver abscess had been replaced by the solid tumor. Microscopic examination of the tumor tissue showed nests of epithelial cells with uniform round hyperchromatic nuclei and high nuclear to cytoplasmic ratio. Immunohistochemical staining was strongly positive for synaptophysin and chromogranin A.     

Abdominal computed tomography findings of malaria infection with Plasmodium vivax

Abdominal computed tomography (CT) findings of malaria are not well-known even though malaria is a serious infectious disease. To identify abdominal CT findings, we selected 34 of 405 patients who had a positive peripheral blood smear for Plasmodium vivax and had underwent abdominal CT as the malaria group. We also selected 80 patients who had fever and a negative peripheral blood smear as the control group and 120 healthy people as the normal group. We reviewed and analyzed their medical records and CT findings retrospectively. The mean spleen and liver length were significantly larger in the malaria group and the incidence of splenomegaly, splenic focal low attenuation, and spontaneous splenic rupture were much higher in the malaria group (P < 0.05). Although abdominal CT is not an indispensable tool for diagnosis, these CT findings will help in the diagnosis of malaria in patients with fever.     

Splenosis--important differential diagnosis in splenectomized patients presenting with abdominal masses of unknown origin

A 40-year-old female patient was admitted for work-up of multiple abdominal masses. The lymphoma-mimicking tumors were detected accidentally during an ultrasound course. The past medical history was unremarkable besides a status post-traumatic splenic rupture and splenectomy. The patient was asymptomatic, especially there were no complaints of fever, night sweats or weight loss. Laboratory tests did not show pathological results. Ultrasound of the abdomen revealed multiple hypoechoic mesenterial and peritoneal enlarged tumors as well as a subhepatic mass (30 x 20 mm). Transmission computed tomography (CT) showed a normal chest, excluded abnormal thoracal masses and confirmed the multiple abdominal nodules. Microparticles were trapped only by tissue with phagocytosis function as cells of the reticulohistiocytary system in liver and spleen. Uptake of (99 m)Tc-labeled microparticles is specific for splenic tissue. All abdominal masses were detectable by single photon emission computed tomography (SPECT) after intravenous administration of this radiotracer. Ultrasound-guided biopsy proved the presence of spleen tissue with follicular hyperplasia. In conclusion, we report a case of post-traumatic splenosis. In 16 - 67 % of patients who experienced traumatic splenic rupture autotransplanted spleen tissue can be detected. Splenosis therefore is an important differential diagnosis of abdominal masses in splenectomized patients.     

16例脾结核临床分析

目的 探讨脾结核的诊断和治疗措施.方法 对16例脾结核患者的临床资料进行回顾性分析.结果 16例病人中表现为发热、盗汗、腹胀、腹痛和脾肿大分别为11例次(68.8%)、6例次(37.5%)、8例次(50%)、10例次(62.5%)和13例次(81.2%).腹部B超及CT检查10例有脾内占位性病变,3例脾内有钙化灶,另3例为脾弥漫性肿大.手术及病理诊断脾结核9例,3例因脾内有钙化灶诊断脾结核,另4例诊断性抗结核治疗有效而诊断脾结核.16例病人中有8例合并脾外结核.所有病人都经过半年以上的抗结核治疗,经随访预后良好.结论 脾结核诊断主要依据睥病理学检查及诊断性治疗,系统性抗结核治疗及选择性手术治疗是脾结核的主要有效治疗手段.     

肝脏神经内分泌肿瘤报道1例并文献回顾

背景肝脏可疑恶性肿瘤行手术切除后病理诊断肝脏神经内分泌肿瘤(hepatic neuroendocrin neoplasmas,HNENs)病例,此病例易与肝癌混淆,了解转移性和原发性HNENs的特性,对临床治疗手段有很好的指导意义.病例简介一名56岁男性患者体检发现肝脏占位性病变复查腹部增强CT提示肝脏转移性恶性肿瘤可能;胃部包块考虑良性.手术切除胃部包块并行术中冰冻病理检查提示梭形细胞肿瘤;完整切除肝脏包块术后石蜡病理检查结果提示NENs(G2),患者术后恢复佳.结论本文报道了一例HNENs,考虑转移性.术前诊断不能排除肝脏转移性恶性肿瘤可能.通过对患者病情的详细分析,最终行手术切除治疗并行病理检查确诊.同时我们对HNENs相关文献进行了分析,进一步了解原发和转移性HNENs的临床特征、治疗方案以及预后,以期对此类疾病有更深入的了解,对临床治疗方案决策做证据支撑作用.     

Juxta-caval hepatic angiomyolipoma masquerading as hepatocellular carcinoma

Hepatic angiomyolipoma is an uncommon primary benign epithelial liver neoplasm. Most patients with angiomyolipomas are middle-aged women who commonly present with epigastric fullness or pain. Angiomyolipomas are tumors consisting of three tissue types: blood vessels, smooth muscle, and fat. Hepatic angiomyolipomas may be difficult to differentiate from other liver neoplasms by noninvasive imaging. We report a 58-year-old asymptomatic woman with a mass in the right lobe of the liver, found incidentally on routine abdominal sonography. Preoperative radiographic evaluation revealed a 6.5-cm hypervascular lesion abutting the inferior vena cava. Preoperative histologic study demonstrated an epithelial neoplasm suspicious for hepatocellular carcinoma. Metastatic workup was negative. At resection, the tumor was found to be an angiomyolipoma composed of lipoid, vascular and smooth muscle cells. Further staining was positive for HMB-45. Resection margins were negative. The woman had an uneventful recovery and was discharged on postoperative day seven. She currently remains well several months after her right hepatectomy.     

Extra-pulmonary Pneumocystis jiroveci infection: a case report

In physical examination abdominal tenderness, gate disturbance and penile herpetic lesions were detected. Decreased disc height at T11-T12 level was detected in chest X-ray. Abdominal sonography and CT scan revealed hypo dense lesions in Lt left Lobe of liver and multiple hypo dense splenic and pancreatic lesions, ascitis, Lt left sided pleural effusion, thickening of jejuneal mucosa and edema of bowel wall. Vertebral body lesion and paravertebral abscess, bony calvarial involvement and adjacent extra axial brain lesion were observed in imaging were other findings. RNA analysis for HIV was positive. Vertebral lesion biopsy and aspiration of splenic lesion were performed and pathology revealed Pneumocystis jirovecii suggestive of extra pulmonary Pneumocystis carinii infection.