肝上皮样血管内皮瘤1例并文献复习

目的提高对肝上皮样血管内皮瘤的认识。方法结合我院诊治的一例肝上皮样血管内皮瘤的患者资料和文献复习,探讨该病的临床特点、诊断、治疗及预后等。结果肝上皮样血管内皮瘤病因尚不明确,最常见的临床表现是右上腹疼痛、肝肿大和体重减轻,MRI或CT特点为:病灶对比增强扫描大于3 cm的病灶增强后有向心性强化倾向,而小于3 cm的病灶呈环形强化,门脉期及延迟期持续环形强化,肝内静脉主干及分支终止于肿瘤的边缘,形成"棒棒糖征"。病理学检查主要特征是具有细胞内血管腔的上皮样瘤细胞并呈血管内皮标志物染色阳性。肝上皮样血管内皮瘤的治疗以手术切除和肝移植为主,预后介于良、恶性肿瘤之间。结论肝上皮样血管内皮瘤发病率低、临床表现复杂,易误诊,临床诊治过程中应予以重视。     

Hepatic haemangioendothelioma: case report and review of literature

A 37 year old-woman complained about persistant pain of right abdominal quadrant. We discovered many hepatic lesions. Histology revealed benign processus. Evolution was marked by peritoneal carcinomatosis. After reexamination of histology we discovered hepatic haemangioendothelioma. The patient died 18 month after diagnosis although chemotherapy.     

Hepatic angiosarcoma of childhood: a case report and review of literature

Angiosarcomas are rare tumors that predominantly affect adults. Hepatic angiosarcoma in a child is an extremely rare event and is associated with a poor prognosis. Herein, we report our experience in a case of hepatic angiosarcoma in a child who presented with a huge unresectable mass that underwent liver transplantation.     

Hepatic malignant epithelioid hemangioendothelioma: a case report and review of the literature

Malignant epithelioid hemangioendothelioma is a rare hepatic tumor of vascular origin. It is most commonly found in young to middle aged women, and the tumors vary in reported malignant potential. Compounds such as oral contraceptive pills, poly vinyl chloride, and Thorotrast have been identified as risk factors for subsequent disease development. Radiologic ("lollipop" sign, capsular flattening) and pathologic (Factor-VIII antigen staining positive) evaluation aids in the diagnosis. As with most mesenchymal tumors, surgical resection is the most effective means of controlling local disease and preventing distant metastasis, though adjuvant therapies have been offered for those that are unresectable or not transplant candidates. We present our case of a hepatic malignant epithelioid hemangioendothelioma and a review of the English-language literature.     

Obstructive uropathy associated with primary ureteral endometrioma: case report and review of the literature

We report the case of a 56-year-old postmenopausal woman who presented with incidental left hydronephrosis during an investigation for a gastrointestinal complaint. The patient denied any history of flank pain or hematuria. Contrast-enhanced computed tomography revealed severe right-sided ureterohydronephrosis as well as renal atrophy. The contralateral kidney was normal, as was the patient’s overall renal function. A retrograde ureterogram demonstrated complete ureteral obstruction 4 cm proximal to the ureterovesical junction. Subsequent ureteroscopy revealed a polypoid mass completely occupying the ureteral lumen, of which the biopsies demonstrated inconclusive atypical urothelial changes. The patient underwent a laparoscopic nephrectomy with open dissection of the distal ureter. The patient recovered well postoperatively. Final pathology revealed a benign obstructing endometrioma without evidence of invasion from periureteral tissue. This appears to be the first reported case of asymptomatic primary ureteral endometrioma with secondary renal atrophy. Earlier investigation and treatment may have allowed for renal preservation earlier in the course of the disease.     

Abdominal wall endometrioma: case report and review

Women with chronic, cyclical abdominal wall pain after gynecologic surgery may present in the plastic surgeon's office requesting body contouring. We present one such case in which an abdominal wall endometrioma was found. Resection of the endometrioma with the abdominal panniculus resulted in symptomatic cure for the patient. Plastic surgeons should be aware of the association of endometriosis with chronic pelvic/abdominal pain. Furthermore, suspicion of abdominal wall endometrioma should be entertained when patients present with chronic intermittent or cyclical abdominal wall pain after surgery of the pelvis or abdomen, especially after gynecologic or obstetric procedures.     

肝滤泡树突细胞肉瘤1例报告并文献复习

目的 探讨肝滤泡树突细胞肉瘤的病理组织学与免疫组化特点。方法 运用组织病理学和免疫组化观察2008年6月首都医科大学附属北京友谊医院普外科诊治的1例肝滤泡树突细胞肉瘤的特点, 并复习相关文献对其进行分析。结果 肝滤泡树突细胞肉瘤无典型临床症状,无特征性影像学表现,术前难于确诊。CT表现为肝内较大单发肿块。光镜可见: 肿瘤细胞呈梭形及卵圆形, 细胞间界限不清。免疫组化: 肿瘤细胞CD35 （+）,而CD21（－）,CD23（－）, S-100（－）,ALK基因（－）,原位杂交法检测EB 病毒（EBER）（－）。 结论 肝滤泡树突细胞肉瘤是一种少见的低度恶性肿瘤,主要依据组织病理学和免疫组化标记进行诊断。手术切除是主要治疗手段。     

Hepatic pulmonary fusion: case report with review of literature

Hepatic pulmonary fusion is a rare congenital anomaly and is associated with abnormal systemic venous drainage and arterial supply. We describe multidetector computed tomography and magnetic resonance angiogram findings of an 11-year-old boy with recurrent cough, fever, and respiratory distress caused by hepatic pulmonary fusion.     

Hepatic angiosarcoma and liver transplantation: case report and literature review

Hepatic angiosarcoma is a rare malignant vascular tumor that accounts for up to 2% of all primary liver tumors. Accurate diagnosis of this tumor is difficult, especially if the patient has no history of exposure to specific carcinogens including thorotrast, arsenicals, and vinyl chloride monomer. Diagnosis of diffuse angiosarcoma by means of liver biopsy has been reported as treacherous and nondiagnostic. Herein, we present a case of a 61-year-old Caucasian male with history of cryptogenic cirrhosis, normal alpha-fetoprotein, and pretransplant abnormal liver MRI who underwent nondiagnostic liver biopsies followed by liver transplantation. High grade diffuse angiosarcoma was diagnosed in the explanted liver. The patient developed bone metastases at 8 months and is alive 1 year posttransplantation. Diffuse liver tissue infiltration seen pretransplant on CT scan or MRI, suggesting the possibility of diffuse liver lesions (HCC, angiosarcoma, etc) must be fully investigated with all techniques available including multiple open liver biopsies to avoid the sacrifice of a liver allograft in these patients.     

Hepatic actinomycosis presenting as a liver tumour: case report and literature review

Hepatic actinomycosis poses a difficult problem in both diagnosis and management. We report the management of a patient with isolated hepatic actinomycosis, and review the clinical features and management of patients with hepatic actinomycosis mimicking liver tumour.     

Hepatic sarcoidosis mimicking cholangiocellular carcinoma: A case report and literature review

IntroductionSarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs. Almost 70% of patients with a sarcoidosis reaction have hepatic involvement. However, evidence-based clinical management or treatment strategies for hepatic sarcoidosis are poorly defined. Here, we present a case of a resected hepatic sarcoidosis patient. Additionally, we review the relevant hepatic sarcoidosis literature and discuss the clinical management of hepatic sarcoidosis.Presentation of caseA 20-mm liver tumor of segment 8 was incidentally detected in a 64-year-old female. Radiological images resembled the enhancement pattern of cholangiocellular carcinoma. Thus, this lesion was assigned a preoperative classification of pT1N0M0 stage I according to the 7th Union for International Cancer Control guidelines. The patient underwent a partial liver resection. Histologically, the tumor contained sarcoidosis lesions indicated by a conglomerate of epithelioid granulomas with giant cells. These histopathological findings were consistent with the diagnosis of hepatic sarcoidosis.DiscussionHistopathological examination has been established as the definitive diagnostic tool for hepatic sarcoidosis. Therefore, liver biopsy or surgical resection of a liver tumor should be considered in cases that are difficult to preoperatively distinguish from malignant tumors.ConclusionWe present the case of a patient with surgically resected hepatic sarcoidosis that was difficult to preoperatively distinguish from cholangiocellular carcinoma.     

Enchondroma: a case report and literature review

An unusual case of enchondroma located in the distal phalanx of the left third toe is presented with a review of literature. An enchondroma is a benign tumor centrally located in a bone. This osseous dysplasia is characterized by an excess of mature hypertrophic hyaline cartilage that has not resorbed or ossified in the normal fashion. Pathologic, clinical, and radiographic findings will be discussed.     

Melorheostosis: a case report and literature review

Melorheostosis is a rare form of cortical hyperostosis that resembles wax dripping down the side of a candle. This disease usually affects the long and short bones of an extremity. Literature review and a case report will be discussed with respect to incidence, clinical presentation, radiographic appearance, and treatment.     

Polyorchidism: a case report and review of the literature

Polyorchidism is defined as the presence of two or more testes. It is a rare anomaly with approximately 77 cases reported in the literature. Polyorchidism is frequently associated with additional urological pathologies such as undescended testis, inguinal hernia, testicular torsion, hydrocoele, malignancy and infertility. Differential diagnosis includes spermatocoele, hydrocoele, epididymal cysts or aberrant epididymis. We report on an interesting case of polyorchidism in a 15-year-old man diagnosed on ultrasound and we review the literature and current management of polyorchidism.     

Meningioangiomatosis: a case report and review of the literature

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.