肺血管肉瘤的临床病理观察

目的 探讨肺原发性血管肉瘤的临床病理特点.方法 对我院收治的1例,发生于肺的血管肉瘤的临床表现、组织形态学及免疫组化进行分析,并复习相关文献.结果 患者男性,67岁,临床表现主要为咳嗽和痰中带血.光镜下上皮样或梭形肿瘤细胞形成不规则且相互吻合的血管腔隙,伴大片的出血坏死.免疫组织化学染色显示肿瘤细胞CD34、CD31和vim阳性;CK( AE1/AE3)、TTF-1和S-100阴性.结论 原发性肺血管肉瘤具有和其他部位血管肉瘤相似的组织形态学特点,免疫组织化学是其必要的辅诊手段.其临床表现无明显特异性,早期诊断困难.     

Primary epithelioid angiosarcoma of the lung presenting as pulmonary hemorrhage

Pulmonary angiosarcomas are usually secondary tumors, and only a few primary cases have been reported. We report a unique case of epithelioid angiosarcoma presenting as a solitary mass in the right upper lobe with pulmonary hemorrhage. Because of its epithelioid histology, this tumor may resemble a carcinoma or a variety of vascular lesions with epithelioid endothelial cells. Therefore, the diagnosis of epithelioid angiosarcoma should be based on immunohistochemical staining.     

Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature

BACKGROUND: Angiosarcoma is a rare high-grade neoplasm that frequently involves the skin and subcutaneous tissue. Rarely, angiosarcoma can occur in the gastrointestinal tract where it frequently exhibits multicentric epithelioid morphology. DESIGN: We report a case of multicentric epithelioid angiosarcoma (EAS) of the small intestine in a 73-year-old male patient who presented with weakness and melena, and was found to have bleeding lesions in the small intestine on upper gastrointestinal endoscopy. In addition to this case, we extensively reviewed the clinical and pathological features of previously reported cases of angiosarcoma of the small intestine in the English literature since 1970. RESULTS: Our patient presented with rare and aggressive EAS of the small intestine. Despite surgical resection of the lesions, the patient continued to worsen and developed rapidly progressive metastatic disease. He died within 4 months of the diagnosis. CONCLUSIONS: Angiosarcoma, especially of the deep tissues and the gastrointestinal tract, is very aggressive and rapidly metastatic. The survival rate in these patients is extremely poor, and most patients die within 6 months to 1 year of the diagnosis. Treatment usually involves surgical resection of the bleeding lesions and frequent blood transfusions for symptom alleviation.     

Five years and 4 months of recurrence-free survival in hepatic angiosarcoma

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 × 42 × 35?mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.     

Angiosarcoma secondary to postirradiation and chronic lymphedema: Case reports

Introduction:Angiosarcoma secondary to post-irradiation and lymphedema is rare, but it is aggressive with a poor prognosis. It is essential to understand these patients’ clinical features and distinguish them from benign diseases or other malignant tumors.Patient concerns:Three patients who had radiotherapy for cancer treatment and chronic lymphedema admitted to the hospital with specific skin lesions at upper or lower extremities.Diagnosis:Excisional biopsies revealed prominent, highly atypical cells with a vasoformative area, composed of atypical, large epithelioid cells with vesicular nuclei, prominent nucleoli, and mitoses. Immunohistochemistry revealed diffuse expression of endothelial cell markers suggestive of angiosarcoma.Interventions:One patient had shoulder disarticulation with wide excision with adjuvant radiotherapy and chemotherapy and other 2 discontinued the treatment.Outcomes:After the treatment, one patient was transferred to rehabilitation department for shoulder disarticulation prosthesis fitting without recurrence sign for 1 year. Two patient refused further treatment and was lost to follow-up.Conclusion:In cases of patients with irratiation and chronic lymphedema, clinical findings suggestive of angiosarcoma, biopsy and imaging studies should be performed as soon as possible.     

Spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis

Aim: We report herein a case of spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis and review the correlative literature. Methods: The resected specimen was examined by histopathological and immunohistochemical evaluation. Results: The final diagnosis was spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis Conclusion: Considering the nature of primary hepatic angiosarcoma, in particular the ruptured hepatic angiosarcoma, it is obviously desirable to avoid any unnecessary delay or definitive surgical treatment. It is presumed that angiosarcoma in the liver has a possible association with S. japonicum and the deposition of ovae in liver.     

Angio-immunoblastic lymphadenopathy with fibrosis of bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph nodes

We report a 47-year-old man diagnosed as angio-immunoblastic lymphadenopathy with dysproteinemia (AILD) with fibrosis of the bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph node. He was admitted to a hospital in May, 1980 because of general fatigue, cough, fever and systemic lymphadenopathy. The diagnosis of AILD was based on a biopsy of right cervical lymph node. His symptoms were improved but recurred with the addition of icterus and progressive pancytopenia with decrement of prednisolone. He was referred to our hospital in July, 1980 and his physical examination revealed generalized lymphadenopathy, icterus and hepatosplenomegaly. Hemogram showed pancytopenia, and needle biopsy of the bone marrow disclosed fibrosis. Sections from the lymph node showed AILD with proliferation of epithelioid cells. Administration of 60 mg/day of prednisolone improved the fever, lymphadenopathy and hepatosplenomegaly. However he died suddenly of acute respiratory failure on July 30. Autopsy showed fibrosis of bone marrow, lymph node, liver and spleen with infiltration of abnormal lymphocytes, and pulmonary aspergillosis.     

Benign and malignant vascular tumors of the liver in adults

Vascular tumors of the liver in adult patients include cavernous hemangioma, a common benign tumor; epithelioid hemangioendothelioma, a rare, usually low-grade malignant tumor; and angiosarcoma, a rare and very aggressive tumor. All these primary mesenchymal tumors develop on a normal liver and may also affect other organs. Their pathogenesis remains largely unknown. Hepatic tumors are increasingly detected incidentally due to widespread use of modern abdominal imaging techniques. Therefore, reliable noninvasive characterization and differentiation of such liver tumors is of major importance for clinical practice. Hemangioma follows a benign course, and a nonoperative approach for the majority of these lesions is recommended. A definitive diagnosis of epithelioid hemangioendothelioma and angiosarcoma requires histopathologic examination. Liver transplantation at an early stage has greatly improved the prognosis of epithelioid hemangioendothelioma. The prognosis of angiosarcoma remains dismal. Designing a worldwide database that contains all data about patients with these rare diseases is recommended.     

Clinical manifestations of primary hepatic angiosarcoma

Malignant tumors of the liver stemming from mesenchymal origins are rare neoplasms, <1% of primary malignant hepatic lesions. Primary hepatic angiosarcoma (PHA) is the most common (36%). This study describes the incidence and clinical characteristics of this rare tumor in two medical centers, over the past 18 years. We reviewed tumor registry files at Jackson Memorial Hospital and oncology data records at Cedar's Medical Center, 1979–1997. A total of 865 primary hepatic tumors were identified, of which five cases (0.58%) were PHA; four were men, and the median age was 53 years. Symptoms and signs included: pain, anemia, fever of unknown origin, weight loss, abdominal mass, and hemoperitoneum. Median survival was only 6 months. In conclusion, primary hepatic angiosarcomas frequently are symptomatic. The presentation and preexisting factors are valuable in establishing a clinical suspicion to diagnose this rare tumor. Although imaging studies are helpful, they are not conclusive, and liver biopsy is usually required.     

肺血管肉瘤的诊断与治疗——附1例报告并文献复习

目的复习肺血管肉瘤的临床表现、病理形态学特点、诊断、鉴别诊断和治疗。方法结合文献报道,回顾性分析5例肺血肉瘤患者临床资料。结果肺血管肉瘤临床症状为胸痛、咳嗽、咯血,肺内多发结节、斑片影,病理检查瘤体主要为血管内衬异型的瘤细胞(血管内皮细胞),可见相互吻合大小不一形态不规则的血管网,免疫组化CD34、CD31和vimentin阳性。结论肺血管肉瘤是罕见的软组织高度恶性肿瘤,预后差。肺血管肉瘤需与肺腺癌、肺结核、真菌等疾病鉴别。     

An unusual cause of diffuse pulmonary haemorrhage

Introduction: Diffuse pulmonary haemorrhage is a potentially life‐threatening complication of a variety of conditions. Tumours, including angiosarcoma lung metastases, are an unusual cause of pulmonary haemorrhage. Methods: Report of a case. Results: This case describes a 38‐year‐old previously healthy male who presented with chest wall bruising followed by haemoptysis and a cerebellar haemorrhage. The patient developed diffuse pulmonary haemorrhage. A biopsy of the chest wall lesion indicated a haematoma and an open‐lung biopsy suggested the diagnosis of vasculitis. The patient died within 3 months after initial presentation and an autopsy revealed a chest wall angiosarcoma with pulmonary and cerebellar metastases. Conclusion: Pulmonary angiosarcoma metastases should be included in the differential diagnosis of diffuse pulmonary haemorrhage, especially in a young, previously healthy patient with normal renal function, particularly as the pathological diagnosis may be difficult and even misleading. Please cite this paper as: Hui C. An unusual cause of diffuse pulmonary haemorrhage. The Clinical Respiratory Journal 2008; 2: 183–186.     

Contrast uptake in primary hepatic angiosarcoma on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging in the hepatobiliary phase

Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.     

Isolated granulocytic sarcoma in the breast

A 16-year-old female, who was diagnosed as having non-Hodgkin lymphoma following a biopsy of a tumor of her right breast, was admitted to our hospital. The diagnosis made by the referring hospital was not reconfirmed because of the pathological findings with suspicion of sarcoma. As another tumor appeared in the contralateral breast 10 days after hospitalization, a second biopsy of the tumor was performed. Touch preparations of the tumor were positive for myeloperoxidase and naphthol AS-D chloroacetate esterase staining. The diagnosis of granulocytic sarcoma (GS) was made. No abnormalities were found in the peripheral blood and bone marrow at this time. Chromosomal examination of the bone marrow was normal. The number of copies for WT-1 mRNA was high both in the bone marrow cells and the tumor cells. The expression of WT-1 mRNA in peripheral blood was not detected. She was treated with the same protocol as for acute myelogenous leukemia and the breast tumor disappeared. The titer of WT-1 mRNA in bone marrow slightly decreased but remained high. Taken together, these findings suggest that the GS seems to be bone marrow origin and the monitoring of WT-1 mRNA may be useful for early diagnosis of any relapse.     

Disseminated histiocytosis with undetermined Langerhans' cells simulating an acute non lymphoid leukemia.

The authors report on a case of disseminated Langerhans' cell histiocytosis with a clinical presentation and a bone marrow simulating, at onset, an acute leukemia non lymphoid. A hepatic needle biopsy performed for the progressive enlargement of the liver oriented the diagnosis towards a Langerhans' cell histiocytosis. The morphological, immunohistochemical and ultrastructural study of these cells showed them to be undetermined, i.e. Langerhans' cell precursors.     

脾边缘带淋巴瘤的临床及肿瘤细胞特征的研究

目的 提高对脾边缘带淋巴瘤（SMZL）的认识和诊疗水平,方法 报告1例典型SMZL,外周血,骨髓及脾脏标本分别采用光镜,相差显微镜,扫描电镜,免疫组化染色,流式细胞术,G显带核型分析及PCR技术研究其肿瘤细胞的生物学特征。结果 本例患者肿瘤细胞为B淋巴细胞,不伴有绒毛,表达CD20,HLA-DR、CD45RA和bcl-2,无异常核型,肿瘤细胞主要浸润脾脏白髓致边缘带明显扩大,脾门淋巴结受累,骨髓和外周血与脾脏有相同的单克隆IgH重排基因,治疗7个月后转为多克隆重排,结论 脾大,外周血或骨髓淋巴细胞比例增高而无淋巴结肿大和白细胞增高患者应疑及SMZL,单克隆IgH基因重排有助于SMZL的诊断,对可疑病例应尽早切脾以明确诊断及防止恶性转化。     

Primary cardiac angiosarcoma treated by complete tumor resection with cardiac reconstruction

Primary cardiac sarcoma is a rare malignant neoplasm, with an incidence of .0001% in collected autopsy series. Angiosarcoma is the most common cardiac sarcoma and is present in up to 33% of the cases that are associated with a poor prognosis. Because angiosarcoma is essentially not responsive to current regimens of chemotherapy and irradiation, early complete resection is recommended as the treatment choice. However, complete resection is difficult because of the limited amount of myocardium and expansion of the tumor at the time of diagnosis. We report a case of right atrial angiosarcoma treated by complete tumor resection with cardiac reconstruction with a bovine pericardium patch.     

Random skin biopsy and bone marrow biopsy for diagnosis of intravascular large B cell lymphoma

Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma in which the lymphoma cells proliferate exclusively in the lumina of small vessels. The diagnosis of IVL requires histological confirmation. Although random skin biopsy from healthy-appearing skin in patients with suspected IVL appeared to be useful, the sensitivity of this method for the diagnosis of IVL remains unknown. We performed a random skin biopsy from 12 consecutive cases of IVL diagnosed at our institution over the past 4 years and evaluate its relevance of clinical and laboratory characteristics, presence or absence of skin lesions, and bone marrow involvement. All 12 patients were diagnosed antemortem by either random skin biopsy or bone marrow biopsy and treated with rituximab-containing chemotherapy. Random skin biopsy was performed in all 12 patients, and the results were positive in ten patients (83.3%). Erythematous skin lesions were seen in 3 of 12 patients, but biopsy was positive for lymphoma lesion in two patients. Bone marrow invasion was seen in 11 of the 12 patients (91.6%) by bone marrow smear and/or flow cytometric analysis, but was detected in only half of the patients by trephine biopsy. We concluded that random skin biopsy from normal-appearing skin is highly sensitive in the diagnosis of IVL comparable to bone marrow trephine biopsy. It should be performed irrespective of the presence or absence of skin lesions in patients who were suspicious of IVL.     

Usefulness of electron microscopy in the diagnosis of cardiac sarcoidosis

Summary A 49-year-old man with cardiac sarcoidosis is presented. He suffered from congestive heart failure, and left ventricular asynergy and reduced function was evident by echocardiogram and left ventriculogram. A light microscopic examination of the endomyocardial biopsy revealed nonspecific myocarditis without giant cells or noncaseating granulomas. Under an electron microscope, however, several epithelioid cells were found in the specimen. The serum level of lysozyme was elevated. The patient had a past history of sarcoidosis of the eyes and lungs 22 years previously. Cardiac diseases presenting epithelioid cells other than sarcoidosis were clinically ruled out. Thus, the diagnosis of cardic sarcoidosis was made based on both clinical and ultrastructural findings, and corticosteroid therapy was initiated. In the second biopsy, performed 4 months later, a noncaseating granuloma was found. Generally, the incidence of histological diagnosis of cardiac sarcoidosis by light microscopy is relatively low in endomyocardial biopsy specimens. The present case suggests that the addition of an ultrastructural examination may improve the diagnostic usefulness of the endomyocardial biopsy in cardiac sarcoidosis, since electron microscopy can clearly identify the presence of even one epithelioid cell.     

Bone marrow histopathology in peripheral T-cell lymphomas

Peripheral T-cell lymphomas (PTCL) account for 10-15% of all lymphoproliferative disorders in the western hemisphere. In PTCL, bone marrow biopsy is performed to establish the diagnosis, rule out other pathology, assess the extent of disease and monitor treatment response. The frequency and histology of bone marrow involvement varies greatly between different clinicopathological entities recognized by the World Health Organisation (WHO) classification, reflecting the differences in the underlying biology. Some lymphomas, such as angioimmunoblastic T-cell lymphoma, show nodular and/or interstitial pattern of infiltration with accompanying reactive changes. Others, including hepatosplenic T-cell lymphoma and large granular lymphocyte leukaemia, are characterized by intrasinusoidal infiltration. In many instances the pathological features are subtle and immunohistochemical and molecular studies are required for the diagnosis. Histological appearances may overlap with a variety of reactive T-cell proliferations and other malignancies. Furthermore PTCL frequently induce secondary changes in the marrow that may obscure the neoplastic infiltrate. The diagnosis often requires critical integration of the information obtained from clinical features, peripheral blood, bone marrow aspirate and biopsy findings. In this article we review the histopathology of bone marrow biopsy in PTCL within the context of the new WHO classification.