Primary pulmonary sarcoma: a clinicopathologic study of 26 cases.

The clinical and pathologic features of 26 primary pulmonary sarcomas were analyzed. Fourteen patients were male and 12 were female; ranging in age from 18 to 75 years (mean, 48 yr). The tumors measured from 0.9 cm in greatest diameter to filling the entire hemithorax. Thirteen tumors were in the left lung and nine in the right lung; one was bilateral, two were in the pulmonary artery, and the location of one tumor was not available. The histologic diagnoses were malignant fibrous histiocytoma (7), synovial sarcoma (6), malignant peripheral-nerve sheath tumor (3), leiomyosarcoma (3), angiosarcoma (2), intimal sarcoma (2), fibrosarcoma (2), and one case of epithelioid hemangioendothelioma. Immunohistochemical and ultrastructural examination supported these diagnoses. Morphologically, the differential diagnosis often included sarcomatoid carcinoma or desmoplastic malignant mesothelioma Patients were treated with surgery, chemotherapy, radiation therapy, or a combination of these. Follow-up was available for 22 patients and ranged from 2 to 183 months (mean, 45 mo). Fourteen patients are free of disease, four died of disease, three are alive with disease, and one died of surgical complications. A variety of sarcomas, especially malignant fibrous histiocytoma and synovial sarcoma, arise within the pulmonary parenchyma. These tumors have the potential to behave aggressively but can be cured by resection, with or without adjuvant therapy. Immunohistochemistry and electron microscopy can be helpful in distinguishing primary pulmonary sarcoma from other tumors in the differential diagnosis.     

Intimal sarcoma of the pulmonary artery: report of an autopsy case

Primary pulmonary artery sarcomas (PASs) are rare and lethal tumors. They are easily misdiagnosed as chronic pulmonary embolism, mediastinal mass or tumor emboli, which delay a proper treatment. Although the advanced technologies are now increasingly being used, their diagnosis is usually hard to establish preoperatively at the present time. We report here a case of a 68-year-old female with PAS with lung metastases, who firstly presented with symptoms of common cold and anemia. Although a PAS had been suspected, the final diagnosis of pulmonary intimal sarcoma was made only postoperatively by histological and immunohistochemical examination. The patient died 8 months after the operation because of tumor growth progression, despite adjuvant chemotherapy and radiation therapy. Although pulmonary intimal sarcomas are usually of poorly differentiated mesenchymal malignancy, most reported cases are immunohistochemically positive for vimentin, alpha-smooth muscle actin (SMA), and/or desmin, therefore resembling leiomyosarcomas. However, the diagnosis of leiomyosarcoma should not be made on the basis of immunostains in the absence of typical morphologic features, and PAS, like the present case, should be more appropriately classified as intimal sarcoma according to the new WHO Classification of Tumours of Soft Tissue and Bone published in 2002.     

Ewing's sarcoma. New clinico-pathological aspects

Ewing's sarcoma is a rare, but highly malignant bone tumor. During the years 1966-1983 we diagnosed and treated this tumor in 84 patients. Diagnosis of the tumor in an early stage is a prerequisite for successful therapy. Clinical and radiological findings contribute to the diagnosis, but histologic confirmation is indispensible. In addition to histomorphology we regard electron microscopy and the demonstration of glycogen as essential for the final diagnosis. The differential diagnosis includes haematogeneous osteomyelitis, malignant lymphoma, metastases from neuroblastomas, rhabdomyosarcoma and some benign bone tumors. New observations concerning the histogenesis of Ewing's sarcoma are discussed. The introduction of chemotherapy as part of a multimodality approach to treatment has substantially improved the prognosis in comparison to the previous results with surgery and radiation therapy. The role of surgery and radiation independently and in combination is discussed. We propose an approach to treatment based on our experience which involves surgical removal of the primary tumor after preoperative chemotherapy. The overall survival rate among our 84 patients was 40%. Among those who were diagnosed early and treated according to the recommended protocol 54% are alive.     

Liposarcoma of the pleural cavity: clinical and pathologic features of 4 cases with a review of the literature

BACKGROUND: Primary liposarcomas of the pleura are extremely rare malignancies, and relatively few reports appear in the world literature. DESIGN: We compiled a small series of 4 cases of primary pleural liposarcoma from the files of the Armed Forces Institute of Pathology (Washington, DC) and compared the histopathologic and clinical features of these 4 cases with those of 9 previously published cases. RESULTS: Our investigation included the case studies of 9 men and 4 women, aged 19 to 80 years (average, 49 years). Histologic subtypes in the 9 cases with available information included 5 myxoid liposarcomas, 1 well-differentiated liposarcoma, and 3 liposarcomas with mixtures of histologic types. Surgical resection with or without chemotherapy appeared to be the most common form of treatment, although radiation therapy was used in some cases and seemed beneficial. Survival information was available for 11 cases; 4 patients died of disease at 7, 9, 12, and 19 months; 1 died of heart failure 2 days after presentation; 1 died of unknown causes 16 months after presentation; and 3 patients were alive without tumor at 5, 16, and 66 months after diagnosis. One patient had local recurrence at 2 years. A second surgical resection in this patient failed, and he died of disease 9 years after initial presentation. A second patient experienced recurrence at 4 years and was free of disease 4 years after the second surgical resection. CONCLUSIONS: Primary pleural liposarcomas occur predominantly in older men, and the myxoid histologic subtype is the most common. Radiographic or surgical evaluation is important to distinguish primary pleural liposarcoma from chest wall or mediastinal sarcomas, as well as metastases from other sites. Although further investigation is needed, evidence from the cases reviewed here indicates that surgical resection with adjuvant radiation therapy may benefit patients with primary pleural liposarcoma.     

Leiomyosarcoma of the head and neck: a clinicopathological study

AIMS: The behaviour of leiomyosarcoma is site-related and there are few data on such tumours located in the head and neck. We studied the clinicopathological features of these lesions. METHODS AND RESULTS: Cases diagnosed as leiomyosarcoma of the head and neck were retrieved from the archives of three institutions. Immunohistochemistry was performed and follow-up information was obtained. There were seven men and six women, aged 21-73 years, and lesions involved the neck (n=3), maxilla (n=4), buccal area (n=3), and maxillary sinus, nose, and pharynx (n=1 each). Tumours ranged from 10 to 80 mm. All tumours showed at least focally typical histological features of leiomyosarcoma with perpendicularly arranged fascicles of smooth muscle cells with blunt-ended nuclei, eosinophilic cytoplasm and paranuclear vacuoles. They expressed muscle-specific actin (8/9), smooth muscle actin (7/9), and desmin (10/12). Follow-up information was available on nine patients. All had surgery, with radiation and/or chemotherapy in seven. Three (27%) recurred at 6-24 months; in one case twice. Five (56%) had metastases, including the three with prior recurrences at 1-128 months. Five (including two who received adjuvant therapy) were disease-free at a median of 47 months, one was alive with metastatic disease at 24 months), and three were dead of disease (median 13 months). CONCLUSIONS: Head and neck leiomyosarcomas are rare and aggressive neoplasms which metastasize. Adjuvant therapy has limited effect.     

Primary and metastatic cardiac sarcomas: a 12-year experience at a German heart center

Background: Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation. To date, there exist no uniform guidelines for their surgical and/or oncological treatment. Methods: We evaluated retrospectively all patients undergoing cardiac surgery for primary or secondary cardiac sarcoma in the period 1999-2011 at the Erlangen Heart Centre to analyze their clinicopathological spectrum, treatment and outcome. Results: Five patients (3 women & 2 men; mean age: 46 years; range: 28-81 years) had primary cardiac sarcomas (6.7% of primary cardiac tumors) and 4 had cardiac metastasis from soft tissue sarcoma (1 case each of osteosarcoma, myxoid liposarcoma, alveolar soft part sarcoma and pleomorphic spindle cell sarcoma). Primary sarcomas were located in the left atrium (n=3), left ventricle (n=1) and right atrium (n=1). Histological types were myxosarcoma (3), pleomorphic undifferentiated sarcoma (1) and angiosarcoma (1). Four patients died at 2-64 months (mean, 24.5 months). Sarcoma metastasis to the heart developed at a mean of 109.5 months from initial diagnosis (range, 5-240 months). Three of them died of disease at a mean of 14 months after cardiac surgery and one is disease free 34 months after heart transplantation for metastasis. Conclusions: Primary and metastatic cardiac sarcomas are very heterogeneous in their histological appearance, clinical presentation and course of the disease. Radical surgery combined with chemoradiation is promising in patients with resectable disease and may significantly prolong survival. Cardiac transplantation represents an emerging strategy for patients with isolated unresectable cardiac involvement.     

单核母细胞肉瘤的临床病理观察及免疫表型分析

目的探讨单核母细胞肉瘤的临床病理特征、诊断和鉴别诊断。方法从1990-2006年诊断的102例髓系肉瘤患者中筛选出5例单核母细胞肉瘤,进行临床病理回顾性研究和随访,以SP法检测其免疫表型。结果5例中男性3人,女性2人,2例为儿童,3例为成人。病程1周～12个月不等。4例有多处浅表淋巴结肿大;2例有多发性皮肤病变。免疫表型：5例瘤细胞均表达CD68（KP1）、CD68（PGM1）、溶菌酶和CD45;均不表达髓过氧化物酶、CD15、CD163、脱氧核苷酸转移酶、CD117以及T和B细胞分化抗原。Ki-67指数为40％～80％。5例患者均有随访,4例死亡,1例存活,平均生存时间6．25个月。结论单核母细胞肉瘤很少见,且预后差;在形态学上难以与粒细胞肉瘤和其他小细胞肿瘤相区别,须借助于免疫表型检测确诊。     

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.     

Rhabdomyosarcomas developing in association with mediastinal germ cell tumours

Summary Two mediastinal rhabdomyosarcomas that arose in association with germ cell tumours are reported. One presented as a small component of a mixed germ cell tumour with yolk sac and immature teratomatous elements. The other appeared as a large mass 4 months after diagnosis of a yolk sac carcinoma that had been treated with chemotherapy. The first patient was alive and free of disease 7 years later, whereas the second died of tumour 3 months post-operatively. The proportion of rhabdomyosarcoma within the germ cell tumours appears to have influenced the prognosis of these patients. This observation emphasizes the necessity of performing a thorough search for sarcomatous elements and quantifying their relative proportion in germ cell tumours of the mediastinum.     

滤泡树突细胞肉瘤的临床病理分析

目的 探讨滤泡树突细胞肉瘤(FDC肉瘤)的临床病理特征及其鉴别诊断.方法 对10例FDC肉瘤行HE染色、免疫组织化学EnVision法染色,对其中5例进行原位杂交,并对7例进行了随访.结果 10例患者中男5例,女5例,平均年龄42岁.发生于结内的6例,主要位于颈部和腹膜后淋巴结;发生于结外的4例,分别位于扁桃体、盆腔、胰尾部和脾脏.镜下观察瘤细胞呈旋涡状、席纹状或片块状排列.肿瘤细胞呈合体状、胖梭形或上皮样,胞质红染,边界不清,细胞核圆形或卵圆形,空泡状,核仁清楚,核分裂象多少不等.偶见核内假包涵体及多核瘤巨细胞.小淋巴细胞或中性粒细胞夹杂于瘤细胞间或围绕血管周围.瘤细胞CD21、CD23、CD35及D2-40呈弥漫或灶状阳性,少数病例瘤细胞上皮细胞膜抗原、CD68、S-100蛋白阳性,白细胞共同抗原、CD20、CD3、CD1a、HMB45、细胞角蛋白均阴性.EBER原位杂交,1例炎性假瘤样FDC肉瘤阳性,另外4例经典型FDC肉瘤为阴性.随访7例,随访2～39个月(平均14个月).2例伴有副肿瘤性天疱疮的患者分别于确诊半年和7个月后死于肺部感染,其余5例经手术切除或辅以放疗和化疗后,无复发和转移.结论 FDC肉瘤是一种少见类型的低至中度恶性肿瘤.CD21、CD35及D2-40的应用有助于其正确的诊断和鉴别诊断.多数病例局部手术切除或辅以放疗和化疗,预后较好,伴有副肿瘤性天疱疮的患者预后差.     

Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature

To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.     

Pararenal leiomyosarcoma of the inferior vena cava

A complete surgical resection is the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma of the inferior vena cava (IVC). Reconstruction of the IVC is not always necessary but is often required to facilitate venous drainage of the kidney for the tumors at the pararenal area of the IVC. Controversy exists in postoperative adjuvant therapy. Recently, we experienced four cases of pararenal leiomyosarcoma of the IVC, of which treatment consisted of a complete resection of the tumor, ringed polytetrafluoroethylene (PTFE) graft interposition, and bilateral renal vein reconstructions in all patients. Postoperative radiation therapy was instituted in 3 of 4 patients. One patient who did not receive the postoperative radiation therapy was treated with adjuvant chemotherapy. The kidneys were preserved in all patients and no deep vein thrombosis (DVT) or venous insufficiency of the lower extremity veins developed. Distant metastasis to the lung was noted in one patient at 18 months after surgery, who was not received the postoperative radiation therapy but chemotherapy. In conclusion, a complete resection of the tumor, IVC reconstruction, and bilateral renal vein reconstruction followed by adjuvant radiation therapy is recommended for the treatment of pararenal leiomyosarcoma of the IVC.     

Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) – analysis of four cases and literature review

In accordance with recent terminology, it is proposed that malignant mesenchymoma should be renamed ‘composite sarcoma’ and defined as ‘a sarcoma composed of two or more cellular types each of which is sufficiently differentiated to permit clear recognition of its histogenetic type microscopically, immunohistochemically or ultrastructurally; excluding fibrosarcomatous and high‐grade pleomorphic undifferentiated sarcomatous component, dedifferentiated sarcoma and the combination of osteosarcoma and chondrosarcoma which is regarded as a single histogenetic type’. Four cases of primary osseous composite sarcoma (POCS) were identified among 928 primary bone sarcomas. Their age ranged from 10 to 87 years, peak incidence in the second decade with equal sex distribution. Most presented with pain, commonest in the knee, affecting the metaphysis, appearing radiologically as expansile infiltrative osteolytic lesions with cortical erosion, periosteal reaction, variable extent of osteoblastic areas and soft tissue extension. All contained variable amounts of conventional high‐grade osteosarcoma with or without chondrosarcoma component; the other constituents were liposarcoma, rhabdomyosarcoma and leiomyosarcoma. In all cases, Ki67 proliferative index was over 35%, there was no CDK4 and MDM2 amplification. The absence of low‐grade component supported the de novo origin of POCS rather than derivation from divergent dedifferentiation. The two older patients with hitherto undescribed osteoleiomyosarcoma died 2 and 10 months after operation, whereas the two younger with osteorhabdomyosarcoma and osteoliposarcoma enjoyed disease‐free survival at 16 and 6 years after chemotherapy despite the latter showing lung metastasis at presentation. Identification of the different lines of differentiation together with their approximate amounts and histological grades is therefore mandatory for POCS as multi‐agent chemotherapy catered for each sarcoma component might offer hope for long‐term disease‐free survival.     

Clear cell sarcoma of the kidney: A clinicopathologic study of 21 patients with long-term follow-up evaluation

The clinical and pathologic features of 21 children with clear cell sarcoma of the kidney are presented. These cases were identified among 517 primary renal tumors collected from four pediatric institutions. Abdominal mass and hematuria were the usual presenting signs. The male to female ratio was 1.3 to 1. Although the mean age at diagnosis was 3 years, the sarcomas were diagnosed earlier in boys (2 years, 7 months) than in girls (3 years, 7 months). Tumor size and weight may have accounted for this difference, since the average tumor weights were 899 g for boys and 635 g for girls. Right kidney locations predominated (14 cases). The most common site for metastasis was the skeleton (12 patients), and the skull was almost invariably involved (10 children). Bone metastases preceded the development of metastases elsewhere in nine patients, in five of whom the involvement was polyostotic. Of the 12 patients with osseous metastases, nine died within five years of diagnosis, and one died eight years and nine months after diagnosis; the remaining two patients were successfully treated with a combination of surgery, radiotherapy, and actinomycin D, vincristine, cyclophosphamide, and Adriamycin. Eight of the nine patients with lung metastases died within five years of diagnosis. Until substantial proof is found that clear cell sarcoma of the kidney is related to nephroblastoma, its identification as a non-Wilms' sarcoma would seem to be appropriate. This tumor is not the only sarcoma of the kidney, but it appears to be the most common renal sarcoma of childhood.     

Leiomyosarcoma with dedifferentiation in a premenopausal patient discovered after uterine artery embolization

Although a hysterectomy is the most common treatment for relieving the symptoms attributable to uterine leiomyomas, uterine artery embolization (UAE) is now being used more frequently as an alternative to a hysterectomy. However, it is difficult to differentiate a leiomyoma from a leiomyosarcoma without performing a pathological examination. Reported herein is a rare case of leiomyosarcoma that showed dedifferentiation of the tumor cells after UAE. A premenopausal 48-year-old woman had been suffering from hypermenorrhea for 4 years before visiting the clinic. She underwent UAE for suspected symptomatic leiomyoma. Two months later, dilatation and curettage was performed because of genital bleeding and a necrotic mass was submitted for pathological examination. Three months after curettage, with renewed symptoms, endometrial biopsy was done, which confirmed pleomorphic sarcoma. Metastatic nodes to the lung were also found at that time. Multiple leiomyosarcomas and a leiomyosarcoma showing dedifferentiation of the uterine body were found on pathological examination. The patient had metastatic nodes to the brain later and died of metastatic disease 20 months in total after UAE. This is a rare case of leiomyosarcoma with dedifferentiation and multiple metastases occurring after UAE, suggesting that dedifferentiation could be derived from ordinary leiomyosarcoma and that the traumatic effect of curettage might cause early metastasis. The present case is a warning that careful and detailed evaluation of the uterine tumor are needed before UEA.     

Inflammatory pseudotumor and sarcoma of urinary bladder: differential diagnosis and outcome in thirty-eight spindle cell neoplasms.

We assessed diagnostic criteria among 38 spindle cell tumors of the urinary bladder and obtained follow-up in 36 patients. Patients comprised 28 males and 10 females aged 2.5 months to 87 years. Hematuria was the commonest presenting symptom (27 patients). After review and immunohistochemical workup, 17 patients had inflammatory pseudotumor (myofibroblastic tumor), 4 postoperative spindle cell nodule, 1 leiomyoma, 13 sarcoma (7 low-grade; 6 high-grade), and 3 carcinoma. Mean age was 38 years for pseudotumor (range 15 to 74), 65 for postoperative spindle cell nodule, 51 for sarcoma, and 76 for carcinoma. Size of pseudotumor averaged 4.4 +/- 0.7 cm (range 1.5 to 13.0), similar to sarcoma, 4.0 +/- 0.6 cm (range 0.5 to 7.0). Similar proportions of benign tumors and sarcomas had muscularis propria invasion. The criteria that best differentiated sarcoma from inflammatory pseudotumor were presence of necrosis at the tumor-detrusor muscle interface in muscle-invasive cases, and nuclear atypia. Sarcoma also had less prominent microvasculature, less variable cellularity, consistently > or =1 mitotic figure per 10 high-power fields, and predominant acute inflammation without plasma cells. p53 protein nuclear immunostaining was moderate, unlike the rare to absent staining in pseudotumors. Because all 12 sarcomas were desmin-negative, we did not call them leiomyosarcoma; they overlapped with benign tumor in epithelial, mesenchymal, and actin immunostaining. Among 12 sarcoma patients, 2 died of tumor (at 3 months). Two of four experienced tumor recurrence after partial cystectomy (2 and 26 months). No pseudotumors recurred after transurethral resection or partial cystectomy, although one patient, 5 months after transurethral resection, had histologically identical pseudotumor that the surgeon considered residual. Another patient with pseudotumor, not a candidate for tumor ablation after transurethral resection, had continued tumor growth and he died of urosepsis. In conclusion, inflammatory pseudotumor, although overlapping with sarcoma in presentation, age range, and size, does not metastasize and remains histologically distinct from low-grade sarcoma.     

Leiomyosarcoma of the inferior vena cava: three case reports and review of the literature

We describe 3 cases of leiomyosarcoma of the inferior vena cava (IVC) and review the literature describing clinicopathologic features of 211 cases and the outcome. Of these, 74% of the cases affected women with median age of 52 years. The most common symptoms were abdominal pain or mass (57%), Budd-Chiari syndrome (17%), and deep vein thrombosis (4%). The most frequent site of tumor origin is the middle segment of the IVC (33%). Tumor size ranged from 2 to 38 cm (mean, 12 cm). Of the tumors with an assigned grade, 46% were high grade, 17% were intermediate grade, and 36% were low grade. Of all patients, 47% underwent complete resection, 24% had complete resection with preoperative or postoperative chemotherapy and/or radiation, and 5% had palliative surgery. Tumor recurrence occurred in 40% of the patients (11% had local recurrence and 29% had metastasis). Perioperative mortality occurred in 4% of the cases. Of those patients who died, 42% died of the disease, 2% died of other causes, 26% were alive and free of the disease, 14% were alive with recurrent disease, and 11% were lost to follow-up. Tumors involving level 2 of the IVC have the best prognosis and tumors of level 1 have the worse prognosis. Although there is no standardized criteria for the grading of extrauterine leiomyosarcoma, we propose to grade based on mitotic activity as follows: high grade, 10 or more mitoses per 10 high-power field (HPF); intermediate grade, 5 to 9 mitoses per 10 HPF; and low grade, 1 to 4 mitoses per 10 HPF.     

上皮样血管肉瘤的临床病理分析

目的 分析上皮样血管肉瘤的临床病理特点及需进行鉴别诊断的肿瘤。方法 利用HE染色,免疫组织化学抗生物素蛋白-生物素复合物法（ABC法）观察15例上皮样血管肉瘤,电镜观察其中12例,结果 15例中12例为男性,3例为女性,发生部位;腹膜后3例,四肢软组织3例,肌骨4例,甲状腺2例,眶上缘1例,胸壁软组织1例,颈部软组织1例。临床症状包括局部肿物及其引起的疼痛,消瘦,乏力。影像学检查显示肿瘤多为实性或囊实性,直径可达3-20cm。镜下观察瘤组织或弥漫成片或聚集成巢,伴有出血和多灶坏死,瘤组织内常见大小不等的不规则腔隙,衬附异型瘤细胞,单个瘤细胞或多个瘤细胞形成空腔包绕红细胞。电镜可观察到胞质内空泡,W-P小体,中间丝及细胞基底膜的存在,瘤细胞可表达CD34,CD31,Ⅷ因子相关抗原,细胞角蛋白,上皮膜抗原,血管周细胞表达平滑肌肌动蛋白,临床主要采用手术切除的治疗方法,偶尔辅以放疗。随访/2例存活,术后存活期分别为19个月,7年;12例已死亡,术后存活期最短为2周,最长38个月,平均19个月;1例失访。8例患者发生肺,骨,淋巴结和腹腔转移。结论 上皮样血管肉瘤是高度恶性肉瘤,免疫组织化学检测和电镜观察有助于确定肿瘤的内皮分化,上皮样血管肉瘤需与转移癌,上皮样血管内皮细胞瘤,恶性黑色素瘤,上皮样肉瘤等鉴别。     

Primary angiosarcomas of the anterior mediastinum:: A clinicopathologic and immunohistochemical study of 9 cases

We report 9 cases of primary angiosarcomas of the anterior mediastinum. Patient ages ranged from 25 to 62 years (mean, 40.7 years); 5 patients were male and 4 were female. Main presenting symptoms included chest pain, dyspnea, and cough. The tumor size ranged from 3 to 12.5 cm. Macroscopically, the lesions were ill-defined hemorrhagic masses. Histologically, the growth patterns ranged from large vascular spaces to capillary-like proliferations. These were either lined by bland or more pleomorphic endothelial cells. The mitotic activity was variable and corresponded to the degree of differentiation. A rim of thymic tissue was observed in 2 cases suggesting a thymic origin of the tumors. No teratomatous components were identified. Immunohistochemical studies showed that all 9 cases were reactive for vascular markers including factor VIII–related antigen, CD31, and CD34, and negative for cytokeratin CAM5.2. All cases were treated by complete resection and 3 patients received adjuvant chemotherapy. Follow-up information available for 6 patients revealed that 4 were alive and free of disease at intervals ranging from 6 to 36 months after diagnosis and 1 was alive with recurrence at 48 months. One patient had died of the disease 10 months after diagnosis. Primary angiosarcomas of the anterior mediastinum are rare tumors that need to be added to the differential diagnosis of primary anterior mediastinal neoplasms. Despite their histologic similarity to angiosarcomas at other sites, primary angiosarcomas of the anterior mediastinum appear to follow a more protracted clinical course than their counterparts in other organ systems.