Clinical behavior of solitary fibrous tumors of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura are rare and present unpredictable clinical behavior. METHODS: Between 1981 and 1998, 11 solitary fibrous tumors of the pleura were resected in 10 patients at the University Hospital of Geneva. Their clinical behavior and outcome were reviewed. RESULTS: Seven tumors arose from the visceral pleura, and three arose from the parietal pleura. Tumors arising from the parietal pleura were revealed to be more difficult to resect than those from the visceral pleura because of their size and adhesion to the chest wall requiring extrapleural resection. Eight tumors showed benign features, whereas two showed distinct features of malignity. One additional patient presented marked pleomorphism that could represent an intermediate form before frank malignity. Four tumors had been followed expectantly for 2 to 10 years before surgery. Although three enlarged rapidly, no signs of malignity were observed on histological examination. All patients are alive, from 2 months to 14 years after surgery (mean 55 months). In one case, however, a malignant tumor recurred 6 years after resection of a benign variant. CONCLUSIONS: Although histologically benign, solitary fibrous tumors of the pleura may enlarge rapidly and occasionally transform into malignant variants after several years. Therefore, complete surgical resection and long-term follow-up is recommended for all patients.     

Giant solitary fibrous tumor of the pleura

Fibrous tumors of the pleura are rare, accounting for <5% of all pleural neoplasms. Although over 80% of pleural fibrous tumors have a benign course, local recurrence postsurgery and occasional malignant transformation have been reported; complete excision of the tumor together with postsurgery follow-up of all patients is therefore recommended. We report on a solitary fibrous tumor of the pleura measuring 30?cm and weighing 3560?g.     

Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome

Objective: The aim of this paper is to study clinical characteristics, surgical treatment and outcome of patients with solitary fibrous tumor of the pleura operated in our institutions in a 20-year period. Methods: Clinical records of all patients operated for solitary fibrous tumors of the pleura between 1981 and 2000 were reviewed retrospectively. Tumors were classified as malignant in the presence of at least one of the following criteria: (1) high mitotic activity; (2) high cellularity with crowding and overlapping of nuclei; (3) presence of necrosis; (4) pleomorphism; otherwise they were considered as benign. Results: Sixty patients (mean age 55 years) were operated in this period. None had asbestos exposure. Symptoms were present in 31 cases. Surgical approaches included thoracotomy (n=53), video-assisted thoracoscopy (n=6), and median sternotomy (n=1). Tumors originated from visceral pleura in 48 cases, from parietal, mediastinal or diaphragmatic pleura in seven, two and three cases, respectively; their mean diameter was 8.5 cm. Tumors could be resected with their implantation basis in 49 patients. In the remaining 11, extended resections were performed, including lung parenchyma (lobectomy, n=4, pneumonectomy, n=2), osteomuscular chest wall structures (n=2), diaphragm (n=2), and pericardium (n=1). Two postoperative deaths (due to myocardial infarction and pulmonary embolism, respectively) occurred. Tumors were pathologically benign in 38 cases and malignant in 22 cases. Mean follow-up was 88 months. Resection was complete in all the patients with benign tumors and no recurrence occurred. Resection was considered as complete in 21/22 malignant tumors. Local recurrence was observed in two cases. Both could be successfully managed by iterative exeresis (no extended resection had been initially performed). Metastatic disease (responsible for patient's death) was observed following the only incomplete resection. Actuarial 5- and 10-year survival rates were 97% for benign tumors and 89% for malignant ones. Conclusions: Surgical resection provided cure in all the patients with benign tumors. As insufficiency of exeresis is associated with all recurrences in malignant tumors, completeness of resection is in our experience the best prognostic factor in these forms.     

Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases

We reviewed 223 localized fibrous tumors of the pleura and divided them histologically into 141 benign and 82 malignant neoplasms. The criteria used for a judgement of malignancy were high cellularity and mitotic activity (more than four mitotic figures per 10 high-power fields), pleomorphism, hemorrhage, and necrosis. The tumors occurred equally in both sexes, most commonly in the sixth to seventh decades of life. Presenting symptoms included chest pain, dyspnea, and cough; they were observed in three-fourths of patients with a malignant tumor. One in every four of these patients had hypoglycemia, clubbed digits, or pleural effusion. Two-thirds of the tumors were attached to visceral pleura, often by a pedicle. The rest arose from the parietal pleura of the chest wall, diaphragm, or mediastinum. Neoplasms in these atypical sites, together with fissural lesions and tumors "inverted" into peripheral lung, were more often malignant. Most neoplasms measured 5-10 cm and weighed 100-400 g. Microscopically, the "patternless pattern," or hemangiopericytic type, was seen in the majority of cases, and mixed patterns were seen in nearly 40% of tumors. Of the 169 tumors where follow-up was available, all of the benign and 45% of the malignant tumors were cured by simple excision. Patients surgically cured of a malignant neoplasm had pedunculated or well-circumscribed lesions. However, 55% of patients with malignant tumors succumbed to their disease secondary to invasion, recurrence, or metastasis. Resectability is the single most important indicator of clinical outcome. No tumor expressed epithelial differentiation, either immunohistochemically or ultrastructurally; therefore, we favor the term "localized fibrous tumor" of pleura instead of "localized mesothelioma."     

Three types of spindle cell tumors of the pleura. Fibroma, sarcoma, and sarcomatoid mesothelioma

The clinicopathologic features of 17 fibrous tumors of the pleura are presented. Eight were benign localized fibrous tumors; all of these were negative when stained with antibodies to keratin. Eight were diffuse malignant tumors that demonstrated intense immunohistochemical staining of the spindle cells with antibodies to keratin. One case presented as a histologically malignant spindle cell tumor that was initially localized but recurred subpleurally three times in 12 years. This spindle cell tumor was consistently negative when stained for keratin. We conclude that there are three types of fibrous tumors of the pleura: (a) a localized, histologically benign, keratin-negative spindle cell tumor that might be termed "fibroma," (b) a diffuse, histologically malignant, keratin-positive neoplasm that might appropriately be termed a "sarcomatoid mesothelioma," and (c) a histologically malignant, keratin-negative, spindle cell tumor with the potential to spread under the pleura that might appropriately be termed a "sarcoma." Immunohistochemistry proved to be useful in distinguishing the sarcomatoid mesothelioma from the sarcoma.     

Solitary Fibrous Tumor of the Pleura: An Analysis of 13 Cases

BACKGROUND: Solitary fibrous tumor of the pleura is a rare soft-tissue tumor. In search of appropriate diagnosis and treatment methods, we present our experience with 13 patients. METHODS: The medical charts of 13 patients treated during the last 5 years were reviewed, as well as pathological records, including immunohistochemical stains. Follow-up data were obtained. In addition, a literature review with regard to treatment and clinical outcome was performed. RESULTS: Our series consisted of four men and nine women with a mean age of 47 years. Two were diagnosed before operation with ultrasonography-guided core needle biopsy. All patients underwent primary surgical treatment, and four of them were resected by video-assisted thoracic surgery. Seven tumors were malignant and the other six were benign. Immunohistochemical staining showed nestin was positive in three malignant solitary fibrous tumors of pleura (3/7), which were negative for CD34. Except for one, all patients were followed-up for 3 to 35 (mean, 14.5) months. Among them, one patient experienced a recurrence and one patient died of brain metastasis. CONCLUSION: Ultrasonography-guided core needle biopsy combined with immunohistochemical analysis might be a safe and rapid method to provide a confirmatory diagnosis before resection. For smaller, pedunculated tumors, video-assisted thoracic surgery could be a powerful and useful approach. We speculate that CD34-negative and nestin-positive might be a malignant marker for solitary fibrous tumor of pleura.     

Giant localized fibrous mesothelioma: an unusual large intrathoracic tumor

BACKGROUND: Localized fibrous mesotheliomas are rare intrathoracic tumors arising from the pleural tissue. They are mostly benign tumors, with dimensions ranging from a small nodule to a large intrathoracic tumor. CASE: This paper describes the presence of giant localized fibrous mesothelioma filling the lower left pleural cavity, which developed over a 20-year period. Surgical resection of the tumor showed a large, localized fibrous mesothelioma 14 cm in diameter. CONCLUSIONS: The clinical manifestations of localized fibrous mesotheliomas are very variable. Small tumors may be asymptotic, while large tumors may cause respiratory, cardiac or metabolic symptoms. Complete surgical resection is the preferred treatment and is usually curative. Careful follow-up is indicated because recurrence may occur, even many years after the initial operation.     

Solitary fibrous tumors of the pleura

Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognized over the past few years. The tumor was initially described in the pleura, but it has been reported in many other sites lately. Although the majority of these tumors have a benign course, the malignant form still remains enigmatic. Indeed, the behavior of these tumors is often unpredictable and does not always correlate with histologic findings. In addition, benign tumors may remain unproblematic for several years before changing into a malignant form. In order to define more precisely the clinical behavior of solitary fibrous tumors of the pleura, we reviewed the literature with particular attention to the clinical presentation, histopathologic characteristics, and cytogenetic differentiation of these tumors. A staging system and an algorithm for the management and follow-up of these patients are proposed.     

Solitary fibrous tumour of the pleura: surgical treatment.

OBJECTIVE: Solitary fibrous tumours (SFT) of the pleura are rare tumours originated from the mesenchimal tissue underlying the mesothelial layer of the pleura. This tumours present unpredictable clinical course probably related to their histological and morphological characteristics. METHODS: Twenty-one patients affected by SFT of the pleura were referred to us for surgical resection from September 1984 to April 2000. They were 15 males and six females with median age of 51 (range 15--73) years. Nine patients (43%) were symptomatic and predominant clinical symptoms or signs were dyspnoea (19%), coughing (14.3%), chest pain (28.5%), finger clubbing (14.3%) and hypoglycaemia (14.3%). Hypoglycaemia was related to a pathological incretion of insulin-like growth factor 2 by the tumour. Chest radiograph and computed tomography of the chest revealed intra-thoracic homogeneous sharply delineated round or lobulated mass sometimes associated with ipsilateral pleural effusion (19%) or causing pulmonary atelectasis with opacification of the complete hemithorax (19%). Surgical excision required 14 posterolateral thoracotomies, six anterior thoracotomies and one video-assisted thoracoscopy. Thirteen tumours arose from visceral pleura and wedge resection was performed, seven tumours arose from parietal pleura and extrapleural resection was carried out without any chest-wall resection, one tumour growth within the upper left lobe and required lobectomy. Tumours weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. At cut section seven cases (34%) revealed focal necrosis and hemorrhagic zones and on light microscopy six cases (28.5%) were characterized by high mitotic count: characteristics related with uncertain clinical behaviour. Immuno-histochemical reactions were in all cases positive for CD34. RESULTS: In all our patients resections were complete. Paraneoplastic syndromes like hypoglycaemia and clubbing receded after surgery. No intraoperative or perioperative medical or surgical complications occurred. Median chest-drain duration timed 3 (range 2--5) days and median hospital stay was 5 (range 4--7) days. Perioperative mortality rate was 0%. Median follow-up was 68 (range 2--189) months: during this period patients were submitted to chest X-ray with 6-months interval to evaluate possible local recurrence. Only one patient experienced tumour recurrence after 124 months follow-up: the tumour was suspected after observation of finger clubbing. The tumour was detected and excised by redo-thoracotomy. CONCLUSIONS: Surgical resection of benign solitary fibrous tumours is usually curative, but local recurrences can occur years after seemingly adequate surgical treatment. Malignant solitary fibrous tumours generally have a poor prognosis. Clinical follow-up and radiological follow-up are indicated for both benign and malignant solitary fibrous tumours.     

Giant Benign Fibrous Tumor of the Pleura in a Pregnant Woman: Report of a Case

While most pleural neoplasms are malignant and associated with asbestos exposure, benign tumors may also occur. Benign fibrous tumors of the pleura are rare and, unless diagnosed and resected early, they may reach an enormous size and cause severe symptoms. We report the case of a pregnant woman with a giant benign fibrous tumor localized in the pleura. Received: February 8, 2002 / Accepted: July 2, 2002 Reprint requests to: A. Cakan, Ege Universitesi Tıp Fakultesi, Gogus Kalp Damar Cerrahisi Anabilim Dali, Bornova, 35100 Izmir, Turkey     

Immunohistochemistry and surgical approaches in solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura (SFTP) is a rare, benign, slow-growing neoplasm that arises from the submesothelial cells of the pleura. Usually, resection of the tumor and adjacent structures are sufficient for resolution. Nowadays, videothoracoscopy (VTC) allows adequate access for the surgical treatment of these tumors. CD34 antigen positivity is a differential feature with mesothelioma. We present our experience with 15 patients with SFTP (nine women and six men) who underwent surgical resection in the last 12 years (10 thoracotomies, one sternotomy and four VTC). Only four patients were symptomatic at diagnosis. In our opinion, VTC is a less invasive diagnostic and therapeutic approach than thoracotomy that provides an adequate approach for the resection of SFTP in selected patients. Because of the malignant potential of this tumor, long-term follow-up is mandatory.     

电视胸腔镜手术治疗胸膜孤立性纤维瘤的临床分析

目的探讨电视胸腔镜手术（VATS）治疗胸膜孤立性纤维瘤（SFTP）的价值,总结微刨治疗经验。方法1999年2月至2010年10月VATS治疗SFFP患者19例,其中全胸腔镜手术14例,胸腔镜辅助小切口（6～8cm）手术3例,胸腔镜联合后外侧大切口（15cm）手术2例。结果全组均完整切除肿瘤,手术顺利,无并发症及围手术期死亡。术中证实13例肿瘤起源于脏层胸膜有蒂,5例起源于壁层胸膜无蒂宽基底,1例起源于纵隔胸膜有蒂。主要诊断依据免疫组化分析,19例患者的Vimentin和CD34均阳性表达;病理诊断良性17例,恶性2例。平均住院时间6d（4-11d）。术后平均随访51个月,无复发和转移。结论VATS创伤小,可作为治疗SFTP的首选方法,值得推荐。     

头皮恶性肿瘤外科切除后的修复

目的 总结不同手术方法修复与重建头皮恶性肿瘤术后缺损,评价其临床效果. 方法 1995 年1 月-2004 年9月,对70例头皮恶性肿瘤患者行手术治疗,其中男41例,女29例;年龄30～85岁,平均50.3岁.病程2周～3年,平均 3.5 个月.基底细胞癌 31 例,鳞状细胞癌24例,恶性黑色素瘤8例,纤维肉瘤4例,脂肪肉瘤2例,血管肉瘤 1 例.病变范围 1.0 cm×0.5 cm～10.0 cm×8.0 cm.根治性切除肿瘤后缺损3 cm×3 cm～12 cm × 11 cm,采用中厚皮片移植 51 例,邻近头皮瓣移位12例,颈肩随意皮瓣移位2例,斜方肌肌皮瓣移位3例,桡动脉逆行岛状皮瓣2例,切取范围 5 cm×4 cm～18 cm × 12 cm. 结果 67 例皮片及皮瓣成活,伤口Ⅰ期愈合;2例皮瓣远端坏死,经过换药后Ⅱ期愈合;1 例桡动脉逆行岛状皮瓣感染,对症处理后愈合.供区均Ⅰ期愈合.55例患者获随访 1～5年,5例复发.植皮患者中鳞状细胞癌患者和纤维肉瘤患者各1例,分别于术后1年及2年半复发,均再次行根治性切除后采用皮瓣移位修复刨面;皮瓣移位患者中血管肉瘤患者及鳞状细胞癌患者各1例于术后6个月及3个月复发放弃治疗,1 例纤维肉瘤患者术后2年复发,再次根治性切除后采用皮瓣移位修复创面.余患者均存活良好. 结论 头皮恶性肿瘤应早期诊断、及时治疗,广泛彻底切除并进行合理的创面修复是有效的治疗方法.     

Solitary fibrous tumor in the extremity: case report and review of the literature

A solitary fibrous tumor is a relatively unusual neoplasm first described as a distinctive tumor arising from pleura. Some reports have shown that solitary fibrous tumors also affect extrathoracic regions. The current study presents a literature review with four additional patients with solitary fibrous tumor arising from the extremities to clarify clinicopathologic features. The current four patients were two males and two females, ranging from 17 to 60 years of age. Magnetic resonance imaging scans of the current patients showed inhomogeneous low to intermediate intensity signal on T1-weighted images and inhomogeneous intermediate to high intensity signal on T2-weighted images. Histologically, the tumors were composed of a haphazard proliferation of spindle cells, although cellularity was variable in each case. Two of the four tumors showed hypercellularity of spindle cells with focally myxomatous or hyaline changes, whereas myxomatous patterns with scattered spindle cells throughout the specimens were observed in the other two tumors. Immunohistochemically, all four patients showed positive immunoreactivity for CD34, and two tumors showed focally positive immunoreactivity for bcl-2 protein. During the followup of 12 to 54 months, neither local recurrence nor distant metastasis was detected after wide resection. Examination of the literature and the current patients suggests that solitary fibrous tumors in the extremities are likely to have a malignant potential, although most patients have a benign clinical course. Local wide resection and careful long-term followup are necessary for patients with solitary fibrous tumor in the extremities.     

Huge malignant localized fibrous tumor of the pleura

Localized fibrous tumor is an unfrequent mesenchymal neoplasm. The malignant variant of the pleura is exceptional and differential diagnosis with the more frequent benign type or with other neoplasms such as soft tissue sarcoma and mesothelioma is rarely possible in a preoperative setting. The best treatment of this disease is radical surgical resection. No definitive data exist about the role of chemotherapy. We report a case of a giant right intrathoracic mass whose preoperative diagnosis, from an open biopsy, was consistent with sarcoma and, in a second review, with fibrous tumor of the pleura without any indication about malignancy. A right pleuropneumonectomy and pericardial resection was performed through a right hemiclam-shell approach. Histology demonstrated an aggressive behaviour: high mitosis rate, Ki 67 of 34% and diffuse necrosis were present. In consideration of the apparent local radicality we did not perform any adjuvant treatment. Six months after the operation a wide local recurrence was evident and a systemic treatment with Ifosfamide and Adriamicina is still in progress. So far a good response has been documented. Preoperative diagnosis of malignancy has an important role as a therapeutic strategy in management of fibrous tumours of the pleura. When there is suspicion of a malignant form neoadjuvant chemotherapy can represent a further tool to control poorly differentiated and large tumors, and a wide surgical resection of the lesion must be performed.     

Localized (solitary) fibrous tumors of the pleura: an analysis of 55 patients

Background. Localized (solitary) fibrous tumors (LFTPs) of the pleura are rare, slow-growing neoplasms thought to originate from submesothelial connective issue. The aim of this article is to present 55 new cases of LFTP, and to discuss the treatment of choice and the clinical behavior of such neoplasms.

Methods. From July 1990 to November 1999, 55 patients (32 male, 23 female) with an LFTP were surgically treated at our Institution. Neoplasms were considered to be malignant if one or more of the following histologic features were present: high cellularity with crowding and overlapping of nuclei; high mitotic activity; or mild, moderate, or marked pleomorphism.

Results. No operative mortality was reported. Forty-eight of the cases arose from the visceral pleura and seven arose from the parietal pleura. A local removal of the neoplasm with free surgical margins was accomplished by video-assisted thoracic surgery in 39 patients and by standard thoracotomy in 10 patients. Four patients underwent formal lung resections, 1 had thymectomy, and 1 had en bloc chest wall resection. Four malignant variants were identified. One patient developed local recurrence and underwent redo surgery with chest wall resection. One patient died of unrelated disease. The remaining patients are alive and disease free at a median follow-up of 53.2 months.

Conclusions. LFTPs show a benign outcome in most of the cases. Video-assisted thoracic surgery, with intraoperative assessment of the surgical margins, represents the treatment of choice.     

63例原发性腹膜后肿瘤的诊断和外科治疗

目的 总结原发性腹膜后肿瘤诊断和外科治疗经验.方法 回顾性分析1990年1月至2007年3月63例经手术治疗且病理证实的原发性腹膜后肿瘤的临床表现、手术治疗、病理类型和随访结果.结果 63例中良性25例,恶性38例.主要临床表现为腹部包块,CT对良恶性判断的约登指数为85%,良性肿瘤完整切除率为88%,恶性肿瘤为68%,联合脏器切除占肿瘤完整切除总数的40%.肿瘤切除程度和病理类型与术后复发密切相关.良、恶性肿瘤完整切除的5年生存率分别为83.6%和27.3%.恶性肿瘤完整切除的患者平均随访36个月(5～168个月),53%局部复发,平均复发时间25个月(3～108个月),恶性肿瘤局部复发再手术完整切除率62.5%.全组有1例术后第1天腹腔内出血死亡.结论 应当重视临床表现争取早期诊断;影像学检查是判断手术范围的重要依据;肿瘤完整切除,必要时联合脏器切除是治疗本病的最佳手段;术后规律复查有助于及早发现复发肿瘤并争取再手术治疗.     

Contacting metastasis of a fibrous tumor of the pleura

A 26-year-old female with a fibrous tumor of the pleura that metastasized to the other site of the pleura due to contact is reported. The primary lesion was a 6-cm pedunculated tumor originating from the diaphragm; the metastasis was a broad-based 1-cm tumor located on the parietal pleura of the lateral thoracic wall, within an area in contact with the primary tumor. The surrounding pleura around the small tumor and the diaphragm—the area that contacted the large tumor—was resected with both of the tumors under a thorascope. Despite a benign pathologic finding, a localized fibrous tumor of the pleura can then metastasize to the pleura due to contact. It was concluded that, because localized fibrous tumors of the pleura can occasionally metastasize to the area in contact with tumors, the intrathoracic cavity should be thoroughly observed using a thoracoscope.     

Multiple recurrent malignant solitary fibrous tumors: long-term follow-up of 24 years

Solitary fibrous tumor (SFT) of the pleura is a rare, generally benign, neoplasm that accounts for less than 5% of all pleural tumors. However, 10% to 30% of SFTs display aggressive behavior with local recurrence, malignant transformation, and distant metastasis. We report a case of multiple recurrent SFTs of the thorax in a 77-year-old Korean woman. During a 24-year period, she underwent six surgical resections for six recurrent SFTs. The tumor eventually underwent morphologically malignant transformation. This unique case highlights the necessity of long-term follow-up in cases of SFT because of the potentially malignant biological behavior of this type of tumor.     

Androgen-secreting adrenal tumors

BACKGROUND: Adrenal tumors that secrete androgens exclusively are extraordinarily rare. The aim of this study was to characterize patients with pure androgen-secreting adrenal tumors. METHODS: A retrospective chart review from January 1946 through November 2002 identified 11 female patients with pure androgen-secreting adrenal tumors. RESULTS: The mean age was 23.4 years (range, 1-52). The most common presenting symptoms were hirsutism, acne, and clitoral enlargement. Elevated 17-ketosteroids were found in seven of nine tested patients. Computed tomogram, ultrasound, or both localized tumors in six of seven patients. All tumors were surgically resected, one laparoscopically, all without complications. Five of the 11 tumors were malignant. Mean weight and mean maximal diameter for benign and malignant tumors were 44 g and 4.2 cm and 232 g and 9.8 cm, respectively. Mean hospital stay was 8.5 days, with excess androgen production resolved in all patients. Recurrence and disease-related death occurred in only one patient who had pulmonary metastases at diagnosis. The remaining patients had no recurrence of tumor at mean follow-up of 11.7 years (range, 0.5-32 years). CONCLUSIONS: Pure androgen-producing tumors are extremely rare. Approximately 50% are benign, and surgical resection provides excellent treatment if the tumors are not metastatic at the time of diagnosis.