Primary hepatic solitary fibrous tumor with histologically benign and malignant areas

Extrapleural solitary fibrous tumor(SFT) is an uncommon mesenchymal neoplasm,presenting most commonly in the intrathoracic sites but which has been reported at numerous extrathoracic locations. The majority of intra-thoracic SFTs are benign,but 10%-15% behave aggressively. We report a case of primary hepatic SFT with histologically benign and malignant areas. A 65-year-old man underwent an abdominal CT scan following a cerebrovascular accident,which demonstrated a sharply demarcated large liver mass with a heterogenous enhancing area and occupying most of the left lobe of the liver. Histological examination following a hemihepatectomy showed an SFT with morphological patterns ranging from benign to malignant areas,including pleomorphism,increased cellularity,herringbone pattern,necrosis and a raised mitotic count. On review of the literature,only an occasional case report with malignant areas in a hepatic SFT was identified. This case highlights that SFT should be included in the differential diagnosis of a hepatic spindle cell lesion,and that on rare occasions,malignant areas can occur in this already uncommon neoplasm.     

Malignant solitary fibrous tumour of the kidney: report of a case and cumulative analysis of the literature

We report the case of a primary metastatic renal solitary fibrous tumour (SFT) and present a cumulative analysis of the literature. A 68-year-old woman presenting with a history of flank pain was diagnosed with a 7 cm renal mass. Further staging showed liver, lung and bone metastases. The patient underwent radical nephrectomy. Microscopically, the tumour consisted of pleomorphic, high-grade spindle cells with high mitotic activity, tumour necrosis and dense collagenous bands. Immunohistochemistry showed the strong expression of CD34 and vimentin, a weak expression of bcl-2 and CD99, and no expression of smooth muscle actin, desmin, S-100, pan-cytokeratin, and epithelial membrane antigen. These findings are consistent with an SFT. For the cumulative analysis, a total of 46 renal SFTs from 35 reports were analysed. Median age at the time of surgery was 52 years and 63% of the patients were female. Sixty-two percent of the tumours were symptomatic, most commonly with flank?/?back pain (24%). Median tumor size was 6.4 cm. Histologically, 91% of the SFTs were benign and 9% were malignant. One patient died of the disease, while 90% are alive without evidence of disease.     

Solitary fibrous tumor of the liver

We report a new case of benign solitary fibrous tumor (SFT) of the liver. A 65‐year‐old man presented to our unit with upper right abdominal discomfort. On examination abdominal distension was present and palpation showed a large firm mass in the right hypochondrium and epigastrium. The patient's past medical history was not significant and laboratory tests were normal. Ultrasonography and computed tomography showed a large tumor, 20 cm in diameter, in the right lobe of the liver. An extended right hepatectomy was performed. The tumor measured 30 × 28 × 14 cm and weighed 4725 g. Microscopic evaluation showed a benign SFT of the liver with tumor cells typically positive for vimentin and CD34. The postoperative course was uneventful, and the patient is alive 30 months after surgery. This is a rare neoplasm of mesenchymal origin that occasionally involves the liver in adult patients. Most SFTs are benign, but some may have malignant histological features and recur locally or metastasize. Because of their rarity, overall experience has not been significant and little has been published concerning this tumor, Including the present one, 28 cases have been reported in the English literature. Surgery is the mainstay of treatment. Little can be said about the benefits of adjuvant radiochemotherapy in these patients. As SFT of the liver is often a benign neoplasm, chemotherapy or radiotherapy should not be necessary, and should be reserved for when resection is incomplete and/or histological examination reveals features of malignancy. Surgeons must be aware of SFT of the liver, and this neoplasm should be included in the differential diagnosis of a single large hepatic mass.     

Intrapulmonary solitary fibrous tumor in an eight-year-old male

We describe an 8-year-old male who had an intrapulmonary solitary fibrous tumor (SFT). SFTs of the pleura are now thought to originate from subpleural mesenchymal cells, and a pathological diagnosis is obtained by a specific marker, i.e., CD34 expression on the tumor cells. The SFT reported here is an extremely rare phenotype in two respects: it originated in a child under age 10 years, and it expanded in an intrapulmonary fashion.     

Giant solitary fibrous tumor arising from greater omentum

Extrathoracic solitary fibrous tumors (SFTs) have been described at almost every anatomic location of human body, but reports of SFT in the abdominal cavity are rare. We herein present a rare case of SFT originating from greater omentum. Computed tomography revealed a 15.8 cm × 21.0 cm solid mass located at superior aspect of stomach. Open laparotomy confirmed its mesenchymal origin. Microscopically, its tissue was composed of non-organized and spindle-shaped cells exhibiting atypical nuclei, which were divided up by branching vessel and collagen bundles. Immunohistochemical staining showed that this tumor was negative for CD117, CD99, CD68, cytokeratin, calretinin, desmin, epithelial membrane antigen, F8 and S-100, but positive for CD34, bcl-2, α-smooth muscle actin and vimentin. The patient presented no evidence of recurrence during follow-up. SFT arising from abdominal cavity can be diagnosed by histological findings and immunohistochemical markers, especially for CD34 and bcl-2 positive cases.     

Solitary fibrous tumor of the liver with CD 34 positivity and hypoglycemia

We report a new case of solitary fibrous tumor (SFT) of the liver, an extremely rare neoplasm. Including the present case no more than ten cases are reported in the English-language literature. To date there is no definite proof of the origin of this tumor. Both mesothelial and fibroblas-tic genesis has been postulated. The monoclonal antibody CD 34 has recently been used for the characterization of SFT. SFT would appear to be histogenetically related to a CD 34 — positive fibroblastic stem cell. A 61-year-old woman was admitted to our department with epigastric and right hypochondriac pain, weight loss, and hypoglycemia. Ultrasonography and computed tomography demonstrated a large heterogeneous mass in the right hepatic lobe. A right hepatectomy was performed. The tumor weighed 2850 g and microscopic section revealed a peculiar random pattern, the so-called patternless pattern of spindle tumor cells separated by abundant thick collagen bands. The tumor presented a number of highly cellular areas composed of plump spindle cell with hyperchromatic nuclei and rare mitotic figures. Ninety percent of the neoplastic cells displayed strong immunoreactivity for CD 34/My 10. The postoperative course was uneventful and the patient is alive and well without recurrence 6 years after surgery.     

Solitary fibrous tumor of the pleura: Ultrasonographic imaging findings of 3 cases

Solitary fibrous tumor (SFT) of the pleura is a rare tumor of mesenchymal origin. Although radiographic findings of thoracic computed tomography and magnetic resonance imaging in the evaluation of SFTs of the pleura have been documented, the value of ultrasonography is uncertain. We presented the ultrasonographic findings of 3 pathologically proven cases of SFTs arising from the visceral pleura.In all the cases, thoracic ultrasonography demonstrated homogeneous, hypoechoic, hemicycle, extrapulmonary lesions, which showed respiratory movement with the adjacent lung, consistent with pedunculated tumors.Preoperative thoracic ultrasonography could be useful in the evaluation of patients with pleural tumors, especially SFTs.     

Unusual solitary fibrous tumors in the central nervous system: a report of two cases

Solitary fibrous tumors (SFTs) are uncommon, and most are found in the pleura. Extrapleural SFTs are rare and have been found in the lung, pericardium, mediastinum, soft tissue of any site, and upper respiratory tract. SFTs of the central nervous system (CNS) are very rare. The biologic features are unknown and remain poorly understood from a clinical standpoint. Most neurosurgeons do not believe that SFTs can present as primary CNS neoplasms. Most SFTs are clinically benign and indolent, and recurrences after surgical excision are scarce. Because malignant transformation or metastasis has been reported, all SFTs should be treated as having malignant potential. Long-term follow-up is recommended. We report two cases, so that surgeons may recognize that this is an entity different from other spindle-cell CNS tumors.     

Solitary fibrous tumor of the liver expressing CD34 and vimentin： A case report

A case of a successfully treated solitary fibrous tumor （SFT） of the liver is reported. An 82-year-old female presented with left upper abdominal discomfort, a firm mass on palpation, and imaging studies revealed a large tumor, 15 cm in diameter, arising from the left lobe of the liver. A formal left hepatectomy was performed. Microscopic evaluation showed spindle and fibroblast-like cells within the collagenous stroma. Immunohistochemistry disclosed diffuse CD34 and positive vimentin, supporting the diagnosis of a benign SFT. The patient remained well 21 months after surgery. SFT of the liver is a very rare neoplasm of mesenchymal origin. In most cases it is a benign lesion, although some may have malignant histological features and recur locally or metastasize. With less than 30 reported cases in the literature, little can be said regarding its natural history or the benefitsof adjuvant radiochemotherapy. Complete surgical resection remains the cornerstone of its treatment.     

Solitary fibrous tumor of the kidney. A propos of a case

Solitary Fibrous Tumors (SFT) are rare spindle cell neoplasm that typically originate from the pleura. However, cases of the SFT are described with origin in other organs, including the urinary and genital apparatus. Within the kidney, except from the renal pelvis, only 19 cases of SFT are described and such rarity of localization makes the histogenesis and the prognosis of the tumor rather unknown. We report the case of a 72-year-old lady who attended our Unit for a mass that was clinically palpable at the level of the left hemiabdomen. The tomodensitographic test indicated a 19cm-diameter mass of likely pertinence of the middle bystender of the left kidney. She had undergone left radical nephrectomy. The histological examination highlighted a solitary fibrous tumor (SFT): the presence of hypercellularity, of cellular pleiomorphism and of a high number of mitosis has led to a histological diagnosis of malignancy for the neoplasm analyzed. The SFT are of rare clinical comparison: this does not allow for a deep knowledge of the lesion histogenesis and prognosis; moreover, the clinical behavior should be more precisely defined.     

Solitary fibrous tumor of the thyroid gland.

Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm more commonly involving the pleura, but recognized also in other tissues. Nineteen patients with SFT arising from the thyroid gland have been reported in the literature. The present report reviews these cases and discusses epidemiology, etio-pathogenesis, clinical-pathologic characteristics, differential diagnosis, therapy, and prognosis of thyroid SFT.     

胸膜孤立性纤维性肿瘤10例临床病理观察及文献复习

目的探讨胸膜孤立性纤维性肿瘤(SFT)的临床病理特征,提高对其临床及病理改变的认识。方法对10例胸膜SFT的临床资料和组织学形态进行回顾性分析,并采用免疫组织化学染色(Envision法)研究其形态及免疫表型特点。结果 10例患者中,男性6例,女性4例,发病年龄47~76岁,平均57.5岁。肿瘤分别位于脏层胸膜(4例),壁层胸膜(4例),叶间裂(2例),患者多因咳嗽,胸痛,胸膜腔肿块,肺部肿块就诊。影像学多显示:占位性病变和软组织团块影,其中2例显示有胸腔积液。病理组织学显示:肿瘤界限清晰,由交替性分布的细胞丰富区及稀疏区组成,部分可有粘液变。免疫组织化学显示:梭形细胞表达Bcl-2、CD34和vimentin。结论 SFT是一种较为少见的间叶性梭形细胞肿瘤,好发于胸膜。其确诊主要依赖于病理组织形态学及免疫组织化学染色。完整的手术切除是临床治疗SFT的必要手段,但少数SFT生长活跃,具有侵袭性,需长期随访。     

Pathology of the pleura: what the pulmonologists need to know

Primary and metastatic pleural neoplasms, and non-neoplastic pleural diseases, can have similar clinical, radiographic and gross features. However, treatments and prognoses of these diverse pleural conditions vary greatly. Accurate diagnosis of pleural disease is therefore extremely important, and histological interpretation of pleural biopsies is vital to rendering an accurate diagnosis. Smaller biopsies contribute to the difficulties in accurately characterizing pleural lesions, and immunostains are frequently employed in their assessment. Diffuse malignant mesothelioma, the most common primary pulmonary neoplasm, is rare; however, other less common primary pleural neoplasms, including solitary fibrous tumour, the most common benign primary pleural neoplasm, occur. These neoplasms are discussed. Also, non-neoplastic pleural diseases, including granulomatous pleuritis, eosinophilic pleuritis and fibrous and fibrinous pleuritis, among other diseases, are discussed.     

Solitary fibrous tumor in the thigh: review of the literature

Solitary fibrous tumors (SFT) of extremities, especially the thighs are very rare. Despite SFTs are generally benign, well-circumscribed soft tissue tumors new cases should be presented and followed up carefully to monitor their biological behavior. In general for tumor classification a biopsy is state of the art. Histological including immunohistochemical patterns for SFTs are defined. MRI and ultrasound are not sufficient for differential diagnosis. Once property identified and defined by size and location, resection with intact tumor capsule may result in full recovery of the patient. Reviewing the literature there are no validated reasons for a wider resection. The current patient was a 41-year-old male. Four years after an arthroscopy of the left knee the patient has been suffering an ongoing swelling of the lateral thigh. Because MRI scan data suggested a synovial sarcoma a biopsy was performed. The tumor was classified as a benign SFT. The diagnosis based on histological findings and the presence of the positive immunohistochemical markers Vimentin, CD34, and CD99. The complete tumor resection with intact capsule was achieved in a final operation. Clinical and in MRI after 54-month outcome period there were no local recurrences.     

Malignant myxoid emboli as the presenting feature of primary sarcoma of the aorta (myxoid malignant fibrous histiocytoma): a case report and review of the literature.

Primary sarcomas of the aorta are rare vascular tumors. About 26 cases have been reported between 1973 and 1989. They are seldom diagnosed antemortem. The present report describes a patient with a myxoid malignant fibrous histiocytoma arising in the intima of the lower thoracic aorta, which was diagnosed premortem in metastatic peripheral emboli. The clinical and pathologic features of this patient along with a summary of the 29 primary aortic tumors previously reported (26 malignant and 3 benign) are discussed. Although few of these tumors have been described histologically as benign, all of them behave as malignant tumors.     

Malignant solitary fibrous tumor involving the liver

Solitary fibrous tumors are predominantly benign and are most commonly found in the thoracic cavity and pleura; while reports exist in the literature of malignant solitary fibrous tumors and those located in extrathoracic organs, these cases are considered extremely rare. Herein, a case is reported of a malignant solitary fibrous tumor involving the liver that was diagnosed and treated in a 62-year-old woman. The patient presented with complaints of upper abdominal pain and unintentional weight loss. Computed tomography scan of the abdomen revealed a remarkably large mass, measuring 15 cm × 10 cm × 20 cm, which appeared to be unrelated to any particular organ. The intraoperative finding of a wide communication with the left liver suggested hepatic origin, and served as an indicator for tumor resection via left hemihepatectomy. The diagnosis of solitary fibrous tumor and its malignant nature was confirmed by histological and immunohistochemical examination of the resected tissues. Hepatic solitary fibrous tumor is very rare, and surgery remains the mainstay of treatment. Due to limited reports of such tumors in the literature, little can be said about the benefit of adjuvant therapy and prognosis for the rare cases with malignant histological findings.     

Pleural malignant solitary fibrous tumor with sarcomatous overgrowth showing PDGFRbeta mutation

Pleural malignant solitary fibrous tumors (SFTs) are uncommon, and little is known about their histogenesis and molecular features. We report a case of pleural SFT with sarcomatous overgrowth that showed expression for PDGFRbeta and a missense mutation on exon 18 of the PDGFRbeta gene. The involvement of the PDGFRbeta gene in SFT is compatible with a pericytic derivation, also supporting a possible role of this tyrosine kinase in malignant transformation and in the adoption of novel molecular therapies.     

Primary Granulocytic Sarcoma of Meninges and Mediastinum as Diagnostic Dilemma

Granulocytic sarcoma is a rare hematological neoplasm which is often misdiagnosed. We report two patients of primary granulocytic sarcoma, one at meninges who was diagnosed radiologically as meningioma and on histopathology as langerhans cell histiocytosis. The second patient presented with an ulcerated chest wall swelling, who on histopathology revealed malignant round cell tumour. Both the patients subsequently proved as primary granulocytic sarcoma on immunohistochemistry. These two cases are reported here due to their rarity.     

Diffuse pulmonary tumor microemboli from prostatic carcinoma with an associated fibrous histiocytic sarcoma

Diffuse tumor microemboli to the lungs from prostatic carcinoma are extremely rare, with only six reported cases in the literature published in English. Here a seventh case is reported. The patient resembled the previously reported cases in the following ways: (1) There were minimal chest x-ray findings; (2) there was no other evidence of significant parenchymal or lymphangitic tumor involvement; and (3) pulmonary hypertension developed, but without signs of cor pulmonale. The case was even more unusual because of the presence of an apparent second neoplasm, ie, a malignant fibrous histiocytic sarcoma primary in the chest wall. There were wide-spread "collisional metastases" (metastases from each malignancy at the same site).     

Solitary fibrous tumor of the pancreas. A case report

Solitary fibrous tumour (SFT), a rare mesenchymal neoplasm usually arising from the pleura, may also occur in many other extra pleural sites. It has exceptionally been described in the pancreas. This report describes the case of a benign SFT of the pancreas occurring in a 41-year-old man who presented with a solid epigastric mass. Pathological and immunohistochemical findings are presented. Imaging features on ultrasonography, CT, MRI, and arteriography are widely detailed. Surgical resection of the tumour was performed, and the patient died from postoperative complications.