Calcifying fibrous tumor (CFT) is usually solitary, involving the soft tissue, pleura, or peritoneum. The cause and pathogenesis are unclear. Cases with multifocal lesions are rare, and a familial occurrence has not been reported. Herein, a family in which 2 sisters developed multifocal peritoneal CFT is described. The sisters each had abdominal pain, and each was found at laparotomy to have multiple nodular lesions on the mesenteric, omental, and small bowel serosal surfaces. Many lesions were composed of collagenous, paucicellular, fibrous tissue with psammomatous or dystrophic calcification, consistent with CFT. Some lesions were composed of more cellular spindle-celled tissue resembling inflammatory myofibroblastic tumors. Both patients were alive and well 19 and 17 years after diagnosis, respectively. One other sibling and 2 children of one of the sisters were speculated to have the same disorder, based on clinical findings. This report suggests that there may be genetic susceptibility to CFT, although chance and common environmental etiologic factors cannot be excluded. (+info)
Solitary fibrous tumour of the falciform ligament containing multiple foci of malignant transformation.
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Solitary fibrous tumour is an uncommon soft tissue tumour initially described in the pleural cavity. In the past decade, it has been described in various extrapleural sites, including the abdomen. Traditionally regarded as benign, cases of histologically or clinically malignant solitary fibrous tumours are rare. This report describes such a case, although the patient is still disease free five years after excisional surgery, without adjuvant treatment. The acute clinical presentation may be related to malignant transformation occurring in a long standing solitary fibrous tumour. (+info)
Solitary fibrous tumor of the urinary bladder.
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Solitary fibrous tumor (SFT) is a distinct spindle cell tumor arising mainly in the pleura, however, SFT of extra-pleural sites had been reported. To our knowledge, five cases of the urinary bladder SFT had been reported. We have recently experienced a case of SFT of the urinary bladder in a 56-year- old man who was admitted to the hospital with a 4-month history of voiding difficulty, frequency, and residual urine sensation. A computerized tomography scan revealed a 12cm sized intravesical mass. The mass was marginally excised under the impression of it being a benign spindle cell tumor by pre-operative needle biopsy. On gross examination, the mass was multinodular, creamy white, and the cut surface was fibrotic with a rubbery consistency. Microscopic examination revealed haphazardly arranged spindle cells with a lace-like deposition of inter- and pericellular collagen. Areas of increased cellularity show nuclear atypia and occasional mitoses (2-3/10HPF). The immunohistochemical staining revealed an intense CD34 and bcl-2 immunoreactivity. Although the exact biologic behavior of these tumors has not been clarified, most of the reported cases have undergone a benign clinical course. (+info)
Solitary fibrous tumor of the liver.
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BACKGROUND: Solitary fibrous tumor of the liver is a rare neoplasm. So far, 23 cases have been described in the English literature. We reported an additional case. METHODS: A 46-year-old woman presented with abdominal mass for 2 weeks. Both abdominal sonography and CT scan showed a solid mass occupying the right lobe of the liver. Right lobectomy was performed and the tumor was resected. RESULTS: Pathological examination showed spindle cell and fibroblast-like cells within the collagenous troma. On immunohistochemical staining, these spindle tumor cells showed diffusely CD34 positive reactivity. The post-operative course was uneventful. The patient recovered smoothly, and was alive half a year without evidence of disease recurrence. CONCLUSIONS: The proper diagnosis was depended on CD34 immunohistochemical study. The number of solitary fibrous tumor of the liver reported to date is too limited to confirm the definite prognosis of the tumor. (+info)
Clinicopathological findings in a case series of extrathoracic solitary fibrous tumors of soft tissues.
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BACKGROUND: Solitary fibrous tumors (SFT) represent a rare entity of soft tissue tumors. Previously considered being of serosal origin and solely limited to the pleural cavity the tumor has been described in other locations, most particularly the head and neck. Extrathoracic SFT in the soft tissues of the trunk and the extremities are very rare. Nine cases of this rare tumor entity are described in the course of this article with respect to clinicopathological data, follow-up and treatment results. METHODS: Data were obtained from patients' records, phone calls to the patients' general practitioners, and clinical follow-up examination, including chest X-ray, abdominal ultrasound, and MRI or computed tomography. RESULTS: There were 6 females and 3 males, whose age at time of diagnosis ranged from 32 to 92 years (mean: 62.2 years). The documented tumors' size was 4.5 to 10 cm (mean: 7 cm). All tumors were located in deep soft tissues, 3 of them epifascial, 2 subfascial, 4 intramuscular. Four tumors were found at the extremities, one each at the flank, in the neck, at the shoulder, in the gluteal region, and in the deep groin. Two out of 9 cases were diagnosed as atypical or malignant variant of ESFT. Complete resection was performed in all cases. Follow-up time ranged from 1 to 71 months. One of the above.mentioned patients with atypical ESFT suffered from local relapse and metastatic disease; the remaining 8 patients were free of disease. CONCLUSION: ESFT usually behave as benign soft tissue tumors, although malignant variants with more aggressive local behaviour (local relapse) and metastasis may occur. The risk of local recurrence and metastasis correlates to tumor size and histological status of surgical resection margins and may reach up to 10% even in so-called "benign" tumors. Tumor specimens should be evaluated by experienced soft tissue pathologists. The treatment of choice is complete resection followed by extended follow-up surveillance. (+info)
Obstructive jaundice caused by secondary pancreatic tumor from malignant solitary fibrous tumor of pleura: a case report.
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A 77-year-old man on systemic chemotherapy against postoperative bilateral multiple lung metastases of malignant solitary fibrous tumor of the pleura suffered from pruritus and jaundice. Blood examination showed elevated levels of hepatobiliary enzymes. Abdominal computed tomography showed a tumor with peripheral enhancement in the pancreatic head, accompanied with the dilatation of intra- and extra-hepatic bile ducts. He was diagnosed as having obstructive jaundice caused by a pancreatic head tumor. The pancreatic head tumor was presumably diagnosed as the metastasis of malignant solitary fibrous tumor of the pleura, because the findings on the pancreatic head tumor on abdominal CT were similar to those on the primary lung lesion of malignant solitary fibrous tumor of the pleura. The pancreatic tumor grew rapidly after the implantation of metallic stent in the inferior part of the common bile duct. The patient died of lymphangitis carcinomatosa of the lungs. Autopsy revealed a tumor that spread from the pancreatic head to the hepatic hilum. Microscopically, spindle-shaped cells exhibiting nuclear atypicality or division together with collagen deposition were observed. Immunohistochemically the pancreatic head tumor cells were negative for staining of alpha-smooth muscle actin (alpha-SMA) or CD117, but positive for vimentin, CD34 and CD99. These findings are consistent with those on malignant solitary fibrous tumor of the pleura. We report the first case of obstructive jaundice caused by a secondary pancreatic tumor from malignant solitary fibrous tumor of the pleura. (+info)
Solitary fibrous tumor of the pleura.
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BACKGROUND: The solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor arising from mesenchymal cells in the areolar tissue subjacent to the mesothelial-lined pleura. Only about 800 cases have been reported in the medical literature. The tumor appears to be unrelated to malignant pleural mesothelioma, the most common primary tumor of the pleura. METHODS: In just over half of these cases, the neoplasm presents as an asymptomatic mass, is often quite large, and is benign in 78% to 88% of patients. The initial evaluation and diagnosis, tumor classification, surgical treatment, results of therapy, and long-term prognosis are reviewed, based on a selective review of the literature from MEDLINE beginning 1980. RESULTS: Complete en bloc surgical resection is the preferred treatment of benign and malignant varieties of the tumor. The pedunculated tumors attached to the visceral pleura can be effectively treated with a wedge resection of lung. Sessile tumors arising on the lung require a larger lung resection. Sessile tumors on the chest wall require wide local excision, often with chest wall resection because of their propensity for local recurrence. Adjuvant therapy remains controversial in SFTP. CONCLUSIONS: Benign SFTP has a high cure rate and an 8% local recurrence rate that is usually amenable to curative re-excision. Malignant SFTP, especially the more common sessile type, has a 63% recurrence rate even with complete resection. The majority of patients with recurrent disease die of the tumor within 2 years. Nevertheless, the overall long-term cure rate for all patients is 88% to 92%. (+info)
Giant solitary fibrous tumour of the meninges: MR-pathological correlation.
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The authors present the clinical, MR imaging and pathological features of the largest intracranial solitary fibrous tumour of the meninges ever reported in the literature. This well-circumscribed dural-based tumour strikingly demonstrated two different solid components. The first one demonstrated a suggestive T2 hypointensity that strongly enhanced after gadolinium administration while the other showed more classic homogeneous T2 hyperintensity and mild enhancement. These two components were also identifiable on the pathological examination, allowing an interesting imaging-histological correlation. Differential diagnoses of this rare extra-axial lesion are discussed. (+info)