Solitary fibrous tumor in the cervical spine with destructive vertebral involvement: a case report and review of the literature

Introduction  Recently, solitary fibrous tumors occurring in spine-related lesions have been reported. However, the destruction of vertebral bodies by this type of tumor has not been reported. Materials and methods  A 71-year-old female presented with pain from a mass on the right side of her neck. Plain radiographs of the cervical spine showed collapse of the C5 vertebral body and dislocation of the C4 vertebral body. The MRI image showed a large mass surrounding C4 and C5, which had low signal intensity in the T1W image and high signal intensity in the T2W image. At first, resection of the tumor and spinal fusion was performed by anterior approach. Results  Histology revealed a solitary fibrous tumor with proliferating spindle cells. Immunohistochemistry showed positive stains for vimentin and CD34. One year postoperatively, a local recurrence manifested extensive destruction of the C4 and C5 vertebral bodies. Then, palliative surgery with posterior cervical instrumentation and radiation therapy were performed. Because the destruction proceeded and the rods were broken 2 years after, she underwent additional occipito-cervical instrumentation. Conclusions  This is the first report of a solitary fibrous tumor that involves the destruction of the spinal structure. An extensive destruction of the vertebral body by the solitary fibrous tumor needs to be aware in treating this tumor with spinal involvement.     

Malignant solitary fibrous tumor with superior vena cava syndrome

Mediastinal solitary fibrous tumors, particularly those that are malignant, are rare. We report a case of malignant solitary fibrous tumor with superior vena cava syndrome and highly aggressive behavior. Postoperative radiotherapy was performed due to incomplete resection, but the tumor relapsed within 4 months. Gemcitabine-based chemotherapy was ineffective.     

Spinal solitary fibrous tumor: seventh reported case and review of the literature

We present the clinical, radiological, and pathological features of a solitary fibrous tumor in the spinal cord. This case is the seventh spinal solitary fibrous tumor in the literature. The tumor caused clinical symptoms in a 70-year-old female, which indicated compression of the spinal cord. Magnetic resonance imaging showed an intradural extramedullary mass at T3 vertebral level. Surgically, the tumor was firm, in an intradural extramedullary location and attached to the dura. Histologically, the tumor was composed of spindle cells in a collagen-rich matrix but exhibited regional variations. CD34 and vimentin were diffusely positive during immunohistochemical stain testing. The tumor displayed no positive staining for epithelial membrane antigen, cytokeratin, S-100 protein, smooth muscle actin or desmin. The Ki-67 labeling index was low. Solitary fibrous tumors have been found in a variety of locations suggesting that a solitary fibrous tumor has a mesenchymal origin. This rare tumor should be considered in the differential diagnosis of spinal tumors.     

Solitary fibrous tumors of the meninges

Central nervous system solitary fibrous tumors are a new pathological entity. To our knowledge, only 60 meningeal solitary fibrous tumors both in the spinal cord and in the brain have been described in the literature. The 56 previously reported cases of meningeal solitary fibrous tumors are critically reviewed. In addition, we report four new cases of solitary fibrous tumors of the meninges. There is a slight male prepoderance. Meningeal solitary fibrous tumors show a tendency to arise in the posterior fossa (26%) and spine (25%). The treatment was mainly total surgical excision. Radiotherapy was given only to four patients with tumors involving the cerebral parenchyma. Sporadic cases of recurrence and distant metastasis have been reported. The prognosis of meningeal solitary fibrous tumors is still unknown because the follow-up of the reported cases is short. It is probable that cases of solitary fibrous tumors of the meninges have been misdiagnosed as other tumors in the past. The best management of these tumors seems to be total surgical excision whenever possible. It is important that every new case of meningeal SFT be reported to throw light on this particular tumor and to affirm its status as a clinicopathological entity.     

Solitary Fibrous Tumor of the Breast and Mammary Myofibroblastoma: The Same Lesion?

Abstract:  Benign stromal tumors of the breast are rare mesenchymal neoplasms that have significant clinical and morphologic overlap. We report the case of a spindle cell tumor occurring in the mammary gland with mixed features of solitary fibrous tumor and mammary myofibroblastoma. The significance of this finding as well as the pathologic and radiologic diagnostic strategies used to differentiate these two lesions are presented based on a review of pertinent literature.     

Solitary Fibrous Tumor of the Larynx

Background True mesenchymal, non-cartilaginous neoplasms of the larynx are rare. Extrapleural solitary fibrous tumor (SFT) is a localized neoplasm characterized by proliferation of thin-walled vessels and collagen-producing cells and is considered within the “hemangiopericytoma-solitary fibrous tumor” spectrum. SFT primary in the larynx is exceptional. Design Case report set in a comparison with other cases reported in the English literature (MEDLINE 1966 to 2007). Results A 49-year old white male presented with difficulty breathing, progressive over the past 2 years. He denied dysphagia and weight loss. Past medical history was significant for asthma. He denied cigarette smoking or alcohol abuse. There were no cervical deformities on physical exam. Fiberoptic laryngoscopy was performed upon stabilization of respiratory function. A smooth, round, submucosal mass measuring 2.3 cm in greatest diameter arising from the inferior surface of left true vocal cord was causing near total obstruction of the endolaryngeal space. The mass was excised. The surface mucosa was intact and unremarkable. A cellular, spindle cell neoplasm was arranged in loose fascicles, associated with heavy collagen fiber deposition. The collagen was wiry and heavy. Cells were bland with cytoplasmic extensions. The nuclei were vesicular to hyperchromatic and elongated with inconspicuous nucleoli. Vessels were prominent and delicate, with patulous spaces. Mitotic figures were easily identified, but atypical forms were not present. The cells were strongly and diffusely immunoreactive with CD34 and bcl-2, while non-reactive with cytokeratin, EMA, actin, ALK-1, S100, desmin, and CD117. These findings confirmed a diagnosis of extraplural solitary fibrous tumor. Without further disease, the patient is alive without evidence of disease, 12 months after surgery. Conclusions The characteristic histologic pattern of solitary fibrous tumor can be noted in extrapulmonary locations. Development in the larynx is uncommon, but the tumor presents as a polypoid mass with characteristic histologic and immunophenotypic features. Conservative local excision is the treatment of choice to yield an excellent prognosis.     

Intradural tumor and concomitant disc herniation of cervical spine

We report a rare patient of a simultaneous extradural and intradural compression of the cervical spinal cord due to co-existent intervertebral disc herniation and an intradural schwannoma at the same level. The intradural lesion was missed resulting in recurrence of myelopathy after a surprisingly complete functional recovery following anterior cervical discectomy. Retrospectively, it was noted that the initial cord swelling noticed was tumor being masked by the compression produced by the herniated disc. A contrast magnetic resonance imaging scan is important in differentiating intradural tumors of the spinal cord. A high index of suspicion is often successful in unmasking both the pathologies.     

肾孤立性纤维瘤1例报告并文献复习

目的 分析肾孤立性纤维瘤的临床病理特征及诊治方式.方法 回顾性分析我院诊治的1例肾孤立性纤维瘤的病理特征、临床表现、影像学检查、治疗方式和术后随访状况.结果 肾孤立性纤维瘤是肾脏较罕见的一种间叶性肿瘤,良性居多,少数为恶性.病理特征多为VIM(+)、CD34(+)、BcL-2(+)、CD99(+).影像学检查显示为类圆形、包膜完整的肿物,与周围组织界限清楚,CT增强后呈现不同程度欠均匀强化.本例患者行机器人辅助腹腔镜下肾部分切除术,术后病理诊断为肾孤立性纤维瘤(中间性),术后随访3月余,未见肿瘤复发、进展和转移.结论 肾孤立性纤维瘤属临床上较罕见的肿瘤,确诊主要通过病理诊断,治疗上首选手术切除肿瘤.     

Solitary fibrous tumor of the thoracic spine

Intraspinal solitary fibrous tumors are rare: to our knowledge, the literature reports only 27 cases. We present a histologically and immunohistochemically confirmed solitary fibrous tumor involving the intradural extramedullary compartment of the thoracic spine. Microsurgical gross-total resection was achieved. A definitive role for adjuvant treatments in this type of tumor has not been established and therefore, they were not used. The patient was well, without clinical or radiological recurrence, 18 months after surgery.     

Supratentorial meningeal melanocytoma mimicking a convexity meningioma

Summary Objective and importance. Meningeal melanocytomas are rare benign neuro-ectodermal tumors arising from melanocytic cells in the leptomeninges. These leptomeningeal melanocytes are found at highest density underneath the brain stem and along the upper cervical spinal cord. Thus, most reported cases of meningeal melanocytomas are located in the posterior fossa and the spinal cord, respectively. Clinical presentation. We report on the rare case of a 55-year-old male patient with a large supratentorial meningeal melanocytoma mimicking a convexity meningioma and a smaller, similarly dura based lesion in the posterior fossa. Intervention. Tumor control to date was achieved by surgery of the large lesion and radiosurgery of the small lesion. Conclusion. Complete tumor resection may be advantageous and second or recurrent lesions may be managed by repeat surgery or stereotactic radiosurgery.     

Solitary fibrous tumors of the pleura presenting satellite tumors

A 29-year-old man presented with a mass in the left lower lung field on a chest radiograph obtained during a medical checkup. Computed tomography revealed a tumor adjacent to the diaphragm. A sessile tumor measuring 10.5 × 8.5 × 4.5 cm arising from the parietal pleura was resected. The tumor was accompanied by several little tumors on the nearby diaphragm. Pathologically, the major tumor consisted of typical spindle-shaped cells with myxoid degeneration. There was no increased cellularity, cellular pleomorphism, or a high mitotic count. In immunohistochemical studies, the spindle cells showed positive staining for CD34 and were negative for bcl-2. The smaller tumors also consisted of myxoid degeneration. We diagnosed benign solitary fibrous tumor of the pleura with satellite tumors. We must be aware of the possibility of satellite tumors when we treat patients with a benign solitary fibrous tumor.     

Giant pelvic solitary fibrous tumor obstructing intestinal and urinary tract: a case report and literature review

We are reporting a giant pelvic neoplasm, a rare solitary fibrous tumor that presented with a large bowel obstruction and bilateral ureteral obstruction because of its size and location. Preoperative diagnosis required complex pathological studies to exclude a high-grade sarcoma suspected clinically. Complete resection was required for resolution of obstructive symptoms. Prognosis for solitary fibrous tumors is usually good after complete resection. Recurrence and metastasis may be related to rare aggressive histological features, including nuclear atypia, hypercellularity, greater than four mitoses/10 high power fields, and necrosis. Because histology is not always a reliable predictor of prognosis, careful long-term follow-up is necessary for this tumor. Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms most likely arising from mesenchymal cells. SFTs were originally described in the pleura, the most common site for this tumor; however, extrathoracic SFTs are seemingly diagnosed with increased frequency. We report a case of a giant pelvic SFT that required complicated clinical management.     

Large solitary fibrous tumor of the seminal vesicle

Solitary fibrous tumors show a classic morphologic pattern ("patternless pattern") consisting mainly of a proliferation of bland spindle cells with varying amounts of thick, often hyalinized or keloid-like, intercellular collagen bundles. Immunohistochemistry shows a strong reactivity for CD34 antigen, vimentin, and, in a variable percentage, bcl-2 antigen. We report the case of a 50-year-old man with a large solitary fibrous tumor located in the pelvic cavity with a rare nonspecific histologic pattern of pseudovascular formations. The patient underwent pelvic exenteration with orthotopic continent urinary diversion and sigmoid-J-pouch bowel reconstruction. No signs of tumor recurrence were noted within 24 months of surgery.     

Intrapulmonary solitary fibrous tumor

Solitary pulmonary nodules are sometimes detected by routine chest radiography. Although many of them are suspected to be benign tumors following noninvasive examinations including computed tomography and positron emission tomography, it is difficult to diagnose them accurately. This report presents a rare case of a solitary fibrous tumor located in the lung that could not be diagnosed preoperatively. More information must be accumulated concerning such rare cases of intrapulmonary solitary fibrous tumors.     

Clear Cell Endocrine Tumor of the Pancreas Which is Not Associated with von Hippel-Lindau Disease: Report of a Case

We describe the case of a pancreatic endocrine tumor with marked clear cell changes. The patient, a 56-year-old woman, presented with appetite loss and nausea but no evidence of either von Hippel-Lindau disease, or a family history of the disease. A radiological examination revealed a hypervascular tumor measuring 5.2 cm in diameter located in the pancreatic head. The patient underwent a pylorus-preserving pancreatoduodenectomy. The cut surface of the tumor was yellowish and well demarcated. Histologically, the tumor was composed of cuboidal cells with clear cytoplasm, thus forming trabecular and insular nests. Immunohistochemically, the tumor cells were strongly positive for chromogranin. An electron microscopic examination revealed the tumor cells to contain neuroendocrine secretory granules, abundant lipid droplets, and some crystalloid bodies in the cytoplasm. We conclusively diagnosed it to be a pancreatic clear cell endocrine tumor. Such tumors not associated with von Hippel-Lindau disease are extremely rare. Received: December 19, 2001 / Accepted: July 2, 2002 RID="*" ID="*" Reprint requests to: M. Sakamoto Acknowledgment. We appreciate the valuable technical assistance provided by Mr. Fumio Hasegawa, Electron Microscopy Laboratory, National Cancer Center Hospital, in performing transmission electron microscopy. This research was supported in part by a Grant-in-Aid for the Second Term Comprehensive 10-year Strategy for Cancer Control from the Ministry of Health, Labour and Welfare, Japan.     

Solitary fibrous pleural tumor

Solitary fibrous pleural tumors are rare masses of mesenchymal origin that may be mistaken for mesothelioma. A positive staining of vimentin, negative staining of cytoplasmic keratin, and expression of the CD34 antigen can confirm the presence of a solitary fibrous pleural tumor. Although most tumors of this type are benign, they possess a malignant potential and thus should be excised. We report a case of a 63-year-old man who had an inconclusive biopsy of a lung lesion 15 years ago. Further testing after excision revealed a solitary fibrous pleural tumor. A brief discussion of the clinical presentation and incidence of these tumors is included.     

Unusual presentations of cerebral solitary fibrous tumors: report of four cases

OBJECTIVE AND IMPORTANCE: Four rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously. CLINICAL PRESENTATION: A 7-year-old child had had 1 year of right-sided weakness at presentation. Magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. Magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation. INTERVENTION AND TECHNIQUE: All four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. Immunohistochemistry confirmed C-SoFTs in all four cases. CONCLUSION: C-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.     

Große solitäre fibröse Tumoren der Pleura als Zufallsbefund

The solitary fibrous tumor (SFT) is a very rare and usually benign neoplasm. This tumor is mostly located in the thoracic cavity, but it has also been reported in numerous sites including liver, skin, and meninges. Due to its rarity diagnosis is often difficult. Accordingly, histopathological differentiation is of major importance. Solitary fibrous tumors consistently express CD 34 and react negatively to epithelial markers. This absence of expression of epithelial markers, especially cytokeratin, is also useful to distinguish these rare entities from sarcomatous mesotheliomas. The treatment of choice for solitary fibrous tumors is extensive surgical resection. Up to now there is no evidence that radiation and chemotherapy are effective. The local recurrence or onset of metastases depends on histologic parameters. In cases of benign classification (60-80%), the recurrence rate was only 2% after surgical excision. One-half of the patients with malignant tumors can be cured; the rest develop recurrences and metastases more often. Especially in those cases, emphasis should be placed on continual follow-up examination. We report two cases of a 65- and a 75-year-old male patient with huge intrathoracic solitary fibrous subpleural tumors, discovered incidentally on routine chest X-ray.     

Malignant solitary tumor in a child: a case report and review of the literature

Solitary fibrous tumor in the mesentery is rare. We report a case of a malignant solitary fibrous tumor in the appendical mesentery of 6-year-old boy. Computed tomography and ultrasound of the abdomen demonstrated a well-defined solid mass, 4.0 cm in diameter, in the lower right abdomen. At laparotomy, an encapsulated tumor was observed in the appendical mesentery and was easily separated from the appendix. Immunohistochemistry stain showed that the spindle-shaped cells were positive for CD34 and neuron-specific enolase. DOG-1, CD117, desmin, S-100 protein, and SMA were negative. The sequence of KIT and platelet-derived growth factor receptor α was presented with wild type, no mutation. The patient received the reoperation 5 months postoperation. Despite these treatments, the relapsed tumor rapidly and markedly enlarged. The patient died of recurrent tumor with liver dissemination 7 months after reoperation.