Posterior fossa solitary fibrous tumour: report of a fetal case and review of the literature

We report a case of solitary fibrous tumour (SFT) involving the posterior fossa in a fetus of 25 weeks’ gestation. SFT is a rare mesenchymal neoplasm, arising in various locations including the meninges. After disclosure of severe ventriculomegaly and posterior fossa mass measuring 45 mm in diameter, termination of pregnancy was performed in accordance with French legislation. Our neuropathological study revealed a tumour covered by meninges, with severe compression of the cerebellum and the brain stem. Microscopically, the tumour was highly cellular, made of packed small fusiform cells with branching vasculature and consistent expression of CD34. No extraneurological lesion was noted. Except cysts and vascular malformations, posterior fossa tumours have been exceptionally reported in fetuses. SFT was distinguished from hemangiopericytoma. In spite of the fact these tumours share many similarities, some criteria such as the staining pattern for CD34 instead indicated a SFT. Histology was distinctive of hemangioblastoma and primitive neuroectodermal tumour. The prognosis of solitary fibrous tumour, which is usually a benign tumour, was there worsened by the precocity of the onset and the local invasion causing disruption of the cerebellum, compression of the brain stem and severe ventriculomegaly.     

Parasagittal solitary fibrous tumor resembling hemangiopericytoma

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor in the central nervous system, and the clinical behavior of this tumor is similar to that of meningioma. We report the case of a Japanese woman with parasagittal SFT that resembled hemangiopericytoma (HPC). Histological examination revealed that the tumor was highly cellular, with cells containing oval- or spindle-shaped nuclei arranged in sheets or a pattern-less growth mode. Focal vascular proliferation was also observed. Some areas showed intercellular stroma containing remarkable eosinophilic collagens. Tumor cells showed a strong immunoreactivity for CD34 but were negative for S-100 protein and epithelial membrane antigen. MIB-1 labeling index of the tumor was 6.6%. Owing to the high cellularity, high MIB-1 labeling index, and focal vascular proliferation, it was difficult to distinguish this lesion from HPC. However, the tumor was finally diagnosed as SFT on the basis of the strong immunostaining for CD34 and absence of pericellular reticulin. HPC. The clinical and pathological features of SFT and HPC and their differential diagnoses are discussed.     

脑膜恶性孤立性纤维性肿瘤临床病理学观察

目的：探讨脑膜恶性孤立性纤维性肿瘤的临床病理特征、核磁共振特点、鉴别诊断及治疗和预后。方法：报道一例脑膜恶性孤立性纤维性肿瘤临床病理、影像学资料并复习文献。结果：患者,男性,65岁,临床主要表现为四肢乏困,无力,记忆力差,MRI示左颞枕叶可见约8.5cm×6.5cm×6cm的囊实性病灶,术中发现肿瘤累及脑膜,与周围脑组织界限清楚,送检肿瘤组织灰白色均质状,有囊性变。镜检：肿瘤由交替性分布的细胞丰富区和细胞稀疏区组成,细胞排列呈条束状、席纹状或无结构样,部分区域呈血管外皮瘤样结构,部分区域细胞丰富密集,有异型性,核分裂象多见（〉4个/10HPF）,未见明显坏死。免疫表型：肿瘤细胞Vimentin、CD99、bcl-2、CD34弥漫阳性,p53弱阳性,Ki-67〉10%。病理诊断：左颞枕叶脑膜恶性孤立性纤维性肿瘤。结论：脑膜恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并与脑的其它梭形细胞肿瘤鉴别。治疗以手术完整切除为主,可预防性放疗并密切随访。     

脑膜恶性孤立性纤维性肿瘤临床病理学观察

目的:探讨脑膜恶性孤立性纤维性肿瘤的临床病理特征、核磁共振特点、鉴别诊断及治疗和预后。方法:报道一例脑膜恶性孤立性纤维性肿瘤临床病理、影像学资料并复习文献。结果:患者,男性,65岁,临床主要表现为四肢乏困,无力,记忆力差,MRI示左颞枕叶可见约8.5cm×6.5cm×6cm的囊实性病灶,术中发现肿瘤累及脑膜,与周围脑组织界限清楚,送检肿瘤组织灰白色均质状,有囊性变。镜检:肿瘤由交替性分布的细胞丰富区和细胞稀疏区组成,细胞排列呈条束状、席纹状或无结构样,部分区域呈血管外皮瘤样结构,部分区域细胞丰富密集,有异型性,核分裂象多见(>4个/10HPF),未见明显坏死。免疫表型:肿瘤细胞Vimentin、CD99、bcl-2、CD34弥漫阳性,p53弱阳性,Ki-67>10%。病理诊断:左颞枕叶脑膜恶性孤立性纤维性肿瘤。结论:脑膜恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并与脑的其它梭形细胞肿瘤鉴别。治疗以手术完整切除为主,可预防性放疗并密切随访。     

Solitary Fibrous Tumor of the Prostate: Case Report and Review of the Literature

Solitary fibrous tumor (SFT), usually described in the pleura, is exceedingly rare in the prostate. We report a 60-year-old man with prostatic SFT revealed by obstructive urinary symptoms, and detected by ultrasonography. Computed tomography (CT) and magnetic resonance imaging suggested a prostatic origin. CT-guided tumor biopsy diagnosed a SFT. A cystoprostatectomy was performed. Pathologic examination showed a 15-cm tumor arising from the prostate and showing histological criteria suggestive of aggressiveness. The surgical resection margins were tumor-free. The patient was then regularly monitored and is still alive in complete remission, 28 months after surgery. In conclusion, we report a new exceptional case of prostatic SFT. We review the literature and discuss the challenging issues of misdiagnosis, prognosis and treatment.Key words: Prostate, Solitary fibrous tumor, Prognosis     

Primary intracranialRhabdomyosarcoma: Case report and review of the literature

Summary Invasion of the meninges is a relatively common complication of head and neck rhabdomyosarcoma (RMS), while RMS arising primarily within the brain or meninges is rare. We report the case of an 11-year old child with a primary “primitive” frontal lobe tumor, subsequent leptomeningeal spread and fatal intratumoral hemorrhage; the diagnosis of RMS was discovered only at postmortem examination. The literature contains a total of 34 reported cases of primary intracranial RMS. This tumor has been observed to arise in a variety of central nervous system (CNS) locations in patients of all ages, but most commonly within the posterior fossa of children. Leptomeningeal dissemination and spontaneous intratumoral hemorrhage are important clinical features. Postoperative chemotherapy and craniospinal radiation may improve the anticipated poor prognosis of patients treated with surgery and radiation alone. The diagnosis of RMS may be missed unless electron microscopic and specific immunohistochemical studies are applied to “undifferentiated” or “primitive” CNS tumors.     

Growth properties of SF188/V+ human glioma in rats in vivo observed by magnetic resonance imaging

SF188/V+ is a highly vascular human glioma model that is based on transfection of vascular endothelial growth factor (VEGF) cDNA into SF188/V? cells. This study aims to assess its growth and vascularity properties in vivo in a rat model. Thirty-two adult rats were inoculated with SF188/V+ tumor cells, and, for comparison, five were inoculated with SF188/V? tumor cells. Several conventional magnetic resonance imaging (MRI) sequences were acquired, and several quantitative structural (T₂ and T₁), functional [isotropic apparent diffusion coefficient (ADC) and blood flow], and molecular [protein and peptide-based amide proton transfer (APT)] MRI parameters were mapped on a 4.7 T animal scanner. In rats inoculated with SF188/V+ tumor cells, conventional T₂-weighted images showed a highly heterogeneous tumor mass, and post-contrast T₁-weighted images showed a heterogeneous, strong enhancement of the mass. There were moderate increases in T₂, T₁, and ADC, and large increases in blood flow and APT in the tumor, compared to contralateral brain tissue. Microscopic examination revealed prominent vascularity and hemorrhage in the VEGF-secreting xenografts as compared to controls, and immunohistochemical staining confirmed increased expression of VEGF in tumor xenografts. Our results indicate that the SF188/V+ glioma model exhibits some MRI and histopathology features that closely resemble human glioblastoma.     

A case of cytokeratin 20-positive large-cell neuroendocrine carcinoma of the breast

We report a rare case of large-cell neuroendocrine carcinoma of the breast. A 63-year-old woman was admitted to our hospital with a firm mass in the right breast. Mammography revealed a high-density mass with specula, and ultrasonography showed a heterogeneous hypoechoic mass with irregular margin and posterior acoustical shadowing. She underwent breast-conserving surgery with axillary lymph node dissection. Histopathologically, the tumor consisted of large cells with polygonal and prominent nuclei and a small amount of eosinophilic cytoplasm, and these cells showed palisading arrangement with fibrous stroma. The tumor cells were positive for neuroendocrine markers such as NSE, synaptophysin, CD56 (NCAM), but not for chromogranin A. Interestingly, the tumor cells showed reactivity for cytokeratin 20 but not for cytokeratin 7. The immunostaining pattern is different from the usual adenocarcinoma of the breast. The patient received postoperative radiotherapy and was given adjuvant chemotherapy. She has remained disease-free for 44?months.     

Recurrent intracranial solitary fibrous tumor initially diagnosed as hemangiopericytoma

We describe a case of an intracranial solitary fibrous tumor that recurred three times consecutively in an 11-year period. A 72-year-old man presented with a headache and gait disturbance. Magnetic resonance imaging (MRI) revealed a dumbbell tumor at the left tentorium. The tumor was removed but recurred. The first diagnosis was hemangiopericytoma, but all specimens showed a “patternless pattern” and few reticulin fibers, which features were not compatible with hemangiopericytoma. All tumors showed immunoreactivity for CD34 and bcl-2. These results point to a solitary fibrous tumor (SFT) and not to hemangiopericytoma. We present here a hypercellular spindle-cell tumor that was very similar to hemangiopericytoma but is better diagnosed as SFT.     

Hemorrhage and VEGF Expression in a Case of Primary CNS Lymphoma

Hemorrhage at presentation in primary CNS lymphoma is exceedingly rare. We describe a patient with primary CNS lymphoma who presented with seizures and was found to have an intracerebral hemorrhage which prompted a cerebral angiogram. Ultimately pathologic evaluation of the lesion revealed a highly cellular B-cell lymphoma with marked hypervascularity and intense expression of vascular endothelial growth factor (VEGF). A review of four other recent cases of primary CNS lymphoma at our institution revealed less-intense VEGF immunoreactivity. This is the first report of VEGF expression in primary CNS lymphoma. The potential significance of VEGF expression with respect to the biology of this tumor is discussed.     

膀胱孤立性纤维性肿瘤 1 例报告并文献复习

目的:探讨膀胱孤立性纤维性肿瘤(solitary fibrous tumor，SFT)的临床表现、影像学表现、病理学形态、免疫表型、生物学行为、诊断、鉴别诊断、治疗方法及其预后。方法:回顾性分析 1 例膀胱 SFT 的临床资料、病理学形态、免疫表型、诊断治疗及随访情况，并结合国内外文献进行分析。结果:本例患者行根治性肿瘤切除术，肿瘤肉眼观表面光滑，切面灰白，质略韧，有包膜，无出血、坏死；镜检:肿瘤界清，无明显侵袭性，细胞稀少区与丰富区交替分布，以梭形细胞为主，核深染，染色质均匀；免疫表型:肿瘤细胞弥漫表达 CD34、Bcl-2；病理诊断为:（膀胱）孤立性纤维性肿瘤。术后随访 24 个月，体格检查正常，行盆腔 MRI 检查未见复发及远处转移。结论:膀胱SFT的诊断依赖临床表现、影像学以及病理组织学检查，确诊则须依靠病理学的免疫组化检查。膀胱 SFT生物学特性大多呈良性表现，根治性切除是主要治疗手段，但少数亦可局部复发或转变为恶性，故有必要对膀胱SFT患者术后进行长期随访。     

胸内孤立性纤维瘤3例报告并文献复习

Objective

The aim of this study was to investigate the imaging findings of intrathoracic solitary fibrous tumor (SFT), so as to improve its diagnosis and differential diagnosis.

Methods

The clinical, imaging and pathological data of three intrathoracic SFTs confirmed by surgical pathology were analyzed retrospectively. There three cases all received spiral CT plain scan and enhanced scan, among which two multi-planar reformation (MPR) and one MR plain scan. And literatures were reviewed to investigate the imaging findings of intrathoracic SFT.

Results

The three intrathoracic SFT located at intra-pulmonary, oblique fissure pleura and posterior chest wall visceral pleura, respectively. All were solitary masses. One case was a peripheral lung mass at dorsal segment of left lower lobe which CT plain scan showed as a soft tissue mass well circumscribed, enhanced scan showed that there were enhanced clustered, lineal small vascular signs in the mass during arterial phase, delayed scanning displayed that the mass showed heterogeneous enhancement and parts of solid content showed gradual enhancement, and there were shorter T₁ signals on MRI T₁WI, map-like high-low mixed signals on T₂WI, mainly with short T₂ signals. One case was an oblique fissure mass which plain scan showed as a homogeneous soft tissue mass with oval in shape and smooth edge, and enhanced scan showed moderate homogeneous enhancement. One case was a mass localized at posterior chest wall visceral pleura which CT plain scan showed as a heterogeneous mass, and enhanced scan showed that there was slight ring-like enhancement, large non-enhancing necrosis area in the mass and facing vessels in the vicinity region. All these three cases had no hilar and mediastinal lymphadenectasis. Operation and pathology results showed that the mass was well circumscribed, with capsule or false capsule. Under microscope, tumor cells were long fusiform, presenting bundle, turbulence or irregular arrangement. Hypocellular and hypercellular area appeared alternately, with interspersed coarse scar-like collagen fibers with hyalinization. There were hemangiopericytoma-like structures under parts of visual fields. There were bronchiole and alveolar epithelium in the legion at dorsal segment of left lower lobe in one case. The results of immunohistochemistry showed that the expressions of CD34 or CD99, Bcl-2 and vimentin were all strong positive.

Conclusion

Intrathoracic SFT might be rare, which imaging findings could have relative characteristic features and diagnosis must depend on histopathology and immunohistochemistry examination.     

Case of epithelioid sarcoma of the lower extremity

A 26-year-old woman with epithelioid sarcoma is reported. An 8 X 4 cm mass was present in the pretibial region of the right leg. The operative specimen revealed a solid mass with necrosis and hemorrhage. Histologically, the tumor consisted of large round or oval cells with rich and eosinophilic cytoplasm and many vascular invasions. Electron-microscopically, the tumor was composed of light and dark cells. The dark cells showed abundant microfibrils in their cytoplasm compared to the light cells. We posit that the tumor was of synovial cell origin because of the presence of cytoplasmic microfibrils, pinocytotic vesicles and filopodias or microvilli-like structures.     

cTNM vs. pTNM: the effect of not applying ultrasonography in the identification of cervical nodal disease

Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells with pericytic differentiation. It accounts for 3-5% of soft tissue sarcomas and 1% of vascular tumors. It usually presents in 5^th to 6^th decade of life. Most common sites are limbs, pelvis and head and neck. About 20% of all hemangiopericytomas are seen in head and neck, mostly in adults. Usually it presents in orbit, nasal cavity, oral cavity, jaw, parotid gland, parapharyngeal space, masticator space and jugular foramen. Long term follow up is important because of imprecise nature of the histological criteria for prediction of biologic behavior. We report herein a case of HPC in 66-year-old man, who presented in our department with headache, nasal obstruction and dysphagia. A neck computer tomography scan and magnetic resonance imaging showed a large left parapharyngeal mass bulging into nasopharynx and oropharynx with extension to pharyngeal mucosal surface and causing narrowing of airways and total obstruction of left posterior nostril. Angiography showed a highly vascular neoplasm. Initially he was managed as a case of schwannoma and embolization was done but with no response. An attempt to do complete surgical resection was made, but due to its critical position, it was not possible. During surgery, highly vascularised tumor was found. The histopathologic examination revealed a vascular tumor consistent with hemangiopericytoma G-II. The patient had normal postoperative course of healing and was given adjuvant radiation. He is on regular follow up without signs of recurrence or metastases. In summary, parapharyngeal space is a rare site of presentation for hemangiopericytoma which is highly vascular tumor, requiring extensive work up including magnetic resonance imaging, computed tomography scan and angiography. Complete surgical excision should be attempted. Postoperative radiation is indicated in cases of incomplete resection.     

Surgical resection using retroperitoneal approach for solitary fibrous tumor in the pelvis

This report concerns a case of solitary fibrous tumor (SFT) for which surgical resection was performed using a retroperitoneal approach. A 41-year-old man was referred to our hospital with urinary retention. Abdominal ultrasound sonography (US) and computed tomography (CT) showed a hypervascular mass lesion in the pelvis. Transrectal biopsy showed SFT. Surgical resection was carried out using a retroperitoneal approach and preserving the neural network related to urinary and erectile functions. Based on immunohistochemical findings, the tumor was diagnosed to be malignant SFT in the pelvic cavity. Urinary function improved post-operation. There was no change to IIEF-5 and it continued to function well. The patient showed no sign of recurrence 12 months after surgery and required no additional therapy.     

A case of metastases to the paranasal sinus from rectal mucinous adenocarcinoma

Metastases of malignancies to the sinonasal tract are rare. We report a case of metastases to the paranasal sinus from rectal adenocarcinoma. A 72-year-old woman, who had a history of therapy with rectal adenocarcinoma, came to our hospital for visual loss in her right eye. Computed tomography revealed soft tissue in the right paranasal sinus. She underwent endoscopic sinus surgery immediately and an easily bleeding tumor was found in the paranasal sinus. Magnetic resonance imaging showed a mass involving the posterior ethmoid, the sphenoid sinus, and the orbit. From the surgical specimen, we found that the tumor was a metastasis of a previously managed rectal mucinous adenocarcinoma. Metastatic carcinomas of the sinonasal tract have a high risk of heavy hemorrhage during endoscopic sinus surgery. When a patient with visual loss has a history of malignancy, a possible metastatic tumor should be considered before surgery.     

Malignant hemangioendothelioma of occipital bone

Epithelioid hemangioendothelioma is a rare vascular tumor of bone, and rarely these lesions can present as unique and extremely aggressive tumor. We report a case of highly aggressive epithelioid hemangioendothelioma and discuss the imaging findings. CT brain plain study revealed a poorly-defined, mixed density expansile and lytic lesion involving the occipital bone with extension to the left side with poorly defined trabecula formation. There was significant but irregular enhancement after intravenous administration of contrast material and also marked bone destruction. Microscopic examination of the fine needle aspiration cytology showed a tumor composed of vascular channels lined by plump endothelial cells, which had enlarged hyperchromatic nuclei. In view of the extensive infiltration the patient was submitted for the radiotherapy.     

Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location

We report a rare case of a peripheral primitive neuroectodermal tumor (PPNET) originating from the left ileopsoas muscle in an adult patient with neoplastic thrombosis of the left external iliac vein, the common femoral vein and the left popliteal vein. We performed a median laparotomy with an oblique left inguinal incision to remove the neoplasm, which consisted of a large mass surrounding the iliac-psoas muscles, extending from the transverse apophysis of the spinal column to Scarpa's triangle, and passing through the lacuna musculorum. Histopathological examination revealed a primitive neuroectodermal tumor (PNET) with focal areas of necrosis, hemorrhage and vascular invasion. Immunophenotyping was positive for CD99, NSE, and focally for CK. Ultrastructural examination of the neoplastic cells showed often multiple nuclei with dense chromatin and very large nucleoli. The patient was discharged ten days after the operation. Adjuvant treatment consisted of radiotherapy at a dose of 2000 cGy in five fractions followed by six cycles of chemotherapy. The venous thrombosis was treated by anticoagulant therapy and recanalization of the occluded veins was obtained after two months of therapy. An MRI scan, carried out 12 months later, showed a local relapse, which was treated with chemotherapy and arterial chemoembolization.