Solitary Fibrous Tumors in the Central Nervous System

2003 ◽  
Vol 127 (4) ◽  
pp. 432-439 ◽  
Author(s):  
Tarik Tihan ◽  
Michael Viglione ◽  
Marc K. Rosenblum ◽  
Alessandro Olivi ◽  
Peter C. Burger

Abstract Context.—Solitary fibrous tumors (SFTs) of the central nervous system are rare neoplasms that usually present as dura-based masses and clinically resemble meningiomas. Histologically, they can be similar to fibrous meningioma or hemangiopericytoma (HPC). In particular, densely cellular regions seen in some SFTs can be indistinguishable from HPC. Little is known about the biological behavior of SFTs, although most seem amenable to total resection. Objectives.—To define the clinicopathologic spectrum of SFTs in the central nervous system and to outline their differences from HPC and meningioma. Design.—We present the clinicopathologic features of 18 patients with SFT and compare them with those of an age- and sex-matched cohort of HPCs. Results.—Eleven SFTs were supratentorial, 3 were infratentorial, and 4 were intraspinal. Four of the 18 tumors were intra-axial (2 in the lateral ventricles and 2 within the spinal cord). Histologically, SFTs were similar to their soft tissue counterparts. Six tumors (6/18) had densely cellular regions, and 1 tumor showed frankly anaplastic features. All but 3 patients underwent gross total resection, and there were no metastases or tumor-related mortalities during the median follow-up of 40 months. In contrast, there were 15 local recurrences (83%), 5 extracranial metastases (27%), and 4 tumor-related deaths (22%) in the HPC cohort. Conclusions.—Our study presents the clinicopathologic features of SFT as a distinct entity from both meningioma and HPC. We also present unusual examples of anaplastic, intraventricular, and intramedullary spinal SFTs that expand the clinicopathologic spectrum of these uncommon and sometimes diagnostically difficult neoplasms.

2021 ◽  
Vol 13 (1) ◽  
pp. 259-266
Author(s):  
Ye-Tao Zhu ◽  
Yang Liu ◽  
Li-Gang Chen ◽  
Da-Ping Song

Solitary fibrous tumor is a very rare mesenchymal tumor that occurs mostly in the pleura, and there are few reported cases of a presence in the central nervous system, particularly in the cerebellum. In 2016, the WHO classified solitary fibrous tumors into grade I. In this article, we present a case of malignant solitary fibrous tumor recurring 8 years after surgery in a 63-year-old male. Magnetic resonance imaging showed low to intermediate mixed signal intensity on T1W1. Immunohistochemical staining positivity for Vimentin, CD99, CD34 and Bcl-2, it is consistent with the immunohistochemical characteristics of solitary fibrous tumor. We resected the patient’s tumor, and the patient was followed up for 3 months with no signs of recurrence. Solitary fibrous tumors are very rare in the central nervous system. Immunohistochemical staining positivity for CD34 and Bcl-2 is strongly expressed in most solitary fibrous tumor. Surgical resection is the preferred treatment. Due to the small number of cases, the biological behavior and prognosis of this tumor need to be further explored.


Neurosurgery ◽  
2007 ◽  
Vol 60 (4) ◽  
pp. 715-722 ◽  
Author(s):  
Philippe Metellus ◽  
Corinne Bouvier ◽  
Jacques Guyotat ◽  
Stéphane Fuentes ◽  
Anne Jouvet ◽  
...  

Abstract OBJECTIVE This is a retrospective study of 18 patients harboring a solitary fibrous tumor of the central nervous system. Therapeutic management and outcome were analyzed. METHODS Between 1999 and 2004, 18 patients harboring central nervous system solitary fibrous tumors were surgically treated at our two institutions. There were nine men and nine women. The patient ages ranged from 33 to 75 years, with a median age of 56.1 years. The locations were spinal in six cases (33.3%), the posterior fossa in six cases (33.3%), supratentorial in four cases (22.2%), and orbital in two cases (11.2%). RESULTS The median follow-up period was 45.3 months. Gross total resection was achieved in 10 cases (55.6%); tumor recurrence or progression occurred in nine cases (50%). Incomplete surgical resection was significantly associated with recurrence (P = 0.018). On univariate analysis, extent of surgery was also associated with a longer progression-free survival (P = 0.05). No statistically significant correlation can be found between histological features and recurrence. CONCLUSION Prognosis of solitary fibrous tumors of the central nervous system remains unclear; consequently, careful and close monitoring of patients and long-term follow-up are mandatory. Radical surgical excision seems to be a significant and reliable prognosis factor, although pathological prognostic features must be defined. In other respects, the role of postoperative radiotherapy in atypical or incompletely resected solitary fibrous tumors of the central nervous system remains to be determined and, therefore, warrants larger series with longer follow-up periods.


1991 ◽  
Vol 75 (1) ◽  
pp. 73-76 ◽  
Author(s):  
Hernando Mena ◽  
Jorge L. Ribas ◽  
Franz M. Enzinger ◽  
Joseph E. Parisi

✓ Angiosarcoma arising in the central or peripheral nervous system has rarely been reported. Eight patients with primary angiosarcoma of the central nervous system are described here; these included five males and three females ranging in age from 2 weeks to 72 years (mean 38 years). Of the eight neoplasms, six were located in the cerebral hemispheres and one was in the meninges; the site was unknown in the other. All patients underwent surgical resection. Five of the eight patients died, four within 4 months after surgery and one after 30 months. Two of the remaining three patients were 17 and 27 years old at the time of diagnosis and were alive at follow-up review 39 and 102 months after surgery, respectively. One patient was lost to follow-up monitoring. Microscopically, all eight tumors demonstrated a well-differentiated pattern with irregular vascular channels and intraluminal papillae; in addition, four showed poorly differentiated solid areas. Immunohistochemical staining of neoplastic cells to factor VIII-related antigen and Ulex europaeus agglutinin I was performed in five tumors and was focally positive in four. No correlation could be shown between the histological features and the growth and biological behavior of the tumors.


2020 ◽  
Vol 6 (2) ◽  
pp. 20190081
Author(s):  
Chi Long Ho ◽  
Jeremy JH Lam ◽  
Robert Chun Chen

Primary intracranial tumours rarely metastasise outside of the central nervous system (CNS). This report describes a rare case of recurrent meningeal haemangiopericytoma with extensive systemic metastases, which eventually resulted in a fatal outcome. We discuss some prevailing theories as to the rarity of extracranial metastases from primary CNS haemangiopericytoma, and elucidate the epidemiology, imaging features, differential diagnosis, treatment, and prognosis of this unusual but surprisingly aggressive meningeal tumour. Besides aggressive treatment for local tumour control, patients with primary CNS haemangiopericytoma require long-term post-treatment surveillance to detect systemic metastases.


2013 ◽  
Vol 11 (2) ◽  
pp. 224-226 ◽  
Author(s):  
Carlos Eduardo Molinari Nardi ◽  
Alexandre Wakil Burzichelli ◽  
Elio Gilberto Pfuetzenreiter ◽  
Rogerio Aparecido Dedivitis

Schwannoma is a benign encapsulated tumor that originates from the Schwann cells lining nerve fibers outside the central nervous system. We report a rare case of schwannoma that arose from the left arythenoid cartilage The patient underwent excision of the mass through microlaryngeal endoscopic procedure. No recurrence was observed during follow-up.


2020 ◽  
Vol 13 (9) ◽  
pp. e235412
Author(s):  
Jesse Mooneyham ◽  
Cesar Gentille ◽  
Andrea Barbieri ◽  
Shilpan Shah

A 33-year-old woman presented to the emergency room with severe headaches. A CT scan of the head revealed two brain lesions with associated vasogenic oedema. Diagnostic resection of one of the lesions followed by pathological analysis revealed grade III lymphomatoid granulomatosis (LYG). Staging investigations elsewhere in the body were negative, isolating this case of LYG to the central nervous system, an atypical presentation. After the resection, she was treated with single-agent rituximab 375 mg/m2. The follow-up MRI demonstrated the resolution of brain lesions and no progression of the disease.


2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Hedia Klaa ◽  
Thouraya Ben Younes ◽  
Hanene Benrhouma ◽  
Sonia Nagi ◽  
Aida Rouissi ◽  
...  

Rasmussen’s encephalitis (RE) is a rare progressive inflammatory disease of the central nervous system. It is characterized by unilateral hemispheric atrophy, pharmacoresistant focal seizures, and progressive neurological deficit. The exact etiopathogenesis still remains unknown. Brain imaging plays an important role in diagnosis and follow-up. Fluctuation of lesions in brain imaging was reported in few cases. Herein, we report an additional pediatric case of Rasmussen encephalitis with fluctuating changes in brain MRI.


Neurosurgery ◽  
1981 ◽  
Vol 8 (3) ◽  
pp. 391-396 ◽  
Author(s):  
Nelson G. Ordóñez ◽  
Alberto G. Ayala ◽  
Milam E. Leavens

Abstract The case of a 33-year-old woman with oligodendroglioma with extraneural metastases involving the masseter muscle, the cervical lymph nodes, the lumbar vertebrae, and the right iliac bone is described. Oligodendroglioma with metastases outside the central nervous system is extremely rare, and only a very few cases have been reported.


1957 ◽  
Vol 35 (6) ◽  
pp. 751-768 ◽  
Author(s):  
Bruce M. McCraw

The topographic anatomy of Lymnaea humilis is described, giving the relative positions of the various internal organs. A description of the anatomy of the cephalic hemocoele is also given. Like Lymnaea stagnalis the central nervous system of L. humilis consists of the paired cerebral, buccal, pedal, pleural, and parietal ganglia and the unpaired abdominal ganglion. The nerves arising from the central nervous system are described and particular attention was devoted to the nerves arising from the pedal ganglia and innervating the foot; fourteen new nerves are named or described. The reproductive system of L. humilis is divided into: (a) the ovotestis and its duct, (b) the female system, and (c) the male system. In reproductively active snails, the female system is the largest of the three portions, and consists of the uterus, oothecal gland, vagina, and seminal receptacle. An accessory structure, the albumen gland, is very large in L. humilis. The male system consists of the upper and lower prostate, vas deferens, and male copulatory organ. The connections between the hermaphrodite duct and the male and female systems show considerable structural consolidation compared to these connections in L. stagnalis. The muciparous gland is not a distinct entity in L. humilis.


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