|Year : 2018 | Volume
| Issue : 7 | Page : 1578-1582
Diagnosis and treatment of hemangiopericytoma in the central nervous system
Fang Liu1, Boning Cai1, Yu Du2, Yurong Huang1
1 Department of Radiation Oncology, Chinese PLA General Hospital, Beijing, China
2 Department of Radiation Oncology, First Affiliated Hospital of PLA General Hospital, Beijing, China
|Date of Web Publication||19-Dec-2018|
Department of Radiation Oncology, Chinese PLA General Hospital, No. 28, Fuxing Road, Beijing-100853
Source of Support: None, Conflict of Interest: None
Objective: This study aimed to explore the clinical characteristics and treatment of the hemangiopericytoma (HPC) in the central nervous system.
Materials and Methods: Clinical data from 14 patients with HPC in the central nervous system who were followed up for 12–107 months were retrospectively analyzed, and relevant literature was reviewed.
Results: All 14 patients underwent surgery and postoperative pathologic diagnosis, including 8 cases of total excision, 3 cases of subtotal excision, and 3 cases of partial excision. There were 7 recurrent cases (50%, 4 cases of total excision, 1 case of subtotal excision, and 2 cases of partial excision, none of them received postoperative radiotherapy) with a median relapse time of 39 months, where the median relapse time after total excision was 41.5 months, and after nontotal excision was 17 months. In addition, patients could survive for a long time after reoperation following relapse and after receiving postoperative radiotherapy.
Conclusion: The diagnosis of HPC depends on the pathology. Currently, the surgery and postoperative radiotherapy provide a good treatment results, while the wholeness of surgical resection is of particular importance.
Keywords: Central nervous system, clinical features, hemangiopericytoma, radiotherapy
|How to cite this article:|
Liu F, Cai B, Du Y, Huang Y. Diagnosis and treatment of hemangiopericytoma in the central nervous system. J Can Res Ther 2018;14:1578-82
| > Introduction|| |
Hemangiopericytoma (HPC) is a soft connective tissue tumor that originates from the hemangiopericytes, and was first identified by Stout and Murray in 1942. The average age of onset is 43 years, and it is more commonly found in males than in females. HPC rarely originates from the central nervous system. It is usually solitary and is mostly connected with dura mater, which is adhered to cerebral falx or sagittal sinus or occurs in the epidural areas, and more rarely in the cerebral parenchyma., Before 1993, intracranial HPC was divided into a subtype of meningioma for a long time. Some scholars have divided vertebral HPC into epidural type (intraspinal and extraspinal), extradural type (intramedullary and extramedullary) and intradural type, among which extradural type and intramedullary type are the most rarely found. Before diagnosis, the disease usually lasts <1 year, and is most likely to be misdiagnosed. In computed tomography (CT) examination, HPC shows irregular high density, with flake low-density foci inside, and the tumors may display lobulated features. However, it presents different manifestations in magnetic resonance imaging (MRI). Most of the cases are irregular or lobulated, with mixed signals and uneven enhancement due to cystic degeneration and necrosis. The HPC substrate is relatively narrow. The intracranial lesions should be differentiated from meningiomas and solitary fibrous tumor., The diagnosis of HPC relies on pathology, which is characterized by staghorn branched cells. The pathological features include CD99, vimentin and reticulated fiber dyeing, CD34 positive, partial CD31 positive, STAT6 positive, glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), and S-100 negative, Ki-67 (+1%–15%). The WHO classification of HPC was based on the cell density, heteromorphism, nuclear mitosis, Ki-67 proliferation index, presence and absence of hemorrhage and necrosis.,
The present study reviewed and analyzed the data from 14 patients HPC in the central nervous system who were admitted to our hospital between June 2007 and December 2016.
| > Clinical Data|| |
A total of 14 patients, including 10 males and 4 females (male to female ratio; 2.5:1) were selected. Their age ranged from 9 to 62 years, with a median age of 47 years. There were 10 cases of intracranial HPC and 4 cases of intraspinal HPC. In intracranial HPC cases, tumors were found in the tentorium cerebelli, parafalcine, and parieto-occipital regions, and while most of them were closely related to the dura mater, in only one case the tumor was found in the cerebral parenchyma. Furthermore, all the 4 cases of intraspinal HPC were of extradural type; multiple intracranial lesions were observed in 2 cases, while all the other cases had single lesion [Table 1].
|Table 1: General information, as well as surgery, treatment and follow-up of the 14 cases|
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Symptoms and signs
The common symptoms included headache (5 cases), unfavorable motor control of limbs (4 cases) and dizziness (3 cases), where headache and dizziness were often accompanied by other symptoms. Other symptoms included: decreased visual acuity (2 cases), pain in neck, shoulder and back (2 cases), olfactory hallucination (1 case), paraplegia (1 case), nystagmus (1 case), slow response (1 case), and other manifestations in the corresponding impaired functional regions. The time frame from the appearance of symptoms to diagnosis ranged from 1 to 24 months, with a median duration of 5.5 months.
| > Imaging Results|| |
MRI scan and enhanced scan were performed in all 14 cases, where CT examination was also performed in the 4 cases of intracranial type and 1 case of intraspinal type. The CT manifestations in five patients were consistent, showing a high or slightly high density of lobulated mass, with flake low-density shadows visible inside. Nevertheless, the MRI showed diverse results: the T1-weighted (T1WI) revealed slightly high, equal, low, or mixed signal; T2WI revealed equal, high, or mixed signal; enhanced scan showed significantly enhanced results most of which were uneven [Figure 1] and [Figure 2]. In one case, a void signal with dural tail sign appeared, while other case was accompanied by lamina bone destruction. None of the patients underwent angiography.
|Figure 1: Computed tomography/magnetic resonance imaging images of patient No. 13 showing lesion in the right middle cranial fossa, (a) high-density lesion in computed tomography, (b) magnetic resonance imaging axial scan, slightly high T1 signal, (c) enhanced result in magnetic resonance imaging corona|
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|Figure 2: Magnetic resonance imaging sagittal scan (high T2 signal) of No. 6 patient showing recurrent lesion in T8-10 spinal|
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| > Treatment and Outcomes|| |
Surgery and radiotherapy
All 14 patients underwent craniotomy or spinal canal excision. During operation, it was found that tumors had abundant blood supply, while most of them had hard texture and clear boundaries. The tumor base was located in cerebral falx, tentorium cerebelli, and extradural. Pathological diagnosis confirmed HPC in all patients. There were 8 cases of total excision (6 cases of intracranial type and 2 cases of intraspinal type), 4 cases of subtotal excision (2 cases of intracranial type and 2 cases of intraspinal type), and 2 cases of partial excision (intracranial type). The patients' symptoms, such as the blurred vision and the unfavorable motor of limbs, slightly improved after the operation. Three patients underwent fractional external radiotherapy after the initial surgery, with a total amount of 50 Gy, while other 11 patients did not undergo radiotherapy due to different reasons [surgery and radiotherapy were shown in [Table 1]. Three patients reported dizziness, while other manifestations of slight brain edema were detected during radiotherapy, all of which improved after symptomatic dehydration treatment.
Under a microscope, cells in the tumor were densely and irregularly arranged, of uniform size, with spindle or polygonal shape, and varying degrees of interstitial fibrosis. In addition, there were abundant mesh fabric surrounding each cell, and a large number of staghorn vessels of different sizes. Nuclei were oval, with visible nuclear mitosis, which presented heteromorphosis. Immunohistochemistry CD99, vimentin, and sweet staining were positive. Thirteen cases were CD34 positive and three cases CD31 positive. All patients had GFAP and EMA negative, as well as Ki-67 (+1%−15%) [Figure 3].
|Figure 3: Hematoxylin-eosin and IHC staining. (a) Spindle cells, vessels with staghorn signs, (Hematoxylin-eosin, ×100) (b) CD99 (+), (Immunohistochemical, ×100), (c) vimentin (+), (Immunohistochemical, ×200), (d) sweet staining (+), (Histochemical, ×200)|
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Patients were followed up for 12–107 months [Table 1], with a median duration of 40.5 months. There were 7 recurrent cases (50%, including 4 cases undergoing total excision, 1 case undergoing subtotal excision, and 2 cases undergoing partial excision, none of them received postoperative radiotherapy), with a median time to relapse of 39 months (10–73 months). The median time to relapse for patients undergoing total excision was 41.5 months, while it was 17 months for patients undergoing nontotal excision. Five patients relapsed within 5 years after the surgery (36%). Recurrent patients underwent the second surgery, and most of them received radiotherapy after the surgery. Two patients with intraspinal HPC selected 40 Gy/5f Cyberknife treatment after relapse, where one case relapsed again at 31 months after postoperative Cyberknife treatment, while other case did not relapse from postoperative Cyberknife treatment to the end of the follow-up (13 months). In two cases, metastasis occurred in other parts, including one case of intraspinal to intracranial metastasis and one case of intracranial to pulmonary metastasis. Among all the patients, two died: one patient who suffered from the second relapse and metastasis of intraspinal lesions and had multiple intraspinal lesions, and another patent who suffered from metastasis of intracranial lesions to the lung with a pathological grading of the WHO II, where the pathological grade did not change after relapse, with Ki-67 (+<10%), respectively. These two patients gave up treatment after learning about metastasis, and died 6 and 4 months after metastasis, respectively. Among all patients, one patient with intracranial lesion who did not receive radiotherapy after partial excision relapsed at 10 postoperative months, and the pathological grading changed to Grade III after relapse. Subsequently, the patients underwent subtotal excision again and received 50 Gy external radiotherapy after the surgery, and did not relapse after another follow-up for 32 months. One patient with intraspinal lesions suffered from five times of recurrence and metastases, and the lesions metastasized to cerebellopontine angle and foramen magnum at the second time of relapse. The patient underwent surgery, and subsequently experienced three times of in situ relapse. Each time after surgery and/or radiotherapy, the pathological grading maintained Grade II, and the patient survived 8 years up to the end of the follow-up.
| > Discussion|| |
HPC that originates from the central nervous system is very rare. The median age of the 14 patients enrolled in the present study was 47 years old. The lesions were in the cerebral parenchyma in all the 14 cases, among which the 4 cases of vertebral HPC were of extradural type. The clinical manifestations mainly included headache and dizziness, and impairment of the corresponding functional regions, such as blurred vision and limb movement disorder.
In the past, the disease was not fully understood, and people were subjected to misdiagnosis. In the present study, imaging diagnosis was not consistent with the pathological diagnosis in 5 cases. Three cases were separately diagnosed as meningioma and solitary fibrous tumor by MRI. Nevertheless, the meningioma had smooth margin and uniform signals, it was rarely lobulated, with visible calcification and dural tail signs. Meanwhile, HPC is often characterized by cystic changes and necrosis, lobulation, and mixed signals. In addition, meningioma often has wide base, while the base of HPC is relatively narrow. Moreover, a low-signal area in T2WI is the characteristic change of solitary fibrous tumor. Although imaging is helpful for diagnosis, the diagnosis mainly depends on the pathology. In the present study, three patients were misdiagnosed with pathological method for the first time, which was corrected after relapse.
Surgical excision and postoperative radiotherapy are widely recognized as effective treatments for HPC. Total excision is main factor affecting the prognosis of HPC, which easily recurs in patients after operation, with an average relapse time of 40–70 months. Liu et al. have found that the median progression-free survival (PFS) time in patients undergoing total tumor excision (PFS, 89 months) was significantly longer than those undergoing partial excision (49 months), and although patients undergoing partial excision received adjuvant radiotherapy after operation, the 5-year relapse rate was as high as 75%. In the present study, the median time to recurrence was 41.5 months in patients receiving total excision, which was significantly longer compared to 17 months in patients undergoing nontotal excision, and was consistent with the existing literature. Although the median PFS in patients receiving total excision was significantly shorter than that reported in the literature, which may be associated with a small number of cases and postoperative auxiliary radiotherapy. The histological grade of HPC is at the level of II-III, where the higher grade implies the shorter relapse time, while with the increase of the relapse frequency, the histological grade also increases. In the present study, 12 patients had a pathological Grade II at initial diagnosis, while two patients were not classified, among which the pathological grade changed to Grade III after relapse in two patients, and it did not change in another patient after multiple relapses. The relapse rate of the whole group was 36%, which might be associated with a short follow-up period. The metastasis related to this disease often occurs in the bone, but it can also occur in lung and liver. Among patients in the present study, the tumor that metastasized from the spinal canal to the intracranial region was observed in one case, and the tumor that metastasized to was observed in another case.
Radiotherapy also has an important role in the treatment of HPC. Due to abundant blood supply and intraoperative bleeding, Schiariti et al. have recommended to perform preoperative 20–30 Gy radiotherapy so as to shrink the tumors by 80%–90% and reduce blood supply for facilitating surgical excision. Consequently, they have reported that postoperative radiotherapy could reduce the local relapse rate from 88% to 12.5%. In the present study, there were no patients undergoing preoperative radiotherapy, while the effect of preoperative radiotherapy on the operation was not observed. Many patients of HPC would be planned for adjuvant radiotherapy in view of high grade of histopathology and doubtful margins. Earlier studies by Guthrie et al. have revealed that a radiation dose of >51Gy could more effectively reduce the relapse rate, while recent studies have shown that a radiation dose of ≥60G could more effectively reduce local relapse rate., Hence, it may be appropriate to use a radiation dose of 51–60 Gy. In the present study, only three patients received radiotherapy after the initial operation, 50–55 Gy. Nevertheless, due to a short follow-up period and a small number of cases, the relationship between radiotherapy and radiation dose with curative effect has not yet been observed. In terms of radiotherapy methods, there was no significant difference between gamma knife and external radiotherapy., Radiosurgery or stereotactic radiotherapy is an effective treatment option for nervous system tumors. It is noteworthy that among two patients with intraspinal lesions who were selected to undergo surgery and a single postoperative CyberKnife treatment after relapse, one patient experienced recurrence at 31 months after surgery + CyberKnife treatment, while the other patient did not experience recurrence until the end of the follow-up after CyberKnife treatment alone. These results revealed a good curative effect. However, due to the small number of cases, the curative effect and the related side effects need to be studied further.
| > Conclusion|| |
HPC in the central nervous system is a rare disease. The use of precise radiotherapy combined with surgery to treat tumors and reduce normal tissue damage should be further investigated.
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Conflicts of interest
There are no conflicts of interest.
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