Clinical features and prognostic factors of primary intracranial malignant fibrous histiocytoma: A report of 8 cases and a literature review

Peng Li, Qiangyi Zhou, Zhijun Yang, Zhenmin Wang, Shiwei Li, Xingchao Wang, Bo Wang, Fu Zhao, Pinan Liu,

1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China

2Department of Neural Reconstruction, Beijing Neurosurgery Institute, Capital Medical University, Beijing 100050, China

Clinical features and prognostic factors of primary intracranial malignant fibrous histiocytoma: A report of 8 cases and a literature review

Peng Li1, Qiangyi Zhou1, Zhijun Yang1, Zhenmin Wang1, Shiwei Li1, Xingchao Wang1, Bo Wang1, Fu Zhao2, Pinan Liu1,2

1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China

2Department of Neural Reconstruction, Beijing Neurosurgery Institute, Capital Medical University, Beijing 100050, China

ARTICLE INFO

Received: 10 July 2016

Revised: 10 July 2016

Accepted: 20 August 2016

© The authors 2016. This article is published with open access at www.TNCjournal.com

clinical features;

Objective: Primary intracranial malignant fibrous histiocytoma (MFH) is rare. We describe the detailed clinical features of 8 cases and fully review the literature to evaluate several prognostic factors.

1 Introduction

Fibrous histiocytomas (FHs) are tumors with fibroblastic and histiocytic components[1]. They usually occur in soft tissues and bones, and are rare in intracranial locations[2]. Intracranial FH can arise from the dura or within the brain parenchyma. FHs are classified as benign (BFH) or malignant (MFH), basedon the absence or presence of anaplasia, respectively[3]. MFH is a high-grade malignant tumor with poor prognosis[4]. To date, only around 60 primary intracranial MFHs have been reported[2]. Most of them are isolated case reports. No single institution is likely to see more than a handful of these rare intracranial tumors over the course of several decades. Therefore, only a careful review of all published cases can provide us with good understanding.

2 Methods

2.1 Case definition and search methods

Between 2008 and 2015, 8 patients with primary intracranial MFH were assessed at the Department of Neurosurgery, Beijing Tiantan Hospital. Their pathological diagnoses were confirmed by 2 independent neuropathologists, according to the 2007 World Health Organization (WHO) criteria. No extracranial sources for these tumors were found clinically, and no extracranial metastases were observed during the follow-up. Data regarding clinical presentation, radiological imaging, pathologic results, and treatment outcome were obtained through a review of the patient records. This retrospective study was approved by the Beijing Tiantan Hospital institutional review board. We searched PubMed for relevant articles containing the term “intracranial malignant fibrous histiocytoma”. Previously reported primary intracranial MFH cases were reviewed in their entirety.

2.2 Follow-up and statistical analysis

All patients were followed up through telephone interview or at an out-patient department. For MFH patients, Kaplan-Meier survival analysis and a logrank test were used to evaluate the survival predictors. The chi-square test was used for analysis of sex predisposition. A P value <0.05 was considered statistically significant. The analyses were performed using the SPSS statistical software, version 19 (SPSS Inc.).

3 Results

3.1 Clinical characteristics

The clinical characteristics of 8 patients (4 women and 4 men) are summarized inTable 1. Three patients had received previous radiotherapy for treatment of Langerhans cell neoplasm (patient 2), meningioma (patient 7), and medulloblastoma (patient 8), respectively. The ages of the patients ranged from 19 to 69years, with a median age of 48 years. Preoperative duration of symptoms varied from acute to 6 months, with a median duration of 1.5 months. The most common preoperative symptom was headache (n = 7), and 1 patient experienced preoperative visual deficiency (patient 7). Most symptoms were slowly progressive, and 2 patients experienced acute onset of intracranial hemorrhage (patient 1 and patient 6).

Table 1Clinical features of 8 cases with primary intracranial malignant fibrous histiocytoma

3.2 Radiological findings

All patients underwent preoperative magnetic resonance imaging (MRI) examination. The tumors were usually located within the brain parenchyma or adjacent to the dura. They lacked specific radiological features and were usually misdiagnosed as glioma or meningioma. Most tumors were isointense or hypointense on T1-weighted images, and isointense or hyperintense on T2-weighted images. Peritumoral edema was evident on the T2-weighted images. The tumors were usually heterogeneously enhanced after contrast administration. Some patients underwent preoperative CT examination; and the tumors were usually slightly hyperdense compared to gray matter.

3.3 Treatment and outcome

All patients underwent surgery. During surgery, the tumors were usually found to be firm and hypervascular. Most tumors could be totally resected; and only one tumor was subtotally resected (patient 7). Six patients received postoperative radiotherapy and 3 patients received postoperative chemotherapy. Most patients died from tumor recurrence within the first year after surgery; and only 1 patient was still alive at the date of the last follow-up (patient 8). Two patients experienced tumor recurrence during the follow-up and 1 patient underwent 2 subsequent surgeries (patient 2).

3.4 Histological findings

Histologically, tumor tissues were usually composed of spindled and plump cells arranged in ill-defined storiform patterns, with malignant biologic behaviors, such as nuclear pleomorphism and histological anaplasia. Multinucleated giant cells were occasionally observed. Histological diagnosis was aided by immune staining. The detailed immunohistochemical results are listed inTable 2. Most tumors were negative for GFAP and S-100, and positive for vimentin and CD68.

3.5 Literature review and survival analysis

In 2004, Hamlat et al.[5]performed a complete review of intracranial MFH reports published before 2003. Based on their study and 12 additional reports[6–17], we identified 55 primary intracranial MFH cases documented with sufficient clinical information. Tumors arising from the skull bone or subcutaneous tissue extending into the brain were excluded. The 7 MFH patients in our report were also included in the analysis (patient 8 was excluded because of suspicious bone or subcutaneous tissue origin), which gave a total of 62 patients. Twenty-nine patients were female, and 33 patients were male (Figure 1a, chi-squared test, P = 0.258). The age of the patients ranged from 4 months to 75 years, with a median age of 43 years(Figure 1b). Sixteen patients who died postoperatively or were lost during the follow-up were excluded from the survival analysis, which left 46 patients. Kaplan-Meier survival analysis showed that most intracranial MFH patients died within the first year after surgery (Figure 1c). Around one quarter of patients have a good prognosis and survive for a long time. Young patients (less than 30 years old) seemed to have a better prognosis and survive longer than older patients (more than 30 years old) (Figure 1d, log-rank test, P = 0.008). However, sex (P = 0.675), extent of resection (P = 0.934), postoperative radiotherapy (P = 0.592), and postoperative chemotherapy (P = 0.424) seemed not affect the patient’s prognosis.

Table 2Results of immunohistochemical staining in 8 cases

Figure 1 (a, b)Bar graph depicting the sex and age distribution in a total of 62 MFH patients.(c)Kaplan-Meier plots of overall survival for 46 MFH patients.(d)Kaplan-Meier plots of overall survival for MFH patients older (dotted line) or younger (solid line) than the age of 30 years old (log-rank test, P = 0.008).

3.6 Illustrative cases

Case 1

A 44-year-old previously healthy man presented with headache and vomiting for 8 days. He did not have any head trauma history. Neurological examination revealed no positive signs. Cranial CT scan showed a hematoma in the left occipital parietal lobe (Figures 2a–2f). The density of the hematoma was heterogeneous on the CT image. A glioma apoplexy was suspected. Immediate surgery with a left parietal approach was performed to clear the hematoma and resect the tumor. The tumor was totally resected. The pathological diagnosis was MFH. The patient recovered well after surgery and received postoperative radiotherapy and chemotherapy. However, 4 months after surgery, he died from the recurrent tumor.

Case 5

A 45-year-old man presented with headache for 6 months. Neurological examination revealed no positive signs. He had no previous history of radiotherapy or head trauma. Cranial MRI showed a large tumor lesion in the left temporal lobe, which was suspected to be meningioma or hemangiopericytoma (Figures 3a–3f). During preoperative preparations, the patient experienced an acute attack of epilepsy and lost consciousness. The pupil size of both eyes was unequal (left:right = 2.5:1.5 mm). A cerebral hernia was suspected. Emergency surgery was performed to resect the tumor and decrease the intracranial pressure. The patient did not recover well and died 3 days after surgery. The pathological diagnosis of the resected tumor was MFH.

Case 7

Figure 2Case 1. Preoperative cranial CT shows an evident hematoma in the left occipital parietal lobe(a–c). Postoperative cranial CT shows total resection of the tumor(d & e). Pathological examination(f)reveals the diagnosis of MFH. (Hematoxylin & eosin staining, ×100)

A 69-year-old woman presented with bilateral visual loss for 1 month. Her symptoms progressed rapidly. In the week prior to consulting our institution, she experienced deteriorating consciousness and couldnot recognize her relatives. Fifteen years ago, a small suspicious meningioma was found at her anterior cranial fossa. Because of its small size and the absence of symptoms, the patient refused surgery and instead received stereotactic radiotherapy (gamma knife). When she came to our institution, a cranial MRI showed an enlarged tumor at the anterior cranial fossa (Figure 4). Immediate surgery with a left frontal approach was performed to resect the tumor. During the operation, the tumor was found to be firm with some indications of previous hemorrhage. The tumor was tightly adhered to the anterior cerebral arteries and other structures. Only subtotal resection could be achieved. The pathological diagnosis was MFH. Although the patient received postoperative radiotherapy, 4 months after surgery she died from the recurrent tumor.

Case 8

A 50-year-old woman presented with headache for 1 month. Neurological examination revealed no positive signs. Eight years ago, the patient underwent surgery for fourth ventricular medulloblastoma and received postoperative radiotherapy. MRI showed a tumor lesion in the posterior cranial fossa (Figures 5a–5c). The tumor extended outward to the subcutaneous tissues through the occipital bone. Recurrent medulloblastoma was suspected. An occipital craniotomy was performed to totally resect the tumor (Figures 5e and 5f). During the operation, the tumor was found to be firmand hypervascular. Most of the tumor tissues were intracranial extradural. The occipital bone and dura mater were eroded by the tumor. The pathological diagnosis was MFH (Figure 5d). The postoperative course was uneventful. The patient received postoperative radiotherapy and chemotherapy. After a 2-year follow-up, the tumor recurred in the extracranial subcutaneous soft tissues (Figures 5g and 5h). The patient refused a second operation and received further treatment with radiotherapy and chemotherapy.

Figure 3Case 5. Preoperative cranial magnetic resonance imaging (MRI) shows a tumor lesion in the left temporal lobe. The tumor is isointense on T1-weighted image(a), and hyperintense on T2-weighted image(b). A peritumoral edema can be observed, and the brain stem is compressed. The tumor is heterogeneously enhanced after contrast administration(c–e). Pathological examination(f)reveals the diagnosis of MFH. (Hematoxylin & eosin staining, ×100)

Figure 4Case 7. Preoperative cranial magnetic resonance imaging (MRI) shows a tumor lesion located at the anterior cranial fossa. The tumor is isointense on T1-weighted image(a), and hypointense on T2-weighted image(b). The tumor is heterogeneously enhanced after contrast administration(c–e). Pathological examination(f)reveals the diagnosis of MFH. (Hematoxylin & eosin staining, ×100)

4 Discussion

FHs are rare tumors of mesenchymal origin[3]. The term “fibrous” is used in a descriptive sense, rather than indicating a cell of fibrotic origin[18]. They may originate from the perivascular pial sheath, mesenchymal cells, deep blood vessel walls, and primitive mesenchymal cells[2]. MFH is defined as a group of pleomorphic sarcomas that show no line of differentiation[12,19]. They are most commonly located in the soft tissues of the extremities and retroperitoneal regions[15]. MFHs account for 10.5%–21.6% of all malignant neoplasias of soft tissue[20]. MFHs in the central nervous system are extremely rare, but have been reported in the brain, dura mater, and spine[5]. Some MFHs arising from the skull bones or subcutaneous tissues can also extend into the cranial cavity[21–23]. A few intracranial MFHs have metastasized from a primary extracranial tumor[19,24,25]. The first intracranial MFH was reported by Gonzalez-Vitale et al. in 1976[26]. Most intracranial MFHs were found arising from the dura or within the brain parenchyma. Rare cases were reported to be intraventricular[27], within thesellar[16], or at the cerebellopontine angle[28]. Intracranial MFHs were usually supratentorial and rarely found in the cerebellum[5,12]. The etiology of MFH is unclear. A few reported cases have been associated with factors such as a previous history of radiation therapy[26,29], chemotherapy[30], or surgical trauma[31]. Stimulation of histiocytes and fibroblasts after damage might induce malignant transformation.

MFHs of the extracranial soft tissues are thought to be tumors of old age, with the peak incidence in the seventh decade of life[12]. But most patients with intracranial MFH are relatively young (median age 44 years), and pediatric patients are common (Figure 1b). As reported, intracranial MFHs do not have sex predominance (Figure 1a). Clinical manifestations of intracranial MFH are various and mainly depend on the location of the tumor[11]. Acute intracranial hemorrhage has been reported in several cases, like the 2 patients in our report (patient 1 & 6)[5,32,33]. Intracranial MFH does not have specific radiological features on MRI and CT images due to its variable degrees of histological differentiation[15]. Arteriography of the tumor can be vascular or avascular[34,35]. Tumors appear hypermetabolic on PET-CT scan, just like other malignant tumors[15].

MFH is a controversial pathological entity, and some authors have expressed considerable doubts about its identity[5,19]. MFH is usually diagnosed after elimination of other possible tumors, such as gliosarcoma, glioblastoma multiforme, xanthoastrocytoma, and malignant schwannoma. Diagnosis should be aided by immune staining. MFH is usually negative for GFAP and S-100, and positive for vimentin and CD68 (Table 2)[5,10,12]. In the past, MFH was thought to have 6 histological subtypes: storiform-pleomorphic, giant cell, inflammatory, atypical, myxoid, and angiomatoid[5]. The latter 2 subtypes were excluded by the WHO classification in 2002 and reclassified as separate entities[12]. All MFH tumors were classified as high-grade sarcomas of grade 2 or grade 3 on a 3-tiered grading system. The most common variant of intracranial MFH is the storiform-pleomorphic type, which comprises spindle cells in a storiform pattern, plump histiocyte-like cells, and pleomorphic multinucleated giant cells[22]. The other 3 subtypes have rarely been reported[35–37]. All fibrous histiocytomas inour report were consistent with the features of the storiform-pleomorphic type.

Figure 5Case 8. Preoperative cranial magnetic resonance imaging (MRI) shows a tumor lesion (arrow) in the posterior cranial fossa extending outward to the subcutaneous tissues(a–c). Pathological examination(d)of the resected tumor reveals the diagnosis of MFH. (Hematoxylin & eosin staining, ×100) Postoperative cranial MRI shows no remnant of the tumor(e, f). Two years after the operation, cranial MRI shows recurrence of the tumor, mainly in the subcutaneous tissues(g, h).

Like extracranial MFH, the prognosis of intracranial MFH is very poor[5,38,39]. Most patients die within the first year after the diagnosis. However, young MFH patients (less than 30 years old) seem to have a relatively better prognosis (Figure 1d), which has also been noticed by several other authors[40,41]. The recommended treatment for MFH is gross surgical removal, followed by radiotherapy and/or chemotherapy[35,42]. Most reported intracranial MFHs could be resected totally. However, the extent of resection seems not to affect the patient’s prognosis. In treatment of soft tissue MFH, the role of chemotherapy and radiotherapy is still debatable[38]; and it has proven difficult to improve the prognosis of intracranial MFH. However, the detailed regimens of chemotherapy and radiotherapy varied a lot in different studies. Their role in MFH treatment still needs to be validated.

5 Limitations

This is a retrospective and literature review study of a rare entity in the intracranial location. The small patient group and inconsistency of different studies may affect the accuracy of statistical results. Fresh tumor tissues are not available for additional genetic analysis to differentiate them from other tumors. As no single institution is likely to see more than a handful of these rare intracranial tumors over the course of several decades, a prospective study seems impractical. Further reports of this rare entity may help us to better understand its clinical features.

6 Conclusions

Primary malignant fibrous histiocytomas are rare intracranial tumors, and pediatric patients are relatively common. The prognosis of MFH is usually poor, and most patients die within the first year after surgery. Young MFH patients (less than 30 years old) seem to have a better prognosis and survive longer than older patients.

Conflict of interests

The authors have no financial interest to disclose regarding the article.

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Li P, Zhou QY, Yang ZJ, Wang ZM, Li SW, Wang XC, Wang B, Zhao F, Liu PN. Clinical features and prognostic factors of primary intracranial malignant fibrous histiocytoma: A report of 8 cases and a literature review. Transl. Neurosci. Clin. 2016, 2(3): 155–164.

* Corresponding author: Pinan Liu, E-mail: pinanliu@ccmu.edu.cn

Supported by the National Science and Technology Support Program of the 12th Five-Year of China (grant number: 2012BAI12B03) and Natural Science Foundation of Beijing (grant number: 7112049).

prognostic factors;

intracranial malignant fibrous histiocytoma;

literature review

Methods: Eight patients with pathologically confirmed primary intracranial MFH were retrospectively reviewed. We searched PubMed for relevant articles with the term “intracranial malignant fibrous histiocytoma”.

Results: Of the 8 patients, 4 were men and 4 were women. Three patients had received previous radiotherapy. The age of the patients ranged from 19 to 69 years, with a median age of 48 years. Most tumors could be totally resected; and only 1 tumor was subtotally resected. Six patients received postoperative radiotherapy and 3 patients received postoperative chemotherapy. Most patients died within the first year after surgery; and only 1 patient was still alive on the date of the last follow-up. We reviewed the literature and included a total of 46 patients in the Kaplan-Meier survival analysis. Young patients (less than 30 years old) seemed to have a better prognosis and survival rate than older patients (more than 30 years old) (log-rank test, P = 0.008). However, sex (P = 0.675), extent of resection (P = 0.934), postoperative radiotherapy (P = 0.592), and postoperative chemotherapy (P = 0.424) did not affect patient prognosis.

Conclusions: The prognosis of MFH is usually poor, and most patients die within the first year after surgery. Younger MFH patients (less than 30 years old) seem to have a better prognosis and improved survival compared to older patients.