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Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review
Journal of Clinical Neuroscience xxx (2018) xxx–xxx
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Review article
Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review Ziquan Li a, Yuzhoujia Deng b, Zhimin Li b, Tianyu Wang b, Jun Gao b,⇑, Weixun Zhou c, Yongning Li b, Yipeng Wang a a b c
Department of Orthopedics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
a r t i c l e
i n f o
Article history: Received 10 August 2018 Accepted 6 October 2018 Available online xxxx Keywords: Hemangiopericytoma Thoracic spine Epidural Recurrence Metastasis Positron emission tomography
a b s t r a c t Hemangiopericytoma (HPC) is a rare tumor originating from pericapillary cells. Rarely found in the central nervous system, it is extremely rare in the spinal canal. Because of the low incidence of this tumor, its radiographic features and clinical manifestations have not been extensively studied and reported, therefore, it is often misdiagnosed as a schwannoma or spinal meningioma. We describe an unexpected HPC in a 35-year-old woman who was admitted to the Peking Union Medical College Hospital with a severe backache, sensory abnormalities, and muscle weakness. Magnetic resonance imaging showed an enhancing lesion at T6–7 with severe compression of the spinal cord. Gross total resection was achieved, and subsequently, a marked neurologic improvement was observed. The diagnosis of primary extradural HPC in our patient was confirmed based on postoperative histopathology and immunohistochemistry. Neither recurrence nor metastasis of the tumor was found during the 14-month follow-up, which did not include radiotherapy. To describe the demography, radiologic features, treatment, and prognosis of spinal HPC, a comprehensive literature review was performed and 105 cases of primary spinal HPC from 1958 to 2017 were collected from 39 articles. Although rare, HPC should be considered in the differential diagnosis of intraspinal lesions. Immunohistologic examination is of decisive importance in making the diagnosis. Adequate surgical resection, when feasible, is the first choice of treatment for all cases of HPC; however, the outcomes of radiotherapy and chemotherapy have yet to be determined. Individualized treatment combined with long-term follow-up for each patient is recommended. Ó 2018 Published by Elsevier Ltd.
1. Introduction Hemangiopericytomas (HPCs) are complex, rare, aggressive neoplasms that were first formally reported by Stout and Murray in 1942 [1]. These are vascular tumors originating from pericapillary cells or the pericytes of Zimmerman. Based on retrospective series research, HPCs may occur in any age group and demonstrate no sex-based predisposition [2]. They tend to occur at any site in the human body that contains capillaries but are commonly present in the lower extremities, retroperitoneum, head, and neck [3,4]. However, they are rarely reported in the central nervous system, where they constitute around 2–4% of all primary meningeal tumors and less than 1% of all HPCs [5,6]. HPC tumors arising in the ⇑ Corresponding author at: Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China. E-mail address: [email protected] (J. Gao).
spinal cord are exceptionally rare. Since Schirger first reported a case of spinal HPC in 1958, retrospective case reports or case series have demonstrated that only about 100 cases of primary spinal HPCs have been published in the literature [7]. In this study, we present a case of primary thoracic spinal HPC in a 35-year-old female who presented with lower limb weakness, hypoesthesia, and backache. Magnetic resonance imaging (MRI) showed an epidural lesion at the T6–7 level with T1 hypointensity and T2 hyperintensity. Marked inhomogeneous enhancement was seen on administration of contrast medium, which led to preoperative misdiagnosis of the condition as a schwannoma. The rarity, absence of radiographic features, and ambiguous clinical manifestations of HPC often lead to the misdiagnosis of lesions in the spinal canal. They may be confused with other, similar diagnoses, such as spinal meningioma, neurofibroma, or schwannoma. The exact demography, radiologic features, treatment, and prognosis of spinal HPC await further investigation. Hence, to the best of our knowledge, our study is the first
https://doi.org/10.1016/j.jocn.2018.10.057 0967-5868/Ó 2018 Published by Elsevier Ltd.
Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057
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Z. Li et al. / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
comprehensive literature review of 105 cases of primary spinal HPC from 1958 to 2017.
2. Case report A 35-year-old Chinese woman without a medical or family history of neoplastic disease presented with a severe backache, which had been noted first 3 months earlier. The pain was diffuse, dull, and paroxysmal and without an identifiable cause. Symptoms were alleviated by rest. The numerical rating scale (NRS) scores for pain were 2 to 3 initially, and then, over the 2 weeks preceding hospital admission, the pain became worse (NRS scores of 5 to 6) in association with lower extremity weakness and a staggering gait. The patient did not seek medical therapy, except for oral analgesics. However, her symptoms recurred after discontinuation of the drug. She denied having any issues with passing urine or changes in bowel habits. The patient was hospitalized at the Peking Union Medical College Hospital owing to the progression of her signs and symptoms. After admission, she underwent a complete physical and neurologic examination combined with blood chemistry and MRI evaluations. There was no abnormality in the curvature or mobility of the spine, but tenderness and pain on percussion were noted at the paravertebral and spinous processes of T5–7. Motor examination revealed paraparesis of both lower extremities graded 4/5 according to the scale of the British Medical Research Council (BMRC). Furthermore, sensory examination showed decreased
sensation of light touch and pinprick below T6. The Babinski sign was elicited on both sides. Otherwise, the patient’s pathologic reflexes, superficial reflexes, tendon reflexes, and coordinated motor and upper limb neurologic examination were unremarkable. There were no rectal disorders, such as difficulty evacuating or urinary incontinence. On biochemical examination, routine blood markers, hepatorenal function, electrolytes, and inflammatory markers, including the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were within normal ranges. Plain radiographic views of the anteroposterior and lateral thoracic spine showed no marked abnormalities. Thoracic-enhanced MRI revealed an oval mass (3.8 1.6 2.5 cm) displaying an inhomogeneous signal at the level of T6–7. This mass was hypointense on T1-weighted imaging (T1WI) and hyperintense or isointense on T2-weighted imaging (T2WI). After the intravenous administration of gadolinium contrast medium, images of the lesion showed marked and asymmetrical enhancement, indicating severe compression of the spinal cord (Fig. 1). Based on the complaints, clinical manifestations, physical examination, and radiologic characteristics, the patient was diagnosed preoperatively with a suspected intraspinal schwannoma. A dark red tumor covered with a thin capsule was found on the right side of the dura mater after T6–7 laminectomy by piezosurgery. The tumor was well-defined, soft, and highly vascular; it had pushed the spinal cord to the left ventral side. Gross total resection was achieved, and marked neurologic improvement was subsequently observed. Histopathologic examination of the
Fig. 1. Preoperative MRI features of primary epidural hemangiopericytoma at the T6–7 level. Sagittal MRI image shows extradural tumor at T6–7. It was hypointense on T1WI (A) and hyperintense or isointense on T2WI (B). Post-gadolinium sagittal MRI depicting inhomogeneous enhancement after gadolinium injection (C). Axial MRI showing that the tumor mass had invaded the spinal canal and compressed the spinal cord on the ventral side (D).
Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057
Z. Li et al. / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
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Fig. 2. Histologic examination of the epidural lesion with hematoxylin and eosin staining. The features seen in the photomicrograph are in accordance with the diagnosis of HPC (A). AE1/AE3 and QBEND10 (CD34) were positive on immunohistochemical staining (B).
tumor tissue showed that the tumor cells were oval or spindleshaped with acidophilic cytoplasm; they were surrounded by numerous branched vessels. The tumor cells were immunepositive for QBEND10 (CD34) and AE1/AE3 but negative for EMA, SMA, CD31, desmin, GFAP, and S-100. The Ki67 index was 10%. The postoperative histopathologic and immunohistochemical features were compatible with a diagnosis of HPC (Fig. 2). After surgery, the patient returned for follow-ups in the outpatient department at 3 months, 6 months and 12 months. Clinical and imaging examinations included NRS scores, as well as evaluations of motor and sensory function. The patient’s symptoms had improved dramatically, and MRI revealed complete resection of the tumor (Fig. 3). Positron emission tomography (PET) was performed during the follow-up period to confirm that there was no recurrence or metastatic spread of the tumor during the followup period.
3. Discussion Pericytes, first described by the Swiss scientist K.W. Zimmerman, are a kind of immature mesenchymal cell with contraction and differentiation potential that wrap themselves around capillaries [8]. Pericytes play an important role in providing mechanical support to capillaries and altering capillaries’ luminal sizes through their contractile power under normal physiologic conditions [9]. It is generally believed that HPC is a vascular tumor derived from pericytes in soft tissues; it could be located at any site that contains capillaries but is rarely found in the central nervous system (CNS). Most CNS HPCs occur in the cranial cavity, and more rarely, in the spinal canal. Because of their rarity, information is scarce regarding their clinical characteristics, preoperative diagnosis, imaging features, and prognosis. Therefore, we report on an uncommon case of primary thoracic epidural HPC and summarize our comprehensive literature review regarding this condition (Table 1) [10–46].
Our research yielded 105 cases of primary spinal HPC from 1958 to 2017. These cases indicate that primary spinal HPC can occur at any age, and that the mean age at diagnosis was 36.3 years (ranging from 2 to 80 years). The most vulnerable age range was 20–39 years (42.9%), followed by 40 to 59 years (28.6%). HPC was slightly more prevalent in males (male-tofemale ratio = 1.33:1), which was in accord with the former articles [46,47]. Primary spinal HPCs were more commonly located in extradural spaces (58 of 105 cases, or 55%), followed by intradural spaces (39 of 105 cases, or 37%) and intra- to extradural regions (8 of 105 cases, or 8%). The intradural lesions are divided into intramedullary and extramedullary subtypes, and the extradural lesions are further classified as being based on either dura or bone. Furthermore, according to statistical data from our literature review, cervical and thoracic segments constitute the most common sites of spinal lesions (37% and 40%, respectively), along with the lumbar segment (18%); the lesions are rarely found in the sacral segment (5%). The clinical presentation of primary spinal HPC is nonspecific. Complaints and symptoms vary depending on tumor size and location, but they are not associated with differing histologic types. The most common presenting symptoms for extradural HPCs are neurologic deficits and weakness of the limbs because the extradural compartment has been occupied, which is similar to other spinal lesions, such as schwannoma, neurofibroma, ependymoma, and meningioma [6,23,34,48]. Conversely, in primary osseous spinal HPCs, pain and mass effects are the most common complaints because osseous HPCs are more likely to involve the paravertebral extension [49]. In our case, the patient was suffering from lower extremity weakness, hypoesthesia below T6, and severe backache, which conforms with previous reports. Spinal HPCs do not demonstrate specific radiographic features and are difficult to differentiate from other tumors. A plain x-ray commonly displays no significant abnormality, unless the vertebral body or pedicles are involved and depending on the extent of the tumor. The typical MRI characteristics of spinal HPCs are
Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057
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Fig. 3. Evaluation by postoperative MRI at 6-month follow up after gross total resection. Sagittal T1WI (A), T2WI (B) and dense enhancement image (C) demonstrating no recurrence or residual tumor. Postoperative axial T2WI displays complete excision of the tumor and decompression of the spinal cord at T6–7 (D).
heterogeneous, predominantly isointense masses on T1WI and T2WI with compression of the spinal cord. Since HPC is a tumor that arises from the pericytes of capillaries, it is possible that marked enhancement with internal vessel voids could be shown on gadolinium-enhanced images [50]. Moreover, some features from previous reports have been described to distinguish intraspinal HPCs [51–53]. The presence of serpentine vessels, a narrow base of dural attachment, a dural tail sign, and absence of calcifications on MRI may point to HPC preoperatively. The description of HPC imaging in our report was not typical, which was why the patient was misdiagnosed with schwannoma. Clinically and radiologically, spinal HPC is a rare disease. It is difficult to diagnose and also easily misdiagnosed. We analyzed the possible causes of the missed diagnoses, including nonspecific radiographic features, variations in morphology and location, and the pathologic categorization of HPCs as it has evolved over recent years [54,55]. In short, the proper diagnosis of this disease calls for close attention to a combination of clinical and radiographic features. Heightened suspicion for spinal HPCs should be elicited by the presence of internal signal voids, heterogeneous enhancement, and adjacent bony erosion. In addition, histopathology and immunohistochemistry are required to confirm the diagnosis. Previous articles have shown that surgical excision is the most widely recommended treatment for intraspinal HPC. HPC has a strong tendency to recur locally and to metastasize because the tumor is almost always attached to the dura. Based on our statistical analysis of 94 intraspinal HPC patients (11 patients were
removed owing to the lack of recurrence-related information), the recurrence rate was 50% (38.5% in the case of intradural tumors, 44.8% in the case of extradural tumors, and 75% in the case of intra- to extradural tumors). Hence, some researchers advocate the complete en bloc resection of the tumor combined with excision of the neighboring dura mater to avoid recurrence and metastasis [5,23,24,33,42,56]. However, a recent study of a 26 case series by Liu et al. proposed that total resection did not have a statistically significant influence on survival or prevention of recurrence [46]. According to our experience, total resection can be attempted for tumors with clear margins and will serve to relieve symptoms; however, for patients at high risk for injury to the nerve root or dura mater, the subsequent adjuvant radiotherapy may be more beneficial than risking total resection. Postoperative radiotherapy and chemotherapy are still controversial. Earlier research suggested that radiotherapy could reduce recurrence and improve survival [6,57–59]. Chou at al. analyzed 16 intraspinal HPC patients who received adjuvant radiotherapy and found that there was no significant difference compared with those who did not receive radiotherapy. Moreover, pathologic grade was the only factor associated with recurrence, a conclusion with which Liu agreed [28,46]. From our statistical analysis of 71 cases selected from 105 cases with detailed records of postoperative therapy and recurrence, our study reached the same conclusions as that of Chou and Liu [28,60]. We are the first to have used PET to evaluate recurrence and metastasis after surgery because PET can monitor these events at an early stage.
Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057
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Z. Li et al. / Journal of Clinical Neuroscience xxx (2018) xxx–xxx Table 1 Summary of previously reported cases of primary spinal HPC. Article
Gender/Age
Schirger (1958) [7] Kruse (1961) [10] Pitlyk (1965) [11]
F/33 T2 Extradural None Yes 1 M/53 C3 Intradural Radiotherapy Yes 4 M/60 C4 Intradural None Yes 2 M/39 T8 Intradural None No 10 F/49 C3 Intradural None Yes 18 M/16 C6–7 Extradural Radiotherapy No 0.75 M/21 T6 Extradural None Unknown Unknown M/62 L5 Extradural Radiotherapy Unknown Unknown M/28 C2–6 Extradural Radiotherapy No 5 F/65 L2 Extradural Radiotherapy No 4 F/31 C6 Extradural None No 1 F/52 C6 Extradural None No 0.1 F/36 C5 Extradural None No 2 M/15 T12 Extradural Radiotherapy Unknown Unknown F/11 T11 Extradural Radiotherapy Yes 6 F/41 L2 Extradural None Unknown Unknown M/30 L3 Intradural Radiotherapy Yes 12 M/48 C4 Intradural None Yes 7 F/36 C6 Extradural Radiotherapy No 2 M/25 S1 Extradural Radiotherapy No 0.75 F/29 L1–3 Extradural Radiotherapy No 10 M/39 T4–6 Extradural Radiotherapy No 3 M/31 L4 Intradural None No 0.5 F/39 L1–2 Extradural None No 2 M/21 T2 Extradural Radiotherapy Chemotherapy Unknown Unknown M/31 T4–6 Intradural None No 3 F/16 T4–5 Extradural Radiotherapy No 0.75 M/80 T10 Intradural None No 3 M/54 L4–5 Intradural Radiotherapy No Unknown M/20 T9–10 Intradural None No 2 M/58 T10 Intradural None No Unknown F/61 C2 Extradural Radiotherapy Unknown 0.25 F/51 T6–7 Intradural None Yes 5 F/51 C3–4 Intra- to extradural None Yes 9 F/27 T7–8 Intradural None No 3 M/56 Occiput to C3 Intradural None Yes 3 M/57 T9–10 Intradural Radiotherapy No 3 M/56 Occiput to C4 Intradural Radiotherapy No 5 M/21 C1–2 Intradural None No 2 M/43 C6–T2 Extradural None No 1 M/28 C3 Extradural Radiotherapy No 1 F/16 T5–6 Extradural None Yes Unknown M/16 T9 Intradural Radiotherapy No 5 F/19 C1–2 Intradural None No Unknown F/15 T9–10 Intradural None No Unknown M/50 C4–5 Intradural Radiotherapy Chemotherapy No 2 M/34 T8–10 Intradural Radiotherapy Chemotherapy No 2 F/37 T7–9 Intra to extradural None No 1 F/12 T11–L1 Intradural Radiotherapy No 0.75 M/37 C5–6 Extradural Radiotherapy Chemotherapy Yes 5 M/63 T9 Intradural None No 1 A report on the clinical course of 60 patients. In 5 cases, a spinal extradural HPC was primarily involved at vertebral levels T1–2, T6–7, T10, T11, and S1. Data regarding recurrence and follow-up period are unknown A report on 23 primary intraspinal HPCs (19 extradural and 4 intradural). The numbers of cases located in the cervical, thoracic, lumbar, and sacral segments were 10, 9, 3, and 1, respectively. Seven patients received radiotherapy. Recurrence rates were 50% for intradural HPCs and 73% for extradural HPCs A retrospective review of 26 patients with HPCs of the spine (14 males and 12 females, mean age: 33.8 years). Nine HPCs were located in the cervical segment, 6 in the thoracic segment, 5 in the lumbar segment, 1 in the cervicothoracic segment, 3 in the thoracolumbar segment and 2 in the lumbosacral segment. Ten patients had intradural HPCs, 10 had extradural HPCs, and 6 had intra- to extradural HPCs. In all, 22 patients accepted postoperative radiotherapy, and 2 patients received chemotherapy; 19 patients had recurrences during the follow-up period
Kriss (1968) [12] Fathie (1970) [13] Gerner (1974) [14] Harris (1978) [15] Stern (1980) [16] Cappabianca (1981) [17] Muraszko (1982) [18]
Ciappetta (1985) [19] Bridges (1988) [20] Salvati (1991) [21] Akhaddar (2002) [22] Betchen (2002) [23] Ijiri (2002) [24] Mohammadianpanah (2004) [25] Kashiwazaki (2007) [26] Kumar (2007) [27] Chou (2009) [28] Fitzpatrick (2009) [4] Moscovici (2011) [29] Ackerman (2011) [30] Santillan (2011) [31] Torigoe (2012) [32] Nakashima (2013) [33] Shirzadi (2013) [34]
Drazin (2013) [35] Lee (2013) [36] Zhang (2014) [37] Raghvendra (2014) [38] Jayashankar (2014) [39] Kaur (2014) [40] Türk (2015) [41] Das (2015) [42]
Chew (2017) [43] McMaster (1975) [44] Zhao (2007) [45]
Liu (2013) [46]
Level
Location
4. Conclusion Primary spinal HPC is a rare disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Primary spinal HPC, with high rates of recurrence and metastasis, causes severe neurologic symptoms and motor disturbances, but it still gets insufficient attention. We have reported an uncommon case of primary epidural thoracic HPC and presented a statistical analysis of earlier cases based on a comprehensive literature review. We emphasize the importance of the proper diagnosis of
Adjuvant therapy
Recurrence
Follow-up (y)
HPC and consider surgical removal to be the best treatment. Postoperative radiotherapy and chemotherapy are not preferred, except in the case of incomplete resection or recurrence. PET may be considered during follow-up to detect recurrence and metastasis as early as possible. Acknowledgments The authors would like to thank all the patients and their family members for their help and informed consent.
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[48] Ecker RD, Marsh WR, Pollock BE, Kurtkaya-Yapicier O, McClelland R, Scheithauer BW, et al. Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. J Neurosurg 2003;98:1182–7. [49] Liu J, Cao L, Liu L, Guo S, Tai H, Chen Z. Primary epidural hemangiopericytoma in the sacrum: a rare case and literature review. Tumor Biol 2014;35:11655–8. [50] Patnaik S, Jyotsnarani Y, Uppin SG, Susarla R. Imaging features of primary tumors of the spine: a pictorial essay. Indian J Radiol Imaging 2016;26 (2):279–89. [51] Osborne DR, Dubois P, Prayer B. Primary intracranial meningeal and spinal hemangiopericytomas: radiological manifestations. AJNR Am J Neuroradiol 1981;2:69–74. [52] Akiyama M, Sakai H, Onoue H, Miyazaki Y, Abe T. Imaging intracranial haemangiopericytomas: study of seven cases. Neuroradiology 2004;46:194–7. [53] Fountas KN, Kapsalaki E, Kassam M, Feltes CH, Dimopoulos VG, Robinson JS, et al. Management of intracranial meningeal hemangiopericytomas: outcome and experience. Neurosurg Rev 2006;29:145–53. [54] Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology 2006;48:63–74. [55] Fletcher CD. The evolving classification of soft tissue tumors: an update based on the new WHO classification. Histopathology 2006;48:3–12. [56] Sundaram C, Shantveer UG, Megha US, Rekha JS, Panigrahi MK, Purohit AK, et al. A clinicopathological and immunohistochemical study of the central nervous system hemangiopericytomas. J Clin Neurosci 2010;17:469–72. [57] Chacko G, Chacko AG, Rajshekhar V, Muliyil JP. Intracranial hemangiopericytomas: correlation of topoisomerase Ilalpha expression with biologic behavior. Surg Neurol 2006;65:11–7. [58] Jaaskelainen J, Servo A, Haltia M, Wahlström T, Valtonen S. Intracranial hemangiopericytoma: radiology, surgery, radiotherapy, and outcome in 21 patients. Surg Neurol 1985;23:227–36. 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Review article
Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review Ziquan Li a, Yuzhoujia Deng b, Zhimin Li b, Tianyu Wang b, Jun Gao b,⇑, Weixun Zhou c, Yongning Li b, Yipeng Wang a a b c
Department of Orthopedics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
a r t i c l e
i n f o
Article history: Received 10 August 2018 Accepted 6 October 2018 Available online xxxx Keywords: Hemangiopericytoma Thoracic spine Epidural Recurrence Metastasis Positron emission tomography
a b s t r a c t Hemangiopericytoma (HPC) is a rare tumor originating from pericapillary cells. Rarely found in the central nervous system, it is extremely rare in the spinal canal. Because of the low incidence of this tumor, its radiographic features and clinical manifestations have not been extensively studied and reported, therefore, it is often misdiagnosed as a schwannoma or spinal meningioma. We describe an unexpected HPC in a 35-year-old woman who was admitted to the Peking Union Medical College Hospital with a severe backache, sensory abnormalities, and muscle weakness. Magnetic resonance imaging showed an enhancing lesion at T6–7 with severe compression of the spinal cord. Gross total resection was achieved, and subsequently, a marked neurologic improvement was observed. The diagnosis of primary extradural HPC in our patient was confirmed based on postoperative histopathology and immunohistochemistry. Neither recurrence nor metastasis of the tumor was found during the 14-month follow-up, which did not include radiotherapy. To describe the demography, radiologic features, treatment, and prognosis of spinal HPC, a comprehensive literature review was performed and 105 cases of primary spinal HPC from 1958 to 2017 were collected from 39 articles. Although rare, HPC should be considered in the differential diagnosis of intraspinal lesions. Immunohistologic examination is of decisive importance in making the diagnosis. Adequate surgical resection, when feasible, is the first choice of treatment for all cases of HPC; however, the outcomes of radiotherapy and chemotherapy have yet to be determined. Individualized treatment combined with long-term follow-up for each patient is recommended. Ó 2018 Published by Elsevier Ltd.
1. Introduction Hemangiopericytomas (HPCs) are complex, rare, aggressive neoplasms that were first formally reported by Stout and Murray in 1942 [1]. These are vascular tumors originating from pericapillary cells or the pericytes of Zimmerman. Based on retrospective series research, HPCs may occur in any age group and demonstrate no sex-based predisposition [2]. They tend to occur at any site in the human body that contains capillaries but are commonly present in the lower extremities, retroperitoneum, head, and neck [3,4]. However, they are rarely reported in the central nervous system, where they constitute around 2–4% of all primary meningeal tumors and less than 1% of all HPCs [5,6]. HPC tumors arising in the ⇑ Corresponding author at: Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China. E-mail address: [email protected] (J. Gao).
spinal cord are exceptionally rare. Since Schirger first reported a case of spinal HPC in 1958, retrospective case reports or case series have demonstrated that only about 100 cases of primary spinal HPCs have been published in the literature [7]. In this study, we present a case of primary thoracic spinal HPC in a 35-year-old female who presented with lower limb weakness, hypoesthesia, and backache. Magnetic resonance imaging (MRI) showed an epidural lesion at the T6–7 level with T1 hypointensity and T2 hyperintensity. Marked inhomogeneous enhancement was seen on administration of contrast medium, which led to preoperative misdiagnosis of the condition as a schwannoma. The rarity, absence of radiographic features, and ambiguous clinical manifestations of HPC often lead to the misdiagnosis of lesions in the spinal canal. They may be confused with other, similar diagnoses, such as spinal meningioma, neurofibroma, or schwannoma. The exact demography, radiologic features, treatment, and prognosis of spinal HPC await further investigation. Hence, to the best of our knowledge, our study is the first
https://doi.org/10.1016/j.jocn.2018.10.057 0967-5868/Ó 2018 Published by Elsevier Ltd.
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comprehensive literature review of 105 cases of primary spinal HPC from 1958 to 2017.
2. Case report A 35-year-old Chinese woman without a medical or family history of neoplastic disease presented with a severe backache, which had been noted first 3 months earlier. The pain was diffuse, dull, and paroxysmal and without an identifiable cause. Symptoms were alleviated by rest. The numerical rating scale (NRS) scores for pain were 2 to 3 initially, and then, over the 2 weeks preceding hospital admission, the pain became worse (NRS scores of 5 to 6) in association with lower extremity weakness and a staggering gait. The patient did not seek medical therapy, except for oral analgesics. However, her symptoms recurred after discontinuation of the drug. She denied having any issues with passing urine or changes in bowel habits. The patient was hospitalized at the Peking Union Medical College Hospital owing to the progression of her signs and symptoms. After admission, she underwent a complete physical and neurologic examination combined with blood chemistry and MRI evaluations. There was no abnormality in the curvature or mobility of the spine, but tenderness and pain on percussion were noted at the paravertebral and spinous processes of T5–7. Motor examination revealed paraparesis of both lower extremities graded 4/5 according to the scale of the British Medical Research Council (BMRC). Furthermore, sensory examination showed decreased
sensation of light touch and pinprick below T6. The Babinski sign was elicited on both sides. Otherwise, the patient’s pathologic reflexes, superficial reflexes, tendon reflexes, and coordinated motor and upper limb neurologic examination were unremarkable. There were no rectal disorders, such as difficulty evacuating or urinary incontinence. On biochemical examination, routine blood markers, hepatorenal function, electrolytes, and inflammatory markers, including the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were within normal ranges. Plain radiographic views of the anteroposterior and lateral thoracic spine showed no marked abnormalities. Thoracic-enhanced MRI revealed an oval mass (3.8 1.6 2.5 cm) displaying an inhomogeneous signal at the level of T6–7. This mass was hypointense on T1-weighted imaging (T1WI) and hyperintense or isointense on T2-weighted imaging (T2WI). After the intravenous administration of gadolinium contrast medium, images of the lesion showed marked and asymmetrical enhancement, indicating severe compression of the spinal cord (Fig. 1). Based on the complaints, clinical manifestations, physical examination, and radiologic characteristics, the patient was diagnosed preoperatively with a suspected intraspinal schwannoma. A dark red tumor covered with a thin capsule was found on the right side of the dura mater after T6–7 laminectomy by piezosurgery. The tumor was well-defined, soft, and highly vascular; it had pushed the spinal cord to the left ventral side. Gross total resection was achieved, and marked neurologic improvement was subsequently observed. Histopathologic examination of the
Fig. 1. Preoperative MRI features of primary epidural hemangiopericytoma at the T6–7 level. Sagittal MRI image shows extradural tumor at T6–7. It was hypointense on T1WI (A) and hyperintense or isointense on T2WI (B). Post-gadolinium sagittal MRI depicting inhomogeneous enhancement after gadolinium injection (C). Axial MRI showing that the tumor mass had invaded the spinal canal and compressed the spinal cord on the ventral side (D).
Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057
Z. Li et al. / Journal of Clinical Neuroscience xxx (2018) xxx–xxx
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Fig. 2. Histologic examination of the epidural lesion with hematoxylin and eosin staining. The features seen in the photomicrograph are in accordance with the diagnosis of HPC (A). AE1/AE3 and QBEND10 (CD34) were positive on immunohistochemical staining (B).
tumor tissue showed that the tumor cells were oval or spindleshaped with acidophilic cytoplasm; they were surrounded by numerous branched vessels. The tumor cells were immunepositive for QBEND10 (CD34) and AE1/AE3 but negative for EMA, SMA, CD31, desmin, GFAP, and S-100. The Ki67 index was 10%. The postoperative histopathologic and immunohistochemical features were compatible with a diagnosis of HPC (Fig. 2). After surgery, the patient returned for follow-ups in the outpatient department at 3 months, 6 months and 12 months. Clinical and imaging examinations included NRS scores, as well as evaluations of motor and sensory function. The patient’s symptoms had improved dramatically, and MRI revealed complete resection of the tumor (Fig. 3). Positron emission tomography (PET) was performed during the follow-up period to confirm that there was no recurrence or metastatic spread of the tumor during the followup period.
3. Discussion Pericytes, first described by the Swiss scientist K.W. Zimmerman, are a kind of immature mesenchymal cell with contraction and differentiation potential that wrap themselves around capillaries [8]. Pericytes play an important role in providing mechanical support to capillaries and altering capillaries’ luminal sizes through their contractile power under normal physiologic conditions [9]. It is generally believed that HPC is a vascular tumor derived from pericytes in soft tissues; it could be located at any site that contains capillaries but is rarely found in the central nervous system (CNS). Most CNS HPCs occur in the cranial cavity, and more rarely, in the spinal canal. Because of their rarity, information is scarce regarding their clinical characteristics, preoperative diagnosis, imaging features, and prognosis. Therefore, we report on an uncommon case of primary thoracic epidural HPC and summarize our comprehensive literature review regarding this condition (Table 1) [10–46].
Our research yielded 105 cases of primary spinal HPC from 1958 to 2017. These cases indicate that primary spinal HPC can occur at any age, and that the mean age at diagnosis was 36.3 years (ranging from 2 to 80 years). The most vulnerable age range was 20–39 years (42.9%), followed by 40 to 59 years (28.6%). HPC was slightly more prevalent in males (male-tofemale ratio = 1.33:1), which was in accord with the former articles [46,47]. Primary spinal HPCs were more commonly located in extradural spaces (58 of 105 cases, or 55%), followed by intradural spaces (39 of 105 cases, or 37%) and intra- to extradural regions (8 of 105 cases, or 8%). The intradural lesions are divided into intramedullary and extramedullary subtypes, and the extradural lesions are further classified as being based on either dura or bone. Furthermore, according to statistical data from our literature review, cervical and thoracic segments constitute the most common sites of spinal lesions (37% and 40%, respectively), along with the lumbar segment (18%); the lesions are rarely found in the sacral segment (5%). The clinical presentation of primary spinal HPC is nonspecific. Complaints and symptoms vary depending on tumor size and location, but they are not associated with differing histologic types. The most common presenting symptoms for extradural HPCs are neurologic deficits and weakness of the limbs because the extradural compartment has been occupied, which is similar to other spinal lesions, such as schwannoma, neurofibroma, ependymoma, and meningioma [6,23,34,48]. Conversely, in primary osseous spinal HPCs, pain and mass effects are the most common complaints because osseous HPCs are more likely to involve the paravertebral extension [49]. In our case, the patient was suffering from lower extremity weakness, hypoesthesia below T6, and severe backache, which conforms with previous reports. Spinal HPCs do not demonstrate specific radiographic features and are difficult to differentiate from other tumors. A plain x-ray commonly displays no significant abnormality, unless the vertebral body or pedicles are involved and depending on the extent of the tumor. The typical MRI characteristics of spinal HPCs are
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Fig. 3. Evaluation by postoperative MRI at 6-month follow up after gross total resection. Sagittal T1WI (A), T2WI (B) and dense enhancement image (C) demonstrating no recurrence or residual tumor. Postoperative axial T2WI displays complete excision of the tumor and decompression of the spinal cord at T6–7 (D).
heterogeneous, predominantly isointense masses on T1WI and T2WI with compression of the spinal cord. Since HPC is a tumor that arises from the pericytes of capillaries, it is possible that marked enhancement with internal vessel voids could be shown on gadolinium-enhanced images [50]. Moreover, some features from previous reports have been described to distinguish intraspinal HPCs [51–53]. The presence of serpentine vessels, a narrow base of dural attachment, a dural tail sign, and absence of calcifications on MRI may point to HPC preoperatively. The description of HPC imaging in our report was not typical, which was why the patient was misdiagnosed with schwannoma. Clinically and radiologically, spinal HPC is a rare disease. It is difficult to diagnose and also easily misdiagnosed. We analyzed the possible causes of the missed diagnoses, including nonspecific radiographic features, variations in morphology and location, and the pathologic categorization of HPCs as it has evolved over recent years [54,55]. In short, the proper diagnosis of this disease calls for close attention to a combination of clinical and radiographic features. Heightened suspicion for spinal HPCs should be elicited by the presence of internal signal voids, heterogeneous enhancement, and adjacent bony erosion. In addition, histopathology and immunohistochemistry are required to confirm the diagnosis. Previous articles have shown that surgical excision is the most widely recommended treatment for intraspinal HPC. HPC has a strong tendency to recur locally and to metastasize because the tumor is almost always attached to the dura. Based on our statistical analysis of 94 intraspinal HPC patients (11 patients were
removed owing to the lack of recurrence-related information), the recurrence rate was 50% (38.5% in the case of intradural tumors, 44.8% in the case of extradural tumors, and 75% in the case of intra- to extradural tumors). Hence, some researchers advocate the complete en bloc resection of the tumor combined with excision of the neighboring dura mater to avoid recurrence and metastasis [5,23,24,33,42,56]. However, a recent study of a 26 case series by Liu et al. proposed that total resection did not have a statistically significant influence on survival or prevention of recurrence [46]. According to our experience, total resection can be attempted for tumors with clear margins and will serve to relieve symptoms; however, for patients at high risk for injury to the nerve root or dura mater, the subsequent adjuvant radiotherapy may be more beneficial than risking total resection. Postoperative radiotherapy and chemotherapy are still controversial. Earlier research suggested that radiotherapy could reduce recurrence and improve survival [6,57–59]. Chou at al. analyzed 16 intraspinal HPC patients who received adjuvant radiotherapy and found that there was no significant difference compared with those who did not receive radiotherapy. Moreover, pathologic grade was the only factor associated with recurrence, a conclusion with which Liu agreed [28,46]. From our statistical analysis of 71 cases selected from 105 cases with detailed records of postoperative therapy and recurrence, our study reached the same conclusions as that of Chou and Liu [28,60]. We are the first to have used PET to evaluate recurrence and metastasis after surgery because PET can monitor these events at an early stage.
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Z. Li et al. / Journal of Clinical Neuroscience xxx (2018) xxx–xxx Table 1 Summary of previously reported cases of primary spinal HPC. Article
Gender/Age
Schirger (1958) [7] Kruse (1961) [10] Pitlyk (1965) [11]
F/33 T2 Extradural None Yes 1 M/53 C3 Intradural Radiotherapy Yes 4 M/60 C4 Intradural None Yes 2 M/39 T8 Intradural None No 10 F/49 C3 Intradural None Yes 18 M/16 C6–7 Extradural Radiotherapy No 0.75 M/21 T6 Extradural None Unknown Unknown M/62 L5 Extradural Radiotherapy Unknown Unknown M/28 C2–6 Extradural Radiotherapy No 5 F/65 L2 Extradural Radiotherapy No 4 F/31 C6 Extradural None No 1 F/52 C6 Extradural None No 0.1 F/36 C5 Extradural None No 2 M/15 T12 Extradural Radiotherapy Unknown Unknown F/11 T11 Extradural Radiotherapy Yes 6 F/41 L2 Extradural None Unknown Unknown M/30 L3 Intradural Radiotherapy Yes 12 M/48 C4 Intradural None Yes 7 F/36 C6 Extradural Radiotherapy No 2 M/25 S1 Extradural Radiotherapy No 0.75 F/29 L1–3 Extradural Radiotherapy No 10 M/39 T4–6 Extradural Radiotherapy No 3 M/31 L4 Intradural None No 0.5 F/39 L1–2 Extradural None No 2 M/21 T2 Extradural Radiotherapy Chemotherapy Unknown Unknown M/31 T4–6 Intradural None No 3 F/16 T4–5 Extradural Radiotherapy No 0.75 M/80 T10 Intradural None No 3 M/54 L4–5 Intradural Radiotherapy No Unknown M/20 T9–10 Intradural None No 2 M/58 T10 Intradural None No Unknown F/61 C2 Extradural Radiotherapy Unknown 0.25 F/51 T6–7 Intradural None Yes 5 F/51 C3–4 Intra- to extradural None Yes 9 F/27 T7–8 Intradural None No 3 M/56 Occiput to C3 Intradural None Yes 3 M/57 T9–10 Intradural Radiotherapy No 3 M/56 Occiput to C4 Intradural Radiotherapy No 5 M/21 C1–2 Intradural None No 2 M/43 C6–T2 Extradural None No 1 M/28 C3 Extradural Radiotherapy No 1 F/16 T5–6 Extradural None Yes Unknown M/16 T9 Intradural Radiotherapy No 5 F/19 C1–2 Intradural None No Unknown F/15 T9–10 Intradural None No Unknown M/50 C4–5 Intradural Radiotherapy Chemotherapy No 2 M/34 T8–10 Intradural Radiotherapy Chemotherapy No 2 F/37 T7–9 Intra to extradural None No 1 F/12 T11–L1 Intradural Radiotherapy No 0.75 M/37 C5–6 Extradural Radiotherapy Chemotherapy Yes 5 M/63 T9 Intradural None No 1 A report on the clinical course of 60 patients. In 5 cases, a spinal extradural HPC was primarily involved at vertebral levels T1–2, T6–7, T10, T11, and S1. Data regarding recurrence and follow-up period are unknown A report on 23 primary intraspinal HPCs (19 extradural and 4 intradural). The numbers of cases located in the cervical, thoracic, lumbar, and sacral segments were 10, 9, 3, and 1, respectively. Seven patients received radiotherapy. Recurrence rates were 50% for intradural HPCs and 73% for extradural HPCs A retrospective review of 26 patients with HPCs of the spine (14 males and 12 females, mean age: 33.8 years). Nine HPCs were located in the cervical segment, 6 in the thoracic segment, 5 in the lumbar segment, 1 in the cervicothoracic segment, 3 in the thoracolumbar segment and 2 in the lumbosacral segment. Ten patients had intradural HPCs, 10 had extradural HPCs, and 6 had intra- to extradural HPCs. In all, 22 patients accepted postoperative radiotherapy, and 2 patients received chemotherapy; 19 patients had recurrences during the follow-up period
Kriss (1968) [12] Fathie (1970) [13] Gerner (1974) [14] Harris (1978) [15] Stern (1980) [16] Cappabianca (1981) [17] Muraszko (1982) [18]
Ciappetta (1985) [19] Bridges (1988) [20] Salvati (1991) [21] Akhaddar (2002) [22] Betchen (2002) [23] Ijiri (2002) [24] Mohammadianpanah (2004) [25] Kashiwazaki (2007) [26] Kumar (2007) [27] Chou (2009) [28] Fitzpatrick (2009) [4] Moscovici (2011) [29] Ackerman (2011) [30] Santillan (2011) [31] Torigoe (2012) [32] Nakashima (2013) [33] Shirzadi (2013) [34]
Drazin (2013) [35] Lee (2013) [36] Zhang (2014) [37] Raghvendra (2014) [38] Jayashankar (2014) [39] Kaur (2014) [40] Türk (2015) [41] Das (2015) [42]
Chew (2017) [43] McMaster (1975) [44] Zhao (2007) [45]
Liu (2013) [46]
Level
Location
4. Conclusion Primary spinal HPC is a rare disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Primary spinal HPC, with high rates of recurrence and metastasis, causes severe neurologic symptoms and motor disturbances, but it still gets insufficient attention. We have reported an uncommon case of primary epidural thoracic HPC and presented a statistical analysis of earlier cases based on a comprehensive literature review. We emphasize the importance of the proper diagnosis of
Adjuvant therapy
Recurrence
Follow-up (y)
HPC and consider surgical removal to be the best treatment. Postoperative radiotherapy and chemotherapy are not preferred, except in the case of incomplete resection or recurrence. PET may be considered during follow-up to detect recurrence and metastasis as early as possible. Acknowledgments The authors would like to thank all the patients and their family members for their help and informed consent.
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Please cite this article in press as: Li Z et al. Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review. J Clin Neurosci (2018), https://doi.org/10.1016/j.jocn.2018.10.057