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Papillary endothelial hyperplasia (Masson tumor) of the petrous and jugulare region: case report and literature review
Surgical Neurology 64 (2005) 55 – 60 www.surgicalneurology-online.com
Neoplasm
Papillary endothelial hyperplasia (Masson tumor) of the petrous and jugulare region: case report and literature review Rong Zhang, MDa,*, Liang-Fu Zhou, MDa,*, Ying Mao, MDa, Yin Wang, PhDb Departments of aNeurosurgery and bNeuropathology, Huashan Hospital, Shanghai Medical College, Fu Dan University, Shanghai 200040, China Received 26 January 2004; accepted 20 August 2004
Abstract
Objective: Intracranial papillary endothelial hyperplasia (PEH) is rare, and only 13 cases have been reported intracranially in the literature. In this article, we present a case of PEH involving petrous and jugular foramen region due to the uncommon incidence. Case Description: A 49-year-old female patient with a 4-year history of left-sided hearing loss and facial palsy. Magnetic resonance (MR) imaging disclosed the presence of a 6 6 5 cm lobular mass occupying the left petrous and jugulare region. This mass was hyperintense on both T1 and T2 weighted MR images and was enhanced strongly with gadolinium. No edema was found around the lesion. Preoperative digital subtraction angiogram examination showed that the lesion had a rich blood supply. Therefore, polyvinyl alcohol embolization was carried out to reduce bleeding during operation. A left-sided suboccipital extreme lateral approach was applied for craniotomy. The lesion was extradural and highly vascular with extension into the petrous bone. It was subtotally removed. Postoperative course of the patient was stable. Her neurologic evaluation was the same as preoperatively. Conclusion: Surgical excision of the lesion is the main therapy. Radiotherapy should be given to patients whose lesion cannot be totally removed. D 2005 Published by Elsevier Inc.
Keywords:
Differential diagnosis; Intracranial neoplasm; Masson tumor; Papillary endothelial hyperplasia; Treatment
1. Introduction Papillary endothelial hyperplasia (PEH) is a rare pathologic entity that was first described by Pierre Masson [7] in 1923 in an inflamed hemorrhoidal plexus as a benign vascular neoplasm. It was also known as intravascular PEH, Masson vegetant intravascular hemangioendothelioma, Masson tumor, or Masson pseudoangiosarcoma. Papillary
endothelial hyperplasia has a propensity for skin and subcutaneous soft tissues, although it rarely occurs in the cranial base. Only 13 intracranial PEH cases have been reported up to now. In this article, we present the 14th case of intracranial PEH, which was located in the petrous and jugulare region.
2. Case report 2.1. History Abbreviations: AVM, arteriovenous malformation; CPA, cerebellopontine angle; CT, computed tomography; DSA, digital subtraction angiogram; MR, magnetic resonance; PEH, papillary endothelial hyperplasia. * Corresponding authors. Tel.: +86 21 6248 9999 6487; fax: +86 21 6249 9412. E-mail addresses: zhang _ [email protected] (R. Zhang)8 [email protected] (L.-F. Zhou). 0090-3019/$ – see front matter D 2005 Published by Elsevier Inc. doi:10.1016/j.surneu.2004.08.091
This 49-year-old right-handed female patient was admitted to the hospital because of left-sided hearing loss and facial palsy. The symptoms started with left-sided hearing loss 4 years ago, then followed with left-sided facial paralysis. She had been treated as Bell’s palsy for a long period but without satisfactory effect. As the
56
R. Zhang et al. / Surgical Neurology 64 (2005) 55 – 60
2.2. Examination and diagnosis
Fig. 1. In endoscopic examination, the tympanic membrane was found to be purple with an eminence at upper and posterior region.
symptoms gradually increased, a CT scan of the head revealed a large massive lesion located at the petrous and jugulare region.
The patient’s neurologic evaluations were significant for loss of left ear hearing, House-Brackmann grade 4 facial palsy of the left side, horizontal nystagmus, and ataxia on the left side associated with positive Romberg’s sign. Electrical ion hearing test showed disappearance of air and bone conduct ion by neuro-otology examination. The tympanic membrane was purple with an eminence at upper and posterior area under endoscope (Fig. 1). MR imaging disclosed the presence of a 6 6 5 cm lobular mass occupying the left petrous region protruding toward jugular foramen. This mass was hyperintense on both T1 and T2 weighted MR images. It enhanced strongly and almost homogeneously after intravenous administration of gadolinium. No edema was found around the lesion (Fig. 2). The lesion was hypodense on CT scan, and CT bone window frame of skull base showed petrous ridge of left side was damaged, with enlargement of jugular
Fig. 2. Preoperative MR images. A: Axial unenhanced T1 weighted MR imaging showing left petrous and jugulare hyperintense mass. B: Axial T2 weighted MR imaging showing the lesion is also hyperintense and without brain edema. C: Axial MR imaging with gadolinium showing enhancement of the lesion. D: Coronal MR imaging with gadolinium showing the lesion has infiltrated the petrous pyramid.
R. Zhang et al. / Surgical Neurology 64 (2005) 55– 60
57
Fig. 3. Preoperative CT imaging. A: Axial enhanced CT scan showing a hypodense mass with ringed enhancement at petrous and jugulare region. B: Cranial base bone window CT showing bone destruction at left petrous pyramid.
foramen (Fig. 3). A diagnosis of glomus jugulare tumor was made. 2.3. Digital subtraction angiogram and embolization DSA demonstrated an obvious blush from the early arterial to the late venous phases. The mass blood supply was from the branches of the left middle meningeal artery and the left posterior auricular artery. Left-sided sigmoid sinus and jugular bulb were not clearly shown in the angiogram. Polyvinyl alcohol embolization was performed by a superselective microcatheter, and 80% to 85% of tumor blood supply was successively occluded, which reduced operative bleeding and lowered the surgical risk (Fig. 4). 2.4. Operation and postoperative course A left-sided suboccipital extreme lateral approach was applied for craniotomy. With a linear incision, left internal carotid vein, left vertebral artery, and left sigmoid sinus were exposed, and the left petrous bone was partially removed by using of high-speed air drill. Because the petrous bone was invaded by the lesion, it is hard to recognize the bone mark that suggested the facial nerve’s position. Therefore, the petrous lesion was unable to be totally resected for the purpose of preserving facial nerve and hearing ability. The
lesion was extradural, highly vascular, attached to the dura in the sigmoid sinus region, invaded into the petrous bone, and extended toward left petrous ridge. After disconnecting the jugular vein and the sigmoid sinus, the lesion was subtotally removed with some residual tumor left at the petrous ridge. Postoperative enhancing MR imaging confirmed this assessment (Fig. 5). The patient’s postoperative course was stable. Her neurologic evaluation was same as before, apart from choking. She had no cerebrospinal fluid leakage but did have intracranial infection, which was cured by continuous lumber drainage and anti-infectious treatment. 2.5. Pathological findings The specimens consisted of highly vascular connective tissue with a complex network of irregular branching. In some areas, the mass was composed of numerous papillary structures, which had a hyaline core representing organized thrombus and was covered by an endothelial monolayer. Thrombi and old area of hemorrhage could be found in some parts of the lesion, and sinus lumens was formed by papillary endothelialized vegetation. The inner wall of the sinus lumina was immunopositive for CD34, whereas it was immunonegative for epithelial membrane antigen, chromo-
Fig. 4. DSA images. A: Left external carotid angiography showing an obvious blush from the branches of left middle meningeal artery. B: Angiography after polyvinyl alcohol embolization showing 80% to 85% of tumor blood supply was occluded.
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R. Zhang et al. / Surgical Neurology 64 (2005) 55 – 60
Fig. 5. Postoperative MR images showing residual tumor is left at the petrous apex. A: Axial enhanced MR imaging. B: Coronal enhanced MR imaging.
granin A, and synopsis. The diagnosis of PEH was made based on these histologic findings (Fig. 6).
3. Discussion 3.1. Review of intracranial PEH Since its first recognition more than 80 years ago, PEH has been diagnosed in a variety of locations such as the liver, uterus, urethra, gastrointestinal tract, etc. Extracranial PEH usually presents as a low-growing nodule that may be somewhat painful. Papillary endothelial hyperplasia is relatively common when associated with thrombosis, either in a normal vessel (the primary type) or in a preexisting vascular lesion (the secondary type). Histologically, the
lesion consists of an intravascular proliferation of numerous papillae that are composed of a core of connective tissue and an endothelial surface, whereas no malignant features exist. The PEH has a predilection for the head and neck, but it rarely involves the central nervous system. In Stoffman and Kim’s [11] report, they included Lam et al’s [6] case, which we decided should be excluded from this article, because it was a case of extracranial scalp mass. However, we included a PEH case reported by Baylor et al [2], in whose case the PEH lesion was located in the internal auditory canal. Among the 14 intracranial PEH cases included in the present case (Table 1), it seemed that PEH arising from extradural space was of primary type, whereas most of the secondary types of PEH were in the brain parenchyma. From a review of the literature on these intracranial lesions,
Fig. 6. Histology. A: The mass is mainly composed of highly vascular connective tissue with numerous small papillary structures (hematoxylin and eosin stain, 4). B: The papillary has a hyaline core, representing organized thrombus, and are covered by a single layer of endothelia (hematoxylin and eosin stain, 10). C: Papillary endothelialized vegetation forming a sinus lumen was found in the lesion (hematoxylin and eosin staining, 40). D: Immunohistochemical study of CD34 marker showed frequent positive staining (10).
R. Zhang et al. / Surgical Neurology 64 (2005) 55– 60
59
Table 1 Summary of intracranial PEH cases reported Authors
Age, sex
Clinical presentation
Location
Type
Nagib et al [8]
16 y, F
Right temporal lobe
Primary
Chen and Kuo [3] Izukawa et al [4] Sickler and Langford [10]
3.5 mo, F 55 y, F 12 d, F
Neurocutaneous disseminated form, seizures Intracranial hypertension, seizures Hemianopsia, hemiparesis, seizures Hydrocephalus, intracranial hypertension
Primary Secondary Primary
Wen et al [13] Patt et al [9]
15 y, F 27 y, F
Frontal meninges In parieto-occipital cavernoma Right middle cranial fossa with extension into frontoparietal region Torcular herophili Fissure orbitalis superior
Tsuji et al [12] Kristof et al [5]
18 70 51 24
Secondary Primary Primary Secondary
Baylor et al [2]
27 y, M
Right facial palsy
Avellino et al [1]
75 y, F
Vertigo, hearing loss
Stoffman and Kim [11] Present case
54 y, F 49 y, F
Headache, dysphasia Loss of hearing and facial palsy on left side
In AVM Cavernous sinus with extension into sellar region Cavernous sinus with extension into sellar region Cavernous sinus with extension into sellar region (with CM) Fundus of the internal auditory canal and the labyrinthine segment of the fallopian canal Left CPA and middle cranial fossa region invading petrous pyramid Meckel’s cave invading petrous apex Left petrous and jugulare region
y, y, y, y,
F F M F
Intracranial hypertension Headache, unilateral deficit of cranial nerves III, V, and VI Seizures, hemiparesis Transient diplopia Diplopia Diplopia, impairment of pituitary function
we found that there was a female predominance, but it varied in age, location, and prognosis. Only 2 male cases were reported among the 14 intracranial PEH cases. The youngest was 12 days, whereas the oldest was 75 years. Our case is like the one in Avellino et al’s [1] article, locates in the cerebellopontine angle (CPA) with destruction of petrous pyramid and extension into the jugular foramen and posterior aspect of the middle ear. We thought these 2 lesions most probably arose from the sigmoid sinus. Besides, Baylor et al confirmed a PEH lesion at the fundus of the internal auditory canal. In our case, we observed that the lesion can be seen in the middle ear with the endoscope. This phenomenon also made us surmise that the origin of the lesion might be in the middle ear. 3.2. Diagnosis and differentiation Like any other intracranial space occupying lesion, the symptoms and signs of presentation are location dependent. Clinically, jugulare PEH is not easily distinguished from glomus jugulare tumor, chordoma, chondroma, petrous apex cholesterol granuloma, or endolymphacysticum. The radiographic findings were not so specific, because hemorrhage is usually present as seen in this case. Most cases of PEH have enhancement on CT or MR imaging. CT scan of this case showed hypodensity lesion with ringed enhancement and petrous bone destruction. No calcification existed in the images. A dense tumor blush can often be seen in angiogram. In the pathologic examination, PEH consisted primarily of innumerable small papillae with hyaline cores associated with an adjacent thrombus. Often, transition from organizing fibrin thrombus into papillae can be seen. Histologically, there are 2 types of PEH classified as the primary type and the secondary type. In primary PEH, thrombosis is in a
Secondary Secondary
Primary Primary Primary Primary
normal vessel such as a venous sinus. However, a secondary PEH develops from a preexisting vascular lesion like an arteriovenous malformation (AVM) or a cavernous malformation. Although the histologic appearance and intravascular location of PEH are usually distinctive, differential diagnosis has to be made from other vascular tumors. The most important differential diagnosis of PEH is angiosarcoma. Angiosarcoma is almost never purely intravascular and rarely has the same degree of papillarity. The typical composition of angiosarcoma is irregularly vascular channels lined by large atypical endothelial cells, which are often hyperchromatic. Other vascular tumors such as capillary hemangioma and spindle cell hemangioma also need to be differentiated. Crowded canalized or uncanalized vascular structures are main features of capillary hemangioma, whereas spindle cell hemangioma is composed of papillary structures with much more cellular areas associated with solid spindle cell areas. Immunohistochemistry is essential for the establishment of the vascular nature of PEH, but it has not played a major role for differential diagnosis among other vascular tumors. 3.3. Treatment and prognosis As PEH are benign nonrecurring processes, complete surgical excision, if possible, will cure the disease. However, it is sometimes difficult for intracranial PEH to be totally removed. On reviewing the literature, only 5 of 13 cases achieved total removal. In those 5 cases, the lesions were located at hemisphere (secondary PEH in cavernoma and AVM), cavernous sinus (secondary PEH with cavernoma), fundus of internal auditory canal (a small lesion b1 cm in diameter), and superior orbital fissure, respectively. Total resection of PEH invading the petrous bone is hardly possible. Most of these lesions could only achieve subtotal
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excision, as in the present case and the other 2 reported cases. Radical excision of these lesions may lead to serious complications such as cerebrospinal fluid leakage, complete facial paresis, and central nervous system infection. Preoperative DSA examination and embolization, if feasible, are recommended to reduce bleeding during operation, which was the practice for most of the authors. A rapid frozen tissue histologic study will always be performed during the operation. Although the pathologic distinction of PEH from angiosarcoma is difficult by frozen tissue, it is greatly suggested so unnecessary aggressive treatments are avoided. Although PEH are benign, postoperative radiotherapy or radiosurgery (g-knife) should be given as main adjuvant therapy for patients with subtotal or partial removal of the lesion and may be effective. Residue of the lesion may shrunk after radiotherapy [5]. Chemotherapy was also applied to patients in the literature. However, its clinical outcome remains unclear. In all intracranial PEH cases reported, 4 cases had local recurrence after first operative intervention. The relapse time varied from 2 months to 9 years. The lesions were at right temporal lobe, right middle cranial fossa with extension into frontoparietal region, left cavernous sinus with extension into sellar region, and left CPA with extension into middle cranial fossa, respectively. With re-resection and radiotherapy or chemotherapy, the lesions were all stabilized in follow-up. Our case was similar to Avellino et al’s, which had recurrence 9 years after first operation and radiotherapy. Therefore, it seems very important for patients to have a longer period follow-up. References [1] Avellino AM, Grant GA, Harris AB, Wallace SK, Shaw CM. Recurrent intracranial Masson’s vegetant intravascular hemangioendothelioma. J Neurosurg 1999;91:308 - 12. [2] Baylor JE, Antonelli PJ, Rojiani A, Mancuso AA. Facial palsy from Masson’s vegetant intravascular hemangioendothelioma. ENT J 1998;77:408 - 17. [3] Chen TJ, Kuo TT. Giant intracranial Masson’s hemangioma. Report of a fatal case. Arch Pathol Lab Med 1984;108:555 - 6.
[4] Izukawa D, Lach B, Benoid B. Intravascular papillary endothelial hyperplasia in an intracranial cavernous hemangioma. Neurosurgery 1987;21:939 - 41. [5] Kristof RA, Van Roost D, Wolf HK, Schramm J. Intravascular papillary endothelial hyperplasia of the sellar region. J Neurosurg 1997;86:558 - 63. [6] Lam CH, Farmer JP, Meagher-Villenmure K, Montes JL. Masson’s vegetant hemangioendothelioma. Pediatr Neurosurg 1997;86:558 - 63. [7] Masson MP. L’Hemangioendotheliome intravasculaire. Ann Anat Pathol 1923;93:517. [8] Nagib MG, Sung JH, Seljeskog EL. Neurocutaneous Masson’s vegetant intravascular hemangioendothelioma. Neurosurgery 1982;11:800 - 3. [9] Patt S, Kaden B, Stoltenburg-Didinger G. Intravascular papillary endothelial hyperplasia at the fissura orbitalis superior: case report. Clin Neuropathol 1992;11:128 - 30. [10] Sickler GK, Langford LA. Intracranial tumor-forming papillary endothelial hyperplasia—case report. Clin Neuropathol 1990;9:125 - 8. [11] Stoffman MR, Kim JH. Masson’s vegetant hemangioendothelioma: case report and literature. J Neurooncol 2003;61:17 - 22. [12] Tsuji N, Tsubokawa T, Katayama Y, Yamamoto T, Nemoto N. Arteriovenous malformation occluded by Masson’s vegetant intravascular hemangioendothelioma and complicated with intracerebral hematoma. Neurol Res 1994;16:148 - 50. [13] Wen DY, Hardten DR, Wirtschafter JD, Sung JH, Haines SJ. Elevated intracranial pressure from cerebral venous obstruction by Masson’s vegetant intravascular hemangioendothelioma. Case report. J Neurosurg 1991;75:787 - 90.
Commentary The authors present a very unusual skull base lesion involving the petrous bone. The case report is very informative and is an excellent review of the limited literature available regarding this problem. Neurosurgeons and neuro-otologists should be familiar with this pathology when facing complex lesions in the petrous region.
Johnny B. Delashaw, MD Department of Neurological Surgery Department of Otolaryngology Oregon Health and Science University Portland, OR 97201, USA
The great secrets of doctors, known only to their wives, but still hidden from the public, is that most things get better by themselves; most things, in fact, are better in the morning. —NY Times 4 Jul 76
Neoplasm
Papillary endothelial hyperplasia (Masson tumor) of the petrous and jugulare region: case report and literature review Rong Zhang, MDa,*, Liang-Fu Zhou, MDa,*, Ying Mao, MDa, Yin Wang, PhDb Departments of aNeurosurgery and bNeuropathology, Huashan Hospital, Shanghai Medical College, Fu Dan University, Shanghai 200040, China Received 26 January 2004; accepted 20 August 2004
Abstract
Objective: Intracranial papillary endothelial hyperplasia (PEH) is rare, and only 13 cases have been reported intracranially in the literature. In this article, we present a case of PEH involving petrous and jugular foramen region due to the uncommon incidence. Case Description: A 49-year-old female patient with a 4-year history of left-sided hearing loss and facial palsy. Magnetic resonance (MR) imaging disclosed the presence of a 6 6 5 cm lobular mass occupying the left petrous and jugulare region. This mass was hyperintense on both T1 and T2 weighted MR images and was enhanced strongly with gadolinium. No edema was found around the lesion. Preoperative digital subtraction angiogram examination showed that the lesion had a rich blood supply. Therefore, polyvinyl alcohol embolization was carried out to reduce bleeding during operation. A left-sided suboccipital extreme lateral approach was applied for craniotomy. The lesion was extradural and highly vascular with extension into the petrous bone. It was subtotally removed. Postoperative course of the patient was stable. Her neurologic evaluation was the same as preoperatively. Conclusion: Surgical excision of the lesion is the main therapy. Radiotherapy should be given to patients whose lesion cannot be totally removed. D 2005 Published by Elsevier Inc.
Keywords:
Differential diagnosis; Intracranial neoplasm; Masson tumor; Papillary endothelial hyperplasia; Treatment
1. Introduction Papillary endothelial hyperplasia (PEH) is a rare pathologic entity that was first described by Pierre Masson [7] in 1923 in an inflamed hemorrhoidal plexus as a benign vascular neoplasm. It was also known as intravascular PEH, Masson vegetant intravascular hemangioendothelioma, Masson tumor, or Masson pseudoangiosarcoma. Papillary
endothelial hyperplasia has a propensity for skin and subcutaneous soft tissues, although it rarely occurs in the cranial base. Only 13 intracranial PEH cases have been reported up to now. In this article, we present the 14th case of intracranial PEH, which was located in the petrous and jugulare region.
2. Case report 2.1. History Abbreviations: AVM, arteriovenous malformation; CPA, cerebellopontine angle; CT, computed tomography; DSA, digital subtraction angiogram; MR, magnetic resonance; PEH, papillary endothelial hyperplasia. * Corresponding authors. Tel.: +86 21 6248 9999 6487; fax: +86 21 6249 9412. E-mail addresses: zhang _ [email protected] (R. Zhang)8 [email protected] (L.-F. Zhou). 0090-3019/$ – see front matter D 2005 Published by Elsevier Inc. doi:10.1016/j.surneu.2004.08.091
This 49-year-old right-handed female patient was admitted to the hospital because of left-sided hearing loss and facial palsy. The symptoms started with left-sided hearing loss 4 years ago, then followed with left-sided facial paralysis. She had been treated as Bell’s palsy for a long period but without satisfactory effect. As the
56
R. Zhang et al. / Surgical Neurology 64 (2005) 55 – 60
2.2. Examination and diagnosis
Fig. 1. In endoscopic examination, the tympanic membrane was found to be purple with an eminence at upper and posterior region.
symptoms gradually increased, a CT scan of the head revealed a large massive lesion located at the petrous and jugulare region.
The patient’s neurologic evaluations were significant for loss of left ear hearing, House-Brackmann grade 4 facial palsy of the left side, horizontal nystagmus, and ataxia on the left side associated with positive Romberg’s sign. Electrical ion hearing test showed disappearance of air and bone conduct ion by neuro-otology examination. The tympanic membrane was purple with an eminence at upper and posterior area under endoscope (Fig. 1). MR imaging disclosed the presence of a 6 6 5 cm lobular mass occupying the left petrous region protruding toward jugular foramen. This mass was hyperintense on both T1 and T2 weighted MR images. It enhanced strongly and almost homogeneously after intravenous administration of gadolinium. No edema was found around the lesion (Fig. 2). The lesion was hypodense on CT scan, and CT bone window frame of skull base showed petrous ridge of left side was damaged, with enlargement of jugular
Fig. 2. Preoperative MR images. A: Axial unenhanced T1 weighted MR imaging showing left petrous and jugulare hyperintense mass. B: Axial T2 weighted MR imaging showing the lesion is also hyperintense and without brain edema. C: Axial MR imaging with gadolinium showing enhancement of the lesion. D: Coronal MR imaging with gadolinium showing the lesion has infiltrated the petrous pyramid.
R. Zhang et al. / Surgical Neurology 64 (2005) 55– 60
57
Fig. 3. Preoperative CT imaging. A: Axial enhanced CT scan showing a hypodense mass with ringed enhancement at petrous and jugulare region. B: Cranial base bone window CT showing bone destruction at left petrous pyramid.
foramen (Fig. 3). A diagnosis of glomus jugulare tumor was made. 2.3. Digital subtraction angiogram and embolization DSA demonstrated an obvious blush from the early arterial to the late venous phases. The mass blood supply was from the branches of the left middle meningeal artery and the left posterior auricular artery. Left-sided sigmoid sinus and jugular bulb were not clearly shown in the angiogram. Polyvinyl alcohol embolization was performed by a superselective microcatheter, and 80% to 85% of tumor blood supply was successively occluded, which reduced operative bleeding and lowered the surgical risk (Fig. 4). 2.4. Operation and postoperative course A left-sided suboccipital extreme lateral approach was applied for craniotomy. With a linear incision, left internal carotid vein, left vertebral artery, and left sigmoid sinus were exposed, and the left petrous bone was partially removed by using of high-speed air drill. Because the petrous bone was invaded by the lesion, it is hard to recognize the bone mark that suggested the facial nerve’s position. Therefore, the petrous lesion was unable to be totally resected for the purpose of preserving facial nerve and hearing ability. The
lesion was extradural, highly vascular, attached to the dura in the sigmoid sinus region, invaded into the petrous bone, and extended toward left petrous ridge. After disconnecting the jugular vein and the sigmoid sinus, the lesion was subtotally removed with some residual tumor left at the petrous ridge. Postoperative enhancing MR imaging confirmed this assessment (Fig. 5). The patient’s postoperative course was stable. Her neurologic evaluation was same as before, apart from choking. She had no cerebrospinal fluid leakage but did have intracranial infection, which was cured by continuous lumber drainage and anti-infectious treatment. 2.5. Pathological findings The specimens consisted of highly vascular connective tissue with a complex network of irregular branching. In some areas, the mass was composed of numerous papillary structures, which had a hyaline core representing organized thrombus and was covered by an endothelial monolayer. Thrombi and old area of hemorrhage could be found in some parts of the lesion, and sinus lumens was formed by papillary endothelialized vegetation. The inner wall of the sinus lumina was immunopositive for CD34, whereas it was immunonegative for epithelial membrane antigen, chromo-
Fig. 4. DSA images. A: Left external carotid angiography showing an obvious blush from the branches of left middle meningeal artery. B: Angiography after polyvinyl alcohol embolization showing 80% to 85% of tumor blood supply was occluded.
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R. Zhang et al. / Surgical Neurology 64 (2005) 55 – 60
Fig. 5. Postoperative MR images showing residual tumor is left at the petrous apex. A: Axial enhanced MR imaging. B: Coronal enhanced MR imaging.
granin A, and synopsis. The diagnosis of PEH was made based on these histologic findings (Fig. 6).
3. Discussion 3.1. Review of intracranial PEH Since its first recognition more than 80 years ago, PEH has been diagnosed in a variety of locations such as the liver, uterus, urethra, gastrointestinal tract, etc. Extracranial PEH usually presents as a low-growing nodule that may be somewhat painful. Papillary endothelial hyperplasia is relatively common when associated with thrombosis, either in a normal vessel (the primary type) or in a preexisting vascular lesion (the secondary type). Histologically, the
lesion consists of an intravascular proliferation of numerous papillae that are composed of a core of connective tissue and an endothelial surface, whereas no malignant features exist. The PEH has a predilection for the head and neck, but it rarely involves the central nervous system. In Stoffman and Kim’s [11] report, they included Lam et al’s [6] case, which we decided should be excluded from this article, because it was a case of extracranial scalp mass. However, we included a PEH case reported by Baylor et al [2], in whose case the PEH lesion was located in the internal auditory canal. Among the 14 intracranial PEH cases included in the present case (Table 1), it seemed that PEH arising from extradural space was of primary type, whereas most of the secondary types of PEH were in the brain parenchyma. From a review of the literature on these intracranial lesions,
Fig. 6. Histology. A: The mass is mainly composed of highly vascular connective tissue with numerous small papillary structures (hematoxylin and eosin stain, 4). B: The papillary has a hyaline core, representing organized thrombus, and are covered by a single layer of endothelia (hematoxylin and eosin stain, 10). C: Papillary endothelialized vegetation forming a sinus lumen was found in the lesion (hematoxylin and eosin staining, 40). D: Immunohistochemical study of CD34 marker showed frequent positive staining (10).
R. Zhang et al. / Surgical Neurology 64 (2005) 55– 60
59
Table 1 Summary of intracranial PEH cases reported Authors
Age, sex
Clinical presentation
Location
Type
Nagib et al [8]
16 y, F
Right temporal lobe
Primary
Chen and Kuo [3] Izukawa et al [4] Sickler and Langford [10]
3.5 mo, F 55 y, F 12 d, F
Neurocutaneous disseminated form, seizures Intracranial hypertension, seizures Hemianopsia, hemiparesis, seizures Hydrocephalus, intracranial hypertension
Primary Secondary Primary
Wen et al [13] Patt et al [9]
15 y, F 27 y, F
Frontal meninges In parieto-occipital cavernoma Right middle cranial fossa with extension into frontoparietal region Torcular herophili Fissure orbitalis superior
Tsuji et al [12] Kristof et al [5]
18 70 51 24
Secondary Primary Primary Secondary
Baylor et al [2]
27 y, M
Right facial palsy
Avellino et al [1]
75 y, F
Vertigo, hearing loss
Stoffman and Kim [11] Present case
54 y, F 49 y, F
Headache, dysphasia Loss of hearing and facial palsy on left side
In AVM Cavernous sinus with extension into sellar region Cavernous sinus with extension into sellar region Cavernous sinus with extension into sellar region (with CM) Fundus of the internal auditory canal and the labyrinthine segment of the fallopian canal Left CPA and middle cranial fossa region invading petrous pyramid Meckel’s cave invading petrous apex Left petrous and jugulare region
y, y, y, y,
F F M F
Intracranial hypertension Headache, unilateral deficit of cranial nerves III, V, and VI Seizures, hemiparesis Transient diplopia Diplopia Diplopia, impairment of pituitary function
we found that there was a female predominance, but it varied in age, location, and prognosis. Only 2 male cases were reported among the 14 intracranial PEH cases. The youngest was 12 days, whereas the oldest was 75 years. Our case is like the one in Avellino et al’s [1] article, locates in the cerebellopontine angle (CPA) with destruction of petrous pyramid and extension into the jugular foramen and posterior aspect of the middle ear. We thought these 2 lesions most probably arose from the sigmoid sinus. Besides, Baylor et al confirmed a PEH lesion at the fundus of the internal auditory canal. In our case, we observed that the lesion can be seen in the middle ear with the endoscope. This phenomenon also made us surmise that the origin of the lesion might be in the middle ear. 3.2. Diagnosis and differentiation Like any other intracranial space occupying lesion, the symptoms and signs of presentation are location dependent. Clinically, jugulare PEH is not easily distinguished from glomus jugulare tumor, chordoma, chondroma, petrous apex cholesterol granuloma, or endolymphacysticum. The radiographic findings were not so specific, because hemorrhage is usually present as seen in this case. Most cases of PEH have enhancement on CT or MR imaging. CT scan of this case showed hypodensity lesion with ringed enhancement and petrous bone destruction. No calcification existed in the images. A dense tumor blush can often be seen in angiogram. In the pathologic examination, PEH consisted primarily of innumerable small papillae with hyaline cores associated with an adjacent thrombus. Often, transition from organizing fibrin thrombus into papillae can be seen. Histologically, there are 2 types of PEH classified as the primary type and the secondary type. In primary PEH, thrombosis is in a
Secondary Secondary
Primary Primary Primary Primary
normal vessel such as a venous sinus. However, a secondary PEH develops from a preexisting vascular lesion like an arteriovenous malformation (AVM) or a cavernous malformation. Although the histologic appearance and intravascular location of PEH are usually distinctive, differential diagnosis has to be made from other vascular tumors. The most important differential diagnosis of PEH is angiosarcoma. Angiosarcoma is almost never purely intravascular and rarely has the same degree of papillarity. The typical composition of angiosarcoma is irregularly vascular channels lined by large atypical endothelial cells, which are often hyperchromatic. Other vascular tumors such as capillary hemangioma and spindle cell hemangioma also need to be differentiated. Crowded canalized or uncanalized vascular structures are main features of capillary hemangioma, whereas spindle cell hemangioma is composed of papillary structures with much more cellular areas associated with solid spindle cell areas. Immunohistochemistry is essential for the establishment of the vascular nature of PEH, but it has not played a major role for differential diagnosis among other vascular tumors. 3.3. Treatment and prognosis As PEH are benign nonrecurring processes, complete surgical excision, if possible, will cure the disease. However, it is sometimes difficult for intracranial PEH to be totally removed. On reviewing the literature, only 5 of 13 cases achieved total removal. In those 5 cases, the lesions were located at hemisphere (secondary PEH in cavernoma and AVM), cavernous sinus (secondary PEH with cavernoma), fundus of internal auditory canal (a small lesion b1 cm in diameter), and superior orbital fissure, respectively. Total resection of PEH invading the petrous bone is hardly possible. Most of these lesions could only achieve subtotal
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excision, as in the present case and the other 2 reported cases. Radical excision of these lesions may lead to serious complications such as cerebrospinal fluid leakage, complete facial paresis, and central nervous system infection. Preoperative DSA examination and embolization, if feasible, are recommended to reduce bleeding during operation, which was the practice for most of the authors. A rapid frozen tissue histologic study will always be performed during the operation. Although the pathologic distinction of PEH from angiosarcoma is difficult by frozen tissue, it is greatly suggested so unnecessary aggressive treatments are avoided. Although PEH are benign, postoperative radiotherapy or radiosurgery (g-knife) should be given as main adjuvant therapy for patients with subtotal or partial removal of the lesion and may be effective. Residue of the lesion may shrunk after radiotherapy [5]. Chemotherapy was also applied to patients in the literature. However, its clinical outcome remains unclear. In all intracranial PEH cases reported, 4 cases had local recurrence after first operative intervention. The relapse time varied from 2 months to 9 years. The lesions were at right temporal lobe, right middle cranial fossa with extension into frontoparietal region, left cavernous sinus with extension into sellar region, and left CPA with extension into middle cranial fossa, respectively. With re-resection and radiotherapy or chemotherapy, the lesions were all stabilized in follow-up. Our case was similar to Avellino et al’s, which had recurrence 9 years after first operation and radiotherapy. Therefore, it seems very important for patients to have a longer period follow-up. References [1] Avellino AM, Grant GA, Harris AB, Wallace SK, Shaw CM. Recurrent intracranial Masson’s vegetant intravascular hemangioendothelioma. J Neurosurg 1999;91:308 - 12. [2] Baylor JE, Antonelli PJ, Rojiani A, Mancuso AA. Facial palsy from Masson’s vegetant intravascular hemangioendothelioma. ENT J 1998;77:408 - 17. [3] Chen TJ, Kuo TT. Giant intracranial Masson’s hemangioma. Report of a fatal case. Arch Pathol Lab Med 1984;108:555 - 6.
[4] Izukawa D, Lach B, Benoid B. Intravascular papillary endothelial hyperplasia in an intracranial cavernous hemangioma. Neurosurgery 1987;21:939 - 41. [5] Kristof RA, Van Roost D, Wolf HK, Schramm J. Intravascular papillary endothelial hyperplasia of the sellar region. J Neurosurg 1997;86:558 - 63. [6] Lam CH, Farmer JP, Meagher-Villenmure K, Montes JL. Masson’s vegetant hemangioendothelioma. Pediatr Neurosurg 1997;86:558 - 63. [7] Masson MP. L’Hemangioendotheliome intravasculaire. Ann Anat Pathol 1923;93:517. [8] Nagib MG, Sung JH, Seljeskog EL. Neurocutaneous Masson’s vegetant intravascular hemangioendothelioma. Neurosurgery 1982;11:800 - 3. [9] Patt S, Kaden B, Stoltenburg-Didinger G. Intravascular papillary endothelial hyperplasia at the fissura orbitalis superior: case report. Clin Neuropathol 1992;11:128 - 30. [10] Sickler GK, Langford LA. Intracranial tumor-forming papillary endothelial hyperplasia—case report. Clin Neuropathol 1990;9:125 - 8. [11] Stoffman MR, Kim JH. Masson’s vegetant hemangioendothelioma: case report and literature. J Neurooncol 2003;61:17 - 22. [12] Tsuji N, Tsubokawa T, Katayama Y, Yamamoto T, Nemoto N. Arteriovenous malformation occluded by Masson’s vegetant intravascular hemangioendothelioma and complicated with intracerebral hematoma. Neurol Res 1994;16:148 - 50. [13] Wen DY, Hardten DR, Wirtschafter JD, Sung JH, Haines SJ. Elevated intracranial pressure from cerebral venous obstruction by Masson’s vegetant intravascular hemangioendothelioma. Case report. J Neurosurg 1991;75:787 - 90.
Commentary The authors present a very unusual skull base lesion involving the petrous bone. The case report is very informative and is an excellent review of the limited literature available regarding this problem. Neurosurgeons and neuro-otologists should be familiar with this pathology when facing complex lesions in the petrous region.
Johnny B. Delashaw, MD Department of Neurological Surgery Department of Otolaryngology Oregon Health and Science University Portland, OR 97201, USA
The great secrets of doctors, known only to their wives, but still hidden from the public, is that most things get better by themselves; most things, in fact, are better in the morning. —NY Times 4 Jul 76