Clinical Imaging 38 (2014) 364–366

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Radiological imaging of florid intravascular papillary endothelial hyperplasia in the mandibule: case report and literature review☆ Sheng-Sheng Xu a,⁎, Dan Li b a b

Department of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China Department of Pathology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China

a r t i c l e

i n f o

Article history: Received 3 October 2013 Received in revised form 8 December 2013 Accepted 13 December 2013 Keywords: Mandible Intravascular papillary endothelial hyperplasia Tomography X-ray computed Panoramic radiography

a b s t r a c t Intravascular papillary endothelial hyperplasia was a rare benign vascular proliferative process as a result of papillary proliferation of the endothelial cells within the vessels. To our knowledge, we reported the second case occurring in the madibule, and the first reported in 1984 in the literature. We discussed manifestations of multislice computed tomography and panoramic radiography about the lesion and relevant literature was reviewed. © 2014 Elsevier Inc. All rights reserved.

1. Introduction Intravascular papillary endothelial hyperplasia (IPEH) was a rare benign vascular lesion with histological characteristics as papillary proliferation of the endothelial cells arising from organizing thrombus [1]. Masson first described this lesion in 1923 and it was also known as Masson’s tumor, intravascular angiomatosis and vegetant intravascular hemangioendothelioma. The term IPEH was first named by Clearkin and Enzinger in 1976 [1]. It had previously been reported occurring in a variety of locations across the body in the literature, and most had discussed its sonographic and magnetic resonance imaging (MRI) findings [2–9]. Mandibule involvement was rare, which Komori reported as the first case in the left madibular body in 1984 [10]. This article presented a new case which located in the ramus of mandibule. We discussed its multislice computed tomography (MSCT) and panoramic radiography features with pathological findings and reviewed relevant literatures. 2. Case report A 14-year-old male patient presented with right facial swelling for one month and trismus for 20 days. On physical examination a firm, painful, fixed mass measuring approximately 5×5×6 cm was detected ☆ Conflict of interest: None. ⁎ Corresponding author. Department of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China. Tel.: +86 23 8901 1721. E-mail address: [email protected] (S.-S. Xu). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.12.006

in the right ramus of mandibule. The patient underwent both panoramic radiography (SOREDEX CRANEX D Finland, 73 kV, 10 mA, 17.6 s) and CT (64-slice Lightspeed Volume CT GE Medical Systems, USA). CT scanned from the skull base to hyoid bone with a 5mm-thick contiguous section, pitch 0.984, matrix 512×512. CT images were reconstructed into 0.625 mm thin slices, then transferred to GE ADW 4.4 postprocess workstation to create volume rendering (VR), maximum intensity projection (MIP) and multiplanar reconstruction (MPR) images. Contrast medium Iopromide injection (370 mg I/ml, Bayer Schering Pharma AG, Berlin, Federal Republic of Germany) was used for contrast enhancement by intravenous injection (0.2 ml/kg of body weight). Panoramic radiography demonstrated a lesion with multilocular radiolucent area in the right ramus of mandibule (Fig. 1). MSCT showed a multilocuar, expansile, well-circumscribed, osteolytic lesion located in the right ramus of mandibule. The lesion had regional cortex destruction but had no sclerosis. On precontrast MSCT it was low density inside the lesion (CT value 43-50HU), and had no enhancement in the center (CT value 43–50 HU) with peripheral slight enhancement on postcontrast CT (Fig. 2a). The lesion protruded inwards into right pterygomandibular space and the pterygoideus were pushed away with right parapharyngeal space narrowing. It involved buccal space downwards and extended into infratemporal fossa upwards. The lesion was ill-defined with the masseter. The madibular lesion was completely removed. Gross view of the specimen displayed a reddish brown colored cystic lesion containing blood clot with fibrous capsule in the bone. There was also focally metaplastic cartilage formation. Microscopic examination revealed

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Fig. 1. Panoramic radiography demonstrated multilocuar radiolucency (thick arrow) with thin septum (thin arrow) in the right ramus of mandibule.

papillary projections and thrombi within the lumen of vessel lined with endothelial cells (Fig. 3a). In the center of the lesion, there were vague papillary configurations of endothelial cells and mixed with

Fig. 3. IPEH histology. (a) Organized thrombi (thick arrow), papillary proliferations of endothelial cells (thin arrow) within vascular lumen, vascular wall around (asterisk) embedded within mandibule (triangle) (H&E ×40). (b) Florid papillary endothelial cells (thick arrow) hyperplasia lined and contained blood clot (thin arrow) (H&E ×400). (c) CD34 stain revealed positive(arrow) in endothelial cells (×400).

hemorrhage and fibrous materials (Fig. 3b). At the periphery of the mass showed a capsule made of parallel lamellae of collagen. The endothelial cells were positive for vascular marker CD34 (Fig. 3c). Histopathological diagnosis was consistent with florid IPEH. 3. Discussion

Fig. 2. MSCT showed a well-circumscribed, expansile, osteolytic mass in the right ramus of madibule. (a) The cystic mass had no enhancement in the center with peripheral slight enhancement on axial postcontrast CT. Volume rendering in sagittal plane (b) and maximum intensity projection in coronal plane (c) showed multiple bony septum and residual crests (arrow) inside the lesion.

IPEH was believed to be a reactive proliferative lesion of endothelial cells in blood vessels, which was usually associated with organization of thrombi [11]. It most commonly occurred in the skin or subcutaneous soft tissue, usually in the head and neck region and extremities. In the literature it had been reported in any other area of

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the body such as oral cavity, intracranial, bladder, orbit, retroperitoneum, spine, skull, breast, thyroid gland, blood vessel, nasal cavity, paranasal sinus, pharynx, larynx, internal auditory canal, labyrinthine, heart valve, digestive tract, liver, lung, kidney, cervix, uterus or female urethra. This case occurring in the mandible was rare. Hashimoto [12] divided IPEH into three different subtypes: the pure form that arised within dilated vessel, which was often located in the finger, head and neck region; the mixed form that existed in preexisting vascular lesions like hemangioma, aneurysm, vascular malformation, or pyogenic granuloma; and the extravascular form, originating from the hematoma. It could affect any age from 9 months to 78 years with female slight predominance, and the mixed form was often seen in younger patients than the pure form [12]. According to its histology, this case was attributed to the pure form, but it occurred in a young male patient. Mandibular osteolytic destruction might be related to this benign vascular lesion’s erosion of spongy bone tissue. The lesion was expansile when it invaded the mandibular cortex, thus the cortex could be interrupted. Bony septum and residual crests existed in the destructive areas due to incomplete osseous destruction. The lesion’s shape was multilocuar, which indicated its aggressive pattern of growth in different directions. Computed tomography revealed a wellcircumscribed cystic mass without enhancement which was consistent with repeated hemorrhagic content. Peripheral slight enhancement represented fibrous capsule of the mass. The mass was ill-defined with the masseter due to adhesion formation. VR image (Fig. 2b) could display stereoscopically the expansile surface and internal multilocuar changes of the whole lesion in three dimensions. MPR image (not shown) could show real osseous destruction and internal cystic content of IPEH on coronal, sagittal or oblique plane at arbitrary angle in two dimensions. MIP image (Fig. 2c) demonstrated abnormal high density osseous structure. MSCT with these postprocess modalities showed not only the lesion itself in detail but also its extent to adjacent structures as masseteric space and large cervical vessels, which was helpful for surgical treatment planning. Besides mandibule cases, IPEH involving skeleton cases documented in the literature were the skull [13,14], tibia [15], spine [6] and skull base [16]. Komori [10] reported left mandibule IPEH as multilocular radiolucency on radiograph, which was similar to this case on panoramic radiography. Skull and tibia IPEH also showed radiolucency without osseous expansion or sclerosis on radiograph [14,15]. IPEH in skull and skull base CT showed well-circumscribed, expansile, punchout, exophytic, osteolytic soft tissue mass located on the inner table, diploic space, transdiploic, clivus and petrous apex [13,14,16]. IPEH in different stages displayed prominent vascular structures [13], well defined hyperintense nodular or lobulated lesion with homogeneous enhancement [13,16], well-circumscribed growing cystic mass with thin peripheral enhancement [13] on gadolinium-enhanced T1WI. Another IPEH MRI disclosed a nodular mass was hypointense on T1WI and heterogeneous hyperintense on T2WI and exhibited inhomogeneous enhancement of the soft tissue filling the punch-out lesion after intravenous administration of gadolinium [14]. Multiple lesions in the spinal column were intermediate signal (smaller lesions) to mixed low central and high peripheral signal (larger lesions) on T1WI, high signal on T2WI, and gadolinium enhancement [6]. Fasina [11] reported a case orbital IPEH, which CT displayed an isodense mass with minimal enhancement. The orbit could be involved from intracranial IPEH mass which extended along the optic nerve into the orbit [3]. The orbital and intracranial mass demonstrated isointense on T1WI with areas of hyperintensity and T2 hyperintense with internal T2 hypointense, which the mass had intense enhancement. But another different enhancement pattern of IPEH masses reported in renal hilum and retroperitoneum was early and progressive enhancement on dynamic gadolinium enhanced images [6].

IPEH arising in extremities was not rare relatively. There were several articles about MRI of IPEH in extremities, and some cases concerning ultrasound (US) also. IPEH soft tissue mass showed various MR signal intensities, which divided hypointensity [4], iso/ intermediate signal intensity [5,7,9], slight hyperintensity [5] on T1WI, and all masses were hyperintensity on T2WI [4,5,7,9]. The soft tissue mass contained nodular foci, which was hyperintensity on T1WI and low signal intensity on T2WI [4,5,7,9]. Nodular foci indicated hemorrhage [4,5,9] or consistency with thrombi [7] in the mass. The thrombi showed hyperintensity on T1WI and T2WI compared with muscle when at the medium stage of thrombosis [9]. Septal [4,5,7,9], central [4,5,9], and peripheral [4,7,9] enhancement patterns of the lesion displayed on gadolinium enhancement image. But imaging findings of the pure form of IPEH were rather nonspecific [5]. On US IPEH mass demonstrated hypoechoic [4,5], intermediate echogenicity [8] and septal vascularity on color Doppler [4,5]. On the other hand, IPEH in dorsal wrist vein displayed hypoechoic compared with the subcutaneous fat and dorsal wrist tendons and hyperechoic compared with the vein lumen. No flow was seen in the lesion on power Doppler sonography [8]. In conclusion, madibular IPEH was a rare case; its radiography displayed multilocular, radiolucent with bony septum and residual thin crests. Computed tomography demonstrated well-circumscribed, expansile osteolytic cystic mass with peripheral enhancement. IPEH should be included in the radiological differential diagnosis of bone fibrous dysplasia, ameloblatoma, and bone giant cell tumors.

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Radiological imaging of florid intravascular papillary endothelial hyperplasia in the mandibule: case report and literature review.

Intravascular papillary endothelial hyperplasia was a rare benign vascular proliferative process as a result of papillary proliferation of the endothe...
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