CASE REPORT

Atypical vertebral Paget’s disease

Constance Beaudouin & Anthony Dohan &

Toufic Nasrallah &Caroline Parlier & Sébastien Touraine &

Korng Ea & Rachid Kaci & Jean-Denis Laredo

Received: 14 June 2013 /Revised: 21 November 2013 /Accepted: 8 December 2013# ISS 2014

Abstract A 40-year-old Mauritanian man consulted for backpain. A computed tomography of the spine showed patchysclerosis of the fifth and seventh thoracic vertebral bodieswith normal neural arch of T5 and sclerosis and hypertrophyof the neural arch of T7, as well as diffuse sclerosis of theT11 vertebral body with a normal neural arch. At MRI, lowsignal-intensity on T1-weighted images and high signal-intensity on T2-weighted images involved the whole T5and T7 vertebrae and the vertebral body of T11. Workingdiagnoses included metastatic disease and lymphoma, and abiopsy of T7 and then T11 was carried out. Both showedpathological findings very suggestive of Paget’s disease.Since CT is usually the more specific radiological examina-tion in vertebral Paget’s disease, we thought it could beuseful to report this atypical CT presentation (patchy sclero-sis of the vertebral body without diffuse bone texture chang-es and isolated involvement of the vertebral body) of verte-bral Paget’s disease.

Keywords Paget’s disease . Vertebral bone sclerosis .

Vertebral Paget’s disease . Nodular bone sclerosis

Introduction

Bone sclerosis in vertebral Paget’s disease (PD) is usuallydiffuse at CT, involving both the vertebral body and theposterior elements. We report a case of Paget disease, present-ing at CT as patchy sclerosis of the vertebral body withoutinvolvement of the neural arch. A malignant process wassuggested and a percutaneous biopsy of T7 and then T11was performed.

Case report

A 40-year-old Mauritanian male consulted for back pain. Hispast clinical history was unremarkable. Clinical examinationonly showed lumbar stiffness. Blood tests including C reactiveprotein, blood cell counts, calcemia, alkaline phosphatases,and protein electrophoresis were within normal limits. Con-ventional radiographs showed a subtle sclerosis of T5 and T7(not T11) vertebral bodies without neural arch abnormalities.A 1.5-T MR examination of the thoracic and lumbar spineshowed several foci of abnormal signal-intensity involving thewhole fifth and seventh thoracic vertebrae and the vertebralbody of T11 with low signal-intensity on T1-weighted images(Fig. 1a), normal signal-intensity on fat-saturated T2-weighted images (Fig. 1b) and moderate homogeneous en-hancement on gadolinium-enhanced T1-weighted images(Fig. 1c). There was no soft tissue involvement.

A computed tomography (CT) of the spine showed abnor-malities of the T5, T7, and T11 vertebrae (Fig. 2a): patchysclerosis of T5 vertebral body with a normal neural arch(Fig. 2b), focal sclerosis of T7 vertebral body together withsclerosis and cortical thickening of the neural arch (Fig. 2c)and diffuse involvement of the T11 vertebral body with anormal neural arch (Fig. 2d). There was no soft tissue involve-ment and the cortical bone was preserved. Despite the absence

C. Beaudouin (*) :A. Dohan : T. Nasrallah :K. EaService de Rhumatologie, Hôpital Lariboisière,2 rue Ambroise Paré, 75010 Paris, Francee-mail: const.beaudouin@gmail.com

C. Parlier : S. Touraine : J.<D. LaredoService de Radiologie Ostéoarticulaire, Hôpital Lariboisière,2 rue Ambroise Paré, 75010 Paris, France

R. KaciService d’Anatomo-Pathologie, Hôpital Lariboisière,2 rue Ambroise Paré, 75010 Paris, France

Skeletal RadiolDOI 10.1007/s00256-013-1799-9

of clinical or biological signs that could indicate an underlyingactive disease, the working diagnoses at that time were scle-rotic metastasis, vertebral lymphoma and, to a lesser extent,

sarcoidosis, mastocytosis, and tuberous sclerosis. A CTof thethorax, abdomen, and pelvis did not show any other abnor-mality. A PET scanner did not favor a malignant disease, but

Fig. 1 Thoracic spine MRI (1.5 T). a Sagittal T1-weighted image (TR=546 ms/TE=11 ms, slice thickness=3 mm) showing low signal intensityinvolving the whole vertebral body of T5, T7, and T11 and mild lowsignal intensity of the spinous processes of T5 and T7. b Sagittal fat-saturated T2-weighted image (TR=3,660 ms/TE=80 ms, slice

thickness=3 mm) showing normal signal intensity of T5, T7, and T11vertebrae (arrows). c Sagittal gadolinium-enhanced T1-weighted image(TR=398 ms/TE=11 ms, slice thickness=4 mm) showing moderatehomogeneous enhancement. The signal intensity of T5, T7, T11 vertebraeis still decreased compared with adjacent vertebrae

Fig. 2 Thoracic spine CT. aSagittal reformation showingfocal sclerotic areas within T5 andT7 vertebrae and diffuse sclerosisof T11 vertebral body (arrows).There is also mild convexity ofthe posterior cortex of T7vertebral body. The neural arch ofT7 exhibits some changessuggestive of Paget’s disease(bone thickening, increased bonedensity, coarse trabeculation andloss of cortical–cancellous bonedifferentiation [arrowhead]),while the neural arches of T5 andT11 have a normal appearance. bAxial CT image at T5 levelshowing sclerotic foci in thevertebral body (arrows) and anormal neural arch. cAxial CTimage at T7 level showing a largesclerotic area in the right part ofthe vertebral body and pedicle(arrow) and a small scleroticfocus in the left transverse process(arrowhead). dAxial CT image atT11 level showing bone sclerosisof the vertebra, sparing the lefthemi-vertebra

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did not help either in ruling out a malignant condition. Itshowed mildly increased activity at the three involved

vertebral levels (T5, T7, T11; maximal SUV ranging from2.5 to 3.3 compared with a mean of 1.8 for normal vertebrae)and possible involvement of L1, without any other site ofincreased activity.

To obtain a pathological diagnosis, a percutaneous biopsyof T7 under CT guidance was performed (Fig. 3). No malig-nant proliferation was found at pathological examination.Pathological findings were very suggestive of PD and includ-ed abundant woven bone and extensive osteoclastic boneresorption together with increased osteoblastic activity(Fig. 4). Owing to the atypical radiological presentation, asecond percutaneous biopsy, at the T11 vertebral body level,was carried out. This second biopsy showed the same patho-logical findings, confirming the diagnosis of PD.

Discussion

The spine is the second most frequent skeletal location of PDafter the pelvis [1, 2]. Vertebral PD involves predominantlythe lumbar spine (62 %), whereas the thoracic and cervical

Fig. 3 CT-guided biopsy at the T11 level with an 11-gauge LAR 2000(LAURANE®Medical) trephine biopsy needle

Fig. 4 Pathological findings from the first percutaneous biopsy of T7vertebra. a This photomicrograph illustrates the thickened bony trabecu-lae and a loose fibrovascular connective tissue filling the medullaryspaces of the bone (low magnification; hematoxylin, eosin, and saffronstain). b Higher magnification of bone reveals increased osteoclastic

activity (hematoxylin, eosin, and saffron stain). c Higher magnificationof bone reveals increased osteoblastic activity (hematoxylin, eosin, andsaffron stain). d Irregular cement lines are well visible (high magnifica-tion; hematoxylin, eosin, and saffron stain)

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spines are affected in only 29.8 % and 8.2 % of the casesrespectively [3]. Vertebral PD is usually polyostotic [2]. Withina given bone, radiological changes may be diffuse or focal [4].

Bone sclerosis is one of the main radiological manifesta-tions of PD. In vertebral PD, bone sclerosis usually involvesthe whole vertebra, producing the typical “ivory vertebra.”Differentiation of PD from other causes of “ivory vertebra,”such as metastatic disease or lymphoma, may be difficult. Inthese situations, enlargement of the vertebral dimensions is akey finding for the diagnosis of PD. Another important clue todifferentiate PD from other causes of “ivory vertebrae” is theinvolvement of the whole vertebra from the vertebral body tothe spinous process. This finding is found in 82 % to 94 % ofvertebral PD cases [2, 5, 6]. Diffuse radiological bone texturechanges seen in vertebral PD include several appearances:multicystic, mesh, or diffuse homogeneous sclerosis [7, 8].Sclerotic foci are also often seen (51 % of vertebral PDlesions) in association with these diffuse bone texture changes[7] and are considered an end-stage abnormality in PD [2, 6].

The case reported here presented as patchy bone sclerosisinvolving the fifth and seventh thoracic vertebral bodies,without diffuse pagetic bone texture changes. Diffuse involve-ment of the vertebral body was present at T11 only, while theneural arch was spared. This radiological presentation is veryunusual in PD and raised the suspicion of malignant disease.

To our knowledge, patchy sclerosis of the vertebral bodywithout diffuse bone texture changes has been reported invertebral PD only twice [6, 9]. In a retrospective analysis of aseries of 79 patients with vertebral PD from our files (submitteddata), two additional cases also presented with patchy sclerosiswithout diffuse bone texture changes, but, in both cases, fattyfoci, a characteristic finding of Paget’s disease at MRI, werecoexisting with the patchy sclerosis within the vertebral body,and were helpful in ruling out a malignant process.

In the present case, MRI demonstrated abnormal signalintensity in the vertebral body and neural arch, both at T5and T7 levels, while the neural arch was involved only at theT7 level at CT. MR findings, however, are less diagnostic thanCT findings in vertebral PD, except when MRI demonstratesthe presence of fat within the abnormal vertebra.

Pagetic changes in the posterior elements usually occur inconjunction with vertebral body abnormalities and less fre-quently as an isolated manifestation of spinal PD [10, 11].Isolated involvement of the vertebral body is uncommon inPD as well, reported in only 12 % of a previous series [5].Demonstration of the concomitant involvement of the spinousprocess at radiography or CT in the case of a non-specificlesion of the vertebral body is a key finding in favor of PD [7].In our case, minimal CT abnormalities involving the neuralarch of the seventh thoracic vertebra were retrospectivelyidentified (Fig. 2a, c), while the neural arch was normal atT5 and T11 levels, even at a retrospective examination.

According to previous classifications, PD starts with a lyticphase, progresses through a mixed phase, and ends with asclerotic phase. In addition, PD is known to progress over timewithin a single bone, extending from one end to the other, andfinally involving the whole bone. The present case demon-strates unusual findings in the context of these different phasesof Paget’s disease: bone sclerosis corresponds to a late stagewhile partial involvement at CT may suggest relatively earlyinvolvement. However, this three-stage classification was de-vised before CTandMRI were available and it is uncertain if itcould be applied to the spine. According to Vande Berg et al.[9], the lytic phase is usually not detected in bones with a hightrabecular/cortex ratio like the vertebra, sacrum and pelvis,and cases of vertebral Paget’s disease presenting in the lyticphase are exceedingly rare [12–15].

In conclusion, since CT is usually the more specific radio-logical examination in vertebral Paget’s disease, knowledge ofatypical CT presentations of the disease is important. Wereport a case of vertebral PD involving three distant vertebraeand presenting, in two vertebrae, as patchy sclerosis of thevertebral body without diffuse bone texture changes of thevertebral body and without neural arch involvement. MRI andPET did not allow a malignant disease to be ruled out and avertebral biopsy at two different vertebral levels had to becarried out. This case shows that patchy sclerosis of thevertebral body without diffuse bone texture changes as wellas isolated involvement of the vertebral body without neuralarch involvement belong to the scope of vertebral PD at CT. Inthe case of several vertebral lesions with a similar appearance,knowledge of these presentations may avoid repeated biop-sies, as were performed in the present case.

Conflict of interest None.

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