Ir J Med Sci (2009) 178:107–110 DOI 10.1007/s11845-008-0133-3

CASE REPORT

MRI, CT, scintigraphic and histological features of a vanishing scapular eosinophilic granuloma S. K. Golla Æ E. C. Kavanagh

Received: 11 June 2007 / Accepted: 8 February 2008 / Published online: 23 February 2008 Ó Royal Academy of Medicine in Ireland 2008

Abstract Introduction Vanishing bone lesions have been previously described in patients with eosinophilic granuloma. Materials and methods We present the magnetic resonance imaging, computed tomography, scintigraphic and histological findings in a 26-year-old woman presenting with a painful scapular mass that subsequently completely resolved, compatible with the diagnosis of eosinophilic granuloma. Conclusion Clinicians should be aware of the multimodality appearances and natural history of resolving eosinophilic granuloma. Keywords

CT
MRI
Scapula

Introduction Vanishing bone lesions have been previously described in patients with eosinophilic granuloma (EG), with radiographic findings well documented [1]. Here, we present the magnetic resonance imaging (MRI), computed tomography (CT), scintigraphic and histological findings in a 26-yearold woman presenting with a painful scapular mass that subsequently completely resolved, compatible with the diagnosis of EG. S. K. Golla
E. C. Kavanagh Department of Musculoskeletal Radiology, University of Pittsburgh Medical Centre, 200 Lothrop St., Pittsburgh, PA 15213, USA E. C. Kavanagh (&) Department of Radiology, Mater Misericordiae Hospital, Eccles Street, Dublin 7, Ireland e-mail: [email protected]

Case report A 26-year-old previously healthy woman presented with a 6week history of right shoulder pain, with no history of trauma. Examination revealed some mild tenderness over the right scapular spine. Shoulder motion was noted to be limited with no evidence of axillary lymphadenopathy. Initial radiographic evaluation with antero–posterior and trans-scapular Y views of the right shoulder was unremarkable. Subsequently, an MRI arthrogram of the right shoulder was requested. This examination showed an aggressive lytic mass within the body and spine of the right scapula with evidence for cortical break-through and involvement of surrounding soft tissue structures (Fig. 1). An isotope bone scan was then performed, which showed this markedly increased uptake of radiotracer in the right scapular body and spine (Fig. 2). There were no other areas of abnormal radiotracer uptake in the axial or appendicular skeleton. Fine needle aspirates and core biopsies of this right scapular lesion were then performed utilizing CT guidance. The planning CT images confirmed the presence of an aggressive appearing mass in the right scapula showing extensive cortical disruption (Fig. 3). Histological analysis showed evidence of EG (Fig. 4). A conservative management approach was taken, as the patient’s symptoms of shoulder pain were beginning to resolve. A repeat CT at 3 months post-diagnosis showed interval reduction in size of the right scapular lesion with adjacent bony sclerotic change within the right scapular body and spine (Fig. 5). A subsequent CT at 6 months post-presentation showed complete resolution of the previously identified mass with reconstitution of normal bone (Fig. 6). At this time, the patient was completely asymptomatic with full range of motion in her scapula and shoulder. The patient remains well and asymptomatic at 12 months post-initial presentation.

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Fig. 1 Twenty-six-year-old woman with right scapular eosinophilic granuloma. MR images are from an MR arthrogram study, performed following the administration of dilute intraarticular Gadolinium. a Axial T1-weighted image without fat saturation shows an expansile mass in the right scapular body with evidence of cortical break-

Fig. 2 Twenty-six-year-old woman with right scapular eosinophilic granuloma. Isotope bone scan shows focally increased uptake in the right scapular body and spine

Discussion EG is one of the triad of the diseases included in the designation of Langerhans cell histiocytosis, previously called histiocytosis X [2, 3]. The other two components of this spectrum of the disease are Letterer–Siwe disease (acute disseminated form) or Hand–Schuller–Christian syndrome (a chronic disseminated form). EG is a disease of young

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through and extension of the mass into the adjacent soft tissues (arrows). b Coronal-oblique T2-weighted image without fat saturation confirms the presence of a mass in the body of the right scapula with extension into the adjacent soft tissues (arrows)

Fig. 3 Twenty-six-year old woman with right scapular eosinophilic granuloma. Planning CT image for CT guided biopsy shows an expansile lesion in the right scapular body and spine with extensive cortical disruption (arrow)

adults and children of unknown etiology. It is described as a solitary mono-ostotic lesion in the majority of the cases [3, 4]. Patients with a single site lesion have a more benign course and favorable prognosis compared to poly-ostotic lesions. It is currently considered to be a disorder of immune regulators, manifested by abnormal proliferation of histiocytes and granuloma formation. The Langerhans Cell, a unique histiocyte, is the distinctive pathologic component of the disease.

109 Fig. 4 Twenty-six-year-old woman with right scapular eosinophilic granuloma. a Fine needle aspirate of the right scapular lesion showing bland histiocytic like cells mingled with eosinophils and vascular spaces (Hematoxyline and Eosin stain, magnification 940). b, c Immunohistochemical stains demonstrating characteristic S100 and CD1a positive cells in the lesion (magnification 920)

Fig. 5 Twenty-six-year-old woman with right scapular eosinophilic granuloma. Axial non-contrast CT image obtained at 3 months post diagnosis shows sclerotic change in the bone surrounding the previously seen scapular lesion with reduction in size of the previously seen mass

Viral and other infectious agents have been considered because of the self-limiting nature of the disease in some patients, and their response to antibiotics and steroids. The non-neoplastic nature of the disease is supported by the response of many patients to steroids, low doses of

Fig. 6 Twenty-six-year-old woman with right scapular eosinophilic granuloma. Axial non-contrast CT image obtained 6 months post presentation shows complete resolution of the previously seen mass with reconstitution of normal bone in the body and spine of the scapula

radiation treatment and spontaneous regression. Most of the localized forms occur between 5 and 15 years of age. They can occur in any bone, with predilection for flat

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bones. The size of these solitary EG bony lesions ranges from 1 to 12 cm. The radiologic features of EG depend upon the site of involvement and stage of the disease [5]. Most of the lesions appear lytic, with well-demarcated margins or poorly defined margins. The early phase may have a more aggressive pattern of osteolysis. Evolving EG may have a less aggressive appearance with sclerotic margins. Chronic lesions may completely disappear or may have sclerotic changes. Spontaneous regression of osseous, parenchymal, skin and central nervous system lesions of histiocytosis X (Langerhans cell histiocytosis) has been reported in the literature [6–9]. Most of the cases reported were in young children under 10 years of age. Our patient presented at the age of 26, with acute onset of shoulder pain of 4–6 weeks duration. MRI examination performed demonstrated an aggressive lesion of the subglenoid region with destruction of the cortex with extension into the surrounding rotator cuff muscles. The diagnosis of a lytic metastasis, lymphoma and other aggressive pathologies, including osteomyelitis, was included in the differential. Subsequent work up, including isotope bone scan and CT scans of the chest, abdomen and pelvis, demonstrated no other lesions. Biopsy of the lesion proved it to be EG. We have decided to report this case of EG because of its presentation at an older age as well as its fast resolution without any treatment. In conclusion, we have demonstrated the MRI, CT, scintigraphic and histological findings

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in a 26-year-old woman presenting with a painful scapular mass with interval complete regression, compatible with the diagnosis of EG. Clinicians should be aware of the multimodality appearances and natural history of EG.

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