Sarcoidosis Presenting as Metastatic Bony Disease

Debra Mangino; Diane E. Stover

doi:10.1159/000077430

Mangino, Debra; Stover, Diane E.

2004-06-01 00:00:00

IntroductionSarcoid of the vertebral bones is rare with less than 30 cases reported. While one of the common denominators of patients with spinal cord sarcoidosis is the paucity of systemic findings, patients with vertebral body sarcoid usually have a known diagnosis of intrathoracic sarcoid. This case presented a diagnostic dilemma because the initial presentation of sarcoidosis was back pain accompanied by low-grade fever. There were no pulmonary signs or symptoms and magnetic resonance imaging (MRI) was highly suggestive of malignancy. Since there are very few case reports on vertebral body sarcoidosis with MR images, we present our case with the MR findings and a review of the literature.Case ReportA previously healthy 42-year-old African-American male was referred to the Memorial Sloan-Kettering Cancer Center for a second opinion regarding the etiology of his back pain. Eight months prior, the patient developed severe thoracic back pain accompanied by low-grade fevers and night sweats. The pain was not associated with any numbness, tingling, weakness or other neurologic symptoms.Although a skeletal survey was unrevealing, findings on a bone scan showed increased radiotracer activity in the thoracic spine raising the possibility of a metastatic disease. The patient underwent an MRI of the spine which revealed abnormal marrow signal intensity within T6, T8 and T10 vertebral bodies with a mass extending into the epidural space and the surrounding paraspinal soft tissues (fig. 1A).Fig. 1A Axial T1-weighted MRI at the level of T10 at the time of diagnosis showing abnormal marrow signal intensity suggesting diffuse infiltration of the marrow. There is also epidural invasion (arrowheads) and a paraspinal soft tissue mass (large arrows) with involvement of the pedical on the left side. There is no cord compression, compression fractures or intrathecal enhancing lesions. B Nine months later and after treatment, there is improvement with a small amount of a residual epidural component (arrowheads) and resolution of the paraspinal mass.These findings were considered to be highly suspicious for metastatic disease and the patient underwent an open biopsy of the paraspinal mass and the vertebral body at T10. Noncaseating granuloma involving both the bone and soft tissue were present. Pathologically, there was no evidence of malignancy, and microbiologic stains and cultures did not reveal any infectious agents. The intradermal purified protein derivative test was negative with a positive anergy panel.Despite the lack of organisms on the stains and cultures of the tissue, the patient was treated for tuberculosis. A gallium scan (GA-67) showed gallium avid tissue in the right upper abdominal quadrant, right paratracheal, bilateral hilar, lacrimal and parotid areas as well as in the mid and lower thoracic spine. A chest radiograph was unremarkable, and a computed tomographic scan of the chest showed slightly enlarged right paratracheal and hilar lymph nodes. Pulmonary function tests were normal. Physical examination showed normal vital signs, bilateral lacrimal hypertrophy, and a large, red, tender keloid on the sternal notch. Neurologic examination was normal. Laboratory values showed negative fungal serologic tests, a normal erythrocyte sedimentation rate and ACE level. Positron emission tomography showed no evidence of abnormal metabolic activity suggestive of malignancy.The patient was placed on 40 mg/day of prednisone for the treatment of sarcoidosis of the vertebral bodies with epidural extension. Two months after starting prednisone therapy, the patient was pain free and able to return to work. The MRI of the spine had normalized (fig. 1B). The patient is on a slow steroid taper and remains pain free.DiscussionSarcoidosis is a multisystem immunologic disorder which predominantly involves the intrathoracic lymph nodes and the lungs. Bony involvement is not infrequent and is seen in up to 13% of sarcoid patients. It is usually a late development and is often associated with cutaneous lesions [1]. The most common manifestations are asymptomatic lytic lesions of the phalanges of the hands and feet. Involvement of the vertebral column is rare, and virtually all publications are limited to case reports [2, 3, 4, 5, 6]. Vertebral sarcoidosis should be distinguished from spinal cord sarcoidosis. The former effects primarily the vertebral bones, while the latter involves the spinal cord and is classified as neurosarcoidosis. Concurrent vertebral and spinal cord sarcoid are rarely found [7]. We present a case of sarcoid of the vertebral bodies with extension to but no involvement of the spinal cord.Almost always patients with vertebral body sarcoid are symptomatic and present with radicular back pain both during activity and at rest. Tenderness over the involved area may be present. Unlike our patient, most patients with vertebral body involvement have a known diagnosis of intrathoracic sarcoidosis. All areas of the vertebral column can be involved, but the thoracic spine is most common. Multiple vertebral bodies are usually affected, and plain radiography can be normal or reveal lytic lesions with or without a rim of sclerosis. An associated paraspinal mass may be present with variable involvement of the pedical. The disc space can be preserved. Bone scans are nonspecific showing uptake by involved bone. Gallium scanning (GA-67) can add diagnostic probability. As seen in our patient, the gallium can be taken up in the vertebral lesions as well as clinically silent areas such as the lacrimal and salivary glands and/or the hilar and mediastinal regions. Computed tomography can show either osteolytic or osteosclerotic lesions.In a few cases, MRI findings of vertebral sarcoidosis have been reported [3, 4, 5, 8]. The MRI usually demonstrates multifocal lesions within the vertebrae that are hypointense (low signal intensity) on T1-weighted images, hyperintense (high signal intensity) on T2-weighted images and enhance following contrast medium administration. The MR images are nonspecific and the differential diagnosis includes metastatic disease, myeloma, lymphoma and granulomatous infection particularly tuberculosis. Biopsy is recommended in all cases to exclude these other diagnoses.Complications of vertebral sarcoidosis are usually limited to pain. Occasionally, vertebral collapse and neurologic impairment can occur. Treatment is anecdotal. Neurosarcoidosis involving the spinal cord as well as vertebral body sarcoidosis have been managed primarily with steroid therapy. For vertebral sarcoid there is no large amount of information or clinical experience concerning specific drug, dose or duration of therapy but symptoms are usually used as a guide. Treatment with agents such azathioprine, cyclophosphamide, methotrexate and cyclosporin have had variable success in the treatment of systemic sarcoidosis including neurosarcoidosis [9]. Interestingly, radiation therapy has been used in some 6 refractory cases of neurosarcoid, with most patients responding [10]. Our review of the literature could find no cases of vertebral sarcoid treated with any of these modalities. If neurologic involvement occurs secondary to vertebral destruction, surgical stabilization and decompression may be required [4]. In summary, vertebral body sarcoidosis is extremely rare and in the absence of pulmonary sarcoidosis, a difficult diagnosis to establish without a biopsy. Even with a biopsy, other diagnoses, e.g. granulomatous infection and malignancy, must be excluded before the diagnosis of vertebral body sarcoidosis can be made with confidence.

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Sarcoidosis Presenting as Metastatic Bony Disease

$IntroductionSarcoid of the vertebral bones is rare with less than 30 cases reported. While one of the common denominators of patients with spinal cord sarcoidosis is the paucity of systemic findings, patients with vertebral body sarcoid usually have a known diagnosis of intrathoracic sarcoid. This case presented a diagnostic dilemma because the initial presentation of sarcoidosis was back pain accompanied by low-grade fever. There were no pulmonary signs or symptoms and magnetic resonance imaging (MRI) was highly suggestive of malignancy. Since there are very few case reports on vertebral body sarcoidosis with MR images, we present our case with the MR findings and a review of the literature.Case ReportA previously healthy 42-year-old African-American male was referred to the Memorial Sloan-Kettering Cancer Center for a second opinion regarding the etiology of his back pain. Eight months prior, the patient developed severe thoracic back pain accompanied by low-grade fevers and night sweats. The pain was not associated with any numbness, tingling, weakness or other neurologic symptoms.Although a skeletal survey was unrevealing, findings on a bone scan showed increased radiotracer activity in the thoracic spine raising the possibility of a metastatic disease. The patient underwent an MRI of the spine which revealed abnormal marrow signal intensity within T6, T8 and T10 vertebral bodies with a mass extending into the epidural space and the surrounding paraspinal soft tissues (fig. 1A).Fig. 1A Axial T1-weighted MRI at the level of T10 at the time of diagnosis showing abnormal marrow signal intensity suggesting diffuse infiltration of the marrow. There is also epidural invasion (arrowheads) and a paraspinal soft tissue mass (large arrows) with involvement of the pedical on the left side. There is no cord compression, compression fractures or intrathecal enhancing lesions. B Nine months later and after treatment, there is improvement with a small amount of a residual epidural component (arrowheads) and resolution of the paraspinal mass.These findings were considered to be highly suspicious for metastatic disease and the patient underwent an open biopsy of the paraspinal mass and the vertebral body at T10. Noncaseating granuloma involving both the bone and soft tissue were present. Pathologically, there was no evidence of malignancy, and microbiologic stains and cultures did not reveal any infectious agents. The intradermal purified protein derivative test was negative with a positive anergy panel.Despite the lack of organisms on the stains and cultures of the tissue, the patient was treated for tuberculosis. A gallium scan (GA-67) showed gallium avid tissue in the right upper abdominal quadrant, right paratracheal, bilateral hilar, lacrimal and parotid areas as well as in the mid and lower thoracic spine. A chest radiograph was unremarkable, and a computed tomographic scan of the chest showed slightly enlarged right paratracheal and hilar lymph nodes. Pulmonary function tests were normal. Physical examination showed normal vital signs, bilateral lacrimal hypertrophy, and a large, red, tender keloid on the sternal notch. Neurologic examination was normal. Laboratory values showed negative fungal serologic tests, a normal erythrocyte sedimentation rate and ACE level. Positron emission tomography showed no evidence of abnormal metabolic activity suggestive of malignancy.The patient was placed on 40 mg/day of prednisone for the treatment of sarcoidosis of the vertebral bodies with epidural extension. Two months after starting prednisone therapy, the patient was pain free and able to return to work. The MRI of the spine had normalized (fig. 1B). The patient is on a slow steroid taper and remains pain free.DiscussionSarcoidosis is a multisystem immunologic disorder which predominantly involves the intrathoracic lymph nodes and the lungs. Bony involvement is not infrequent and is seen in up to 13% of sarcoid patients. It is usually a late development and is often associated with cutaneous lesions [1]. The most common manifestations are asymptomatic lytic lesions of the phalanges of the hands and feet. Involvement of the vertebral column is rare, and virtually all publications are limited to case reports [2, 3, 4, 5, 6]. Vertebral sarcoidosis should be distinguished from spinal cord sarcoidosis. The former effects primarily the vertebral bones, while the latter involves the spinal cord and is classified as neurosarcoidosis. Concurrent vertebral and spinal cord sarcoid are rarely found [7]. We present a case of sarcoid of the vertebral bodies with extension to but no involvement of the spinal cord.Almost always patients with vertebral body sarcoid are symptomatic and present with radicular back pain both during activity and at rest. Tenderness over the involved area may be present. Unlike our patient, most patients with vertebral body involvement have a known diagnosis of intrathoracic sarcoidosis. All areas of the vertebral column can be involved, but the thoracic spine is most common. Multiple vertebral bodies are usually affected, and plain radiography can be normal or reveal lytic lesions with or without a rim of sclerosis. An associated paraspinal mass may be present with variable involvement of the pedical. The disc space can be preserved. Bone scans are nonspecific showing uptake by involved bone. Gallium scanning (GA-67) can add diagnostic probability. As seen in our patient, the gallium can be taken up in the vertebral lesions as well as clinically silent areas such as the lacrimal and salivary glands and/or the hilar and mediastinal regions. Computed tomography can show either osteolytic or osteosclerotic lesions.In a few cases, MRI findings of vertebral sarcoidosis have been reported [3, 4, 5, 8]. The MRI usually demonstrates multifocal lesions within the vertebrae that are hypointense (low signal intensity) on T1-weighted images, hyperintense (high signal intensity) on T2-weighted images and enhance following contrast medium administration. The MR images are nonspecific and the differential diagnosis includes metastatic disease, myeloma, lymphoma and granulomatous infection particularly tuberculosis. Biopsy is recommended in all cases to exclude these other diagnoses.Complications of vertebral sarcoidosis are usually limited to pain. Occasionally, vertebral collapse and neurologic impairment can occur. Treatment is anecdotal. Neurosarcoidosis involving the spinal cord as well as vertebral body sarcoidosis have been managed primarily with steroid therapy. For vertebral sarcoid there is no large amount of information or clinical experience concerning specific drug, dose or duration of therapy but symptoms are usually used as a guide. Treatment with agents such azathioprine, cyclophosphamide, methotrexate and cyclosporin have had variable success in the treatment of systemic sarcoidosis including neurosarcoidosis [9]. Interestingly, radiation therapy has been used in some 6 refractory cases of neurosarcoid, with most patients responding [10]. Our review of the literature could find no cases of vertebral sarcoid treated with any of these modalities. If neurologic involvement occurs secondary to vertebral destruction, surgical stabilization and decompression may be required [4]. In summary, vertebral body sarcoidosis is extremely rare and in the absence of pulmonary sarcoidosis, a difficult diagnosis to establish without a biopsy. Even with a biopsy, other diagnoses, e.g. granulomatous infection and malignancy, must be excluded before the diagnosis of vertebral body sarcoidosis can be made with confidence.$

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Sarcoidosis Presenting as Metastatic Bony Disease

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Sarcoidosis Presenting as Metastatic Bony Disease

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