Primary intraosseous paraganglioma of the sacrum: a case report

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The Spine Journal 7 (2007) 733–738

Primary intraosseous paraganglioma of the sacrum: a case report Ilya Laufer, MDa,*, Mark A. Edgar, MDb, Roger Ha¨rtl, MDa a

Department of Neurological Surgery, Weill Medical College of Cornell University, New York Presbyterian Hospital, 435 East 70th Street, #27L, New York, NY 10021, USA b Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA Received 26 January 2006; accepted 20 September 2006


BACKGROUND CONTEXT: Paragangliomas are neuroendocrine tumors that most frequently arise in the adrenal medulla, carotid body, and glomus jugulare. However, they have been reported in many other sites throughout the body. Within the central nervous system, the overwhelming majority of paragangliomas arise intradurally in the area of the cauda equina. We report a case of a woman who presented with back pain and radiculopathy and was found to have an intraosseous sacral mass on magnetic resonance imaging (MRI). Operative biopsy revealed that this lesion was a paraganglioma. Chest, abdomen, and pelvis computed tomography scans revealed no other tumors. This lesion was treated with limited resection, decompression, stabilization, and radiation therapy. PURPOSE: To report a rare case of an intrasacral paraganglioma that presented with back pain and radiculopathy and was treated with intralesional decompression, stabilization, and radiation. STUDY DESIGN: Observational case report. METHODS: A 69-year-old woman presented with back and leg pain. An MRI scan revealed a grade II spondylolisthesis at L5/S1 and a left-sided intraosseus mass at S1 with significant obstruction of the left S1 nerve root foramen. The patient underwent initially an open biopsy of this lesion that revealed a paraganglioma. Subsequently, she underwent transarterial embolization of this lesion, partial resection with decompression of the nerve root, and stabilization and arthrodesis from L4 to the ilium. RESULTS: Intraoperatively, a highly vascular lesion was encountered. The patient’s back and leg pain improved significantly after surgery. She was referred for intensity-modulated radiation therapy. One year after surgery, she was neurologically intact without evidence of growth of the lesion. CONCLUSIONS: Only two cases of primary intraosseous sacral paragangliomas were reported in the past. We believe that paragangliomas should be included in the differential diagnosis of intraosseous sacral tumors. Optimal treatment of the lesions can be difficult because of their hypervascular nature and the high morbidity associated with complete surgical resection. Treatment should combine preoperative embolisation, surgical resection if possible, and radiation therapy. Ó 2007 Elsevier Inc. All rights reserved.


Sacrum; Paraganglioma; Intraosseous

Introduction Paragangliomas are tumors that arise from the diffuse neuroendocrine system and thus have been reported in FDA device/drug status: not applicable. Nothing of value received from a commercial entity related to this manuscript. * Corresponding author. Department of Neurological Surgery, Weill Cornell Medical College, New York Presbyterian Hospital, 435 East 70th Street, #27L, New York, NY 10021, USA. Tel.: (212) 706-7499; fax: 212 746-8416. E-mail address: [email protected] (I. Laufer) 1529-9430/07/$ – see front matter Ó 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.spinee.2006.09.008

a multitude of locations throughout the body. They are most frequently found in the adrenal medulla (pheochromocytoma), carotid body, and jugular glomus but have also been reported in liver, urinary bladder, urethra, gastrointestinal tract, retroperitoneum, and other sites throughout the body. Within the central nervous system, they were reported in the sella turcica, cavernous sinus, cerebellopontine angle, pineal gland, and throughout the spinal cord [1–3]. The majority of spinal cord paragangliomas are found in the cauda equina, but they have been reported in the cervical, thoracic, and lumbar spine [2,4–15]. We report the case of a patient who was found to have an intraosseous extradural


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paraganglioma of the sacrum. The differential diagnosis of tumors of the sacrum includes metastases, chordomas, giant cell tumors, and several other less common lesions [16,17]. Based on this and two previous case reports [18,19], paraganglioma should be added to the differential diagnosis of intraosseous sacral masses.

Case report A 69-year-old woman presented with a year-long history of progressive low back and left leg pain. She developed weakness of her left lower extremity and had to use a cane to ambulate. She reported normal bowel and bladder function. A physical examination revealed 5/5 strength in all lower-extremity muscle groups, with intact sensation. Magnetic resonance imaging (MRI) of her lumbosacral spine revealed a large left-sided osseous sacral mass with central areas of necrosis and a soft-tissue component extending into the left S1 foramen. Incidentally, a grade II spondylolisthesis was noted at the L5/S1 junction with significant foraminal stenosis (Fig. 1). A computed tomography (CT) scan of her sacrum revealed a partially sclerotic, partially lytic lesion in the S1/2 vertebral bodies (Fig. 2). A CT scan of her chest, abdomen, and pelvis showed no other lesions. A subsequent F-18 fluorodeoxyglucose positron emission tomography (FDG PET) scan showed increased FDG uptake only in the sacral area. The patient underwent an open biopsy of the lesion via a small laminotomy of S1 to obtain definitive tissue diagnosis. A tan, hypervascular mass was found that had eroded the bone of the sacrum. Tissue samples were obtained from the intra- and extraosseous components of the mass and were sent for pathology. Histological sections of tumor showed epithelioid cells with a modest amount of pink, granular cytoplasm, and focal mild cytologic atypia (Fig. 3A); no mitoses were seen. Cells were arranged in cords and nests with close relationship to sinusoidal vascular channels. Immunostains for chromogranin and synaptophysin strongly labeled most of the tumor cells (Fig. 3B), and S-100 protein immunostain highlighted the epithelioid cells (chief cells) and a second population of slender, spindly cells at the periphery of many cell nests (sustentacular cells). Immunostains for cytokeratins were negative, and tumor cells did not label for CD31 or inhibin. The findings were typical for paraganglioma. The patient was subsequently taken to the operating room for complete decompression of the L5 and S1 nerve roots via laminectomies and foraminotomies and lumboiliac fusion (Fig. 4A). During this procedure, pedicle screws were placed in L3, L4, and L5 and into both iliac wings and connected with rods. Arthrodesis was performed by using a combination of local autograft and allograft bone. Intraoperatively, the patient was noted to have porous, weak bone. Therefore, fusion construct was extended up to L3 in the hope that additional instrumentation level would provide greater

stability. Postoperatively, the patient did well and was discharged to a rehabilitation facility. Preoperatively, the patient underwent transarterial embolization of the lesion. The patient recovered well after the operation and reported improved left lower-extremity strength. She stopped using a cane for ambulation. She was referred for postoperative stereotactic radiosurgery (intensity-modulated radiation therapy) after adequate wound healing. Because of personal reasons, the patient underwent the planned intensity modulated radiotherapy at a significantly later date. She received a total dose of 3,000 cGy in 10 fractions during a 2-week treatment course, which she completed 1 year after the second operation. On follow-up, she was ambulating without assistance and had only minimal back pain. A follow-up CT scan showed evidence of posterolateral arthrodesis (Fig. 4B). Repeat imaging studies did not reveal growth of the lesion.

Discussion Paragangliomas of the spine Paragangliomas of the spine comprise a multifaceted body of tumors. Although most spinal tumors have a strong predilection for either intradural or extradural locations, paragangliomas may be found on either side of the dura. They may originate in the spine or metastasize there from a remote location. This mutable nature of paragangliomas may be explained by their cell of origin. Paragangliomas arise from neuroendocrine cells found in the adrenal medulla and in the diffuse extra-adrenal paraganglion system. Some of the more commonly discussed members of the diffuse paraganglion system include the organ of Zuckerkandl; jugular ganglion; ganglion nodosum; and intracarotid, jugulotympanic, coronary, aorticopulmonary, and urinary bladder paraganglia. All of these paraganglia may give rise to paragangliomas [3]. In the central nervous system, primary or metastatic paragangliomas were reported in the cavernous sinus, pineal gland, sella turcica, petrous ridge, and cerebellopontine angle [3], but the most common location is the region of filum terminale and cauda equina [20]. Controversy surrounds the origin of paragangliomas within the central nervous system because it does not normally contain neuroendocrine organs. It has been suggested that they result from latent paraganglion cells in the cauda equina, but many authors point to the lack of histologic evidence of such cells and propose aberrant cell migration, residual neuroblasts, or ependymal cells as potential culprits [3]. Within the spine, the majority of paragangliomas are intradural extramedullary tumors mostly found in the cauda equina. Over 190 such tumors are reported to date [1–3]. In their review of 77 cases of cauda equina paragangliomas, Aghakhani et al. [1] note that 74 of the tumors are intradural, whereas the remaining 3 have intra- and extradural components. Paragangliomas at this site frequently have

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Fig. 1. MRI of the patient’s lumbosacral spine. (A) Axial T1 imaging revealed a left-sided intaosseous mass with central areas of necrosis. (B) A gadolinium-enhanced image showed heterogeneous enhancement. (C, D) Saggittal images revealed a grade II spondylolisthesis at the L5/S1 junction.

a neuronal component (gangliocytic paraganglioma). Because of their location, cauda equina paraganglioma may be difficult to distinguish from myxopapillary ependymoma, but the former is typically much more vascular. Cauda equina paragangliomas do not ordinarily cause symptoms

by overproduction of catecholamines, with only four such cases reported [5]. Spine paragangliomas may also be extradural. They are largely found in the vertebrae and have been reported in the cervical, thoracic, and lumbar spine [2,4–15]. The majority


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from Sundgren et al. [19] in 1999 of another case of a primary intraosseous sacral paraganglioma that was found in the S1 vertebral body. This patient underwent external radiotherapy (60 Gy) without resection. However, the tumor grew, and two courses of I131 metaiodobenzylguanidine (MIBG) were administered. After this treatment, 5-year follow-up revealed no growth of the tumor. We now add the third reported case of a sacral paraganglioma. Our patient presented with progressive low back pain and radicular symptoms. Her MRI revealed a heterogeneously enhancing mass in the vertebral body of S1. Low back pain is by far the most common presenting symptom of spinal paragangliomas [20]; however, several cases of progressive or traumatic myelopathy were also reported with intravertebral paragangliomas [4,6,7,11]. Differential diagnosis of sacral tumors Fig. 2. A CT scan revealing an osteolytic sacral tumor.

of these lesions appear to metastasize to the spine from numerous locations throughout the body. However, several reports exist of primary intraosseous paragangliomas of the spine. Jindel at al. [6] reported a multifocal intraosseous paraganglioma that originated either in T7 or L3 and went on to metastasize throughout the skeleton. In 2002, Houten et al. [4] reported a case of an intraosseous thoracic paraganglioma that presented with spinal cord compression and metastases to the liver. They cite five more cases of primary thoracic intraosseous paragangliomas. Sacral paragangliomas Paragangliomas in the sacrum are certainly rare, and little is known about their biological behavior. In 1998, Coles et al. [18] reported the first case of a primary sacral intraosseous tumor, found in S3. After preoperative embolization, the tumor was completely enucleated via the posterior approach. At 2-year follow-up, the patient was disease free and did not require postoperative radiation. This was followed by a report

The differential diagnosis of extradural sacral tumors is rather broad, with metastases being the most frequent malignant sacral tumor [21]. Lung, breast, renal, and colorectal carcinomas reach the sacrum via hematogenous route; however, colorectal along with genitourinary cancer may also directly invade the sacrum via contiguous spread. Chordoma is the most common primary malignant sacral tumor, whereas giant cell tumor is the most common benign sacral tumor and the most common sacral tumor in general, representing 70% of all sacral tumors [22]. These are followed by cavernous hemangiomas, osteoid osteoma, osteoblastoma, multiple myeloma, Ewing’s sarcoma, lymphoma, teratoma, aneurysmal bone cyst, chondrosarcoma, fibrosarcoma, and osteosarcoma [16,17,22]. The aforementioned neoplasms arise within the osseous elements of the sacrum, whereas neurofibromas, schwannomas, ependymomas, meningiomas, dermoid, and epidermoid cysts arise within the sacral canal [16,17,22]. Treatment of spinal paragangliomas The recommended treatment for spinal paragangliomas is complete enucleation [23–25]. When removal of the

Fig. 3. (A) Polygonal cells arranged in nests and cords in relation to numerous sinusoidal vascular channels (hematoxylin and eosin stain). (B) Immunostain for synaptophysin labels chief cells (avidin-biotin peroxidase method).

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Fig. 4. (A) AP X-ray after lumboiliac fusion displaying the construct. (B) Follow-up CT showing the extent of fusion.

entire tumor is not possible, radiation treatment is recommended [6,7]. The general recommended dose is 4,000 to 4,500 cGy; however this is largely based on studies done on giant cell tumors and chordomas [6,22]. Gerszten et al. [26] report successful treatment of spinal paragangliomas with single-fraction stereotactic radiosurgery. Iodine131 administration has been shown to be an effective method for controlling malignant paragangliomas [27,28]. Several chemotherapy regimens have also been attempted [29,30]. U-King-Im et al. [7] reported successful therapeutic use of octreotide in a patient with carotid body paraganglioma that metastasized to multiple vertebrae. In the case of our patient, complete enucleation of the tumor would have required complete sacrectomy using an anterior combined with posterior surgical approaches with potentially unacceptable neurological deficits. Given her previous transabdominal procedure for uterine cancer with the presence of extensive scar tissue, the decision was made to decompress the symptomatic nerve roots posteriorly and to treat the lesion with stereotactic radiosurgery.

Conclusions To a very limited literature on sacral intraosseous paragangliomas, we add the third case report. Management of these lesions can be very challenging given their vascular nature and the significant morbidity associated with complete surgical removal. Optimal treatment therefore relies on surgical resection when feasible followed by radiation

and chemotherapy. We believe that paragangliomas should be included in the differential diagnosis of sacral intraosseous tumors.

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