Calvarial metastasis of a paraganglioma

Calvarial Metastasis of a Paraganglioma CASE REPORT AND REVIEW OF THE LITERATURE Ferruh Gezen, M.D.,* Ibrahim M. Ziyal, M.D.,* Alper Baysefer, M.D.,† ¨ zcan C¸iklatekerlioglu, M.D.† Serdar Kahraman, M.D.,† and O *Department of The Abant Izzet Baysal University, Du¨zce School of Medicine, Neurosurgery, Du¨zce, Turkey; and †Department of Neurosurgery, Gu¨lhane Medical School, Ankara, Turkey

¨. Gezen F, Ziyal I, Baysefer A, Kahraman S, C¸iklatekerlioglu O Calvarial metastasis of a paragangloioma: case report and review of the literature. Surg Neurol 2000;53:61– 63. BACKGROUND

Metastasis of a paraganglioma (PRG) to the calvarium is very rare. In this paper, the case of a 25-year-old male with metastasis of a PRG to the frontoparietal bone is described. CASE DESCRIPTION

The patient presented with bulging on the left side of the head, headache, and weight loss. Magnetic resonance imaging (MRI) revealed a mass lesion in the left frontoparietal region that had destroyed both the external and internal table of the bone, extending under the skin and above the dura mater. After a frontoparietal craniotomy the tumor was removed totally. Histopathological examination revealed the “Zellballen,” which are pathognomonic for a PRG. Systemic examination and radiological investigation revealed no primary tumor source. CONCLUSION

Metastasis of a PRG to the calvarium is possible; radical removal of the tumor will provide a cure. © 2000 by Elsevier Science Inc. KEY WORDS

Bone metastasis, cranial metastasis, paraganglioma.

aragangliomas (PRG), also known as chemodectomas, are usually benign tumors of neuroectoderm cells. The neck is the most common location, although some tumors have been found within the abdominal cavity [12–14]. Even though central nervous system involvement is rare, some intrasellar [11,15], and some intraspinal cases in the cauda equina or filum terminale [1,2,16] have been reported.

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Address reprint requests to: Dr. Ferruh Gezen, Barbaros Bulvari, 61/2, Besiktas Istanbul, Turkey. Received May 3, 1994; accepted January 4, 1999. © 2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010

The majority of PRGs are histologically benign and have favorable postoperative outcomes. Local recurrence is possible in up to 50% of those originating from the glomus jugulare, up to 17% of those from vagal bodies and up to 10% of those from the carotid body [9]. Malignancy is manifested by local tissue invasion and distant metastasis to cervical and mediastinal lymph nodes, the lungs, and the bone marrow [9,16]. PRGs may also originate from the temporal bone [4] or skull base [10,18]. However, bony metastasis of a PRG is rare and occurs most often in the spine [3,8,14]. As far as we know, this is the first case report of a PRG with calvarial metastasis in the literature.

Case Report A 25- year-old male presented with bulging on the left side of the head, headache, and weight loss, which had begun 3 months previously. Systemic and neurological examinations and routine laboratory investigations were normal. There was no familial history of a systemic disease or malignancy. Direct x-rays of the calvarium showed destruction of the left frontoparietal bone. Magnetic resonance imaging (MRI) revealed a mass lesion in the left frontoparietal region, with a diameter of 2.5 cm, which had destroyed both the external and internal table of the bone, and extending under the skin and above the dura mater (Figure 1). Cervical, chest, and abdominal examinations, CT scans, and MRI investigations demonstrated no mass lesion or malignancy. A frontoparietal craniotomy was performed. The tumor was not invading the dura and was well vascularized. It was removed totally. 0090-3019/00/$–see front matter PII S0090-3019(99)00200-1

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T1 weighted coronal (A) and sagittal (B) MR images after gadolinium–DTPA injection reveals a mass in the left frontoparietal region that destroyed the external and internal table, bulging under the skin and compressing the dura.

The tumor was grayish-white in color, highly vascularized, and mixed with lamellar bone. Histopathological examination revealed that the tumor tissue consisted of clusters of chief cells surrounded by sustentacular cells arranged in socalled “Zellballen,” which is pathognomonic for PRG. The tumor cells had abundant pale cytoplasm and vesicular nuclei; some cells showed prominent pleomorphism (Figure 2). The patient did well after the operation. Postoperative clinical and radiological examinations of the whole body showed no primary source for the tumor. Follow-up MRI examination 6 months, 1, 2, and 4 years later, and systemic examinations also re-

Histopathological section of lamellar bone infiltrated by paraganglioma. Clusters of chief cells are visible, surrounded by sustentacular cells arranged in so-called “Zellballen” (Hematoxylin-eosin ⫻ 100).

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vealed no recurrence of the tumor, and no additional disease or malignancy.

Discussion The broad group of neoplasms under the term “neuroendocrine tumors” are classified in three main groups: pheochromocytomas originating from the adrenal medulla; sympathetic PRGs originating from neuroendocrine cells associated with the sympathetic chain; and parasympathetic PRGs, synonymous with chemodectomas, originating from similar cells that have parasympathetic innervation and chemoreceptor function [7]. The majority of these tumors are benign, hormonally nonfunctioning, and have favorable postoperative outcomes. Parasympathetic PRGs are usually nonchromaffin, secrete small amounts of biogenic amines, and are rarely physiologically active [7]. Some cases may have a familial background. There may be loss of heterozygosity predominantly at the chromosome 11q22-q23 region, where the disease-causing gene PGL1 has been identified by linkage analysis [17]. The tumor may originate from anywhere a parasympathetic ganglia can be found [6,9]. Despite their different locations in the body, they are morphologically similar. They are characterized by a uniform organoid growth of cells with argyrophilic and ultrastructurally demonstrable dense core granules. The cells are arranged in a classical “Zellballen” pattern [17,18]. There are several reports of primary central ner-

Calvarial Metastasis of Paraganglioma

vous system involvement of PRGs in the columna vertebralis, cauda equina, or filum terminale [1,2]. Metastasis of these tumors is not common. Of 71 patients with PRGs of the head or the neck, only one patient with a carotid body PRG had an intracranial extention 5 years after the surgery [9]. There has been one case report of intravagal PRG metastasis to a regional lymph node [5]. A case of thoracic spinal cord metastasis has been also reported [6]. A few cases of bony involvement have been reported [3,8,14]; however, we found no reports of calvarial metastasis in the literature. Our case is the first report of a PRG metastasizing to the calvarium. He had no familial history of any disease or malignancy. During preoperative evaluation of the patient and also during 5-year clinical and radiological follow-up examinations, no other malignancy or disease in any location could be found. No recurrence of the calvarial tumor was observed during a 5-year follow-up. We thank Gu¨zin Evren, M.D. for preparation and evaluation of histopathological sections and Altay Bedu¨k, M.D. for his assistance.

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COMMENTARY

Paragangliomas are rare lesions that are presumed to arise from the extra-adrenal ganglionic system. They may be chemically active and produce noradrenalin. These tumors are not often encountered by neurosurgeons, for not only are they rare, but involvement of the CNS is highly unusual. In this case, the tumor was found in the frontoparietal cranium and thus reached neurosurgical attention. The authors describe the classification of these tumors and the classic “Zelballen” arrangement of cells that serve to characterize these lesions. An interesting aspect of this case is the invasion of bone, a mesenchymal tissue, by paraganglionic cells, presumably of neurectodermal origin. This invasion of tissues of different origin is in itself unusual. Whereas meningiomas commonly invade bone, even the most malignant glioblastomas tend not to invade the cranial vault. This specificity of invading cells to different sites is of great interest and this case raises the question as to why this particular tumor was able to grow in the cranium. Paul L. Kornblith, M.D. Neurosurgeon Pittsburgh, Pennsylvania