Carcinoid call-point gGmbH

Zahnärztliche Mitteilungen ot 6.3.2010
www.zm-online.de

Nyeĭroendokrinnyh opuholyeĭ golovy i shyei
regione glomusnaya opuholʹ v Carotisbifurkation

1. August 2007 - A 74-year-old patient was assigned to the surgical removal of a mass in the Carotis bifurcation. Reported a history of the patient on blood pressure crises with nausea and vertigo attacks and the occurrence of syncope. These phenomena were first appeared suddenly two years ago and has since been observed in ever shorter intervals. The doctor was mediozervikal on the clinical examination of the very thin patients, swelling, and he led a first imaging by MRI. Here was a first indication of a tumor located in the carotid bifurcation, and the patient was assigned to further treatment.

		Figure 1: sonography of the neck in two planes. Directly in the carotid bifurcation (white asterisk marks the common carotid artery) can be found by projection on the external carotid, a spherical mass with well-defined relatively homogeneous within textures.
		Figure 2: Magnetic Resonance Imaging (A) with contrast administration (B) after reconstruction of the vascular conditions. There comes a host muskelintense contrast, well-defined mass (A) for the representation, which fork to the carotid fork and projected these plumes (B).
		Figure 3: Intraoperative situs. According to the vessels in the carotid triangle, there is a well-defined, the external carotid adherent mass.
		Figure 4: Surgical resected. The resected tissue shows a spherical tumor with a thin connective tissue capsule, the incision in the very inhomogeneous, sometimes dark red to brown or bright impressed with the internal parts.

At the initial examination was mediozervikal a small, firm mass recognizable, which could immediately recognize the pulsations of the carotid artery. The further clinical examination findings were unremarkable, in particular, no pathologically enlarged lymph nodes were present. Sonography was found in close proximity to a roundish Carotisbifurkation relationship to tissue proliferation of about 1.5 centimeters in diameter (Figure 1). Preoperatively, the relationship was added to the vascular system through an advanced imaging by magnetic resonance imaging with contrast administration. This also was a clearly demarcated, contrast enhancing mass within the bifurcation of the presentation, which was superposed the external carotid and the spreading of the fork, leading (Figure 2). Therapeutically, the resection of the tumor from the carotid bifurcation (Figure 3) was carried out, whereby, according to the preoperative imaging, left the vessel walls separating the A. carotis dissection with a low burden of tumor. Figure 4 shows the bearing of a capsule Resektionsbefund, impressed at the gate by some dark, fat and some bright areas. Postoperatively, no fresh blood crises have occurred more and since then the patient is stable under a typical anti-hypertensive medications and remained recurrence.

Figure 5: Histopathology

In the HE staining (A: original magnification 200x) to show the typical structure of the glomus tumor of two cell populations, so that the impression formed cell nests in a reticular stroma.	The characteristic "cell balls" come with large epithelioid cells in the enlarged (B: original magnification 400x) for access.
The central type I cells show in immunohistological staining significantly Expression of synaptophysin (C: original magnification 400x)	whereas the peripheral type II cells (Expressing D: original magnification 400x) S-100. The histological specimens were fixed by Dr. Hansen, Institute of Pathology, Johannes Gutenberg University (Director: Univ. Professor Kirkpatrick) made available.

In the histologic evaluation of resected tissue (Bleed: Figure 4) was in the HE staining, the dual of a typical glomus tumor cell population to display. These are the epithelioid cells in so-called "cell balls" arranged type 1 cells, which can be identified in the HE staining than large, epithelioid cells with eosinophilic, slightly granular cytoplasm are, and in the periphery of this "cell balls" arranged like a net population of type II cells, called supporting cells. Immunohistochemistry reveals the significant differences between the two cell types. Type I cells express, among other things, and synaptophysin are negative are in the S-100 staining, type II cells show a strong positive reaction to S-100, whereas synaptophysin is not expressed (Figure 5).

Discussion
"Glomus tumors" of the carotid glomus or glomus jugulare be combined with other sympathetic and parasympathetic paraganglioma of the trunk and head and neck region by the WHO, the so-called "paraganglionären tumors of the system" together [Barnes et al., 2005].

In the head and neck area, the glomus tumors represent only about 0.6 percent of neoplasms is a rare tumor entity, which by the paraganglionären glomus cells of the carotid glomus, N. vagus, the middle ear or jugular foramen proceed. Of these, the localization in the area of carotid body and glomus jugulare with 80 percent of most common [is Axmann et al., 2004]. The endocrine activity with Katecholaminausschüttung and consecutive cardiovascular response that was seminal in the present case the diagnosis is in glomus tumors of the head and neck region, otherwise relatively rare (4 percent). Therefore, most of the regional glomus tumors are discovered as incidental findings on clinical examination or by imaging techniques [Young, 2006]. Due to the good visualization of the cervical vascular sheath, most glomus tumors in ultrasound diagnostics are displayed. In magnetic resonance imaging muskelisointens they are presented with a strong enhancement of contrast medium administration [Axmann et al., 2004].

From the biological behavior of glomus tumors are usually benign and slow-growing tumors of adulthood with a peak incidence between the fourth and fifth decade of life and a preference for the female sex (2.7 to 5:1) [Tali et al., 1991]. Clinically, the patients fall on depending on the location and size of the lesion by different symptoms.

While small, endocrine inactive glomus tumors are usually clinically completely inconspicuous, may be associated mediozervikale, large tumors with swallowing, hoarseness, and stridor. Neuroendocrine tumors can not be active by influencing the current path and pressure on the carotid glomus lead with his Presso-and chemoreceptors can tilt to dizziness and syncope. Glomus jugular and tympanic glomus tumors can also cause dizziness and can also lead to tinnitus [Young, 2006]. While it was described for the cervical glomus tumors an association Hypoxemic states, for example, in chronic obstructive pulmonary disease or a long stay at high altitudes, there is an accumulation of peripheral hereditary paragangliomas, for example, in neurofibromatosis or multiple endocrine neoplasia (MEN), so that in case of suspicion the search for the presence of additional tumors and genetic screening may be indicated [Young, 2006].

The curative treatment of glomus tumors is, as in this case, the surgical resection. For locally advanced tumors or high medical risk factors using a radiation therapy can stop the growth and possibly to achieve a partial remission [Axmann et al., 2004].

With the development of selective catheter techniques, the embolization alone or in combination with surgical procedures has become increasingly important [Gjuric et al., 1997].

Only about 2 to 13 percent of the glomus tumors display a malignant phenotype with metastatic disease, with the malignant clinical behavior is not yet possible to predict on the basis of classical histological parameters. In this respect are also shown for initially well-defined, clinically benign appearing tumors long-term follow-up examinations.

For dentists, this case indicates that can manifest neoplasms of the head and neck region, in rare cases not only have the classic signs of tissue proliferation, but also about paraneoplastic phenomena that include, among other things, an endocrine activity of catecholamine secretion counts.

Dr. Maximilian Moergel
Prof. Dr. Dr. Martin Kunkel
Department of Oral and Maxillofacial Surgery
Hospital of the Johannes Gutenberg University
Augustusplatz 2
55131 Mainz
; Этот e-mail адрес защищен от спам-ботов, для его просмотра у Вас должен быть включен Javascript

Conclusion for practice:

• Although glomus tumors are rare tumors of the head and neck region, but can blood pressure crisis due to their proximity to vascular structures or through a neuroendocrine activity, vertigo, tinnitus, or even cause syncope.
• In most cases the clinical symptoms of glomus tumors is low. Often these are incidental findings, for example, under the diagnosis of a thyroid disease.
• Although a malignant phenotype in glomus tumors very rare, is a known occurrence of relapse even after years, so that the patients should be cared for in a long-term follow-up concept.
• Not all tumors of the head and neck region, manifesting itself primarily on the clinical signs of tissue proliferation, including paraneoplastic phenomena may provide important clues for tumor detection.

We thank the editors and the ZM "German - Publishing company" for the release for publication!