2013 February; 2(2): 50–52. ISSN: 2240-2954
Published online 2014 April 2.

Carotid body paraganglioma: early diagnosis

Raffaele Capoano, Maria Chiara Tesori, Giada Lacroce, Karen Llange, Erminio Mastroluca, Andrea Police, Anna Rita Forcione, and Bruno Salvati

Department of Surgical Sciences, “Sapienza” University of Rome, Italy

Corresponding Author: Raffaele Capoano, Department of Surgical Sciences, “Sapienza” University of Rome, Italy, E-mail: raffaele.capoano@uniroma1.it

Summary

Background:
paragangliomas (PGLs) are rare neuroendocrine tumors which arise from the extraadrenal chromaffin tissue of the autonomous nervous system, located in the carotid body or glomus (CB). This is a little corpuscle localized at carotid bifurcation; it contributes to the regulation of blood pressure, cardiac and respiratory frequency by giving information to the nervous system bodies located in the brain stem. Istologically, PGLs remind of the CB normal architecture, with clusters cells known as Zellballen, highly vascularized. Rarely secreting vasoactive amines, they come more often to clinical observation as a mass located at the mandibular angle, asymptomatic. Based on the size and connections with the surrounding vascular structures, they are divided into three groups according to the Shamblin classification.

Case report:
57-year-old patient, smoker. He reports about a year before, presence of vertigo associated with cold sweat and general malaise that lasts about 20 min resolved spontaneously (blood pressure in association with these episodes is high with maximum values recorded around 180/110 mmHg). He is submitted to a Holter blood pressure investigation, ECD of the neck vessels (TSA), then it was performed a neck MRI. The patient underwent surgery. The final histological examination showed a “paraganglioma composed of nests of chromogranin positive cells with round nuclei and finely dispersed chromatin with abundant eosinophilic, granular cytoplasmic portion”.

Discussion and Conclusions:
the PGLs are rare neoplasms, with an incidence of 0.012%. Very rarely (5% of cases) the patients come to medical attention for the appearance of a “pressor amine syndrome” with tachycardia, palpitations, flushing, hypertensive crisis, dizziness, malaise, diarrhea, amino-linked activity consequences of PGLs secreting. According to the Shamblin classification are divided into three groups based on the size and relationships contracted with the surrounding vascular structures (group III belongs to the larger and it forms persistently adhesions to the carotid bifurcation). In most cases, the patients having a PGLs come to medical attention for the appearance of a mass in the lateral region of the neck, at the mandibular angle, often completely asymptomatic. When the tumor reaches considerable size, it may be responsible for nerve deficits (especially for X, XI and XII nerves), dysphagia, neck pain. Early diagnosis of PLGs asymptomatic and small reduces the risk of perioperative lesions of the cranial nerves and carotid arteries. In our case, early identification was made possible by the biological and functional characteristics of the tumor and the consequent clinical manifestations, allowing to carry out the surgery safely for the patient. Despite the size of PLG taken into account, it was ranked to the Shamblin group I.

Keywords: neuroendocrine tumors, paraganglioma, carotid

Background

Paragangliomas (PGLs) are rare neuroendocrine tumors which arise from the extraadrenal chromaffin tissue of the autonomous nervous system, located in the carotid body or glomus (CB). This is a little corpuscle localized at carotid bifurcation; it contributes to regulating blood pressure, cardiac and respiratory frequency by giving information to the nervous system bodies located in the brain stem. Istologically, PGLs remind of the CB normal architecture, with clusters cells known as Zellballen, highly vascularized. Rarely secreting vasoactive amines, they come more often to clinical observation as a mass located at the mandibular angle, asymptomatic. Even more rarely they behave as malignant lesions giving distant metastases. Based on the size and relationship with the surrounding vascular structures, are divided into three groups according to the Shamblin classification (1) (Tab. 1).

Table 1Table 1
Shamblin Classification.

Case Report

57-year-old patient, smoker. Reports about a year before the presence of vertigo associated with cold sweat and general malaise that lasts about 20 min resolved spontaneously (blood pressure in association with these episodes is high with maximum values recorded around 180/110 mmHg). He was submitted to a Holter blood pressure investigation, which documents “mean daytime systolic and in 24 hours values more than referenced ones”, and ECD of the neck vessels (TSA) highlighting “at the right carotid bifurcation presence of nodularity, size 27mm X 17mm”. Then a neck MRI was performed which “confirms the presence of nodularity at the carotid bifurcation of the size of 27mm X 20mm (Fig. 1), with moderate post-contrast enhancement and that displaces the external and internal carotid with no stenosis; this nodularity is also not infiltrating the surrounding tissues” (Fig. 2). The patient underwent surgery. The surgery, conducted in loco-regional anesthesia, consisted of the cleavage of the “nodularity” from the carotid bifurcation after hemostasis with metal clips of glomus vessels and removal of this nodularity, in its entirety, without the need for clamping the carotid arteries. The final histological examination showed a “paraganglioma composed of nests of chromogranin positive cells with round nuclei and finely dispersed chromatin with abundant eosinophilic, granular cytoplasmic portion” (Fig. 3). In the one year follow-up the patient has no longer presented episodes of vertigo or general asthenia, and a new Holter blood pressure investigation documented the normalization of blood pressure both day and night average values, while the control of TSA Doppler ultrasound showed no evidence of disease recurrence.

Figure 1Figure 1
Right glomus tumor: long and transverse sections without contrast.
Figure 2Figure 2
Right glomus tumor: long and transverse sections with contrast.
Figure 3Figure 3
Paraganglioma.

Discussion

PGLs are neuroendocrine structures located in the adventitia carotid bifurcation, posterior-medial side. They are sensitive to hypoxia, acidosis and hypercapnia; their stimulation results in increased frequency and respiratory rate and a rise in blood pressure through vasoconstriction and the production of circulating catecholamines. The PGLs are rare neoplasms, with an incidence of 0.012% (2). They generally occur between the fourth and fifth decade of life. The average size is of 4.5 cm × 3.5 cm × 3 cm but can grow up to 15 cm and a weight of 200 g. According to the Shamblin classification they are divided into three groups based on the size and connection with the surrounding vascular structures (group III belongs to the larger and forms persistently adherent to the carotid bifurcation). In most cases, the patients having a PGLs come to medical attention for the appearance of a mass in the lateral region of the neck, at the mandibular angle, often completely asymptomatic. When the tumor reaches considerable size, it may be responsible for nerve deficits (especially for X, XI and XII nerves), dysphagia, neck pain (3). Very rarely (5% of cases) the patients come to medical attention for the appearance of a “pressor amine syndrome” with tachycardia, palpitations, flushing, hypertensive crisis, dizziness, malaise, diarrhea, amino-linked activity consequences of PGLs secreting (4). The biological behavior of PLGs, characterized by their tendency to grow and gradually incorporate the surrounding vascular and nerve structures, justifies their complete removal whenever they are identified (5, 6).

Conclusions

Early diagnosis of PLGs asymptomatic and small, reduces the risk of perioperative lesions of the cranial nerves and carotid arteries (2). Unfortunately, most of the PLGs, when identified, belong to group II or III Shamblin, making the operation much more difficult (7). In our case, early identification was made possible by the biological and functional characteristics of the tumor and the consequent clinical manifestations, allowing to carry out the surgery safely for the patient. Despite the size of PLG taken into account, it was ranked to the Shamblin group I.

References
1.
Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr. Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg. 1971;122(6):732–739.
2.
Grotemeyer D, Loghmanieh SM, Pourhassan S, et al. Dignity of carotid body tumors. Review of the literature and clinical experiences. Chirurg. 2009;80(9):854–863.
3.
Padberg FT Jr, Cady B, Persson AV. Carotid body tumor. The Lahey Clinic experience. Am J Surg. 1983;145(4):526–528.
4.
Pellitteri PK, Rinaldo A, Myssiorek D, et al. Paragangliomas of the head and neck. Oral Oncol. 2004;40(6):563–575.
5.
Krupski WC, Effeney DJ, Ehrenfeld WK, Stoney RJ. Cervical chemodectoma. Technical considerations and management options. Am J Surg. 1982;144(2):215–220.
6.
Lim JY, Kim J, Kim SH, et al. Surgical treatment of carotid body paragangliomas: outcomes and complications according to the shamblin classification. Clin Exp Otorhinolaryngol. 2010;3(2):91–95.
7.
Chapman DB, Lippert D, Geer C, et al. Clinical, histopathologic, and radiographic indicators of malignancy in head and neck paragangliomas. Otolaryngol Head Neck Surg. 2010;143(4):531–537.