Paragangliomas Treated at
the Clinic for ENT and Surgery of Head and Neck of the First Medical Faculty Charles University
in 1990-2000
Salač V., Betka J., Zvěřina E., Taudy M., Skřivan J., Belšan T.*, Kodet R.**
Klinika otorinolaryngologie a chirurgie hlavy a krku 1. LF UK a FN Motol, Praha, katedra otorinolaryngologie IPVZ, Praha, přednosta prof. MUDr. J. Betka, DrSc. Klinika zobrazovacích metod 2. LF UK, Praha, přednosta doc. MUDr. J. Neuwirth, CSc.* Klinika patologické anatomie 2. LF UK, Praha, přednosta prof. MUDr. R. Kodet, CSc.** |
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Summary:
Paragangliomas or chemodectomas are according to their origin classified as
neuroectodermal tumours. As to their basis and structure they are near to ganglioneuromas which
is differentiated near to the structure of the adrenal medulla. In the ENT area, contrary to classical
pheochromocytoma, we encounter the achromaffine extraadrenal variant which develops from
tissue of sympathetic and parasympathetic ganglia. In the area of head and neck evidence of pressor
substances produced by tumours is rare in the same may as malignant variants of these tumours.
Macroscopically glomus tumours can be characterized as encapsulated, lobular, redish to violet
coloured formations depending on the vascularization of the tumour. Microscopically similarly as
in paraganglia there are two types of cells, the main (eptheloid) - type I and sustentacular - type II.
The basic structural unit is a defined agglomeration of main cells which is called Zellballen. The
extraadrenal variant of the tumour is characterized in addition to inadequate argentaffinity by
a good and sharply defined separation of alveolar tumourous foci connective tissue strips with wide
sinusoid capillaries. Only rarely it is possible to observe a periteliomatous pattern. In the microscopic structure of these tumours participates the axial ending of a nonmyelinized nerve immersed
into cells of the Schwann type. When investigating the incidence of glomus tumours within the
framework of genealogical studies a certain hereditary relationship was found whereby the prob-
able transfer between different generations is autosomally dominant. There is also an increased
predisposition of the incidence of paragangliomas in women and an increased incidence (up to 50%)
of multiple incidence of tumours in different affected members of the family, while in patients
without a family load of the basic disease the multifocal incidence is slightly lower, ca by 10%. The
authors observed also a tendency of the multiple incidence of these tumours in conjunction with
advancing age of the patients and a tendency of a possibly higher incidence of paragangliomas in
children of affected men. Genetic predisposition of familial glomus depends on one allele. The most
frequent familial type of chemodectoma is the glomus caroticum tumour (up to 80%). A typical
manifestation of paragangliomas is slow progression of growth. The first clinical signs of these
neoplasias are usually observed several years after development of the tumour. Only about 3% of
these cases can be considered malignant, in particular because of metastases. These tumours are
known by their very slow locoregional spread.
The authors present a group of patients examined and treated at a clinical ENT department in the
course of 10 years on account of the diagnosis of paraganglioma. The group comprises a total of 15
patients, incl. 11 women (73.3%) and 4 men (20.7%). The average age of the operated patients was
37.5 years, range 20 tp 73 years. As to sites, tympanojugular chemodectomas were found in 33.3%,
a glomus vagale tumour in five instances (26.7%), a paraganglioma of the carotid sinus was proved
in five instances (33.3%) and in one case the finding was described as a metastasis of a paraganglioma which was originally in the aortic arch and which was already previously treated at a surgical
department. In two patients (13.3%) anamnestically multiple incidence of paragangliomas was
described incl. one female patient who was treated previously at another department on account of
chemodectoma at another site, primarily by radiotherapy with two relapses in this area which were
subsequently treated by a combination of surgery embolization and radiotherapy. In another
patient in the past repeatedly surgery was used at the same site for of chemodectoma, in another
department. However ther was multicentric incidence, i.e. first a glomus vagale tumour and later
a carotid paraganglioma which was confirmed on operation and histological examination. The
preoperative diagnosis of glomus tumours was based on a detailed case-history, clinical picture and
imaging methods. In the cervical region ultrasonography was used, digital subtraction angiography
twice supplemented by CT examination. In the area of the cranial base always digital subtraction
angiography was used supplemented by CT or possibly MR examination. In three patients with
suspected endocrine activity catecholamine breakdown products were examined in urine and in
one case raised levels were found in a female patient with netastases of an aortal paraganglioma in
the cervical region. All patients were treated primarily by radical removal of the tumour.
After surgery in none of the patients actinotherapy was indicated nor treatment with Leksell’s
gammaknife. In conjunction with surgery no inflammatory complications were observed in the area
of the operational field. Postoperative clinical complications involved discontinuation of anatomical structures which the tumour infiltrated and which had to be removed to achieve a primary
radical operation. The follow-up in the investigated patients is on average 2.8 years with a range of
7 months to 10 years and during the follow-up period no relapse of the basic disease was observed
in conjunction with the administered treatment.
Key words:
paragangliomas, surgical treatment, complications, Leksell’s gammaknife, actinotherapy.
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