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Review
. 2009 Apr;29(2):97-102.

Primary paraganglioma of thyroid gland: a clinicopathologic and immunohistochemical study with review of the literature

E Ferri  1 , R Manconi, E Armato, F Ianniello
Affiliations
Review

Primary paraganglioma of thyroid gland: a clinicopathologic and immunohistochemical study with review of the literature

E Ferri et al. Acta Otorhinolaryngol Ital. 2009 Apr.

Abstract
in English, Italian

Primary paraganglioma of the thyroid is a very rare neuroendocrine tumour. Only 24 cases have been reported in the Literature. A case of a primary paraganglioma of the thyroid is presented in order to provide a review of the Literature, an update on current knowledge and to emphasize the key diagnostic role of immunohistochemistry. A 63-year-old female presented with a 6-month history of right-sided solitary thyroid nodule. Ultrasonography and fine needle aspiration biopsy were not diagnostic. The patient underwent right hemithyroidectomy. The tumour cells showed a strongly positive staining for chromogranin A, synaptophysin and neuron specific enolase, whereas S-100 protein was positive in sustentacular cells. A diagnosis of primary paraganglioma of the thyroid was made. Radiotherapy for suspected local tumour persistence was planned. At 18-months follow-up, the patient is alive without evidence of recurrence. This case highlights the need to include primary paraganglioma of the thyroid in the differential diagnosis of neuroendocrine intra-thyroidal tumours. Immunohistochemistry is essential for diagnosis. Surgery is the treatment of choice.

Il paraganglioma primitivo della tiroide (PTPG) è un rarissimo tumore neuroendocrino. In Letteratura ne sono stati descritti appena 24 casi. Questo caso è stato presentato al fine di evidenziare il ruolo chiave dell’immunoistochimica nella fase diagnostica ed al fine di realizzare una revisione della Letteratura e un aggiornamento sulle attuali conoscenze. La paziente è una donna di 63 anni, giunta alla nostra osservazione in seguito alla comparsa, da circa 6 mesi, di un nodulo solitario a carico dell’emitiroide destra. L’ecografia e l’ago-aspirato risultarono non dirimenti ai fini diagnostici. La paziente fu sottoposta ad un’emitiroidectomia destra. Lo studio immunoistochimico evidenziò una marcata positività delle cellule tumorali per la cromogranina A, la sinaptofisina e l’enolasi neurono-specifica, mentre le cellule di sostegno risultarono positive per la proteina S-100. Tale indagine permise di formulare la diagnosi di PTPG. La paziente fu sottoposta ad un ciclo di radioterapia post-operatoria nel sospetto di una persistenza locale di malattia. A 18 mesi dall’intervento, non sono state evidenziate recidive. In conclusione, il PTPG va incluso nella diagnosi differenziale dei tumori tiroidei. L’immunoistochimica è fondamentale ai fini diagnostici. La chirurgia rappresenta il trattamento di scelta.

Keywords: Differential diagnosis; Neck mass; Neuroendocrine tumours; Paraganglioma; Thyroid tumours.

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Figures

Fig. 1
Fig. 1
Tumour exhibits variably sized nests in a vascularized stroma (Haematoxylin and Eosin, [H&E], X10, orig. magn.).
Fig. 2
Fig. 2
Cellular detail showing moderately pleomorphic tumour cells embraced by sustentacular cells (H&E, X40, orig. magn.).
Fig. 3
Fig. 3
A dendritic pattern is focally evident with S-100 protein immuno-staining due to presence of sustentacular cells (X20, orig. magn.).
See this image and copyright information in PMC

References

    1. Buss DH, Marshall RB, Baird FG, Myers RT. Paraganglioma of the thyroid gland. Am J Surg Pathol 1980;4:589-93. - PubMed
    1. Lack EE, Cubilla AL, Woodruff JM, Farr HW. Paraganglioma of the head and neck region: a clinical study of 69 patients. Cancer 1977;39:397-409. - PubMed
    1. Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-7. - PubMed
    1. Kliwer KE, Wen DR, Cancilla PA, Cochran AJ. Paragangliomas: assessment of prognostic by histologic, immunohistochemical and ultrastructural techniques. Hum Pathol 1989;20:29-39. - PubMed
    1. Van Miert PJ. The treatment of chemodectomas by radiotherapy. Proc R Soc Med 1964;57:946-51. - PubMed