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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

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