Abstract:Paragangliomas are very occasionally located in the nasal cavity. In this article, we present a 32-year-old female case with a sinonasal paraganglioma with atypical histopathological morphology. In our case, sinonasal paraganglioma consisted of lipoblast-like vacuolated cells instead of the typical Zellballan histological pattern. The prognosis of a nasal paraganglioma is not very clear. The present article is important, as it describes the first case report of a sinonasal paraganglioma with atypical lipoblast… Show more
“…Other markers are Synaptophysin, neuron specific Enolase and S-100 protein, the last one stains the peripheral sustentacular cells. 9 Chromogranin was negative in our case as extra-adrenal paragangliomas are usually non-functional.…”
“…Other markers are Synaptophysin, neuron specific Enolase and S-100 protein, the last one stains the peripheral sustentacular cells. 9 Chromogranin was negative in our case as extra-adrenal paragangliomas are usually non-functional.…”
“…This pattern was observed in our case, but an uncommon morphology was described with lipoblast-vacuolated cells instead of the typical pattern [23]. Other variations in the tumor architecture include diffuse and trabecular growth patterns, extensive sclerosis that may mimic invasive carcinoma, and unusual vascular patterns that can mimic angiomas [51].…”
Section: Discussionmentioning
confidence: 82%
“…These are all hypervascular tumors, with contrast enhancement, presenting the classic “salt-and-pepper” appearance and variable patterns of intensity on MRI [5,10,20,21,22,23,24,25,26,27,28,29,30,31,32]. …”
Paragangliomas can be found from the skull base to the sacrum. Sinonasal paragangliomas are infrequent. A 16-year-old female reported spontaneous discrete bilateral epistaxis once a month beginning when she was 3 years of age. Computed tomography showed an expansive hypervascular mass occupying the right nasal cavity and nasopharynx. Sinonasal paragangliomas usually occur in middle-aged women. Radiologic investigation is essential for the diagnosis of sinonasal paragangliomas and evaluating extension of the lesion. Endoscopic and conventional approaches are effective, and preoperative embolization is paramount for reducing bleeding risk. Histopathological features cannot differentiate benign from malignant paragangliomas, and since metastasis may eventually occur, follow-up must be carried out for a long period of time.
“…However, atypical morphology may be encountered. [11] This variability, however, creates a challenge for both treating physician and histopathologists alike, especially if there is an association with systemic disease, i.e. Cushing's syndrome or Addison's disease, as seen in our case.…”
Paraganglioma of the nasal and paranasal sinuses are quite rare neuroendocrine tumours, usually hormonally inactive, presenting as nasal polyps with or without epistaxis. We present a rare case of a 23-year old male, with Addison's disease, who was referred from endocrinology department with a complaint of right nasal blockage associated with recurrent epistaxis. A provisional clinical diagnosis of vascular tumour was made, computed tomography (CT) and magnetic resonant imaging (MRI) suggested hemangioma/inverted papilloma. However, biopsy reported a highly vascular tissue mimicking angiofibroma, further immunostaining studies with Synaptophysin and S-100 confirmed the diagnosis of paraganglioma. This rare tumour in the paranasal sinuses may mimic vascular tumour and can only be confirmed with histopathological immunostains studies. According to our knowledge, this is first case reported to be associated with Addison's disease in the English literature.
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