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CORRESPONDENCE
Year : 2018  |  Volume : 14  |  Issue : 10  |  Page : 812-814

Sinonasal paraganglioma and Cushing's syndrome: A rare association


Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Web Publication24-Sep-2018

Correspondence Address:
Puneeth Kumar
Department of Radiology, Seth GS Medical College and KEM Hospital, Room No. 107, Main Boys Hostel, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.187354

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 > Abstract 


In general, paragangliomas are symptomatic tumors, which may be clinically taken for other tumors, benign or malignant lesions. Paragangliomas of the nasal cavity and paranasal sinuses are an extremely rare entity and what is even rarer is its association with ectopic adrenocorticotropic hormone production. We report this very rare case to highlight the rare association of Cushing's syndrome with nasal paraganglioma and the importance of total surgical resection in its treatment.

Keywords: Cushing, nasal, paraganglioma, resection


How to cite this article:
Kumar P, Dey AK, Mittal K, Sharma R, Badhe P, Kale S. Sinonasal paraganglioma and Cushing's syndrome: A rare association. J Can Res Ther 2018;14, Suppl S3:812-4

How to cite this URL:
Kumar P, Dey AK, Mittal K, Sharma R, Badhe P, Kale S. Sinonasal paraganglioma and Cushing's syndrome: A rare association. J Can Res Ther [serial online] 2018 [cited 2020 Oct 27];14:812-4. Available from: https://www.cancerjournal.net/text.asp?2018/14/10/812/187354




 > Introduction Top


Head and neck paragangliomas are very rare, with an incidence of 0.0012%.[1] Most of them are benign but can lead to hormone production ectopically which includes Cushing's syndrome (adrenocorticotropic hormone [ACTH]), hypercalcemia (parathyroid-related peptide), inappropriate secretion of antidiuretic hormone syndrome (antidiuretic hormone), diarrhea (intestinal vasoactive peptide), and acromegaly (growth hormone).[2] We report this very rare case to highlight the rare association of Cushing's syndrome with nasal paraganglioma and the importance of total surgical resection in its treatment.


 > Case Report Top


A 46-year-old female presented with leg edema and recurrent episodes of epistaxis. On physical examination, the patient appeared cushingoid, with hyperpigmentation and facial rounding, purple striae on the abdomen and central obesity. There was mildly elevated blood pressure. Hyperglycemia >190 mg/dl and increased white blood cell count were reported, and so Cushing's syndrome was suspected. Laboratory investigations were ordered. The blood results revealed highly elevated ACTH and cortisol levels (ACTH, 312.0 pg/ml; AM cortisol, 94.6 μg/dl; and 24-h urine cortisol 1650.4 μg/24 h total volume). In the presence of very high ACTH, a pituitary tumor was suspected. She also had some difficulty in vision and also had complaints of headache besides the main complaint of recurrent epistaxis. There were no other constitutional symptoms. There is no significant personal history. In the meantime, the patient was started on ketoconazole.

Paranasal computed tomography (CT) revealed a soft tissue mass filling right frontal sinus, ethmoid sinus, and anterosuperior nasal cavity. Magnetic resonance imaging was done which showed an enhancing expansile T2 isointense soft tissue mass with salt and pepper appearance seen involving the right frontal sinus, ethmoid sinus, and anterosuperior nasal cavity and extending up to subcutaneous plane causing bulging of subcutaneous plane. The mass is extending into anterior cranial fossa. Extension into medial and superior aspect of the right orbit is also noted with avid contrast enhancement on postcontrast studies [Figure 1] and [Figure 2]. CT scan of the chest and abdomen was unremarkable except for hyperplasia of the adrenals. Diagnosis of ectopic ACTH was made in lieu of the mass, and biopsy of the mass was performed which showed focal expression of ACTH and chromogranin A. A diagnosis of sinonasal paraganglioma with ectopic ACTH was made. Patient was sent for surgery and is doing remarkably well on 6 monthly follow-up. On follow-up, the patient had normal ACTH and cortisol levels.
Figure 1: (a) Magnetic resonance imaging, T2-weighted axial image showing isointense to hyperintense soft tissue mass involving the right frontal sinus, ethmoid sinus, anterosuperior nasal cavity, and extending up to the subcutaneous plane causing bulging of the same (solid white arrow). (b) Magnetic resonance imaging, T2-weighted coronal image showing isointense to hyperintense soft tissue mass involving the right frontal sinus, ethmoid sinus, anterosuperior nasal cavity, and extending up to the subcutaneous plane causing bulging of the same (solid white arrow). (c) Magnetic resonance imaging, T1-weighted coronal image showing isointense to hyperintense soft tissue mass involving the right frontal sinus, ethmoid sinus, anterosuperior nasal cavity, and extending up to the subcutaneous plane causing bulging of the same (solid white arrow)

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Figure 2: (a) Magnetic resonance imaging, T2-weighted sagittal image showing salt and pepper appearance of the lesion (solid white arrow). (b) Magnetic resonance imaging, postcontrast T1-weighted coronal image showing avid enhancement with mass extending into anterior cranial fossa and medial and superior aspect of the right orbit (solid white arrow). (c) Magnetic resonance imaging, postcontrast T1-weighted sagittal section showing avid enhancement with mass extending into anterior cranial fossa and medial and superior aspect of the right orbit (solid white arrow)

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 > Discussion Top


Paragangliomas of the paranasal sinuses usually are very rarely hormonally active and usually present with hearing problems or tinnitus.[2] In fact, they are so rare that <10 cases of association of Cushing's syndrome with nasal paraganglioma has been made till date.[3] Ectopic Cushing's syndrome is usually caused by small cell carcinomas of the lung, carcinoid tumors, pancreatic islet cell tumors, medullary thyroid carcinomas, and rarely paragangliomas which can be cured with tumor resection.[4] In all previous reported cases, the characteristic alveolar pattern of a paraganglioma, the ACTH granules and the presence of sustentacular cells made the diagnosis clear.[3] Owing to the high prevalence of mutations in the succinate dehydrogenase gene in people suffering from paraganglioma, genetic testing is suggested but could not be done in our center.[5] Total resection of the mass is the treatment of choice.[3]


 > Conclusion Top


The diagnosis of ectopic ACTH syndrome secondary to nasal paraganglioma was established based on the presence of ACTH seen on immunohistochemical staining of the tumor; the disappearance of symptoms as well as decrease and normalization of plasma ACTH and cortisol levels after resection of the tumor. The rare association of nasal paraganglioma and ectopic hormone production should be kept in mind.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Lack EE, Cubilla AL, Woodruff JM, Farr HW. Paragangliomas of the head and neck region: A clinical study of 69 patients. Cancer 1977;39:397-409.  Back to cited text no. 1
    
2.
Colen TY, Mihm FG, Mason TP, Roberson JB. Catecholamine-secreting paragangliomas: Recent progress in diagnosis and perioperative management. Skull Base 2009;19:377-85.  Back to cited text no. 2
    
3.
Serra F, Duarte S, Abreu S, Marques C, Cassis J, Saraiva M. Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma. Endocrinol Diabetes Metab Case Rep 2013;2013:130038.  Back to cited text no. 3
    
4.
Isidori AM, Lenzi A. Ectopic ACTH syndrome. Arq Bras Endocrinol Metabol 2007;51:1217-25.  Back to cited text no. 4
    
5.
Joynt KE, Moslehi JJ, Baughman KL. Paragangliomas: Etiology, presentation, and management. Cardiol Rev 2009;17:159-64.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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