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Open access

Marinos C Makris, Konstantinos C Koumarelas, Apostolos S Mitrousias, Giannos G Psathas, Athanasios Mantzioros, Stratigoula P Sakellariou, Panagiota Ntailiani and Evripides Yettimis

Summary

Until now, less than ten cases of extra-adrenal chromaffin cell tumors have been reported to be localized to the spermatic cord area. All published studies report benign tumors with a diameter <2–3 cm and no invasion of the testis. In this article, we present one case of a giant malignant paraganglioma in the testis of a patient who had initially been operated for a giant mass in the scrotum. The mass developed in approximately 4 months. This is the first study reporting the following findings: i) paraganglioma was found exclusively in the testis, invading the testicle and not the spermatic cord, ii) it was malignant with lung metastasis, and iii) its size was 17.5 cm×10 cm×9.5 cm. We present the first – giant – malignant paraganglioma. Moreover, it is the first case report of a paraganglioma in the testis.

Learning points

  • This is the first study reporting the following findings:

  • Paraganglioma found exclusively in the testis, invading the testicle and not the spermatic cord.

  • It is malignant with lung metastasis.

  • It is of the size 17.5 cm×10 cm×9.5 cm.

Open access

F Serra, S Duarte, S Abreu, C Marques, J Cassis and M Saraiva

Summary

Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature.

Learning points

  • Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies.

  • Most paraganglioma of the head and neck are not hormonally active.

  • Nasal paraganglioma, especially ACTH producing, is a very rare tumour.