A composite tumor in the adrenal gland: Pheochromocytoma and ganglioneuroma

Year 2018, Volume: 35 Issue: 2, 49 - 52, 25.10.2019

Seda Gün Duygu Kuru Mehmet Kefeli

Abstract

Composite tumor in the adrenal medulla is a rare tumor containing endocrine
and neural components. Pheochromocytoma together with ganglioneuroma
combination is the most common compound tumor in the adrenal medulla.
Pheochromocytoma originates from the chromaffin cells in adrenal medulla and
the ganglioneuroma originates from autonomic ganglion cells. A 49-years-old
male patient refers to hospital with the complaint of abdominal pain which had
started 1month ago. A mass was detected in his right adrenal gland, and he
underwent right adrenalectomy. Macroscopic study of the adrenalectomy ma-
terial section revealed a 7x5x4.5 cm dark brown-yellow mass. Histopatholog-
ical studies revealed that the tumor was consisted of two components 1) areas
compatible with pheochromocytoma characterized by islands of polygonal cells
with eosinophilic cytoplasm, round-oval nucleus and a prominent nucleolus,
and forming small nodules with well-defined margins in a fibrovascular stroma
and 2) mature ganglion cells scattered in a stroma with Schwann-like cells. In
immunohistochemical study, pheochromocytoma foci were stained positive for
chromogranin, synaptophysin and tyrosine hydroxylase. Schwann and ganglion
cells were stained positive with S100. On the basis of histomorphological and
immunohistochemical findings, the patient diagnosed with compound tumor of
pheochromocytoma and ganglioneuroma. Although ganglioneuroma is encoun-
tered as a rare tumor, it should be kept in mind in the differential diagnosis of
the adrenal masses. 

Keywords

Adrenal Medulla, Composite Tumor, Ganglioneuroma, Pheochromocytoma

References

• Choi, E.K., Kim, W.H., Park, K.Y., 2006. A Case of a Composite Adrenal Medullary Tumor of Pheochromocytoma and Ganglioneuroma Masquerading as Acute Pancreatitis. The Korean Journal of Internal Medicine. 2 (21), 141-145.
• Comstock, J.M., Willmore-Payne, C., Holden, J.A., Coffin, C.M., 2009. Composite pheochromycitoma. A clinicopathologic and molecular comparison with ordinary pheochromocytoma and neuroblastoma. Am J Clin Pathol. 132, 69-73.
• Gorgel, A., Çetinkaya, D.D., Salgur, F., Demirpence, M., Yılmaz H., Karaman, E.H., Tutuncuoglu, P., Oruk, G., Bahceci, M., Sari,A.A., Altinboga, A.A. and Paker, İ., 2014. Coexistence of Gastrointestinal Stromal Tumors (GISTs) and Pheochromocytoma in Three Cases of Neurofibromatosis Type 1 (NF1) with a Review of the. Intern Med. 53, 1783-1789.
• Gücin, Z., Geçer, M.Ö., Aksoy, B., 2003. Nörofibromatozis eşliğinde non-sporadik adrenal kompozit feokromositom. Feokromositom-ganglionörom birlikteliği. Olgu sunumu. Türk Patoloji Dergisi. 19 (1-2): 42-44.
• Hu, J., Wu, J., Cai, L., Jiang, L., Lang, Z. et al., 2013. Retroperitoneal composite pheochromocytoma- ganglioneuroma: a case report and review of literature. Diagnostic Pathology. 8, 63.
• Lam, K.Y., Lo, C.Y., 1999. Composite Pheochromocytoma-Ganglioneuroma of the Adrenal Gland: An Uncommon Entity with Distinctive Clinicopathologic Features. Endocr Pathol. Winter; 10 (4),343-352.
• Mahajan, H., Lee, D., Sharma, R., Chin, P. et al., 2010. Composite phaeocromcytoma-ganglioneuroma, an uncommon entity: report of two cases. Pathology. 3 (42), 295-297.
• Menon, S., Manajan, P., Desai, S.B., 2011. Composite adrenal medullary tumor: A rare cause of hypertension in a young male. Urol Ann. 3 (1), 36-38.
• Rai, R., Gajanthody, S., Jayaram, J., Chauhry, R.K., 2012. Composite pheochromycitoma. South Asian J Cancer. 1 (2), 98-99. Rao, R.N., Singla, N., Yadav, K., 2013. Composite pheochromycitoma-ganglioneuroma of the adrenal gland: a case report with immunohistochemical study. Urol Ann. 5 (2), 115–118.
• Shida, T., Igawa, T., Abe, K., Hakariya, T., Takehara, K., Onita, T. and Sak, H., 2015. Composite pheochromocytoma of the adrenal gland: a case series. BMC Res Notes. 8, 257.