Endocrine Abstracts

Background: The occurrence of multiple endocrine tumors in the same patient is not always typical and cannot always be classified into a multiple endocrine neoplasia (MEN) type, McCune-Albright syndrome or the Carney complex. Schwannomas are mesenchymal tumors that originate from Schwann cells of peripheral nerve fibers that can associate in some syndromes. 90% cases are benign, involving usually the head, neck and extremities. They are rare in the retroperitoneal region (1–3%).

Case report: We report the case of a 49-year-old female patient, Caucasian, non-smoker, who was previously diagnosed (in 2008) with ACTH-independent Cushing’s syndrome due to a cortisol secreting adenoma of the right adrenal gland (30/25mm). She had undergone right adrenalectomy (histopathologic exam and immunohistochemistry confirmed the diagnosis) and received one year of hydrocortisone replacement. In 2016, due to recurrence of malignant hypertension, the CT scan revealed in the right adrenal lodge a multilobulated, well-defined, heterogeneous mass, measuring 50/23 mm, that was compressing the superior vena cava and also a small adenoma (0.7/1 cm) on the left adrenal gland and left renal cysts. The patient had only a mild discomfort in the right flank. Adrenal function evaluation results were normal, excluding recurrent Cushing’s syndrome, pheocromocytoma or primary aldosteronism. Pituitary function was also normal. Further testing revealed hypercalcemia (11.3 mg/dl), elevated parathormone level (103 pg/ml), insufficient level of 25-OH-Vitamin D (17.02 ng/ml), normal renal function (CKD-EPI eGFR=97.02 ml/min per 1.73 m² and normal calcitonin (excluding medullary thyroid cancer). The anterior cervical ultrasonography showed a hypo-echogenic nodule (1.23/1.6 cm), suggestive for parathyroid adenoma. The patient was then referred to surgical service in order to remove the retroperitoneal mass of unknown origin by laparoscopic approach. The histopathologic and immunohistochemistry (S100, SYN, Melan A and VIM diffusely positive) exams indicated an ancient schwannoma. The patient is scheduled for sestamibi parathyroid scintigraphy. Further on, depending on the scintigraphy result, the patient will be advised to undergo parathyroidectomy and will be periodically screened for other occurrences.

Conclusion: The association between ACTH-independent Cushing’s, schwannoma finding in the same adrenal lodge after adrenalectomy and recently discovered primary hyperparathyroidism, is very rare and it may be genetically determined in the context of MEN or other syndromes, but can also be sporadic. Also, retroperitoneal ancient schwannomas are very rare, and the occurrence of it after adrenalectomy has not yet been reported, so we cannot know for sure if there is a connection with these endocrine conditions.

Keyword: Cushing’s syndrome, hyperparathyroidism, ancient schwannoma