Familial Adenomatous Polyposis Associated With Bilateral Adrenocortical Tumors and Agressive Desmoid Tumor

Background: Familial adenomatous polyposis (FAP) is an autosomal dominant condition caused by germline mutation in the tumor suppressor gene APC. FAP occurs in 1 to 10.000 individuals, and is characterized by hundreds to thousands of colonic adenomatous polyps with a high risk of developing into colorectal cancer. Extracolonic manifestations can be malignant or benign. The major causes of morbidity and mortality in patients with FAP are abdominal desmoid tumors, with incidences ranging between 7% and 17%. Adrenal incidentaloma are frequently discovered in these patients, generally as benign lesions when they undergo abdominal CT-scan in the course of surveillance. Adrenal lesions in FAP ranged from 7.4% to 16%. We described an unusual patient with FAP, associated with autonomous cortisol production due to bilateral adrenal tumors and the development of aggressive desmoid tumor after unilateral adrenalectomy. Clinical Case: A 33-years-old female FAP-patient presented with abdominal pain, weight gain (10kgs), humor instability, paroxysmal of chest pain, dizziness and tremors. The abdominal MRI showed a heterogeneous, left adrenal mass (9.0 x 7.9 x 6.7cm), suspected for malignant tumor, and right adrenal mass with 3.6 x 1.8 cm suggestive of adenoma. Abdominal CT and PETCTFDG revealed on the left adrenal lesion with 33UH and maxSUV 3.9 and a right adrenal lesion 13UH and maxSUV 3.1. Serum hormone levels were as follows: cortisol after DST (1mg-dexamethasone) 4.8 ug/Dl, ACTH 8,8pg/Ml with no other abnormal hormone secretion detected. Patient underwent left adrenalectomy. Histological analysis revealed Weiss 1, modified Weiss 2 and Ki67 <1% compatible with adenoma. On follow-up, abdominal MRI revealed a 4.3cm-solid-homogeneous mass at the surgical incision, suspected of malignance. The mass progressively enlarged to 6.3 cm in diameter. Histological analysis of the biopsy identified a desmoid tumor. The contralateral adrenal tumor maintained stable during the follow-up; however, it began to produce cortisol autonomous secretion as observed on DST. The patient developed metabolic syndrome and did not present classical Cushing’ syndrome. Contralateral adrenalectomy was contraindicated because of concern of emergence of a new desmoid tumor. Discussion: FAP-associated with adrenal tumors can produce mineralocorticoids, corticosteroids, or both. Although adrenal cortical tumors have been reported frequently in FAP patients, the presence of bilateral commitment tumors on adrenal glands is extremely rare. Conclusion: We reported a woman with FAP and bilateral adrenal tumors with non-synchronic cortisol secretion associated with an aggressive desmoid tumor developing after the adrenalectomy. The patient is taking an adrenal inhibitor of steroidogenesis to control cortisol secretion and to provide clinical improvement.

Bilateral Adrenal Lymphoma: A Diagnostic Challenge

Bilateral primary adrenal lymphoma is rare with no specific clinical or imaging features. We report herein the case of a 44-year-old man presenting with massive bilateral adrenal tumors associated with normal adrenal function. Computed tomography findings were nonspecific. Because pheochromocytoma was ruled out and the patient had a normal coagulation test, we performed an ultrasound-guided adrenal biopsy for diagnostic purposes. Pathology confirmed adrenal non-Hodgkin's lymphoma. By reporting our case, we aimed to propose a practical approach for this rare entity and to show that there are still situations in which biopsy is necessary to make a diagnosis.

Cushing’s syndrome due to bilateral oncocytic adrenal tumor

Cushings syndrome accounts for approximately 2030% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushings syndrome due to bilateral adrenal tumors is extremely rare. Adrenal oncocytomas are another rare cause of endogenous hypercortisolism: about 13 cases are described in the literature. Oncocytomas are rare epithelial neoplasms, characterized by abnormally excessive accumulation of defective mitochondria in the cytoplasm of cells, and make up 1.8% of all adrenal neoplasms. We describe a 58-year old patient with Cushings syndrome and bilateral adrenal tumors. Multispiral computed tomography of the adrenals showed signs suspicious of lipid-poor atypical adenomas or malignant tumors. Surgical treatment was the method of choice, and the larger tumor was excised first. Due to the absence of remission of endogenous hypercortisolism the excision of the second tumor was performed. Morphological and immunohistochemical examination confirmed the diagnosis of bilateral oncocytic adrenocortical tumors with uncertain malignant potential. Cases of bilateral hormone-producing adrenal oncocytomas have not been described in the literature.

ADRENAL SURGICAL APPROACH IN A WOMAN WITH SYNCHRONOUS BILATERAL ADRENAL TUMORS

Bilateral adrenal tumours are very rare and display various endocrine profiles. The unilateralapproach is indicated if a nonsecretor contralateral pattern is suspected in order to avoid adrenal insufficiency.We present a 48-year-old woman with a history of bradimenorrhea at the age of 44.The gynecological evaluation was negative but two adrenal tumours were found. CT revealed aright adrenal tumour of 2 cm and a left one of 2.4 cm. Low-normal ACTH and high serumchromogranin A were confirmed. After one year, the left tumour increased to 4 cm so laparoscopictransabdominal left adrenalectomy (with 3 trocars) was successfully performed without anyincident. No conversion was required. Neither intraoperative nor postoperative complicationsoccurred. The patient was hospitalised 5 days and the wounds healed normally. The pathologicalreport revealed a benign corticoadenoma. The endocrine evaluation after one week showed thatafter surgery ACTH de-suppressed to normal and chromogranin A normalised. The right adrenaltumour was followed-up for another year and the diameters were stationary. The patient was stillhypertensive and she was offered adequate therapy (which we considered essential for high blood pressure). Life-long periodical check-up is necessary.Unilateral adrenalectomy in patients with both side tumours allows an adequate pathological report and avoids unnecessary contralateral surgery if an incidentaloma is revealed, thus allowing the preservation of adrenal function.

A case of primary aldosteronism with secondary hyperparathyroidism and bilateral adrenal tumors

Summary A 43-year-old Japanese woman was admitted to our hospital with weakness. Laboratory findings showed hypokalemia, hypocalcemia and elevation of the serum creatinine phosphokinase levels, but intact parathyroid hormone levels. Further evaluations suggested that she had primary aldosteronism (PA), secondary hyperparathyroidism and bilateral adrenal tumors. She was treated successfully by laparoscopic right adrenalectomy. This case not only serves to the diagnosis of bilateral adrenal tumors in which selective adrenal venous sampling (SAVS) proved to be useful, but also for physicians to be aware of secondary hyperparathyroidism and the risk of secondary osteoporosis caused by PA. Learning points The classic presenting signs of PA are hypertension and hypokalemia. Hypokalemia can induce rhabdomyolysis. PA causes secondary hyperparathyroidism. Patients with PA have the risk of osteoporosis with secondary hyperparathyroidism. SAVS is useful in bilateral adrenal tumors.