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Feminizing Adrenocortical Carcinoma with Distant Clinics and Practice 2014; volume 4:651 Feminizing adrenocortical (either virilization or feminization), although mixed clinical features can be observed.1,5 Correspondence: Alessandro Fancellu, Unit of carcinoma with distant Feminizing ACCs, i.e. malignant adrenal General Surgery 2 - Clinica Chirurgica, metastases: can surgery tumors causing features of estrogen excess, Department of Clinical and Experimental be considered? are an extremely rare cause of abdominal Medicine, University of Sassari, v.le San Pietro mass. 43, 07100 Sassari, Italy. Tel.: +39.079.228432 - Fax: +39.079 228394. Alessandro Fancellu, Antonio Pinna, We herein present a case of a young man E-mail: [email protected] Alberto Porcu having a giant feminizing ACC with distant metastases, which posed serious management Key words: adrenocortical carcinoma, feminizing, Unit of General Surgery 2, Department problems principally related to the burden of treatment, gynecomastia, rare tumor. of Clinical and Experimental Medicine, the primary tumor. University of Sassari, Italy Conflicts of interest: the authors declare no potential conflicts of interest. Case Report Contributions: the authors contributed equally. Abstract Received for publication: 9 May 2014. A 41-year-old gentleman presented com- Accepted for publication: 12 June 2014. plaining of a 6-month history of bilateral Functioning adrenocortical carcinomas are gynecomastia without galactorrhea, and a sig- This work is licensed under a Creative Commons rare diseases with dismal prognosis. A 41-year- Attribution NonCommercial 3.0 License (CC BY- nificant weight loss (8 kg in the last three old man presenting with gynecomastia had a NC 3.0). months). In the last two months he also giant feminizing adrenocortical carcinoma at noticed abdominal and back pain, dyspepsia, stage IV. Although surgical resection was con- ©Copyright A. Fancellu et al., 2014 and some episodes of vomiting, bloating and Licensee PAGEPress, Italy troversial, we removed the primary tumor to difficult passage of stools. The rest of his med- Clinics and Practice 2014; 4:651 reduce the mass effects. He lived for 12 ical history was not relevant and he was on no doi:10.4081/cp.2014.651 months with an acceptable quality of life. medications. His family history revealed the Gynecomastia may be the first sign of feminiz- presence of a 37-year-old sister with an estab- ing adrenal malignancies. Surgery may ame- lished diagnosis of MEN1 (primary hyper- liorate the quality of life in selected patients parathyroidism, pancreatic gastrinoma, and adrenal tumor, in an attempt to reduce the with metastatic disease. prolactinoma) with germline mutation 894-9 symptoms related to the mass, principally pain GA, and a 50-year-old brother affected by an and recurrent subocclusive episodes. Before adrenocorticotropic hormone (ACTH)-secret- the operation, a medication with ketoconazole ing pituitary adenoma causing Cushing’s dis- was started for hypercortisolemia; however, Introduction ease. As the patient was in good health before replacement of ketaconazole with metyrapone the appearance of gynecomastia, he had previ- was necessa ry to normalize the cortisol levels. While adrenal adenomas are quite frequent ously refused to undergo laboratory and genet- The patient underwent an exploratory celioto- in the general population, adrenal cortical car- ic testing. Laboratory tests showed: i) extreme- my where a large encapsulated mass was cinomas (ACCs) are rare malignancies affect- ly high estradiol plasma levels (E2>500), encountered, displacing the kidney inferiorly ing only 2 per million per year in the world pop- together with undetectable gonadotropin levels and the distal pancreas and spleen superiorly. ulation.1,2 A bimodal age distribution has been and low testosterone; ii) the likely coexistence The mass and the left kidney were resected en recognized, with first peak in childhood (<5 of Cushing’s disease (hypercortisolemia with bloc. On surgical exploration, the right adrenal years of age) and second peak in 4th-5th decade increased ACTH levels) (Table 1). In relation also appeared increased in size, thus a bilater- of life. Most of the ACCs are sporadic; however to the latter, an overnight dexamethasone sup- al adrenalectomy was performed. On gross they may also arise wit hin the context of famil- pression test (1 mg at 11.00 p.m.) was carried pathology examination, the left adrenal tumor ial or hereditary diseases.2-4 out, which failed to show serum cortisol sup- (which included approximately half of the left Although different studies have been under- pression (19 g/dL at 08.00 a.m.). On abdomi- kidney, with diffusely infiltrated areas) taken in order to elucidate the molecular nal examination, a large, firm mass adherent weighed 3.3 kg, and measured 27×17×12 cm pathogenesis of sporadic ACCs, to date none of to the deep layers was palpable in the left quad- (Figure 2). The histological examination them has been proven to be completely rants. The total body computed tomographic revealed an ACC with oncocytic changes with a exhaustive.2,4 In particular, the genetic alter- (CT) scan showed a large tumor of the left Weiss score of 8 (Figure 3). Based on the his- ations characteristic of the adenoma-carcino- adrenal (25 cm in its maximum diameter) tological finding, the laboratory tests, and the ma sequence has not clearly established. To with colliquative areas infiltrating the left kid- presence of distant metastases, we made the date, modification in three main molecular ney (Figure 1), left renal vein thrombosis, and diagnosis of feminizing adrenocortical carci- patterns has been identified: insulin-like multiple liver and lung metastases. No pancre- noma at stage IV according to the American growth factor (IGF)-2, the metabolic pathway atic lesions were visible. Pelvic CT scan Joint Committee on Cancer (AJCC) classifica- Wnt/-catenin and TP53.4 ACCs can be either revealed no abnormality, while breast and tes- tion. The right adrenal weighed 14 g and had functioning (i.e. producing hormones of the ticular ultrasound confirmed the presence of aspects of cortical hyperplasia. adrenal cortex) or non-functioning. Adrenal gynecomastia and reduced testicular volume. The postoperative course was uneventful, malignancies are more likely to be functioning Neck ultrasound showed neither enlarged and the patient was discharged on postopera- with respect to adenomas and can have differ- parathyroid glands nor thyroid alterations. tive day 12. He was given replacement therapy ent clinical presentations, depending on the Pituitary magnetic resonance imaging and with hydrocortisone, fludrocortisone, and type of hormonal secretion. In fact, patients parathyroid sestamibi scan were also normal. mitotane therapy without any importan t side may have symptoms of hypercortisolism, After a multidisciplinary discussion, it was effects. Six months after surgery, laboratory hyperaldosteronism, or sex hormone excess decided to operate on the patient to remove the tests showed a normalization of cortisol and [page 30] [Clinics and Practice 2014; 4:651] Article estrogens levels. Gynecomastia was markedly The main presenting signs and symptoms of patient.6 While AACs are for the most part spo- improved, and a mild reduction of the size of feminizing ACC are gynecomastia, testicular radic, they can occur as part of various familial liver and lung metastasis was observed. He atrophy, decreased libido and impotence. In tumor syndromes, such as MEN1, Li-Fraumeni went on to live 12 months with an acceptable line with other reported cases, gynecomastia syndrome, Beckwith-Wiedemann syndrome, quality of life before dying from metastatic dis- was the presenting symptom of ACC in our and Carney complex.2,4 The familiar history of ease. patient.5,7 Gynecomastia recognizes various our patient denoted the presence of well- causes, being most commonly due to medica- defined endocrine pathologies (MEN1 and tions, imbalance between testosterone/estro- ACTH-producing pituitary adenoma). Adrenal gen levels, kidney failure, cirrhosis, and much neoplasms are not commonly included in Discussion and Conclusions more rarely to pituitary and adrenal tumors.7 It MEN1; however, non-functioning macronodu- has been suggested that feminizing symptoms lar hyperplasia is observed in up to 40% of Feminizing ACCs are very rare, accounting can be linked to aberrant aromatization of patients with MEN1 and, even rarely, ACCs can for only 1-2% of all malignant neoplasms of adrenal androgens in estrogens, principally occur.4 As a consequence, we investigated the 1 adrenal cortex. In 1994, Zayed reported fewer E1.6 Feminization is often combined with exist ence of MEN1 syndrome in our patient, 6 than 100 cases described in male sex. Since hypercortisolism, as we observed in our but the laboratory and imaging studies pre- then, only a few cases have been added to the literature. Clinical features related to steroid excess occur in about 60% of ACCs, while feminizing symptoms due to estrogen secretion are extremely uncommon.1,5 Figure 1. Computed tomographic scan of the abdomen revealed a large tumor of the left adrenal (25 cm in its maximum diame- ter) with colliquative areas, occupying almost entirely the hemiabdomen. Figure 2. The adrenal mass measured Figure 3. Histological examination with standard hematoxylin and eosin showing a pro- 25×17 cm, and appeared encapsulated, yel- liferation of large epithelial cells and pleomorphic oxyphil nuclei (A, 200×; B, 400×), lowish with extensive areas of necrosis and with
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