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The coexistence of Cushing syndrome and gynecomastia as the manifestations of adrenocortical carcinoma

2018; Wiley; Volume: 34; Issue: 12 Linguagem: Inglês

10.1016/j.kjms.2018.06.004

2410-8650

Mongkontida Umphonsathien, Panudda Srichomkwun, Patinut Buranasupkajorn, Thiti Snabboon,

Male Breast Health Studies

Dear editor, A 43-year-old man presented with gynecomastia for 4 months. He also complained about loss of libido and 3-kg weight gain. Bilateral gynecomastia with testicular atrophy was noted as well as his Cushingoid appearance on the physical exam. Computed tomography scan showed a huge heterogeneous hypoechoic left suprarenal mass (Fig. 1A) corresponding to the palpable abdominal mass. Multiple metastatic lung nodules were also detected (Fig. 1B). The diagnosis of feminizing adrenocortical carcinoma (ACC) with Cushing syndrome was proposed by elevated UFC (606.02 μg/24 h, 21–143) with low ACTH level, and hyperestrogenemia (1221 pmol/L, 0–130). Left adrenalectomy, nephrectomy with splenectomy and IVC thrombectomy were performed. The pathological study showed a tumor size of 12 × 13 × 15 cm with extensive necrosis and capsular invasion, Ki-67 was 10% positive. Postoperatively, mitotane was commenced to control his advanced tumor. His gynecomastia subsequently regressed as well as his estradiol levels decreased. Unfortunately, he died six months later due to the uncontrolled infection. (A) Computed tomography scan showed a huge heterogeneous hypoechoic left suprarenal mass, (B) multiple metastatic lung nodules were demonstrated. Gynecomastia, an enlargement of male breast, is a common disorder in clinical practice. It represents a feminizing condition due to either absolute or relative estrogen excess. Causes of hyperestrogenemia include exogenous administration and endogenous overproduction from secreting tumors or increased peripheral aromatization; whereas relative estrogen excess refers to a decrease in androgen levels or its actions. An initial step in making the diagnosis is to differentiate from lipomastia and breast cancer. True gynecomastia is characterized by a rubbery mass concentric within the nipple-areolar complex [1]. Most cases have a benign etiology from physiologic changes or medications; however, few cases can be a result of underlying endocrine disorders or tumors. Feminizing tumors can be categorized into estrogen-producing testicular or adrenal tumors and hCG-producing testicular or non-testicular tumors. Several possible causes can be uncovered with an extensive history and physical examination: onset/duration, tenderness, medications, family history of gynecomastia or neurological diseases, systemic diseases (liver or renal failure), and endocrine disorders (hyperestrogenemia, hypogonadism, thyrotoxicosis). Initial investigations should include a liver/renal function, hormonal profiles (testosterone, estradiol, LH, TSH, prolactin) and tumor markers (hCG, α-fetoprotein); nonetheless, about one-fourth would be classified as idiopathic. ACC is a rare tumor with dismal prognosis. Adjacent organ invasion or metastasis is crucial to distinguish from the benign lesions. Larger size (>4 cm), sex hormone excess (virilization/feminization), high mitotic figures or capsular/vascular invasion are also characteristics of ACC [2]. Nearly 60% of ACC is functional, predominantly with a cortisol excess, a whereas feminizing tumor is found in only 2–6% [2], [3]. Hyperestrogenemia in ACC has been attributed to an increased tumoral aromatase activity. Another possible mechanism is the peripheral conversion of weaker androgens from the tumor [4]. Feminizing tumor is manifested mainly by symptoms of hyperestrogenism (e.g. gynecomastia, testicular atrophy, and erectile dysfunction), mass symptoms or deterioration of the general conditions (fatigue, weight loss). As described in our patient, multiple hormonal secretions represent its de-differentiated tumorigenesis and steroidogenic dysregulations. Therefore, complete hormonal evaluation is requisite in ACC patients even if clinical symptoms are absent to prevent adrenal insufficiency postoperatively and provide as a marker of tumor recurrence [5]. Complete surgical removal is the only curative treatment. Mitotane and chemotherapy are adjunctive modalities to control an advanced disease. In conclusion, gynecomastia is common and self-limited but few cases can be from a malignant cause. Further investigation is required when it occurs in an unusual age group or is progressive. Supplementary data related to this article can be found at https://doi.org/10.1016/j.kjms.2018.06.004. Multimedia component 1 Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.