ACCs are a rare type of malignancy (incidence of 1 to 2 cases per 1 million population) with a heterogeneous presentation and generally have a poor prognosis [1, 6]. Women are affected more often than men [7, 8]. The diagnosis of malignancy of adrenocortical tumors depend on careful investigations of clinical, biological, and imaging features before surgery and pathological examination. The assessment of the Weiss score is important for the diagnosis of malignancy [9, 10]. Adrenocortical tumors might be seen as a component of several hereditary tumor syndromes such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia 1, Carney complex, and congenital adrenal hyperplasia. A lot of specific genes might have a role in the pathogenesis of sporadic ACC, and some of them might be seen in the pathogenesis of the aforementioned syndromes [11, 12].
Patients present with evidence of adrenal steroid hormone excess in approximately 60% of cases. Rapidly progressing Cushing’s syndrome with or without virilization is the most frequent presentation. Hormonal inactive ACC usually presents with abdominal discomfort (nausea, vomiting, and abdominal fullness) or back pain caused by a mass effect of the large tumor.
PTC comprises about 85% of cases with differentiated thyroid carcinoma . PTC usually presents with a thyroid nodule or cervical lymphadenopathy. Nodules more than 1cm in diameter have a greater potential to be malignant. On the other hand, patients with nodules less than 1cm in diameter, suspicious ultrasound findings, lymphadenopathy, a history of neck and head irradiation, or a history of thyroid cancer in first-degree relatives must be evaluated for cancer .
The ultrasonographic features suggesting the presence of malignancy in a thyroid nodule have been described clearly. These include microcalcifications, marked hypoechogenicity, absent “halo” sign, extraglandular extension, an irregular or microlobulated margin, and a heterogeneous echo structure [15, 16]. Individual sonographic features have limitations for prediction of thyroid cancer. Elastosonography is a newly developed, promising sonographic method for evaluating suspicious nodules. It gives information about the stiffness of a nodule by measuring the amount of distortion that occurs when the nodule is subjected to external pressure . Tissue stiffness is scored from 1 to 5 based on subjective analysis of the elastographic image. Rago et al. reported sensitivity of 97% and specificity of 100% for a score of 4 or 5 as being predictive of malignancy .
PTC generally metastasizes to the lymph nodes. Distant organ metastasis is rare, but lung, bone, brain, liver, and adrenal gland metastases have been reported [19, 20]. ACC may metastasize to the thyroid gland . Immunohistochemical staining is used to differentiate the primary and metastatic tumors. Melan-A, inhibin, and some other markers have been found to be positive in patients with ACC [22–24], and thyroglobulin and TTF-1 have been found to be positive in patients with thyroid neoplasms in immunohistochemical studies [25–28]. In our case, melan-A and inhibin were positive, but thyroglobulin and TTF-1 were negative, in the patient’s adrenal gland tumor. The thyroid lesion was thyroglobulin-positive. Therefore, these lesions were evaluated as primary tumor neoplasms of those organs.
In the literature, there are few case reports of patients with with comorbid ACC and PTC. Fukushima et al. reported a case of a middle-aged woman with virilizing adrenocortical adenoma complicated with Cushing’s syndrome, PTC, and hypergastrinemia . They did not study genetic analyses in their patient. Wanta et al. reported the case of a patient with synchronous presentation of an aldosterone-secreting metastatic ACC and PTC . That patient was diagnosed as having Li-Fraumeni syndrome and multiple endocrine neoplasia type I, but both p53 and menin gene mutations were absent.