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Feminizing Adrenocortical Carcinoma with Distant Metastases: Can Surgery be Considered?

Fancellu A, Pinna A, Porcu A - Clin Pract (2014)

Bottom Line: He lived for 12 months with an acceptable quality of life.Gynecomastia may be the first sign of feminizing adrenal malignancies.Surgery may ameliorate the quality of life in selected patients with metastatic disease.

View Article: PubMed Central - PubMed

Affiliation: Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari , Italy.

ABSTRACT
Functioning adrenocortical carcinomas are rare diseases with dismal prognosis. A 41-year-old man presenting with gynecomastia had a giant feminizing adrenocortical carcinoma at stage IV. Although surgical resection was controversial, we removed the primary tumor to reduce the mass effects. He lived for 12 months with an acceptable quality of life. Gynecomastia may be the first sign of feminizing adrenal malignancies. Surgery may ameliorate the quality of life in selected patients with metastatic disease.

No MeSH data available.


Related in: MedlinePlus

Computed tomographic scan of the abdomen revealed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas, occupying almost entirely the hemiabdomen.
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fig001: Computed tomographic scan of the abdomen revealed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas, occupying almost entirely the hemiabdomen.

Mentions: A 41-year-old gentleman presented complaining of a 6-month history of bilateral gynecomastia without galactorrhea, and a significant weight loss (8 kg in the last three months). In the last two months he also noticed abdominal and back pain, dyspepsia, and some episodes of vomiting, bloating and difficult passage of stools. The rest of his medical history was not relevant and he was on no medications. His family history revealed the presence of a 37-year-old sister with an established diagnosis of MEN1 (primary hyperparathyroidism, pancreatic gastrinoma, and prolactinoma) with germline mutation 894-9 G→A, and a 50-year-old brother affected by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma causing Cushing’s disease. As the patient was in good health before the appearance of gynecomastia, he had previously refused to undergo laboratory and genetic testing. Laboratory tests showed: i) extremely high estradiol plasma levels (E2>500), together with undetectable gonadotropin levels and low testosterone; ii) the likely coexistence of Cushing’s disease (hypercortisolemia with increased ACTH levels) (Table 1). In relation to the latter, an overnight dexamethasone suppression test (1 mg at 11.00 p.m.) was carried out, which failed to show serum cortisol suppression (19 µg/dL at 08.00 a.m.). On abdominal examination, a large, firm mass adherent to the deep layers was palpable in the left quadrants. The total body computed tomographic (CT) scan showed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas infiltrating the left kidney (Figure 1), left renal vein thrombosis, and multiple liver and lung metastases. No pancreatic lesions were visible. Pelvic CT scan revealed no abnormality, while breast and testicular ultrasound confirmed the presence of gynecomastia and reduced testicular volume. Neck ultrasound showed neither enlarged parathyroid glands nor thyroid alterations. Pituitary magnetic resonance imaging and parathyroid sestamibi scan were also normal.


Feminizing Adrenocortical Carcinoma with Distant Metastases: Can Surgery be Considered?

Fancellu A, Pinna A, Porcu A - Clin Pract (2014)

Computed tomographic scan of the abdomen revealed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas, occupying almost entirely the hemiabdomen.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4202184&req=5

fig001: Computed tomographic scan of the abdomen revealed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas, occupying almost entirely the hemiabdomen.
Mentions: A 41-year-old gentleman presented complaining of a 6-month history of bilateral gynecomastia without galactorrhea, and a significant weight loss (8 kg in the last three months). In the last two months he also noticed abdominal and back pain, dyspepsia, and some episodes of vomiting, bloating and difficult passage of stools. The rest of his medical history was not relevant and he was on no medications. His family history revealed the presence of a 37-year-old sister with an established diagnosis of MEN1 (primary hyperparathyroidism, pancreatic gastrinoma, and prolactinoma) with germline mutation 894-9 G→A, and a 50-year-old brother affected by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma causing Cushing’s disease. As the patient was in good health before the appearance of gynecomastia, he had previously refused to undergo laboratory and genetic testing. Laboratory tests showed: i) extremely high estradiol plasma levels (E2>500), together with undetectable gonadotropin levels and low testosterone; ii) the likely coexistence of Cushing’s disease (hypercortisolemia with increased ACTH levels) (Table 1). In relation to the latter, an overnight dexamethasone suppression test (1 mg at 11.00 p.m.) was carried out, which failed to show serum cortisol suppression (19 µg/dL at 08.00 a.m.). On abdominal examination, a large, firm mass adherent to the deep layers was palpable in the left quadrants. The total body computed tomographic (CT) scan showed a large tumor of the left adrenal (25 cm in its maximum diameter) with colliquative areas infiltrating the left kidney (Figure 1), left renal vein thrombosis, and multiple liver and lung metastases. No pancreatic lesions were visible. Pelvic CT scan revealed no abnormality, while breast and testicular ultrasound confirmed the presence of gynecomastia and reduced testicular volume. Neck ultrasound showed neither enlarged parathyroid glands nor thyroid alterations. Pituitary magnetic resonance imaging and parathyroid sestamibi scan were also normal.

Bottom Line: He lived for 12 months with an acceptable quality of life.Gynecomastia may be the first sign of feminizing adrenal malignancies.Surgery may ameliorate the quality of life in selected patients with metastatic disease.

View Article: PubMed Central - PubMed

Affiliation: Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari , Italy.

ABSTRACT
Functioning adrenocortical carcinomas are rare diseases with dismal prognosis. A 41-year-old man presenting with gynecomastia had a giant feminizing adrenocortical carcinoma at stage IV. Although surgical resection was controversial, we removed the primary tumor to reduce the mass effects. He lived for 12 months with an acceptable quality of life. Gynecomastia may be the first sign of feminizing adrenal malignancies. Surgery may ameliorate the quality of life in selected patients with metastatic disease.

No MeSH data available.


Related in: MedlinePlus