Adrenocortical carcinoma presenting as varicocele and renal vein thrombosis: a case report

Abstract

IntroductionAdrenocortical carcinomas are rare aggressive tumors. Their annual incidence is approximately one to two per million among the population of the United States of America. Patients with active endocrine tumors often present with Cushing's syndrome accompanied by virilizing features. Conversely, patients with non-functioning tumors may present with symptoms related to a mass-occupying lesion, such as abdominal pain and flank pain. Although varicoceles and acute kidney injuries are common problems in medicine, they are uncommon presentations of these rare tumors and easy to miss. We report a case of a large adrenocortical carcinoma that presented as testicular pain, varicocele, and acute kidney injury secondary to renal vein thrombosis.Case presentationA 54-year-old Caucasian man with a left-sided varicocele presented to our emergency department with lower abdominal pain and a decrease in urination. Four months previously, he had noticed pain and swelling in his left groin and had been diagnosed with left-sided varicocele. For one week, he began developing left-sided abdominal pain and decreased urination frequency, so he came to our emergency department for evaluation. His physical examination revealed a hard mass occupying the entire left side of his abdomen, crossing the midline, and extending to the pelvic brim. His blood tests showed acute kidney injury and mild anemia. Computed tomography of his abdomen showed a large retroperitoneal mass on the left side, displacing the left kidney inferiorly and the spleen superiorly with thoracic epidural compression. Thrombus was also identified in his left renal vein and inferior vena cava. Computed tomography of his chest showed bilateral pulmonary nodules. A computed tomography-guided abdominal mass biopsy was performed, and the diagnosis of adrenocortical carcinoma was made on the basis of pathology and immunohistochemistry. His hormonal evaluations were normal. His kidney function improved with intravenous hydration and anti-coagulation treatment. Unfortunately, the adrenal mass was unresectable because of the extent of the tumor. Treatment with mitotane, an adrenocorticolytic drug, was started with concomitant with irradiation of a lesion at T5, followed by combination chemotherapy thereafter.ConclusionUnilateral right-sided varicoceles are rare and should alert the clinician to possible underlying pathology causing inferior vena caval obstruction. Left-sided varicoceles, in contrast, are common secondary to the venous anatomy of the left testis; however, the enlargement of the left testicle can be associated with blockage of the left testicular vein by tumor invasion of the left renal vein. Varicoceles could be an early presentation of a non-functioning adrenocortical carcinoma. Acute kidney injury can occur as a result of mass effect or thrombosis of renal vessels. Large tumors can cause abdominal pain as a late manifestation. Physicians should perform a complete abdominal examination in every patient with varicocele or testicular pain.

Left renal vein compression as cause for varicocele: prevalence and associated findings on contrast-enhanced CT

“…In one small case series, over 75% of patients with pancreatic adenocarcinoma involved the LRV [21]. Varicoceles have also been documented as presenting symptoms in renal and adrenal carcinomas, Wilms tumors, lymphoma, and aortic pseudoaneurysms [22][23][24][25][26]. Although a new presentation of a right-sided varicocele typically prompts a workup for malignancy, the higher than expected prevalence of malignancy in our left-sided varicocele population suggests that in adult patients with a new left-sided varicocele and other potential signs and symptoms of malignancy, and then further workup may be indicated.…”

Diagnosis and surgical treatment of adrenocortical cancer with invasion into great veins

“…Объем операции будет заключаться в уда-лении опухоли с опухолевым тромбом. При этом инвазия опухоли в магистральные сосуды не влияет на продолжительность жизни больного [6,12].…”

Organüberschreitendes Nebennierenrindenkarzinom mit Beteiligung der Vena cava inferior

“…Das NNR-Ca tritt mit einer weltweiten jährlichen Inzidenz von 0,7-2/Mio (1-2 bis 4-12/Mio -unterschiedliche Literaturangaben [2,3,4,5,6,7,8]) und mit einer geringen Präferenz des weiblichen Geschlechts (1,5 : 1) [7] und der linken Nebennierenrinde auf [7]. Das sporadische Vorkommen zeigt zwei Gipfel -in der 1. und 4./5.…”

“…Tumorthromben entsprechend der VCI-Klassifikation nach Staehler[16].VCI: V. cava inferior.Tab 4. Diagnostischer Score für das Nebennierenrinden-Karzinom (histopathologisches Weiss-System[21].DtschMed Wochenschr 2013; 138: 260-265 · J. Dillner et al, Organüberschreitendes Nebennierenrindenkarzinom mit …Die 5-Jahres-Überlebensrate ist unbefriedigend mit abweichenden Angaben zwischen 23-60 % (je nach Literaturangabe auch 37-47 %[2,4,7,12,14,18,20]). Bei inkompletter Resektion beträgt die mediane Überlebensrate jedoch weniger als ein Jahr.…”