Retroperitoneal paraganglioma presenting as right-sided varicocele: case report.

Varicocele, the most important identifiable pathological cause of male infertility, is rarely observed on the right side. In isolated cases of right-sided varicocele, the presence of retroperitoneal masses compressing the inferior vena cava should be considered by further investigation. The retroperitoneal masses that are detected tend to grow rapidly due to their location and may be very large at the time of diagnosis. Soft tissue sarcomas are the most common cause of retroperitoneal masses, while paragangliomas are rare causes. Retroperitoneal paragangliomas should be diagnosed via detection of symptoms caused by hormonal activity, observation of the compression effect of the mass, or incidentally during imaging studies. SIMILAR CASES PUBLISHED: This study presents the first case of retroperitoneal paraganglioma in a 41-year old man as isolated right-sided varicocele that has been described in the published reports.

Varicocele is the most important identifiable pathological cause of male infertility. Although presenting on the left side in 85% to 90% of all cases, varicocele has rarely been observed on the right side in isolated cases.1 In cases of right-sided varicocele, the presence of retroperitoneal masses should be considered and further studies be conducted for its detection. Although rarely observed, these retroperitoneal masses that are detected grow rapidly due to their location, and may be very large at the time of diagnosis. Soft tissue sarcomas are the most common cause of retroperitoneal masses, while paragangliomas are rare causes.2 In this study, we present the first case of retroperitoneal paraganglioma as isolated right-sided varicocele that has been described in the published reports.

CASE

A 41-year-old man was admitted to our outpatient clinic suffering from severe right-sided scrotal pain. Physical examination revealed grade 3 isolated right-sided varicocele (Figure 1), while abdominal examination revealed no palpable masses. The review of his medical history and records revealed no personal or family history of variococele or related diseases. Right-sided varicocele showing reflux flow via the Valsalva maneuver was confirmed by scrotal Doppler ultrasonography. The parameters of the spermiogram test were normal. Contrast-enhanced CT detected an irregularly shaped mass measuring 92×87 mm2 in the precaval and aortocaval areas at an infrarenal level contiguous with the duodenum (Figure 2). Although the mass was compressing the inferior vena cava (IVC), no invasion was detected.

Figure 1

Grade 3 isolated right-sided varicocele.

Figure 2

CT image of a 92×87 mm2, irregularly shaped, contrast-enhanced mass detected in the precaval and aortocaval areas at an infrarenal level contiguous with the duodenum.

With an initial diagnosis of retroperitoneal mass, laparotomy was performed. During the removal of the retroperitoneal mass contiguous with the IVC and duodenum, no symptoms of catecholamine discharge, such as hypertension or abnormalities in electrocardiography measurements, were observed. The pathological report described the detection of a capsulated, well-grained tumor with cells containing pleomorphic–hyperchromatic nuclei and wide cytoplasm and displaying intercellular mitosis. Histochemical staining revealed the sustentacular cells to be S100 protein positive and the tumor cells to be synaptophysin positive and chromogranin positive. Based on these histochemical findings, paraganglioma was diagnosed (Figure 3). Postoperative urinary metanephrine and normetanephrine levels were normal, and the patient was discharged without any complications on postoperative day 4. Metaiodobenzylguanidine (MIBG) scintigraphy performed on the second postoperative week showed no metastasis or local enhancement.

Figure 3

Positive staining with chromogranin in immunohistochemical examination.

DISCUSSION

Defined as the dilatation of pampiniform plexus veins, varicocele affects 10% to 15% of the male population and is an important cause of male infertility.3,4 The most common type of varicocele is isolated left-sided disease due to the entrance angle of the left testicular vein into the left renal vein. Bilateral varicocele is observed in 10% of cases, while right-sided varicocele is rarely alone.1 As the right gonadal vein enters directly into the IVC, the presence of retroperitoneal masses compressing the IVC should be considered in cases of isolated rightsided varicocele. Although the most common causes of these retroperitoneal masses are renal masses and sarcomas, adrenocortical carcinoma,5,6 Burkitt lymphoma,7 Wilms tumor,8 and IVC malformation caused by situs inversus9 appear in the published reports. Review of the MEDLINE database indicates that the case reported here is the first case of retroperitoneal paraganglioma presenting as isolated right-sided varicocele, as described in the published reports.

Tumors originating from extra-adrenal chromaffin cells are defined as paragangliomas and those from adrenal chromaffin cells as pheochromocytomas. Paraganglioma has an incidence of 2 to 8 per million cases per year10 and is primarily diagnosed between the third and fifth decades of life.11 Most (approximately two thirds) of paragangliomas are seen in the head and neck. Approximately 75% of paragangliomas occupying the sympathetic nervous chain originate from the abdomen.12 Paragangliomas might occur sporadically or as symptoms of several hereditary syndromes. Approximately 25% of cases of pheochromocytoma and paraganglioma are associated with classical hereditary syndromes.10 Multiple endocrine neoplasia type 2 (RET mutations), von Hippel Lindau disease (VHL mutations), hereditary paraganglioma/pheochromocytoma syndrome (succinate dehydrogenase [SDHx] mutations), and, rarely, neurofibromatosis type 1 (NF1 mutations) are some of these syndromes.13 Retroperitoneal paragangliomas should be diagnosed via detection of symptoms caused by hormonal activity, observation of the compression effect of the mass, or incidentally during imaging studies.

Patients with hormonally active tumors might present with headache, sweating, and hypertension associated with severe catecholamine release, symptoms that also present in pheochromocytoma. To date, no consensus regarding the histological criteria for determining malignancy in paraganglioma has been reached. Nevertheless, the most applicable criterion is believed to be metastasis. The most preferred local imaging methods for detecting and examining paraganglioma are currently CT and MRI. MIBG scintigraphy is the preferred imaging method due to its high specificity and sensitivity for examining residual tissues and detecting metastasis. Because of its cost-effectiveness, especially in examining paragangliomas, which are related to SDHx mutations, fluorodeoxyglucose–positron emission tomography (FDG-PET ) is another option for imaging.14 In some series, the 18F-DOPA PET scan was reported superior to MIBG scintigraphy.15 Urinary metanephrine and normetanephrine levels are more significant in determining hormonal activity than plasma catecholamin levels because of their high specificities.

The gold standard treatment for paraganglioma is the aggressive surgical removal of the primer mass and metastatic masses. In hormonally active tumors, the administration of preoperative alpha-blockers is mandatory for preventing intraoperative arrhythmia and hypertension. The use of this treatment option, together with embolization,17 radiotherapy,18 and chemotheraphy19 as adjuvant treatment options, has resulted in an intraoperative mortality rate for paraganglioma of less than 1%.16

In conclusion, isolated right-sided varicocele is a rare pathologic condition that may be accompanied by the compression of the IVC by retroperitoneal masses. Although this pathologic condition is mostly caused by the growth of renal masses and sarcomas, other rare causes, such as paragangliomas, should be considered. While the patient described here was diagnosed with hormonally inactive paraganglioma, preoperative hormonal studies in cases of retroperitoneal masses should be considered due to the risk of intraoperative cardiac complications.