Retroperitoneal ganglioneuroma presenting as lower back pain

Abstract

Retroperitoneal ganglioneuroma (RGN) is a rare benign tumor that arises from the retroperitoneal sympathetic ganglia composed of mature Schwann cells, ganglion cells and nerve fibers. These tumors can occur anywhere along the paravertebral sympathetic plexus and occasionally from adrenal medulla. Although they grow in excessive size, they may cause compression to adjacent organ or structures thus giving rise to symptoms. Resecting RGN’s is a challenging endeavor, as they tend to encase neighboring vessels to their site of origin. The reported case is a 43-year-old male who presented with lumbar pain that increased progressively in intensity over the last 6 months. Preoperative investigations revealed a large tumor with encasement of the origins of the superior mesenteric artery and bilateral renal arteries. The tumor was completely resected and the final pathology confirmed the diagnosis of RGN.

Large retroperitoneal ganglioneuroma revealed by a left ovarian endometrioma: A case report

“…Nevertheless, site-specific symptoms may manifest as the tumor enlarges, compressing and displacing adjacent organs and vessels, with prevalent symptoms including abdominal back discomfort and abdominal distension. 5 , 6 While GNs are generally nonsecretory tumors, some instances involve the secretion of catecholamine and/or vasoactive intestinal polypeptides, leading to symptoms such as hypertension and diarrhea. 6 , 7 …”

“… 5 , 6 While GNs are generally nonsecretory tumors, some instances involve the secretion of catecholamine and/or vasoactive intestinal polypeptides, leading to symptoms such as hypertension and diarrhea. 6 , 7 …”

“…Imaging methods are crucial for surgical planning, providing insights into the tumor’s size, origin organ, local expansion, and conjunction with surrounding organs and arteries. 2 , 4 , 6 However, it is imperative to note that the misinterpretation of a benign neuroblastic tumor as a sarcoma by a CT scan has been documented, underscoring the inadequacy of imaging features in providing an accurate diagnosis for this condition.…”

“…Besides, the presence of adipose cells in these tumors, previously reported in the literature and due to the lack of distinguishing characteristics, may make it difficult to differentiate between GNs and other aggressive tumors using cytology that leads to delay a proper treatment. 6 Consequently, histopathological examination of the specimen remains the sole specific test for a conclusive diagnosis of GN, identifying an admixture of ganglion cells and Schwann cells without immature elements. 6 , 7 …”

Ganglioneuroma presents as the absence of sweating on the face and the opposite trunk in a child

“…3 GNs more commonly occur in children, which are occasionally found during physical examination and may present generally with chest pain, low back pain, abdominal pain, and limb numbness; a few patients may develop Horner's syndrome, characterized by sweatlessness on the affected side. 4,5 However, this case emphasizes that mediastinal GN should not be overlooked to avoid false or missed diagnoses in children who experienced gradual aggravation or are at high risk.…”