Surgical management of retroperitoneal schwannoma complicated with severe hydronephrosis

Abstract

Rationale:Schwannomas are usually benign tumors arising from well-differentiated schwann cells, which rarely occur in the retroperitoneal space. The lack of specific signs and radiologic imaging characteristics makes preoperative diagnosis rather difficult. Most retroperitoneal schwannomas are benign and the primary treatment choice for retroperitoneal schwannomas is surgical excision, however, the involvement of the urinary system is scarcely reported.Patient concerns:A 34-year-old woman presented with progressive left abdominal pain and rebound abdominal mass at the left lower quadrant for 1 month. Radiological imaging suggested capsulated solid mass with cystic and necrotic areas in the retroperitoneum accompanied by severe left kidney hydronephrosis and preoperative biopsy result was inconclusive.Diagnoses:We believe this is a rare case of retroperitoneal schwannoma complicated with severe hydronephrosis.Interventions:After preparation, the patient underwent laparoscopy exploration and converted to open surgical exploration. The patient accepted complete surgical excision of the retroperitoneal tumor and left kidney. Postoperative pathology diagnosis of the mass was proven to be benign retroperitoneal schwannoma.Outcomes:Postoperative course of the patient was uneventful and the left abdominal pain was greatly improved. After 12-month follow up, no evidence of recurrence or any other complication including renal failure was observed.Lessons:Preoperative imaging and preoperative ultrasound-guided biopsy are helpful to make accurate diagnosis. The final diagnosis is based on postoperative histological and immunohistochemical findings. The primary treatment option is complete surgical resection of the retroperitoneal schwannoma and the involved upper urinary system when severe hydronephrosis occured. Local recurrence and overall survival are closely correlated with negative resection margins and pathology types.

Retroperitoneal schwannoma sandwiched between abdominal aorta and inferior vena cava. A case report

“…However, controversy exists over attaining negative margins [ 8 , 16 ]. Some surgeons recommend en bloc excision with negative margin because malignancy cannot be accurately ruled out in pre-operative biopsy or even during intra-operative frozen section [ 4 ]; while other group of surgeons recommend simple enucleation/ partial excision without extensive resection of adjacent tissue [ 2 , 7 ]. The surgical approach can be either open or laparoscopic [ 17 ].…”

“…Retroperitoneum is the second most common location for occurrence of sarcomas, and it is important to distinguish them from schwannomas so that the patients can be managed conservatively under surveillance [ 3 ]. The imaging techniques (ultrasound, computed tomogram (CT) abdomen, magnetic resonance imaging (MRI) and PET-CT abdomen) and histologic tests may help in pre-operative diagnosis but are not always conclusive [ 4 ]. Likewise, schwannomas at the time of surgery, are generally large (10–20 cm in diameter) because the patients are either asymptomatic or report vague abdominal symptoms [ 5 ].…”

Laparoscopic resection of primary retroperitoneal schwannoma: A case report

“…Schwannomas are rare tumors deriving from Schwann cells found in peripheral nerve sheaths[ 1 - 8 ]; they are often diagnosed in patients’ head, neck and extremities[ 1 - 10 ]. Schwannoma incidence in the retroperitoneal region is quite rare; it accounts for only 0.5% to 5% of all cases of it[ 7 ]. Such incidence prevails among women, mainly in patients in the age group 20-60 years, although it can affect individuals at any other age[ 1 - 10 ].…”

“…Early schwannoma diagnosis is unusual due to lack of specific signs and symptoms capable of characterizing the disease and of leading patients to seek medical help[ 1 - 5 , 7 , 9 - 11 ]. In addition, findings of ultrasound images, computed tomography (CT) and magnetic resonance imaging (MRI) directly linked to the disease are poorly described.…”

“…Thus, many cases are diagnosed incidentally during imaging exams. Advanced stage schwannomas can be associated with abdominal pain or palpable abdominal tumor, and even with atypical symptoms such as secondary hypertension, hematuria, renal colic and headache[ 1 - 7 , 9 , 11 ]. Schwannomas are often benign; however, they can lead to malignant tumor transformation when they are associated with Von Recklinghausen’s disease[ 1 , 2 , 4 , 6 , 7 ].…”