Surgery without Blood Transfusion for Giant Paraganglioma in a Jehovah's Witness Patient
In February 2011, a retroperitoneal tumor was suspected by a health check in a 57-year-old Japanese woman and subsequent examination led to the suspicion of a right pheochromocytoma. As the patient was a Jehovah's Witness, she wished to undergo bloodless surgery and consulted our hospital in June 2011 after being refused surgery by several hospitals.\nThe characteristics of the patient recorded at the time of hospital admission were as follows: height: 154 cm, weight: 56 kg, blood pressure: 115/65 mm Hg and heart rate: 78 bpm and regular. The tumor was palpable in the right hypochondrium. There were no physical signs of Cushing's syndrome such as moon facies or buffalo hump. There were no abnormal findings in the peripheral blood and biochemical tests. Endocrine tests revealed high levels of urinary normetanephrine (1.60 mg/day). There were no abnormal findings in any other items, including blood adrenaline, cardiac function, and pulmonary function.\nAbdominal contrast-enhanced computed tomography (CT) revealed a retroperitoneal tumor (size: 12 cm) touching the upper pole of the right kidney with an uneven contrast-enhanced effect inside the tumor. The tumor had ill-defined borders with the surrounding liver, right kidney, inferior vena cava, right renal artery and vein, and left renal vein, which indicated adhesion (fig. ). There was no clear lymph node metastasis or distal metastasis on imaging. The tumor had a low signal intensity on T1-weighted magnetic resonance images and high signal intensity on T2-weighted magnetic resonance images. Marked accumulation was found on 131I-metaiodobenzylguanidine scintigraphy.\nThe above findings were suggestive of a right adrenal pheochromocytoma. Abdominal contrast-enhanced CT findings revealed that tumorectomy required a right renal combined resection, sectioning of the right renal artery and vein, left renal vein, and inferior vena cava, and detachment of the tumor from the lower hepatic surface. Despite explaining the importance of blood to the patient once again as an outpatient, she did not give consent. However, we were able to gain her consent for the use of albumin preparations, intraoperative diluted autotransfusions, intraoperative recovery-type autotransfusions, the use of a heart-lung machine, and hemodialysis. A preoperation conference was held with the four hospital departments including anesthesiology, gastrointestinal surgery (because of the detachment of the tumor from the liver), and cardiovascular surgery (extracorporeal circulation is required when sectioning the inferior vena cava). The type of surgical procedure was confirmed, and the risks involved were sufficiently discussed. Then, the patient and her friends were provided the details of the surgery. The patient also acknowledged the risk of bleeding to death because of bloodless surgery, loss of renal function because of the removal of the right kidney and left renal vein, the possibility of hemodialysis, the risk of partial liver removal, and eventual exploratory laparotomy. After the patient understood all risks and consented, a written consent and waiver of liability for blood transfusion refusal were signed. In accordance with the surgical policy, an erythropoietin preparation, iron, and doxazosin mesylate were administered prior to surgery.\nAfter the diagnosis of right pheochromocytoma, open surgery was conducted with an abdominal midline incision in August 2011. During operation, the tumor was found adhered to the peritoneum, diaphragm, lower hepatic surface, inferior vena cava, and right renal vein. It engulfed the right renal artery and vein.\nFirst, the adhesion site of the tumor was detached from the lower hepatic surface. Partial hepatic resection was not necessary. Then, the anterior surface of the tumor was detached from the left renal vein, and the left renal vein was secured. Next, the right renal artery was tied, and blood flow to the right kidney was blocked. A clamp test was performed on the inferior vena cava and left renal vein, and as there were no changes in blood pressure, the bypass was considered adequate. Next, the tumor was very carefully detached from the inferior vena cava, which was tied to protect the right renal vein as tumor detachment progressed. Lastly, the tumor vessels flowing into the inferior vena cava were tied, and both the right kidney and tumor were removed. The inferior vena cava and left renal vein were preserved. The operative times were 8 h and 18 min and blood loss was 1,770 ml.\nThe resected specimen (size: 10.5 × 11.9 cm) was macroscopically brown in appearance with multiple cysts. There was no continuity of the tumor in the normal adrenal gland, and it was diagnosed as paraganglioma (fig. ).\nOn histological findings, the tumor consisted of a hyperplasia of cells with basophilic cytoplasm and was arranged in an alveolar pattern around fibrous blood vessels (fig. ). Histologically, there was no clear continuity of the tumor in the adrenal gland tissue, and an extra-adrenal paraganglioma was diagnosed. No invasion into the right adrenal gland or right kidney was observed.\nFollowing surgery, the patient progressed without any major changes in blood pressure or other complications and the high urinary normetanephrine level returned to normal. After discharge, the patient continues to have follow-ups at her home hospital.