Konuş Ali Hakan, Uğur Kader, Aygen Erhan, Tektaş Cihat, Durumlu Fatih, Çalık Muhammet
Department of Cardiology, Bingöl State Hospital, Bingöl, Türkiye.
Department of Endocrinology, Faculty of Medicine, Fırat University, Elazığ, Türkiye.
Front Cardiovasc Med. 2025 Mar 4;12:1541880. doi: 10.3389/fcvm.2025.1541880. eCollection 2025.
Giant cystic pheochromocytoma and giant adrenal myelolipoma are two highly uncommon masses. There are difficulties in diagnosis and management of both types of giant (>20 cm) adrenal tumors.
CASE 1: A 56-year-old male patient applied with complaints of headache and high blood pressure. A mass was palpated in the left upper quadrant. The average 24-hour ambulatory blood pressure was 146/93 mm Hg. Computed tomography revealed a huge left adrenal cystic mass measuring 22 × 17 cm. A large left renal parapelvic cyst measuring 6 × 5.5 cm was also observed. Urine metanephrine and normetanephrine values were high. The patient was diagnosed with pheochromocytoma. It was decided to remove the adrenal cystic mass and renal parapelvic cyst with open surgery. Severe hypotension occurred during the intraoperative and early postoperative periods, and severe orthostatic dizziness occurred during the in-hospital stay and two months of outpatient follow-up. The patient's urine metanephrine and normetanephrine levels returned to normal. The average 24 h ambulatory blood pressure was 122/69 at six months.
CASE 2: A 53-year-old male patient was admitted with complaints of high blood pressure, accompanied by mild headache and dizziness that had been ongoing for several months. A mass was palpated in the right upper quadrant. The average 24 h ambulatory blood pressure was 151/91 mm Hg. Abdominal computed tomography revealed a 24 × 16 × 22 cm solid mass with diffuse fat density originating from the right adrenal gland. Laboratory studies and endocrine investigations were normal. With the diagnosis of adrenal myelolipoma, a mass weighing 4,229 g was surgically removed. The patient was normotensive without medical treatment during the two-year follow-up after the operation.
To our knowledge, our case of giant cystic pheochromocytoma accompanied by ipsilateral large renal parapelvic cyst, which is the first in the literature, reports the management of severe hypotension in the perioperative period and severe orthostatic dizziness in the two-month follow-up. Non-functional adrenal myelolipomas can cause hypertension with mass effect. Our second case is one of the largest adrenal myelolipomas in literature. Although surgical removal of giant masses is difficult, successful surgeries have resulted in resolution of hypertension in our cases at mid-term follow-up.
巨大囊性嗜铬细胞瘤和巨大肾上腺髓质脂肪瘤是两种极不常见的肿块。这两种类型的巨大（>20 cm）肾上腺肿瘤在诊断和治疗方面都存在困难。
病例1：一名56岁男性患者因头痛和高血压前来就诊。左上腹可触及一个肿块。24小时动态血压平均值为146/93 mmHg。计算机断层扫描显示左肾上腺有一个巨大的囊性肿块，大小为22×17 cm。还观察到左肾旁较大的肾盂旁囊肿，大小为6×5.5 cm。尿间甲肾上腺素和去甲间肾上腺素值升高。患者被诊断为嗜铬细胞瘤。决定通过开放手术切除肾上腺囊性肿块和肾旁囊肿。术中及术后早期出现严重低血压，住院期间及门诊随访两个月期间出现严重直立性头晕。患者的尿间甲肾上腺素和去甲间肾上腺素水平恢复正常。六个月时24小时动态血压平均值为122/69。
病例2：一名53岁男性患者因高血压前来就诊，伴有持续数月的轻度头痛和头晕。右上腹可触及一个肿块。24小时动态血压平均值为151/91 mmHg。腹部计算机断层扫描显示一个起源于右肾上腺的大小为24×16×22 cm的实性肿块，密度弥漫性脂肪样。实验室检查和内分泌检查均正常。诊断为肾上腺髓质脂肪瘤，手术切除了一个重4229 g的肿块。术后两年随访期间，患者未经药物治疗血压正常。
据我们所知，我们报道的巨大囊性嗜铬细胞瘤伴同侧巨大肾旁囊肿病例为文献首例，报道了围手术期严重低血压及两个月随访期严重直立性头晕的处理情况。无功能肾上腺髓质脂肪瘤可因占位效应导致高血压。我们的第二个病例是文献中最大的肾上腺髓质脂肪瘤之一。虽然切除巨大肿块手术困难，但在我们的病例中，中期随访时成功手术使高血压得到缓解。
