Cerović Snezana, Cizmić Milica, Milović Novak, Ajdinović Boris, Brajusković Goran
Institute of Pathology, Military Medical Academy, Belgrade.
Srp Arh Celok Lek. 2002 Jul;130 Suppl 2:47-51.
Adrenal incidentalomas are a heterogeneous group of pathological entities, including benign or malignant adrenocortical or medullary tumors, hormonally active or inactive lesions, which are identified incidentally during the examination of nonadrenal-related abdominal complaints. About 1.5% to 23% of adrenal incidentalomas are pheochromocytomas. Composite pheochromocytoma is a rare tumour of adrenal medulla with divergente clinical course. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether pheochromocytoma and nonpheochromocytoma components show the same embryologic origin. Nonpheochromocytoma components found in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The biologic behavior of composite pheochromocytomas may be as difficult to predict as more traditional pheochromocytomas; based on the number of cases reported to date the presence of areas resembling ganglioneuroblastoma or neuroblastoma does not necessary indicate a poor prognosis. Some may behave in a malignant fashion with metastasis by a component of the tumour which has neural features. Pheochromocytomas and paragangliomas are well-defined entities. Some of their nonsporadic associations and unusual morphological appearances are not universally appreciated. We report on a rare association of left adrenal CP, with typical right adrenal phochromocytoma and retroperitoneal paraganglioma, and a review of literature. We analyzed the clinical and immunohistochemical features in a 24-year-old woman with composite pheochromocytoma localized in the left adrenal gland and associated with blood pressure of 200/140 mmHg. Abdominal computed tomography and 131-J MIBG revealed a 65 x 60 mm mass in the right adrenal gland, but no revealed 45 x 40 mm retroperitoneal mass and 20 x 20 mm mass in the left adrenal region. Serum and urinary adrenaline levels were high, and catecholamine levels in the blood sample of the selective adrenal vein, were also high. Bilateral adrenalectomy and retroperitoneal mass were surgically removed without complications. Clinical symptoms were absent 6 years after surgery. After surgery the patient gave birth to two healthy babies. Immunohistochemical analyses revealed that tumour cells of right adrenal pheochromocytoma and retroperitoneal paraganglioma were strongly positive for neurone specific enolase, synaptophisin and chromogranin A. The left adrenal tumour showed pheochromocytoma, ganglioneuroma and neuroblastoma components. Immunoreactivity of this tumour added several features to the wide immunohistochemical spectrum. This case demonstrates the indolent behavior of sporadic-type CP and retroperitoneal paraganglioma in an adult patient. Unusual morphological features of CP occur in a substantial number of cases and may cause diagnostic problems.
肾上腺偶发瘤是一组异质性的病理实体，包括良性或恶性肾上腺皮质或髓质肿瘤、有或无激素活性的病变，这些病变是在检查非肾上腺相关的腹部疾病时偶然发现的。约1.5%至23%的肾上腺偶发瘤为嗜铬细胞瘤。复合型嗜铬细胞瘤是一种罕见的肾上腺髓质肿瘤，临床病程多样。根据嗜铬细胞瘤和非嗜铬细胞瘤成分是否具有相同的胚胎起源，这种类型的嗜铬细胞瘤被称为“复合型”或“混合型”。复合型嗜铬细胞瘤中发现的非嗜铬细胞瘤成分包括神经节瘤、神经节神经母细胞瘤、神经母细胞瘤和恶性神经鞘瘤。复合型嗜铬细胞瘤的生物学行为可能与更传统的嗜铬细胞瘤一样难以预测；根据迄今为止报道的病例数量，出现类似神经节神经母细胞瘤或神经母细胞瘤的区域并不一定预示预后不良。有些可能表现为恶性，肿瘤具有神经特征的成分会发生转移。嗜铬细胞瘤和副神经节瘤是明确的实体。它们的一些非散发性关联和不寻常的形态表现并未得到普遍认识。我们报告了一例罕见的左肾上腺复合型嗜铬细胞瘤，伴有典型的右肾上腺嗜铬细胞瘤和腹膜后副神经节瘤，并对文献进行了综述。我们分析了一名24岁女性复合型嗜铬细胞瘤的临床和免疫组化特征，该肿瘤位于左肾上腺，伴有200/140 mmHg的血压。腹部计算机断层扫描和131-J MIBG显示右肾上腺有一个65×60 mm的肿块，但未发现腹膜后45×40 mm的肿块和左肾上腺区域20×20 mm的肿块。血清和尿肾上腺素水平升高，选择性肾上腺静脉血样中的儿茶酚胺水平也升高。双侧肾上腺切除术和腹膜后肿块被手术切除，无并发症。术后6年临床症状消失。术后患者生下两个健康婴儿。免疫组化分析显示，右肾上腺嗜铬细胞瘤和腹膜后副神经节瘤的肿瘤细胞对神经元特异性烯醇化酶、突触素和嗜铬粒蛋白A呈强阳性。左肾上腺肿瘤显示有嗜铬细胞瘤、神经节瘤和神经母细胞瘤成分。该肿瘤的免疫反应性为广泛的免疫组化谱增加了几个特征。本病例显示了成人患者散发性复合型嗜铬细胞瘤和腹膜后副神经节瘤的惰性行为。复合型嗜铬细胞瘤的不寻常形态特征在相当数量的病例中出现，可能会导致诊断问题。
