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首页> 外文期刊>Journal of Clinical Research in Pediatric Endocrinology >Two Subsequent Metachroneus Solid Tumors: Oncocytic Variant Adrenocortical Carcinoma and Rhabdomyosarcoma of Childhood: Case Report and Literature Review
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Two Subsequent Metachroneus Solid Tumors: Oncocytic Variant Adrenocortical Carcinoma and Rhabdomyosarcoma of Childhood: Case Report and Literature Review

机译：两种后续甲状腺类固体肿瘤：儿童生物变异肾上腺皮质癌和童年的横纹肌瘤：病例报告和文献综述

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Most cases of malignancies appear to be sporadic, but some syndromes are associated with malignancies with germline variants. Herein, a child with an unusual association of oncocytic variant adrenocortical carcinoma (ACC) and rhabdomyosarcoma (RMS) was presented. An 18-month-old-boy was admitted with virilization of the genital area, penis enlargement and erection, which had begun six months earlier. Serum total testosterone (457 ng/dL; NR &10), androstenedione (3.35 ng/mL; NR &0.5) and dehydroepiandrosterone- SO4 (206 mcg/dL; NR&35) were above the normal ranges. Right adrenal mass was detected. After adrenalectomy, histopathological examination revealed an oncocytic variant ACC. Three-month after surgery, he then presented with 6x8 cm sized swelling of the left leg. Histopathological examination revealed embryonal RMS. Testing for tumor protein (TP53) variant by DNA sequence analysis was positive; however; fluorescence in situ hybridization analysis was negative. After chemotherapy and local radiotherapy, the patient is in good condition without tumor recurrence. Only about one-third of these tumors have a variant of TP53. This status also applies to other genetic variants related to cancer. However, a significant association of malignancies strongly suggests a problem in tumor suppressor genes or new variants. Another as yet unidentified suppressor gene may also be present and effective in this locus. The occurrence of ACC as a part of a syndrome and positive family history of malignancies in patients are clinically important. These patients and their families should be scanned for genetic abnormalities. The patient with ACC should be followed-up carefully for other tumors to detect malignancy early.
机译：大多数恶性肿瘤似乎都是散发性的，但有些综合征与具有种系变体的恶性肿瘤有关。这里，提出了一种具有异常变异肾上腺皮质癌（ACC）和横纹肌糖瘤（RMS）的异常关联的儿童。一个18个月大的男孩在六个月前开始，阴茎扩大和勃起的激发区域，阴茎扩大和勃起。血清总睾酮（457 ng / d1; NR＆＆amp; 10），androstentione（3.35 ng / ml; nr＆amp; 0.5）和脱氢哌啶-O4（206mcg / dl; nr＆amp; 35）高于正常范围。检测到右肾上腺质量。肾上腺切除后，组织病理学检查显示了一种鼻咽炎变异。手术后三个月，他随后呈现左腿6x8厘米尺寸的肿胀。组织病理学检查揭示了胚胎rms。 DNA序列分析对肿瘤蛋白（TP53）变体进行阳性;然而;原位杂交分析的荧光是阴性的。化疗和局部放疗后，患者处于良好的状态，没有肿瘤复发。只有约三分之一的这些肿瘤具有TP53的变体。该状态也适用于与癌症相关的其他遗传变异。然而，重大的恶性肿瘤关联强烈表明肿瘤抑制基因或新变种的问题。尚未识别的抑制基因也可以存在并有效地存在于该基因座中。 ACC作为综合征和患者恶性肿瘤阳性家族史的一部分的发生在临床上是重要的。应扫描这些患者及其家庭以遗传异常。应仔细仔细随访患者，以便早期发现恶性肿瘤。

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