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Its occurrence in an adrenal gland is unusual.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Most of these tumors are benign, with malignant ones being more common in cases of Von Recklinghausen’s disease. They are more prevalent among women than men (1.2:1), and the mean age of onset is 49 years. Most are discovered as incidental findings, and their symptoms seem to be related to the size of the tumor, such as abdominal pain caused by the mass effect. Hormonal test findings tend to be normal. Computed tomography (CT) images tend to show a well-defined, encapsulated, homogeneous mass and magnetic resonance imaging (MRI) reveals a heterogeneous mass, with either cystic degeneration or hemorrhagic areas with a hypointense T1 signal and a hyperintense T2 signal, which, therefore, lead to a differential diagnosis of pheochromocytoma or adrenal carcinoma.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We hereby present the case of a 55-year-old woman with hypertransaminasemia secondary to alcohol consumption. A requested MRI revealed a heterogeneous, left adrenal mass with a diameter of 3.5 × 3 cm, with some irregular areas of hyperintense T2 signal and others of hypointense cystic degeneration/central necrosis. The lesion contained no adipose or hemorrhagic material, and exhibited no signs of invasion of the adjacent structures or adenopathies. A differential diagnosis of either pheochromocytoma or adrenal carcinoma was proposed as the main option.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was referred to the Endocrinology Department for further examination. She reported a three-year history of arterial hypertension, with a worse control of her blood pressure levels over the previous year (mean 160/100 mmHg), owing to which she is currently under treatment with two antihypertensive drugs. A hormonal study was requested to determine the concentrations of renin mass, aldosterone, urinary free cortisol, plasma cortisol after administering 1 mg of dexamethasone, urinary metanephrines, dehydroepiandrosterone sulphate, total testosterone, androstenedione, and 17-hydroxyprogesterone, all of which fell within normal limits.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was subsequently referred to the Surgery Department to undergo a left adrenalectomy and reach a definitive pathological diagnosis. This procedure was performed laparoscopically in March 2017, with the pathological study of the samples obtained yielding a diagnosis of a schwannoma limited to the adrenal gland. The macroscopic description was reported as an adrenal gland measuring 8.5 × 4 × 3 cm, with a 3.5 × 3.2-cm nodule delimited by a capsule. The nodule corresponded to a solid mass with whitish-grayish cystic areas. The microscopic description reported a tumor in the adrenal medulla that was displacing its cortex and was comprised by a proliferation of spindle cells with a fascicular pattern, elongated nuclei, and an ill-defined cytoplasm, with areas of increased cellularity, nuclear “pseudo-palisading”, myxoid areas with poor cellular representation, and blood vessels with thick and hyalinized walls. Areas of ischemia, cystic degeneration, and polymorphous nuclei without mitosis were also observed. The mitotic index was less than 1 mitosis/10 high power fields (HPFs), and the Ki-67 index was 5%. In addition, an immunohistochemistry study revealed a positive result for the S-100 protein and a negative one for actin, caldesmon, desmin, AE1-AE3, and inhibin.</p><p id="par0025" class="elsevierStylePara elsevierViewall">After the procedure, the patient was re-examined in the Endocrinology Department with the aid of a Holter blood pressure device, which revealed mean blood pressure levels of 107/74 mmHg and, therefore, a need for decreasing her antihypertensive medication. A physical examination revealed no “<span class="elsevierStyleItalic">café au lait</span>” spots, but several axillary freckles were detected in a region where, according to the patient, several, small, pedunculated, benign tumors had previously been removed. The corresponding pathology report could not be retrieved from her medical history.</p><p id="par0030" class="elsevierStylePara elsevierViewall">To conclude, schwannomas are solid, encapsulated tumors, frequently presenting with areas of bleeding, necrosis, and cysts, which are generally located in the cranial and cervical cutaneous nerves, with their presence in the retroperitoneum and adrenal glands being rare. Because CT or MRI images rarely allow their preoperative differentiation from pheochromocytomas or adrenal carcinomas,<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> preoperative hormonal testing is advised to rule out an increase in metanephrine levels requiring prior treatment with alpha-blockers. Only a single case of a schwannoma associated with increased levels of urinary catecholamines in the laboratory tests has been described. Pathological studies often reveal hemorrhagic and cystic areas within the tumors. In addition, despite not being pathognomonic, positivity for the S-100<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> protein in the immunohistochemistry studies is a fundamental finding. The clinical evolution of this condition is generally benign, except in cases associated with Von Recklinghausen’s fibromatosis, where malignancy is more frequent.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The patient was also examined in the Ophthalmology Department to have an optic glioma ruled out owing to the suspected diagnosis of type 1 neurofibromatosis, although she did not fulfill the criteria for genetic testing.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Fuentes C, Garaizabal L, Pérez O. Schwannoma suprarrenal: descripción de un caso. Med Clin (Barc). 2021;157:91–92.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary adrenal schwannoma: a series of 31 cases emphasizing their clinicopathologic features and favorable prognosis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Zhou" 1 => "D. Zhang" 2 => "W. Li" 3 => "L. Zhou" 4 => "H. Xu" 5 => "S. Zheng" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s12020-019-01992-z" "Revista" => array:6 [ "tituloSerie" => "Endocrine." 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Letter to the Editor
Adrenal schwannoma: A case report
Schwannoma suprarrenal: descripción de un caso
Clara Fuentes
, Leire Garaizabal, Octavio Pérez
Corresponding author
Servicio de Endocrinología, Hospital Universitario Araba, Vitoria-Gasteiz, Spain