Academic Journal
First report of ectopic ACTH syndrome and PTHrP-induced hypercalcemia due to a hepatoblastoma in a child
| العنوان: | First report of ectopic ACTH syndrome and PTHrP-induced hypercalcemia due to a hepatoblastoma in a child |
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| المؤلفون: | Grunewald, Thomas G P, von Luettichau, Irene, Welsch, Ulrich, Dörr, Helmuth-Günther, Höpner, Frank, Kovacs, Kalman, Burdach, Stefan, Rabl, Wolfgang |
| المصدر: | European Journal of Endocrinology ; volume 162, issue 4, page 813-818 ; ISSN 0804-4643 1479-683X |
| بيانات النشر: | Oxford University Press (OUP) |
| سنة النشر: | 2010 |
| الوصف: | Context Only occasionally, endocrine-active tumors develop directly from hepatic tissue, and may lead to paraneoplastic syndromes (PNS). PNS mostly accompany malignancy of adulthood and are exceedingly rare in children. Patient A girl aged 6 years and 9 months presented with a 2-month history of rapidly progressive weight gain, abdominal distension, and polyuria/pollakiuria accompanied by short episodes of abdominal pain. She showed the typical clinical features of Cushing's syndrome and a huge hepatic mass. An abdominal computed tomography (CT) scan revealed a large liver tumor. Blood glucose and serum calcium were greatly elevated. Design and objective Case report describing the causative relationship of the clinical findings. Methods Physical examination; ultrasound of the abdomen; CT scan of the abdomen and the chest; conventional X-rays; routine hematology; blood chemistry and multiple parameters of calcium and phosphorus metabolism; multisteroid analysis in serum and urine; adrenocortical stimulation and suppression tests; histopathological assessment of the resected tumor; immunohistochemistry for ACTH, β-endorphin, corticotrophin-releasing hormone (CRH), and PTH-related peptide (PTHrP); electron microscopy of tumor cells; ACTH and CRH extraction from the tumor tissue; and clinical follow-up for more than 20 years. Results Giant hepatoblastoma (HB; ∼1000 ml volume) of the right lobe of the liver with combined ectopic ACTH syndrome and PTHrP-induced tumor-associated hypercalcemia. Wide local excision and polychemotherapy led to complete reversal of the paraneoplastic phenotype. Conclusions This is the first report of an endocrine-active HB causing both Cushing's syndrome and PTHrP-related ‘humoral hypercalcemia of malignancy’. This information should be added to the well-known β-human chorionic gonadotropin-related paraneoplastic effects of HB in children. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | unknown |
| DOI: | 10.1530/eje-09-0961 |
| الاتاحة: | http://dx.doi.org/10.1530/eje-09-0961 https://eje.bioscientifica.com/view/journals/eje/162/4/813.xml |
| رقم الانضمام: | edsbas.8592948E |
| قاعدة البيانات: | BASE |
| DOI: | 10.1530/eje-09-0961 |
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