Photographic Journey of Cushing Syndrome
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PICTURE OF THE MONTH
Photographic Journey of Cushing Syndrome Jaivinder Yadav 1 & Saniya Gupta 1 & Rakesh Kumar 1 & JK Mahajan 2 & Kushaljit Sodhi 3 & Balamurugan Thirunavukkarasu 4 & Devi Dayal 1 Received: 22 June 2020 / Accepted: 4 September 2020 # Dr. K C Chaudhuri Foundation 2020
Cushing syndrome (CS) occurs due to excessive secretion of cortisol either secondary to Adrenocorticotropic hormone (ACTH) or independent of it. It manifests clinically as facial plethora, moon like facies, buffalo hump, skin striae, excess weight gain and height retardation. Biochemical estimation of hormones such as cortisol, ACTH and imaging of pituitary and adrenals helps in localizing the cause for CS [1, 2]. A patient presented with excessive weight gain and cushingoid features at the age of 3 mo. The patient was diagnosed with adrenocortical carcinoma and the manifestations resolved after surgical resection. Figure 1 (a-f) shows the clinical features of the patient and Fig. 1 (g-k) displays the radiological and laboratory features of the lesion.
CS in infancy and childhood age usually occurs secondary to exogenous steroid administration. Endogenous CS is exceedingly rare, and the causes include adrenocortical tumors and benign bilateral adrenocortical hyperplasia. Genetic defects like TP53 mutation and cyclic -AMP protein kinase pathways predispose to adrenocortical tumors. Complete surgical resection of tumor is curative, and the age of onset, volume of mass and the presence of metastasis are major prognostic factors. The patient requires continuous follow-up for years to detect tumor recurrence and screening for extraadrenal malignancies in patients with genetic variations for tumor predisposition [3–5].
* Jaivinder Yadav [email protected]
2
Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3
Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
4
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
1
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
Indian J Pediatr
Fig. 1 (a) Normal appearance at birth (b & c) Cushingoid facies, excessive skin folds with central obesity at 2 mo and 3 mo of age. (d & e) Resolution of features of Cushing syndrome at 5 mo and 10 mo of age (f) Normal appearance at 1.5 y of age. (g) Antenatal ultrasonography revealed Right adrenal gland hypoechoic lesion. (h & i) CT and PET
CT showing a large (6.4X4.9X4.6 cm) right adrenocortical tumor (arrows) (j) A friable grey white tumor with areas of hemorrhage. (k) Section shows round to oval monomorphic polygonal shaped tumor cells in trabeculae, nests, and solid sheets
Compliance with Ethical Standards
3.
Consent Information The consent had been taken for all the photographs of the child.
4.
Conflict of Interest None.
References 1. 2.
Stratakis CA. An update on Cushing syndrome in pediatrics. Ann Endocrinol (
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