Two subsequent metachroneus solid tumors: Oncocytic variant adrenocortical carcinoma and rhabdomyosarcoma of childhood: Case report and Literature Review.

Two subsequent metachroneus solid tumors: Oncocytic variant adrenocortical carcinoma and rhabdomyosarcoma of childhood: Case report and Literature Review. J Clin Res Pediatr Endocrinol. 2020 Jun 16;: Authors: Akin O, Atas E, Atasoy İA, Durmaz N, Kartal Ö Abstract 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 was presented. An 18-month-old-boy was admitted with virilization of the genital area, penis enlargement, and erection which had begun six months ago. Serum total testosterone: 457 ng/dl (<10), androstenedione: 3.35ng/ml (<0.5) and DHEA-SO4: 206 mcg/dl (<35) were measured higher than normal ranges. Right adrenal mass was detected. After adrenalectomy, histopathological examination revealed oncocytic variant ACC. Three-month later from surgery, the child then presented with 6x8 cm sized swelling of the left leg. Histopathological examination revealed embryonal RMS. Testing for tumour protein (TP53) variant by DNA sequence analysis was positive; however; FISH analysis was negative. After chemotherapy and local radiotherapy, the patient is in good condition without tumour recurrence. Only about one-third of these tumours have a variant of TP53. This status also applies to other genetic variants related to cancer. However, a sign...
Source: JCRPE Journal of Clinical Research in Pediatric Endocrinology - Category: Endocrinology Tags: J Clin Res Pediatr Endocrinol Source Type: research