Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty:A case report and review of literature / Journal of the ASEAN Federation of Endocrine Societies

@#Oncocytic adrenocortical tumours (OATs) or oncocytomas are extremely rare and are usually benign and nonfunctional. We report the case of a 4-year-old male with a right-sided, functional oncocytic adrenocortical adenoma, who presented with precocious puberty and Cushing’s syndrome. After work-up, the patient underwent laparoscopic adrenalectomy. The excised adrenal mass weighed 21 g and measured 3.5 cm in maximum dimension. Histological examination demonstrated no features suggestive of aggressive biological behaviour. The patient had no features of recurrent or metastatic disease and had prepubertal testosterone levels with suppressed hypothalamic-pituitary-adrenal axis twelve months after the surgery. A discussion of this case and a review of the literature on functional OATs in the pediatric population are presented.

Basal like carcinoma of breast in patient with neurofibromatosis I: An association or co-existence.

Neurofi bromatosis I (NF I), an autosomal dominant disorder is associated with increased risk of benign and malignant peripheral nerve sheath tumors and central nervous system tumors. There are only few case reports of breast carcinoma in known patients of NF I. We report a case of basal like carcinoma of the breast in a 69-year-old lady who had NF I. Considering the rare association of carcinomas with NF I and fi nding that both the NF I gene and a breast cancer pre-disposition gene, BRCA 1 are located in close proximity on chromosome 17q makes the association of these two conditions intriguing.

Spontaneous regression of a congenital melanocytic nevus.

Congenital melanocytic nevus (CMN) may rarely regress which may also be associated with a halo or vitiligo. We describe a 10-year-old girl who presented with CMN on the left leg since birth, which recently started to regress spontaneously with associated depigmentation in the lesion and at a distant site. Dermoscopy performed at different sites of the regressing lesion demonstrated loss of epidermal pigments first followed by loss of dermal pigments. Histopathology and Masson-Fontana stain demonstrated lymphocytic infiltration and loss of pigment production in the regressing area. Immunohistochemistry staining (S100 and HMB-45), however, showed that nevus cells were present in the regressing areas.

Significance of p53 expression in ovarian tumors and its correlation to the morphological differentiation.

Cancer of breast and cervix reign supreme among the fatal diseases in women globally. Interestingly the ovarian cancer has the highest mortality rate. This is attributed to its late clinical presentation and delayed diagnosis. The expression of p53 throws light on the prognosis of ovarian tumors. The surface epithelial tumors, especially the serous cystadenocarcinoma and non Hodgkin lymphoma of ovary where in the expression p53 was high compared to the benign and borderline tumors. Further the expression of p53 in tumors arising from germinal cells, sex-cord stromal cells are observed to be very low. The expression of p53 and its correlation to the morphology enhances the prognostic significance.