Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.

Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a high-grade malignant neoplasm commonly affecting bones of the thoracic region. Primary Ewing sarcoma/PNET of the kidney is exceptional; it commonly affects young adults and is rarely reported in children. Here we describe a case of renal PNET in a 10-year-old girl who presented at our institute with haematuria and abdominal lump. Computer tomography scan revealed a huge mass in the right kidney. Computer tomography-guided aspiration from the mass showed cytomorphological features of a small blue round cell tumor. The patient underwent right radical nephrectomy. Histopathology with supplementary immunohistochemistry confirmed the diagnosis of PNET. She has been receiving treatment with adjuvant chemotherapy post-surgery and is currently disease free. Primary renal PNET is a distinct and rare entity. This tumor is very aggressive with low survival rate, even with a multi-disciplinary approach. We reported the case because it is rare in children, poses diagnostic challenges, is aggressive in behaviour and responds poorly to treatment.

Benign Fibrous Histiocytoma: An Uncommon Presentation.

Intracranial fibrous histiocytomas are rare; Benign Fibrous Histiocytoma (BFH) being uncommon than its malignant counterpart. BFH comprises fibroblasts and histiocytes without any nuclear pleomorphism or atypia. We present a case of a 42-year-old male who had swelling over the occipital region for the past five years, which progressively increased in size. He developed headache, dizziness, and gait disturbance over the last six months. Computed tomographic scan revealed a posterior fossa space-occupying lesion. Fine-needle aspiration cytology from the swelling revealed spindled fibroblasts along with histiocytes and multinucleated giant cells. Later, histopathology showed presence of spindle-shaped cells in storiform pattern admixed with histiocytes and giant cells. The giant cells and histiocytes were immunopositive for CD68 and spindled cells were positive for vimentin, but immunonegative for CD34, epithelial membrane antigen, CD1a and S100. The final diagnosis was intracranial BFH. We present this case because of its extreme rarity and unusual location.

Ectopic Paratubal Adrenal Cell Rest Associated with Mucinous Cystadenoma of Ovary.

Ectopic adrenal cortex is a rare entity. Usually found in male children; commonly located around kidney, retroperitoneum, spermatic cord and para-testicular region. Rarely, adults with heterotopic adrenal glands are described. Incidence in females is very less; though sometimes detected accidentally in hysterectomy specimens. We describe a case of ectopic adrenal cortical cell in paratubal region in a patient with mucinous cyst adenoma of ovary. A 26-year-old female presented with complains of menstrual irregularities and abdominal discomfort for 6 months. Investigations suggested a right ovarian cyst. Right ovarian cystectomy with partial salpingectomy was performed; histopathology revealed mucinous cyst adenoma. Sections from tube showed presence of ectopic adrenal cortical rest in the paratubal region, incidentally discovered on microscopy. We present this case because of its rarity in females, interesting presentation with another unrelated gynaecological pathology, its potentiality for malignant transformation and possible complications.