Atypical Teratoid/ Rhabdoid Tumor of Brain: a Clinicopathologic Study of Eleven Patients and Review of Literature.

Background: Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare aggressive embryonal central nervous system (CNS) tumor of infancy and early childhood. Majority of the cases arise in the posterior fossa, and remaining in the cerebrum. Aims: To analyze the clinicopathologic features of AT/RT on a cohort of cases.

Isolated adult hypoganglionosis presenting as sigmoid volvulus: a case report.

Introduction: Isolated hypoganglionosis is a rare cause of intestinal innervation defects. It is characterized by sparse and small myenteric ganglia, absent or low acetylcholinesterase activity in the lamina propria and hypertrophy of the muscularis mucosae, principally in the region of the colon and rectum. It accounts for 5% of all intestinal neuronal malformations.

CNS neoplasms in Pakistan, a pathological perspective.

The Section of Histopathology, Aga Khan University is the largest center for histopathology in Pakistan and is the major reporting and referral center for CNS neoplasms in the country. Over the years, a significant increase has been noted in the number of CNS neoplasms reported annually. This increase most likely represents increased number of neurosurgical procedures being performed.

Rhinocladiella mackenziei as an emerging cause of cerebral phaeohyphomycosis in Pakistan: a case series.

Six cases of Rhinocladiella mackenziei cerebral phaeohyphomycosis are being reported for the first time in Pakistan. Identification was confirmed by DNA sequencing (isolates and fixed tissue). Diabetes, head trauma, immunosuppressive treatment, and postpartum state were present in 4 cases.

Diffuse ganglioneuromatosis of intestine in a 15-year-old girl.

The authors present the case of a 15-year-old girl with diffuse ganglioneuromatosis, principally involving the caecum. This is a rare condition and usually associated with multiple endocrine neoplasm IIb and neurofibromatosis type I. In our case, the myentric nerve plexus was exclusively involved.

Spindle epithelial tumor with thymus-like differentiation of thyroid gland: report of two cases with follow-up.

Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare malignant thyroid tumor showing thymic or related branchial pouch differentiation. The tumors are composed predominantly of spindle cells along with focal epithelial component and ductular formations. SETTLE occurs in young patients, with indolent growth and a tendency to develop delayed blood-borne metastases.

Spectrum of malignancy in mixed tumors of salivary gland: a morphological and immunohistochemical review of 23 cases.

Malignancy arising in mixed tumors of the salivary gland is a distinct entity that can occur sporadically or in association with a background of pleomorphic adenoma. Carcinoma arising with a background of pleomorphic adenoma is well documented. However, there are rare occurrences of aggressive de novo carcinosarcomas of the parotid that have been reported.

Fibromatous periorchitis.

We report a case of diffuse fibrous pseudotumour/fibromatous periorchitis, in a 43 year old male, that completely encased the right testis and was adjacent to a hydrocoele cavity. Although fibrous pseudotumours of this region are uncommon, they are reported to be the second most common benign paratesticular lesion after adenomatoid tumours. These comprise approximately 6 percent of paratesticular lesions, and are accepted as reactive lesions secondary to trauma, hydrocoele, infections or inflammation.