Ependymomas are mostly infratentorial, intraventricular tumor, accounting for 2–9% of all central nervous system tumors. Supratentorial pure cortical ependymoma are extremely rare tumor with definite ependymal morphology and uncertain histogenesis. They are mostly low grade tumor and are cured with resection, rendering them favorable prognosis. Our case is of 14-year-old female presenting with headache and convulsion of short duration. She underwent gross total excision of the tumor without radiation therapy and her follow-up is uneventful.
Clear-cell meningioma (CCM), an unusual subset of meningioma has prominent, clear-cell morphology. It is a wolf in sheep's clothing characterized by benign histologic attributes, but tendency for recurrence (61%) and metastasis. Therefore, WHO has classified it as grade II meningioma. Fine-needle aspiration cytology diagnosis is simple, rapid, cost-effective and reliable procedure primarily aimed at preoperative diagnosis of advanced and metastatic extracranial tumor. Preoperative and/or intraoperative cytodiagnosis of CCM demand expertise in the evaluation of cytology smears. However in case of intra operative evaluation of squash smears there is a time constraint and a very small tissue material obtained by stereotactic biopsy are available for interpretation. Knowledge of clinical features including age, anatomical locations, neuroimaging findings and cytomorphologic features, are prerequisites for arriving at definitive cytodiagnosis. We describe intra operative squash cytology of CCM in a 16-year-old female, located in cerebello-pontine angle. The diagnosis of CCM offered on squash cytology was subsequently confirmed on histopathology and immunohistochemistry. It typically showed pattern less sheets and groups of polyhedral, clear cells with monomorphic, round nuclei having a bland chromatin and inconspicuous nucleoli. Separation of CCM from other tumors having clear -cell morphology and variants of meningioma is important because of its high recurrence rate and mortality.
Ependymomas are tumors derived from ependymal cells lining the ventricles or from the central canal of the spinal cord. It usually arises in the ventricles with extra ventricular extension. Less than 15 cases of purely cortical ependymomas are reported. We report a rare case of purely cortical anaplastic ependymoma in a pediatric patient, which is rarely reported.
Complete androgen insensitivity syndrome (CAIS) is an AIS condition that results in the complete inability of the cell to respond to androgens. Individuals with complete androgen insensitivity syndrome are born phenotypically female, without any signs of genital masculinization, despite having a 46,XY karyotype.
Case report
We present a case of a 66 year nulliparous female with primary amenorrhea with complaints of bilateral inguinal mass for 5 years and bilateral breast lump for 3 years. Patient developed a sudden increase in the size of left breast lump. An MRI chest revealed a large 15.4x11.5cm heterogeneous mass in the left breast and 9.9x6.1 cm lobulated mass in the right breast. MRI abdomen and pelvis revealed soft tissue structures in the bilateral inguinal region near the deep inguinal ring with blind ending vaginal tract and absent uterus and ovaries. karyotyping revealed 46XY. We received specimens of left MRM, right lumpectomy and bilateral inguinal mass. Left MRM measured 32x20x12cm. On the cut section showed a large tumor with variegated appearance. Right lumpectomy showed a tumor with leaf-like appearance was noted. Bilateral inguinal mass measured 5x3x3 cm each, cut section showed yellowish nodules. Histopathological examination reveals malignant phyllodes of left breast with benign phyllodes of the right breast and leydig cell hyperplasia of bilateral testes. Conclusion
This is the first case reported of malignant phyllodes in a case of complete androgen insensitivity syndrome with bilateral leydig cell hyperplasia of testes.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.