Primary pure yolk sac tumors (YST) arising in the central nervous system (CNS) are extremely rare and generally arise in pineal (62%) and suprasellar (31%) regions. To best of our knowledge, till date only 10 cases of Primary YST arising in cerebellum are reported in the literature. YSTs have very poor prognosis. Most of them occur in childhood, between 7 months to 3 years of age. Pathologist should be aware that the primary yolk sac tumors can present at unusual anatomical sites like cerebellum, which would aid its early diagnosis and appropriate treatment. Herein, we report a case of Primary YST involving cerebellar vermis and right cerebellar hemisphere in a 4-year-old female.
Cerebellar liponeurocytoma is a rare clinicopathological entity, included in the 2000 World Health Organization (WHO) classification of tumors of the central nervous system in the category of glioneuronal tumors. These are WHO Grade II, slow-growing tumors in adults with known recurrences. To date, about 70 cases of liponeurocytomas have been reported. This tumor needs to be differentiated from oligodendroglioma as well as medulloblastoma which are more common and aggressive, requiring postoperative chemoradiation. A high index of suspicion is thus advocated for the diagnosis. We present one such case of this rare entity reported in our institute. A 42-year-old female presented with a 4-month history of headache, neck pain, reduced vision, and cerebellar signs. Imaging showed a midline lesion involving the cerebellar vermis and left lobe suggestive of anaplastic ependymoma/hemangioblastoma. The final diagnosis of cerebellar liponeurocytoma was rendered on routine histopathological examination supported by immunohistochemical analysis.
Sex cord stromal cell tumours constitute 5-8% of all ovarian neoplasms of which pure leydig cell tumours constitute 0.1%. These tumours are most commonly found in the post-menopausal age group and patients present with a rapid onset of symptoms of androgen excess like hoarseness of voice, clitoromegaly and hirsutism. We present a case of 39 year old female, who presented with virilising symptoms since 2 years. Serum Testosterone levels were raised and CT revealed homogeneously enhancing mass of 2.8 cm in size in the right ovary. An unilateral oophorectomy was done and sent for histopathological examination. On Gross examination, the ovary revealed the presence of a well circumscribed greyish white tumour with multiple yellowish areas. Microscopy revealed features suggestive of pure leydig cell tumour with reinke crystals. Post operatively, the patient improved symptomatically. The unusual features seen in this case were younger age at presentation and insidious onset of symptoms. The presentation of pure leydig cell tumour-hilar type at such a young age is extremely uncommon.
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