Solitary Intracranial Juvenile Xanthogranuloma Masquerading as Low-Grade Glioma - A Rare Case Report.

Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic disorder of young children. It is most often present with cutaneous involvement of the head, neck and trunk region. Systemic JXG causes cutaneous lesions with extracutaneous involvements frequently seen in the orbit, liver, spleen, lung, kidney and bones. Central nervous system (CNS) involvement is uncommon and is usually seen as a component of systemic disease. Isolated JXG of CNS is very rare and only few cases have been reported till date. Here we report a case of isolated solitary intracranial JXG mimicking clinically and radiologically as a low-grade glioma with no signs of cutaneous or other systemic involvement. Gross total excision of the tumour was done and final diagnosis was made by histopathological and immunohistochemical examination. CNS involvement of JXG can cause significant morbidity and mortality. These cases are usually misdiagnosed on radiology as glioma or meningioma and the diagnosis is usually made by histopathology. Surgery is the mainstay of treatment and these patients should be on long-term follow-up since the natural history of the disease is still unknown. The case is presented here for its rarity and for its clinical significance.

"Endolymphatic sac tumour": A case report with review of literature.

Endolymphatic sac tumour (ELST) is a non-metastasizing low grade adenocarcinoma of endolymphatic sac origin. It is also known as Heffner tumour, low grade adenocarcinoma of endolymphatic sac origin and aggressive papillary middle ear tumour. These tumours are closely associated with Von Hippel Lindau (VHL) disease. Here we report a case of Endolymphatic sac tumour in a 63 yr old lady who presented with left sided facial palsy. Since the tumour was highly vascular and required preoperative embolization, initial clinicoradiological diagnosis was Jugulotymphanic paraganglioma. Histopathology showed features of Endolymphatic sac tumour, which was confirmed by immunohistochemistry. Since this tumour is locally aggressive low grade adenocarcinoma, the diagnosis is difficult in advanced cases where there is erosion of petrous temporal bone or the lesion shows extension into cerebellopontine angle as in our case. Since the association of this tumour with VHL disease is well established, it is important to screen all the patients of VHL disease for this lesion and also all the patients of ELST should be screened for other lesions of VHL disease to aid in early diagnosis and treatment. The case is presented here for its rarity and difficulty in initial diagnosis.

Psammomatous melanotic schwannoma as a component of Carney complex.

Melanotic schwannoma is a rare form of pigmented neural tumor commonly arising from the posterior spinal nerves and ganglia. Two variants have been described, psammomatous and nonpsammomatous. 50% of psammomatous tumors are associated with Carney complex. The biologic behavior of the tumor is difficult to predict and slightly over 10% of the tumors follow malignant course. We present a case of psammomatous melanotic schwannoma as part of Carney complex in a 67-year-old male. Clinical examination revealed oral mucosal and abdominal skin pigmentation. Magnetic resonance imaging showed an intradural extramedullary lesion at D8-D12 level. Intraoperative squash smear study showed sheets of spindle cells with abundant intracytoplasmic melanin pigmentation and few psammoma bodies. Based on clinical, radiologic, and histopathological findings with immunohistochemistry correlation a final diagnosis of psammomatous melanotic schwannoma was rendered.