ABSTRACT. We report the first case of extensive involvement of isolated intracranial Rosai-Dorfman's disease (RDD) in a child. Our case is unique because it presents with involvement of the middle cranial fossa, cavernous sinus, pituitary gland, orbit, ethmoid and sphenoid sinuses. Previous cases of intracranial RDD in children have reported separate involvement of cavernous sinus, suprasellar region, and frontal and petroclival regions. Involvement of the pituitary gland has so far not been reported. A 14-year-old male presented with a medical history of loss of vision, raised erythrocyte sedimentation rate (ESR), and abnormal prolactin and cortisol levels. Radiologically the diagnosis was meningioma. The histopathological diagnosis was RDD with emperipolesis and S-100 positivity. RDD is a histiocytic proliferation of unknown aetiology, which commonly affects lymph nodes. Uncommonly it involves the extranodal sites and rarely the central nervous system (CNS). 80 cases of RDD involving CNS have been reported in the literature, and only 5 were in children. Although the definitive diagnosis of RDD disease is based on the histopathology report, it should be included in the differentials of a lesion mimicking meningioma especially in children.
The co-occurrence of two or more brain tumors with different histological features is rare. The authors report three rare cases of intracranial tumors associated with pituitary adenomas. Two of the pituitary tumors were functioning adenomas: a prolactinoma and a thyrotropin secreting adenoma. Two of the associated intracranial neoplasms were gliomas and one was a meningioma. Radiological and clinical examination for syndromal association was negative in all cases. We briefly discuss the presentation and treatment options of these cases and review the 19 previous publications in the literature of pituitary tumors occurring in association with other neoplasms and explore the possible links underlying these co-occurring neoplasms. Our three cases represent 0.86% of all pituitary tumors operated at our institute over a 9-year period.
This study aimed to identify (1) the thalamic gliomas suitable for surgical resection and (2) the appropriate surgical approach based on their location and the displacement of the posterior limb of the internal capsule (PLIC). A retrospective study over a 5-year period (from 2006 to 2010) was performed in 41 patients with thalamic gliomas. The mean age of these patients was 20.4 years (range, 2-65 years). Twenty (49 %) tumors were thalamic, 19 (46 %) were thalamopeduncular, and 2 (5 %) were bilateral. The PLIC, based on T2-weighted magnetic resonance axial sections, was displaced anterolaterally in 23 (56 %) cases and laterally in 6 (14 %) cases. It was involved by lesion in eight (20 %) cases and could not be identified in four (10 %) cases. Resection, favored in patients with well-defined, contrast-enhancing lesions, was performed in 34 (83 %) cases, while a biopsy was resorted to in 7 (17 %) cases. A gross total resection or near total resection (>90 %) could be achieved in 26 (63 %) cases. The middle temporal gyrus approach, used when the PLIC was displaced anterolaterally, was the commonly used approach (63.5 %). Common pathologies were pilocytic astrocytoma (58 %) in children and grade III/IV astrocytomas (86 %) in adults. Preoperative motor deficits improved in 64 % of the patients with pilocytic lesions as compared to 0 % in patients with grade III/IV lesions (P value, 0.001). Postoperatively, two patients (5 %) had marginal worsening of motor power, two patients developed visual field defects, and one patient developed a third nerve paresis. Radical resection of thalamic gliomas is a useful treatment modality in a select subset of patients and is the treatment of choice for pilocytic astrocytomas. Tailoring the surgical approach, depending on the relative position of the PLIC, has an important bearing on outcome.
Tuberculosis, a common cause for chronic intracranial infections can mimic varied intracranial pathologies including tumours. Pituitary tuberculomas are rare lesions and are often diagnosed pre-operatively as pituitary tumours. We report a case of a 31-year-old lady with a sellar-suprasellar lesion who presented with panhypopituitarism. The patient underwent a trans-nasal, trans-sphenoidal surgical decompression of the lesion. Histopathology revealed a tuberculous lesion in the pituitary. The characteristic radiological features of sellar tuberculomas are discussed along with a review of literature. Atypical sellar radiology in the presence of a thickened pituitary stalk could point to pathology other than pituitary adenoma, possibly a chronic inflammatory condition like tuberculoma.
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