Non-Hodgkin Lymphoma Causing Hypopituitarism Can Imaging Help Diagnosis and Management?

Non-Hodgkin lymphomas of the hypothalamus and pituitary are rare. They usually remain clinically silent until onset of compressive features affecting surrounding structures. When symptomatic, patients most commonly present with diabetes insipidus, headaches, ophthalmoplegia and/or bilateral hemianopia. We report a case of a 67-year-old Caucasian female with a history of B-cell lymphoma in complete remission. She presented with left oculomotor nerve palsy and was subsequently found to have a sellar/suprasellar mass lesion on MRI. Alongside hypocortisolism and hypogonadotropic hypogonadism, she developed transient diabetes insipidus during her illness. Her clinical course was characterized by rapid intracranial progression of the sellar mass. MR spectroscopy suggested a diagnosis of lymphoma. Diagnostic biopsy confirmed high-grade diffuse large B-cell CNS lymphoma; this changed the definitive management from surgical excision to chemotherapy. Despite treatment, she succumbed to her illness within 7 months of initial presentation. This case highlights the aggressive nature of CNS lymphomas and the need for a high index of suspicion in an unusual presentation of sellar/suprasellar mass lesions. LEARNING POINTS: Novel imaging techniques such as MR spectroscopy might help to differentiate some brain tumours from pituitary macroadenomas, but these are not diagnostic.Tissue diagnosis with biopsy and histopathology is the gold standard for deciding management of pituitary fossa mass lesions with atypical presentation.

Facial twitching: calcium or concussion conundrum? Hypocalcaemia in a young American football player masking an internal carotid artery dissection.

A 30-year-old male American football player presented to the acute medical unit with left-hand and hemifacial spasms. History and examination revealed hemifacial spasms in keeping with seizure-like activity possibly due to symptomatic hypocalcaemia. Subsequent investigations revealed an adjusted calcium of 1.87 mmol/L and, hence, he was managed with intravenous calcium replacement. He presented two further times in a 1-month period, with subjective limb weakness, despite normal adjusted calcium. During his third admission, he developed slurred speech and a marked facial droop, with absence of power in the right upper limb. Imaging revealed acute and old infarctions in the left middle cerebral artery territory and appearances consistent with left internal carotid artery dissection. This presentation of arterial stroke is atypical but with potentially grave consequences if missed. There is limited literature on the presentation of hemifacial spasm, and its association with ischaemic or haemorrhagic stroke represents a key learning point.

The value of prolactin in predicting prolactinοma in hyperprolactinaemic polycystic ovarian syndrome.

BACKGROUND: To identify a serum prolactin (PRL) cut-off value indicative of a PRL-producing adenoma in women with polycystic ovarian syndrome (PCOS) and hyperprolactinaemia and characterize such patients. MATERIALS AND METHODS: In the present retrospective case-control study, the medical records of 528 PCOS women were reviewed. Pituitary magnetic resonance imaging (MRI) was performed in PCOS patients with PRL levels ≥94.0 ng/mL and/or symptoms suspicious of a pituitary adenoma (PA). Prolactinoma diagnosis was made in the presence of an MRI-identifiable PA with biochemical and radiological response to dopamine agonists. Receiver operating characteristic (ROC) curve analysis was performed to determine a serum PRL threshold that could identify hyperprolactinaemic PCOS subjects with prolactinomas. Clinical, metabolic and endocrine parameters were also analysed. RESULTS: Among 528 patients with PCOS, 60 (11.4%) had elevated PRL levels. Of 44 (73.3%) patients who had pituitary imaging, 19 had PAs, 18 normal MRI and 7 other abnormalities. Patients harbouring prolactinomas had significantly higher PRL levels compared to patients without adenomas (median PRL 95.4 vs 49.2 ng/mL, P < .0001). A PRL threshold of 85.2 ng/mL could distinguish patients with prolactinomas with 77% sensitivity and 100% specificity [Area Under the curve (AUC) (95%) 0.91(0.8-1.018), P = .0001]. PCOS women with prolactinomas were younger and had lower LH levels compared to women without prolactinomas. CONCLUSIONS: In women with PCOS, PRL levels exceeding 85.2 ng/mL are highly suggestive of a prolactinoma warranting pituitary imaging. Pituitary MRI could also be considered in young PCOS patients with milder PRL elevation and low LH levels.

Infected intracranial meningiomas.

OBJECTIVE: Infection associated with an intracranial meningioma is an extremely rare condition. Only six cases have been described in the literature. Because of its dual pathologies, initial radiologic diagnosis can be difficult. We present the first reported case of multiple infected intracranial meningiomas and correlate the radiologic and histologic findings. METHODS: A 70-year-old woman presented with sepsis and a left hemiparesis following ureteroscopy and lithotripsy. A large right parietal lesion and a smaller left frontal lesion were diagnosed on magnetic resonance imaging. Diffusion-weighted imaging and an apparent diffusion coefficient map demonstrated features of cerebral metastases. RESULTS: A 2-stage excision confirmed atypical meningiomas containing an intratumoral abscess secondary to Escherichia coli. The patient made a full neurologic recovery. Despite the additional techniques, the radiologic diagnosis was initially challenging because of the dual pathologies. Nonetheless, the radiologic appearance was consistent with the complex histologic findings. CONCLUSIONS: In the appropriate clinical context, diffusion-weighted imaging and apparent diffusion coefficient map aid the diagnosis of infected intracranial meningiomas.

Post-radiotherapy radionecrosis of the temporal bone resulting in delayed CSF otorrhoea: a case report.

The authors report a case of temporal glioblastoma multiforme, who received post-operative radiotherapy. The patient presented to neurosurgery 8 months post-radiotherapy with CSF otorrhoea. This was shown to be from a defect of the right tegmen tympani secondary to radionecrosis of the petrous temporal bone. This was successfully repaired via craniotomy and repair of the middle cranial fossa floor. The authors have been unable to find previously published examples of isolated radio-necrosis of the temporal bone following radiotherapy for glioblastoma multiforme.

Congenital hypopituitarism: clinical, molecular and neuroradiological correlates.

OBJECTIVE: Recent studies have suggested that mutations in genes encoding several hypothalamo-pituitary (H-P) transcription factors result in hypopituitarism [isolated GH deficiency (IGHD) and combined pituitary hormone deficiency (CPHD)], which may in turn be related to the neuroanatomy revealed by magnetic resonance (MR) imaging. Although studies have focused on patients with either optic nerve hypoplasia (ONH) or isolated hypopituitarism with normal optic nerves, few studies have compared the two groups. We aimed to relate the clinical phenotype of a large cohort (n = 170) of children with congenital hypopituitarism including septo-optic dysplasia (SOD) attending a single centre to the neuroradiological and genetic findings. DESIGN: Clinical, biochemical, MR imaging and molecular data were analysed retrospectively in 170 patients with or 'at-risk' (with ONH) of hypopituitarism to determine predictors of hypopituitarism. RESULTS: The presence of ONH was significantly associated with an absent septum pellucidum [odds ratio (OR) 31.5, 95% confidence intervals (CI) 7.3-136.6, P < 0.001], an abnormal corpus callosum (OR 10.5, 95% CI 3.8-28.6, P < 0.001) and stalk abnormalities (OR 2.3, 95% CI 1.2-4.2, P = 0.009). The risk of hypopituitarism was 27.2 times greater in patients with an undescended posterior pituitary (95% CI 3.6-205.1, P < 0.001). Anterior pituitary hypoplasia (OR 3.1, 95% CI 1.3-7.0, P = 0.006) and an absent pituitary stalk (P < 0.001) were also significantly associated with hypopituitarism. With respect to the type or severity of hypopituitarism, CPHD was more often associated with an abnormal corpus callosum (OR 6.1, 95% CI 1.4-27.4, P = 0.008) and stalk abnormalities (OR 2.8, 95% CI 1.3-6.1, P = 0.006). Male to female ratio was significantly greater in patients with normal optic nerves (3.3:1) as compared with those with ONH (1.2:1). The prevalence of diabetes insipidus, thyrotrophin and ACTH deficiencies was significantly greater in patients with ONH as compared with 'idiopathic' hypopituitarism. Mutations in pituitary transcription factors and genes regulating GH secretion were rare (5/170) in this cohort of patients with sporadic hypopituitarism. CONCLUSION: Our data suggest that individuals presenting with ONH are at high risk for neuroradiologic and endocrine abnormalities. The neuroradiologic features are predictive not only of the presence, but also of the type, of hypopituitarism. The association of midline abnormalities with hypopituitarism in this cohort suggests a common developmental origin for these features, the aetiology of which remains unidentified in the majority of cases.