MRI findings in childhood neurohypophyseal germinomas

Transcription

1 MRI findings in childhood neurohypophyseal germinomas Poster No.: C-1587 Congress: ECR 2015 Type: Scientific Exhibit Authors: C. Laganâ, S. I. Sirvent, M. A. Lopez-Pino, G. Albi, I. Solis Muniz, E. García Esparza, T. Cañas, G. Gómez Mardones; Madrid/ES Keywords: Paediatric, Neuroradiology brain, CNS, MR, Diagnostic procedure, Neoplasia, Metabolic disorders DOI: /ecr2015/C-1587 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 21

2 Aims and objectives Neurohypophyseal germinoma is a rare pediatric central nervous system neoplasia which imaging features are poorly reported in radiological literature. Approximately 90% of germ cell tumors occur in patients under 20-years-old. The pineal gland is the most common site of origin, followed by the suprasellar region. Other less common origines sites include the basal ganglia, thalamus, brainstem and spinal cord. When suprasellar region is involved, germ cell tumor affect the hypothalamo-neurohypophyseal axis, infundibulum and posterior lobe of the pituitary gland. Our objective was to describe hypothalamo-neurohypophyseal germinoma MR findings in pediatric patients. Page 2 of 21

3 Images for this section: Fig. 1: 15 years old female with headache, secondary amenorrhea and polydipsia. MR images sagital T1 weighted image (a) and sagital (b), axial (c) and coronal (d) T1 post gadolinium weighted images show sellar tumor extending into the pituitary stalk, which is also thickened. Laterally contact with both cavernous sinuses, especially the left, which surrounds in more than 50% of its circumference. Pathologically proven dysgerminoma. Page 3 of 21

4 Methods and materials Patients: MR images obtained in a pediatric hospital of 8 patients (2 males, 6 females; mean age: 12,5 years old; range: 7-15 years) with germinoma diagnosis were retrospectively analyzed. Most of cases (87,5%) have histologic confirmation and in the remaining case diagnosis was made by clinical and analytical criteria. MR imaging protocol: MR imaging studies were performed using a 1.5-T superconducting magnet. Our protocol include sagittal and coronal T1-weighted images, coronal T2-weighted, diffusion weighted image (DWI) and sagittal and coronal contrast-enhanced T1-weighted images after intravenous injection of gadopentetate dimeglumine (Gd-DTPA) at a dose of 0.1 mmol/kg bodyweight. The variables under study were: Pituitary gland and pituitary stalk morphology is classified according to the image in the coronal plane in 4 different types: normal (< 4mm thickness), V-shaped, rounded morphology and triangle morphology (Figure 2). Page 4 of 21

5 Fig. 2 References: Pediatric Radiology, Hospital Universitario del Niño Jesus - Madrid/ES Rest of variables were: neurohypophysis absence or presence, maximum transverse and longitudinal pituitary stalk axes, cystic transformation, craneal and spinal leptomeningeal spread, presence of concomitant injury to diagnosis. MR images obteined prior to diagnostic were available in all patient and we study their evolution. Analysis of all variables has been performed on MR obteined prior to treatment. Page 5 of 21

6 Results Results: Infundibular thickening was observed in all cases; transversal measurement was: mean 12,8 mm, range: 4-30 mm;longitudinal measurement was: mean 13,7mm, range 5-21 mm. (Figure 13) Fig. 13 References: Pediatric Radiology, Hospital Universitario del Niño Jesus - Madrid/ES Page 6 of 21

7 Hyperintensity on T1- weighted image of neurohypofysis in the posterior pituitary gland was absent in 7 cases (87,5%). According to the reported morphologic pattern, 4 cases (50%) present rounded morphology, 2 cases (25%) triangle morphology, 1 case V-shaped morphology (25%) and 1 case normal morphology (25%). (Figure 14) Fig. 14 References: Pediatric Radiology, Hospital Universitario del Niño Jesus - Madrid/ES 37,5% has a concomitant lesion in the pineal gland. Craneal meningeal dissemination was found in one case (12,5%) (figure 12); no patient with spinal meningeal dissemination was found. Page 7 of 21

8 MR images obteined prior to the diagnosis, were available in all patients and in three of themes (37,5%) the only abnormality detected was the absence of neurohypophysis. (examples in figures ) Results resume in figure 15. Fig. 15 References: Pediatric Radiology, Hospital Universitario del Niño Jesus - Madrid/ES Page 8 of 21

9 Images for this section: Fig. 3: 12 years old female under study for low height. MR images : coronal T2 (a) and T1 (b) post gadolonium weighted images, sagital T1 pre (c) and post (d) gadolinium weighted images. Images show pituitary stalk diffusely thickened, about 4-5 mm, with enhancement after intravenous gadolinium. No abnormalities in morphology and sive of pituitary gland except no visible neurohypophysis. Image control 6 month after in next picture show findings progresión. Page 9 of 21

10 Fig. 4: Same patient of previous picture six month later. MR images: sagital T1 pre (a) and post (b) gadolinium inyection and coronal (c) T1 weighted images show enlargement of the pituitary stalk. Increased enhancement in the supraoptic recess of the third ventricle with nodular morphology is also visible. Dysgerminoma was histologically proven. Page 10 of 21

11 Fig. 5: 15 years old male with suprasellar lesion. MR images: axial FLAIR (a) and T1 post gadolinium inyection (b), sagital T1 pre (c) and post (d) gadolinium inyection weighted images. Pictures show small tumor in the pineal gland predominantly isointense on T1 and hyperintense on FLAIR sequences, and with intense enhancement after gadolinium administration.suprasellar small nodule is detected region tuber cinereum. Postsurgical changes right fronto-temporal craniotomy. Images control after several quimiotherapy treatment in the next picture. Page 11 of 21

12 Fig. 6: 14 years old female with Central diabetes insipidus. MR images: axial (a), coronal (b) and sagital (d) T1 post gadolinium weighted images and T1 weighted image (c). The posterior hyperintense signal in T1 corresponding with the pituitary gland is not detectable in its usual location. No ectopic neurohypophysi is identifies. Rest of gland with normal characteristics. See image control in next picture. Page 12 of 21

13 Fig. 7: Same patient of previous picture. MR images control 2 year later. Coronal T1 pre (a) and post (b) and sagital T1 pre(c) and post (d) gadolinium weighted images. Images show focused and thickened pituitary stalk, which has increased in volume over previous scans. It has increased at the expense of its most cranial portion, which combines present nodular morphology and occupies the recess hypothalamic supraoptic and reaching the third ventricle. Dysgerminoma histologically proven. Page 13 of 21

14 Fig. 8: 8 years old female patient with severe headache and vomiting for two days of evolution. MR images: coronal T1 weighted images pre (a) and post (b) and sagital pre (c) and post (d) gadolinium inyection. Images show absence of focal hyperintense T1 neurohypophysis normal or ectopic. Enlargement of the pituitary gland to the age of the patient, signal strength homogeneous equal to brain parenchyma on T1, but with heterogeneous enhancement after administration intravenous gadolinium. Pathologically proven dysgerminoma. Page 14 of 21

15 Fig. 9: 15 years old male with polydidipsia symptoms. Axial T1 weighted image (a), coronal T2 weighted image (b) and axial (c) and sagital (d) post contrast weighted images show pituitary mass with suprasellar extension, bulging the sellar diaphragm and infiltrating the pituitary stalk, with infilración the right cavernous sinus where the mass surrounding the carotid artery. After administration of gadolinium homogeneous uptake is seen. Dysgerminoma pathlogically proven. Page 15 of 21

16 Fig. 10: 15 years old female patient studied because underweight. MR images: coronal T1 weighted image (a), coronal (b), axial (c) and sagital (d) post gadolinium weighted images show pituitary gland size, morphology and normal.normal T1 neurohypophysis hyperintensities is not identified in in his usual or ectopic location.nodular thickening of the pituitary stalk of approximately 6 mm with enhancement in post gadolinium images. Linear pathological gadolinium uptake in the suprachiasmatic recess of the third ventricle suggestive of leptomeningeal cranial dissemination.nodular image in the pineal area of about 12mm, with post contraste enhancement. Cranial germ cell tumor with signs of cranial leptomeningeal dissemination. Page 16 of 21

17 Fig. 11: 12 years old female with hormonales troubles. MR images: Axial FLAIR (a), axial T1 post gadolinium (b), coronal T2 (c) and sagital T1 (d) weighted images show supra sellar nodular solid lesion with printing on the optic chiasm and post contrast enhancement. Lesion was a dysgerminoma, histologically proven. Page 17 of 21

18 Fig. 12: Same patient of previous pciture. MR images: Axial FLAIR (a), axial T1 post gadolinium (b), coronal T2 (c) and sagital T1 (d) weighted images. Images control after radio-quimiotherapy demonstrate pituitary gland of normal morphology with absence of the neurohypophysis, not visible like in normal population as hyperintense on T1 Pituitary stalk centered, gauge is normal. No suprasellar mass displayed in the previous study. Page 18 of 21

19 Conclusion Thickening of the pituitary stalk is a radiological nonspecific finding. Although we have not found in the radiological literature a point of cutoff to establish the diagnosis of neurohypophyseal germinoma, infundibular thickening is observed in all cases and thickness depends on the time when the diagnosis is made. The normal hyperintensity signal of the posterior pituitary on T1-weighted images is usually absent. This finding may be an early sign of tumor infiltration even in absence of infundibular thickening, reason for wich is suitable to perform images controls in these patients. Less frequently this pathological entity may present as a multifocal form observing concomitant lesions in pineal gland area. On contrast-enhanced T1-weighted imaging, neoplasm show significant contrast enhancement. In any cases can observe enhancement of the ventricular walls in relation to neoplastic dissemination. Leptomeningeal dissemination in brain and in the spinal canal cord may occur but is rare. In the differencial diagnosis of pituitary stalk thickening should be considered more commonly Langerhans cell histiocytosis, a systemic disease and lymphocytic infundíbular neurohypophysitis, an autoinmune mediated inflammatory disorder. More rarely many other diseases of several origins can cause infundibular thickening: malignant like lymphoma, leukemia and metastasis; granulomatouse diseases, such tuberculosis, Wegener's granulomatosis and sarcoidosis. Although germinomas are fatal if untreated, be consider that are neoplasms highly susceptible to irradiation, chemotherapy, surgical treatement and are potentially curable with combinantions of themes. Post-treatment MRI studies can assess the degree of response and thus provide prognostic information. Page 19 of 21

20 Personal information Dr. Claudio Laganá 4th year radiology resident Hospital Niño Jesus - Hospital de La Princesa Madrid, Spain claudiolaga@hotmail.it Page 20 of 21

21 References MRI and CT findings of neurohypophyseal germinoma Kanagaki et. coll. European Journal of Radiology 49 (2004) High Signal Intensity of the Infundibular Stalk on Fluid-Attenuated Inversion Recovery MR Yutaka Araki et coll. AJNR Am J Neuroradiol 18:89-93, January 1997 MRI and CT findings of neurohypophyseal germinoma Mitsounori Kanagachi et coll. European Journal of Radiology March 2004, Volume 49,Issue 3, Pages Primary intracranial germ cell tumors Shagufta tahir Mufti et coll. Asian J Neurosurg Oct-Dec; 7(4): Page 21 of 21