Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient

Bokhari, Rakan; Ghanem, Ahmed I; Al-Ahwal, Mahmoud; Baeesa, Saleh

doi:10.1155/2013/614658

Cited by 11 publications

(19 citation statements)

References 11 publications

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“…PCNSLs within fourth ventricle are relatively seldom seen in immune-competent patients. To the best of our knowledge, only nine cases of PCNSL located at fourth ventricle have been reported in the literature (Table 1) [1][2][3][4][5][6][7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Primary Central Nervous System Lymphoma in the Fourth Ventricle

Xinyi¹,

Qiu²,

Rong³

et al. 2017

J Neuroinfect Dis

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Primary central nervous system lymphoma (PCNSL), a highly malignant and infrequent tumor, is rarely found within the fourth ventricle. Here we report a case of isolated lymphoma within the fourth ventricle: a 61-year-old male presented with headache, mental disorder, abnormal gait and urinary incontinence. Based on the clinical symptoms and neuroimage, He was initially diagnosed as choroid plexus papilloma. However, post-surgery pathology corrected the diagnosis as a diffuse large B-cell lymphoma. To the best of our knowledge, only nine cases are documented in the literature and all of them were initially diagnosed as other tumours or not determined. Therefore, Thesis unconventional location and atypical clinical manifestation of PCNSL in the fourth ventricle should be included on the list of differential diagnosis of the fourth ventricle tumors. Case ReportA 61-year-old man presented to our hospital complaining of headache for one month and somnolence, abnormal gait and urinary incontinence for one week. During admission, he was in a state of somnolence and physical examination only revealed the mild cervical resistance. His past medical history and laboratory tests including routine blood parameters, blood biochemistry (including lactate dehydrogenase (LDH) HIV testing), routine coagulation parameters and erythrocyte sedimentation rate were normal as well.Brain magnetic resonance imaging (MRI) revealed one irregular nodule occupying the fourth ventricle with a hypo intensity on T1 weighted image (T1WI) and is intensity on T2 weighted image (T2WI) (Figure 1). Post-contrast T1WI showed well-defined and marked homogenous enhancement without necrosis. Furthermore, contrast enhancement image demonstrated that the primary nidus of the mass originated from the roof of the forth ventricle, which was surrounded with choroid plexus (Figure 2). The intraventricular location of the mass and MRI manifestation led us to clinical diagnosis as choroid plexus papilloma (CPP). PET-CT of the whole body showed high metabolism in the fourth ventricle. The special location of the mass prompted our surgical team to choose surgical excision over stereotactic biopsy.Interestingly, post-surgery pathology diagnosed as a diffuse large Bcell lymphoma (Figure 3). The patient refused to undergo radiotherapy or chemotherapy immediately after the surgery. At 6-week follow-up after the resection, brain MRI demonstrated enhanced nodules in the third ventricle and midbrain aqueduct area, which were considered as implantation metastases. Consequently, the patient was administrated with chemotherapy combined with radiotherapy. Another month later, these lesions amazingly disappeared on brain MRI. Currently, 20 months post-surgery, the patient is still under active follow-up.

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Section: Discussionmentioning

confidence: 99%

Primary Central Nervous System Lymphoma in the Fourth Ventricle

Xinyi¹,

Qiu²,

Rong³

et al. 2017

J Neuroinfect Dis

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“…1 Few cases of PCNSL (five affecting the hypothalamus and six the fourth ventricle) have been described so far. [10][11][12][13][14][15] To the best of our knowledge, mutual involvement of the hypothalamus and fourth ventricle by PCNSL has not been reported yet.…”

Section: Discussionmentioning

confidence: 99%

Unusual primary central nervous system lymphoma location involving the fourth ventricle and hypothalamus

et al. 2015

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A previously healthy 65-year-old man presented with a two-week history of weight loss, headaches, blurred vision, asthenia and quickly worsening walking impairment. He denied photophobia, neck stiffness, fever, nausea or vomiting.Neurological examination showed global motor slowing, tendency to fall asleep during the clinical examination, generalized weakness against resistance to head and limbs, and osteotendon reflexes present in the upper limbs, but not evoked in the lower limbs. No sensitive deficit or focal neurologic sign was recognizable.Non-contrast multislice computed tomography (MSCT) of the head was performed in the emergency department, showing diffuse periventricular white matter and thalamic mild hyperdensity.Lumbar puncture, blood tests, including serology for HIV and other infections, were negative.On the third day the patient, showing decreased consciousness, underwent magnetic resonance imaging (MRI) with contrast medium injection. MRI revealed the presence of multiple pseudonodular avidly enhancing lesions, supra and infratentorial, crossing the midline, involving the ventricular system, including the fourth ventricle, with extension into the surrounding white matter, the corpus callosum, the thalamus and the hypothamalus.A stereotactic biopsy led to a diagnosis of diffuse large B-cell lymphoma, primarily located in the central nervous system (PCNSL).After the completion of the first phase of treatment (immunotherapy with intravenous Rituximab and corticosteroid), the MRI showed a marked regression of tumor masses.

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“…Les LCP impliquant les ventricules sont généralement développés dans la zone périventriculaire et font saillie dans les cavités ventriculaires. La localisation pure et isolée du quatrième ventricule (V4) est exceptionnelle, sept cas ayant été rapportés à ce jour dans la littérature [4][5][6][7][8][9][10]. Ces cas sont résumés dans le tableau 1.…”

Section: Discussionunclassified

“…À l'instar des autres auteurs, malgré l'aspect de la lésion à l'IRM, qui et a posteriori était compatible avec un lymphome, celui-ci n'avait pas été évoqué parmi les hypothèses diagnostiques du fait de la rareté de cette localisation. Certains proposent d'inclure le lymphome primitif dans la liste des diagnostiques différen-tiels des lésions du quatrième ventricule, bien limitées et se rehaussant de façon intense et homogène après gadolinium [5][6][7][8]10].…”

Section: Discussionunclassified

“…Contrairement aux localisations classiques du LCP souvent profondes, l'exérèse d'un lymphome du V4 peut être obtenue sans traverser le parenchyme cérébelleux et donc avec une morbidité moindre. Certains pensent que, dans les cas de lésion du V4 bien limitée et prenant le contraste de façon intense et homogène, la chirurgie d'exérèse suivi d'une chimiothérapie peut être une option intéressante [8,10]. On peut noter à ce sujet, que la plupart des LCP du V4 publiés avaient été opérée avec des suites simples (tableau 1).…”

Section: Discussionunclassified

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Lymphome primitif à grandes cellules B du quatrième ventricule : cas clinique et revue de la littérature

et al. 2015

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Nous rapportons le cas d'un lymphome B diffus primitif isolé du quatrième ventricule. Il s'agissait d'une femme de 70 ans adressée pour céphalées, vertiges et une démarche instable évoluant depuis trois semaines. L'imagerie cérébrale par résonance magnétique objectivait une lésion du quatrième ventricule bien limitée, se rehaussant de façon intense et homogène après gadolinium faisant évoquer un gliome ou une métastase. Elle a été opérée après qu'un bilan systémique n'a pas retrouvé d'autres localisations (imagerie, biologie sanguine…). L'exérèse complète était réalisée. L'histopathologie a conclu à un lymphome B diffus à grandes cellules avec un phénotype de cellules B activées. La recherche d'autres localisations était négative. Une polychimiothérapie à base de Méthotrexate a été administrée. L'évolution était favorable avec un recul de 10 mois sans récidive. A la lumière de ce cas rare de localisation cérébel-leuse lymphomateuse du 4ème ventricule, une revue exhaustive de la littérature est proposée. Mots clés Lymphome B · Quatrième ventricule · ChimiothérapieAbstract We herein present the case of a 70 year-old woman admitted with a 3 weeks history of headaches, vertigo and gait instability. Brain imaging revealed an isolated wellcircumscribed and enhancing lesion of the fourth ventricle suggesting either a glioma or a metastasis. A systemic checkup including hole body CT-scan did not reveal any other lesion. Therefore a complete microsurgical removal of the tumor was performed with uneventful postoperative course. The pathological assessment confirmed the diagnosis of a diffuse B cell lymphoma with an activated B cells' phenotype. The patient underwent a successful polychemotherapy including Methotrexate. A 10 months follow-up is available with disease-free course. Emphasizing on this very rare case, we provide an exhaustive review of the literature.

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Primary Isolated Lymphoma of the Fourth Ventricle in an Immunocompetent Patient

Cited by 11 publications

References 11 publications

Primary Central Nervous System Lymphoma in the Fourth Ventricle

Primary Central Nervous System Lymphoma in the Fourth Ventricle

Unusual primary central nervous system lymphoma location involving the fourth ventricle and hypothalamus

Lymphome primitif à grandes cellules B du quatrième ventricule : cas clinique et revue de la littérature

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