Fig. 15.1
Enhanced CT scan on axial view (a) and sagittal T2-weighted MRI (b) showing a bilateral hypothalamic lymphoma with suprasellar extension, optic chiasm infiltration and brainstem involvement
Fig. 15.2
Axial (a) and sagittal coronal (b) T1-weighted MRIs with gadolinium infusion demonstrate a suprasellar-hypothalamic lymphoma with bifrontal extension
It’s well known that if a CNS lymphoma is suspected, administration of corticosteroids therapy should be delayed until a definitive biopsy has been performed.
The differential diagnosis of a hypothalamic mass includes craniopharyngioma, Rathke cleft cyst, germ cell tumour, granulomatous processes, glioma, hamartoma, germinoma and aneurysm (Samuels and de la Monte 1994; Akhaddar et al. 2001, 2011; Oweity et al. 2002; Chourmouzi et al. 2005; Binning et al. 2008; Rennert J and Doerfler 2007; Raoa et al. 2008; Fadoukhair et al. 2010; Belfquih et al. 2012).
Craniopharyngioma has a heterogeneous appearance with solid and cystic elements tumour in the suprasellar region. Rathke cleft cysts usually have the following imaging features: a sellar epicentre, smooth contour, lack of calcification, lack of internal enhancement and a homogeneous signal intensity within the lesion. The characteristic features of granulomatous diseases are thickening of the pituitary stalk and absence of a normal bright posterior pituitary signal. Imaging of germ cell tumour shows generally an infiltrative, solid, homogeneous mass in the midline with intense contrast enhancement. Hypothalamic glioma tends to be solid with microcyst formation and is iso- or hypointense on T1-weighted images, hyperintense on T2-weighted images and demonstrates enhancement with contrast. Hypothalamic hamartoma presents as precocious puberty in a young child with a nodular mass in the suprasellar cistern. The mass is isointense with normal brain on T1-weighted images and isointense or mild hyperintense on T2-weighted images. These lesions usually do not enhance after contrast administration. Suprasellar germinoma appears as a well-marginated lobulated homogeneous tumour with prolonged T1 and T2 relaxation times which strongly enhances after gadolinium administration. The presence of these imaging findings along with the presence of diabetes insipidus and a suprasellar mass is a clue to the diagnosis of germinoma. Although metastatic tumour could not be ruled out, absence of any primary malignancy on chest, abdominal and pelvic computed tomography scan (CT scan) made it unlikely. Aneurysms of the sellar region usually originate from the cavernous or supraclinoid portions of the internal carotid artery and account for up to 10 % of all cerebral aneurysms. In selected cases, they can mimic other supra-, para- or intrasellar lesions. MRI or CT scan is useful in differentiating tumour from thrombosed and nonthrombosed aneurysms.
The differential diagnosis of primary versus secondary CNS lymphomas may include a complete neurological staging, including cerebrospinal fluid examination and ophthalmological evaluation with slit-lamp examination to exclude vitreous or retinal involvement. In addition, an abdominopelvic CT scan and bone marrow biopsy may be obtained to exclude systemic lymphoma (Giustina et al. 2001).
15.6 Pathology
Histologically, hypothalamic lymphomas are similar to other CNS lymphomas: predominant B-cell non-Hodgkin lymphoma. In our review of 15 reports of patients with hypothalamic lymphoma, 2 did not provide detailed histological description (Patrick et al. 1989; Schwingel et al. 2011), and 13 were B-cell immunophenotype which 2 patients had Burkitt’s lymphoma (Table 15.1).
15.7 Treatment and Outcome
The management of hypothalamic lymphoma is multimodal requiring judicious use of observation, surgery, chemotherapy, radiotherapy and hormone substitution. The initial challenge is establishment of the diagnosis by surgical biopsy: open or stereotactic to differentiate from other suprasellar tumours. Most patients underwent surgical biopsy through stereotactic approach.
In our review, treatments were not observed in three cases, four received chemotherapy alone and one patient received radiotherapy alone. Chemotherapy followed by radiotherapy was performed in four cases. It seems that in immunocompetent patients treated with chemotherapy followed by radiotherapy have considerable improvements in survival (Hobson et al. 1986; Patrick et al. 1989; Jonkhoff et al. 1993; Pioltelli et al. 1996; Lee et al. 2004). Regimens used were different and were in the most cases extrapolated from the protocol used in CNS lymphoma. Despite the increasing number of studies published since a decade on CNS lymphoma and recent therapeutic advances, several questions still remain unanswered about the optimal management of these unusual tumours.
Treatment of immunocompromised patients is aimed at reversing the immunosuppression. If it is reversed, the tumour may regress. In patients with AIDS-associated primary CNS lymphoma, therapy of this type is not possible and the prognosis is very poor as with secondary CNS lymphoma (Samuels and de la Monte 1994).
We attempted to clarify the relationship between treatments and prognosis. However, the treatments varied case by case. In addition, in many reports, the description of clinical outcomes was limited (Matsuda et al. 1999).
Conclusion
Hypothalamic lymphoma is a rare but an increasing clinical entity in recent epidemiological data. It may be a primary tumour or after spreading from an established systemic lymphoma. The clinical presentations are always atypical. Acute presentation may mimic a pituitary apoplexy. MRI findings are largely nonspecific and the tumours are rarely limited to the hypothalamic area. The definite diagnosis depends on histopathologic description. The more common immunophenotype are B-cell non-Hodgkin lymphoma. The overall outcome is favourable with significant survival. Early diagnosis and prompt treatment combination of surgery, chemotherapy, radiation therapy and hormonal substitution can ensure maximal quality of life over the long term. Great attention must be paid to the lasting morbidity associated with pituitary/hypothalamic insufficiencies.
Table 15.1
Summary of demographic data, clinical presentation, imaging characteristics, histopathologic findings, therapy and outcomes of the 15 cases with hypothalamic lymphoma reported in the literature since 1988
Author/year | Sex, age | Primary or secondary localization | Pituitary dysfunction | Neurological dysfunction | Affected sites | Histopathology findings | Treatment | Follow-up/outcome |
---|---|---|---|---|---|---|---|---|
Patrick/1989 | F, 30 y | Primary | Anterior and posterior | Ataxic with spasticity, hyperreflexia and diminished vibration sensation in the lower limbs | Normal CT scan Autopsy: cerebellum, thalamus, cingulated gyri and hypothalamus | Lymphoma (autopsy) | Corticosteroids | No clinical response. Died 26 months after onset |
Balmaceda/1994 | M, 65 y | Primary | Anterior | Declined cognitive function and urinary incontinence | Multiple lesions in lateral, third ventricles, right thalamus, hypothalamus and cerebellum | B-cell lymphoma Kappa light chain immunoglobulin (Stereotactic biopsy) | Intraventricular chemotherapy, systemic chemotherapy Radiotherapy | Clinical improvement Normal pressure hydrocephalus Recurrence 22 months later |
Samuels/1994 | M, 49 y | Primary | Anterior and posterior | Headache, diminished hearing, blurred vision, visual field defect, dysarthria, gait difficulty Cold intolerance | Hypothalamic lesion (20 mm) with extension to suprasellar cistern and optic chiasm | B-cell lymphoma Kappa light chain immunoglobulin (Stereotactic biopsy) | Chemotherapy (6 cycles of methotrexate) Hormonal therapy replacement | Clinical improvement Complete resolution of the mass |
Silfen/2001 | M, 15 y | Primary (Family history of leukaemia, sarcoma and a brain tumour) | Anterior and posterior | – | Nine mm lesion in region of pituitary stalk | B-cell lymphoma suggestive of Burkitt’s lymphoma | Intrathecal chemotherapy (methotrexate) and cytarabine, prednisone, vincristine, cyclophosphamide, doxorubicin and hydrocortisone Hormonal therapy replacement | Clinical improvement Complete mass resolution after 11 months Remission for 17 months |
Lee/2004 | M, 64 y | Primary | Anterior Hyperprolactinemia | Stiff-man syndrome and visual field defect | Hypothalamic tumour (18 mm) with optic chiasm involvement | B-cell lymphoma (Partial resection) | Radiotherapy Hormonal therapy replacement | Clinical improvement Complete mass resolution after 1 year No recurrence after 2 years |
Chourmouzi/2005
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