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CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 6  |  Page : 1435-1438

Primary Suprasellar Hypothalamic CNS Lymphoma in an Immunocompetent Adult: A Case Report and Review of Literature


Department of Neurosurgery, Bombay Hospital and Medical Research Centre, 12, New Marine Lines, Mumbai, Maharashtra, India

Date of Web Publication19-Dec-2020

Correspondence Address:
Dr. Chandrasekhar E Deopujari
Department of Neurosurgery, Bombay Hospital and Medical Research Centre, 12, New Marine Lines, Mumbai - 400020, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.304120

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 » Abstract 


Background: Primary suprasellar central nervous system lymphoma (PCNSL) of the hypothalamus is a rare entity.
Material and Methods: We report a case of a 49-year-old, healthy male presented with features of diabetes insipidus. Imaging features showed a mass in the suprasellar region involving the hypothalamus mainly tuber cinereum and infundibulum.
Results: Preoperative fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) showed increased uptake in the mass. Biopsy revealed a diffuse type-B PCNSL. The present case emphasizes the importance of considering the diagnosis of hypothalamic lymphoma and the utility of FDG PET-CT in such situations.
Conclusion: To our knowledge, only eight cases of suprasellar hypothalamic PCNSL have been reported in the literature.


Keywords: Hypothalamus, lymphoma, PET, review, suprasellar
Key Messages: Hypothalamic PCNSL is an extremely rare entity sparingly reported in the literature. PET CT can be of value in the diagnosis.


How to cite this article:
Mohanty CB, Muley KD, Deopujari CE. Primary Suprasellar Hypothalamic CNS Lymphoma in an Immunocompetent Adult: A Case Report and Review of Literature. Neurol India 2020;68:1435-8

How to cite this URL:
Mohanty CB, Muley KD, Deopujari CE. Primary Suprasellar Hypothalamic CNS Lymphoma in an Immunocompetent Adult: A Case Report and Review of Literature. Neurol India [serial online] 2020 [cited 2021 Jan 27];68:1435-8. Available from: https://www.neurologyindia.com/text.asp?2020/68/6/1435/304120




Primary central nervous system lymphoma (PCNSL) is an uncommon intracranial lesion that accounts for 1–2% of all malignant lymphomas and 3% of intracranial tumors.[1],[2],[3] This disease occurs more frequently in patients who are immunocompromised, including those with diseases such as acquired immune deficiency syndrome (AIDS), and rarely occurs in immunocompetent patients.[1] However, the incidence of PCNSL in the immunocompetent population has been reported to have increased >10 fold from 2.5 cases to 30 cases per 10 million population between 1973 and 1992.[2],[3],[4] The cause for the increase in the incidence of this disease in the immunocompetent population is unknown. Surgical resection is generally ineffective due to the deep location of the tumor. Furthermore, treatment with radiotherapy and corticosteroids often only produce a partial response, and tumors recur in >90% of patients. There have been few reports of suprasellar PCNSL isolated to the hypothalamus without infiltration of the brain and in the absence of extracranial systemic signs of non-Hodgkin's lymphoma (NHL). Although hypothalamic masses may be low-grade tumors, the differential diagnoses also include germ cell tumors, CNS lymphoma, and cerebral inflammatory lesions such as neurosarcoidosis and histiocytosis.[5],[6],[7] Invasive biopsy, with its associated risks, is required for definitive diagnosis of these lesions. In the current paper, we report an extremely rare case of isolated suprasellar lymphoma of the hypothalamus in an immunocompetent patient and review the pertinent literature.


 » Case Report Top


A 49-year old male patient was referred to our clinic with 8-weeks history of excessive thirst, polydipsia, polyuria, and the occasional headache. He was otherwise well, with no significant medical history, and diabetes mellitus had been excluded. On examination, he did not have any neurological deficits and had a normal vision. Initial investigations demonstrated normal serum osmolality (293 mmol/kg) with dilute urine (91 mmol/kg) while other pituitary function tests showed panhypopituitarism with an increase in prolactin (53.06 ng/mL) and decrease in vasopressin (6.30 pg/mL). A diagnosis of central diabetes insipidus was made and an oral desmopressin was commenced, with immediate symptomatic improvement. The patient was also started on hormonal supplementation. Fundus examination revealed no papilledema while perimetry showed partial visual field defect in the bilateral temporal superior quadrant. Magnetic resonance imaging (MRI) of this patient revealed a focal nodular lesion in the midline suprasellar, hypothalamic region abutting the mamillary bodies [Figure 1]. Loss of high signal intensity within the posterior pituitary gland was noted along with a normal radiologic appearance of the anterior pituitary. Further examination of the MRI did not reveal any meningeal enhancement. Thus, the varied differential diagnoses of this hypothalamic lesion were high-grade lesions like metastasis, lymphoma, germinoma and low-grade lesion like glioma, autoimmune, or inflammatory hypophysitis (histiocytosis/sarcoidosis). A chest radiograph, cerebrospinal fluid examination, autoimmune profile, antineutrophil cytoplasmic antibody, C-reactive protein, erythrocyte sedimentation rate, beta-human chorionic gonadotrophin, prostate-specific antigen, carcinoembryonic antigen, and alpha-fetoprotein did not suggest sarcoidosis or inflammatory or malignant disease. Therefore, it was difficult to differentiate between a high grade and low-grade lesion in this patient. Hence, a whole-body FDG PET-CT scan was performed. The other aim of this test was to detect systemic site or focus of lesion before undertaking a biopsy of the suprasellar lesion. PET-CT showed increased uptake only in the suprasellar region characteristic of a high-grade lesion [Figure 2] without any other focus elsewhere in the body. The patient underwent right pterional craniotomy and biopsy of the hypothalamic lesion. The histopathology showed a diffuse B-cell type non-Hodgkin's lymphoma [Figure 3] which was confirmed by immunohistochemistry (CD20 +, CD45 +, CD 79a +, CD30 - and CD117 -). The patient was further subjected to methotrexate-based chemotherapy and made a good recovery. Follow-up MRI at 2 years showed complete resolution of the lesion [Figure 4]. Presently patient is on hormonal supplementation without any other neurological deficits.
Figure 1: T2-weighted sagittal (a) and coronal MRI (b) showing suprasellar lesion with intact anterior pituitary. Contrast coronal MRI (c) showing intense enhancement of suprasellar lesion with mild compression of the optic chiasma

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Figure 2: FDG PET-CT scan showing increased uptake in the suprasellar region

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Figure 3: Histopathology reveals typical perivascular aggregation of lymphoma cells

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Figure 4: Follow-up contrast coronal (a) and sagittal (b) MRI after 24 months showing regression of lesion (Arrow indicates the previous site of the tumor)

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 » Discussion Top


Suprasellar hypothalamic lymphoma

Hypothalamic lymphoma can be divided into primary hypothalamic lymphoma, (PCNSL) and secondary metastatic involvement from systemic NHL. PCNSL is predominantly of B-cell origin and is associated with invasion and poor prognosis.[8] However, PCNSL in the suprasellar location does not have a characteristic radiological appearance and thus radiological diagnosis is quite challenging.[9] In the current case, the lesion was suprasellar in location and was isointense on T1-weighted MRI with mild hyperintensity on T2-weighted MRI. It also showed mild diffusion restriction and homogeneous post-contrast enhancement. To our knowledge, there have been only eight reported cases of suprasellar hypothalamic PCNSL in the literature [Table 1].[10],[11],[12],[13],[14],[15],[16] The reported age variation in literature has been 9–71 years and is more commonly seen in males. Hormonal deficiency is the most common presentation. Therefore, hormonal deficiency in the presence of an enhancing mass in the hypothalamus and pituitary stalk should always raise suspicion of PCNSL, irrespective of the age group. All cases reported in the literature were biopsy-proven, except in a 9-year-old boy, where the diagnosis was obtained on lumbar puncture.[12] Thus, lumbar puncture may be useful to diagnose hypothalamic lymphoma before subjecting the patient to a more invasive procedure. The duration of follow-up in the published literature is extremely short ranging from 15 days to 17 months. Therefore, it is difficult to comment if hypothalamic lymphomas have a different outcome compared to PCNSL at other sites. The optimal treatment of these lesions is still unknown, but chemotherapy is the preferred mode of treatment for these patients.
Table 1: Clinical summary of nine patients reported in the literature

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 » Role of PET-CT in PCNSL Top


In view of its high cellularity and high metabolism, PCNSL shows high uptake of FDG. Moreover, FDG also helps in differentiation from other high-grade lesions like metastatic lesions and glioblastoma by the presence of homogenous uptake.[17],[18] Preoperative PET-CT showed increased uptake confirming a high-grade lesion. To our knowledge, the present case is the first case in literature where a preoperative FDG PET-CT was performed for a hypothalamic lymphoma. Thus, this test is especially useful in young patients where a low-grade lesion is usually suspected at this location and can result in delayed treatment.

A large series of 166 patients, initially diagnosed with PCNSL, demonstrated the presence of systemic lymphoma in about 7% of cases, evaluated by PET-CT, which were missed by routine CT and bone marrow biopsies.[19] PET-CT is also helpful to rule out a possible extracranial disease. Identification of a systemic lymphoma is critical since it can alter the staging of the lymphoma and its management strategy.[19],[20] Furthermore, PET has also been shown to have prognostic significance in PCNSL. PCNSL with high 18F-FDG uptake usually signifies a poor prognosis.[18] Patients with a low maximum standardized uptake value (SUVmax) of < 12 has a better prognosis than patients with a SUVmax > 12.[18] In patients treated with chemotherapy, the treatment response depicted by FDG PET predates the changes on MRI. Hence, it has been proposed that appropriate early change in the chemotherapeutic agents can be made based on the degree of uptake in FDG PET.[18]


 » Conclusion Top


The hormonal imbalance with an enhancing hypothalamic mass warrants a biopsy if tumor markers are negative. PCNSL must be considered in the differential diagnosis of hypothalamic masses irrespective of the age group. Preoperative FDG-PET is a useful test to determine the grade of the lesion and degree of extracranial disease and thus is an extremely useful adjunct in the management of hypothalamic lymphomas.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Hochberg FH, Baehring JM, Hochberg EP. Primary CNS lymphoma. Nat Clin Pract Neurol 2007;3:24-35.  Back to cited text no. 1
    
2.
Lee AG, Tang RA, Roberts D, Schiffman JS, Osborne A. Primary central nervous system lymphoma involving the optic chiasm in AIDS. J Neuroophthalmol 2001;21:95-8.  Back to cited text no. 2
    
3.
Eby NL, Grufferman S, Flannelly CM, Schold SC, Vogel FS, Burger PC. Increasing incidence of primary brain lymphoma in the US. Cancer 1988;62:2461-5.  Back to cited text no. 3
    
4.
Corn BW, Marcus SM, Topham A, Hauck W, Curran WJ. Will primary central nervous system lymphoma be the most frequent brain tumor diagnosed in the year 2000? Cancer 1997;79:2409-13.  Back to cited text no. 4
    
5.
Deangelis LM, Hormigo A. Treatment of primary central nervous system lymphoma. Semin Oncol 2004;31:684-92.  Back to cited text no. 5
    
6.
Mascalchi M, Roncaroli F, Salvi F, Frank G. Transient regression of an intracranial germ cell tumour after intravenous steroid administration: A case report. J Neurol Neurosurg Psychiatry 1998;64:670-2.  Back to cited text no. 6
    
7.
Alderson L, Fetell MR, Sisti M, Hochberg F, Cohen M, Louis DN. Sentinel lesions of primary CNS lymphoma. J Neurol Neurosurg Psychiatry 1996;60:102-5.  Back to cited text no. 7
    
8.
Lexa FJ, Grossman RI. MR of sarcoidosis in the head and spine: Spectrum of manifestations and radiographic response to steroid therapy. AJNR Am J Neuroradiol 1994;15:973-82.  Back to cited text no. 8
    
9.
Chimienti E, Spina M, Vaccher E, Tirelli U. Management of immunocompetent patients with primary central nervous system lymphoma. Clin Lymphoma Myeloma 2009;9:353-64.  Back to cited text no. 9
    
10.
Fadoukhair Z, Amzerin M, Ismaili N, Belbaraka R, Latib R, Sbitti Y, et al. Symptomatic hypopituitarism revealing primary suprasellar lymphoma. BMC Endocrine Disorders 2010;10:19.  Back to cited text no. 10
    
11.
Silfen ME, Garvin JH, Hays AP, Starkman HS, Aranoff GS, Levine LS, et al. Primary central nervous system lymphoma in childhood presenting as progressive panhypopituitarism. J Pediatr Hematol Oncol 2001;23;130-3.  Back to cited text no. 11
    
12.
Baleydier F, Galambrun C, Manel AM, Guibaud L, Nicolino M, Bertrand Y. Primary lymphoma of the pituitary stalk in an immunocompetent 9-year-old child. Med Pediatr Oncol 2001;36:392-5.  Back to cited text no. 12
    
13.
Capra M, Wherrett D, Weitzman S, Dirks P, Hawkins C, Bouffet E. Pituitary stalk thickening and primary central nervous system lymphoma. J Neurooncol 2004;67:227-31.  Back to cited text no. 13
    
14.
Singh VP, Mahapatra AK, Dinde AK. Sellar-suprasellar primary malignant lymphoma: Case report. Indian J Cancer 1993;30:88-91.  Back to cited text no. 14
    
15.
Samuels MA, De la Monte S. Case records of the Massachusetts general hospital. Weekly clinicopathological exercises. A 49-year-old man with hypopituitarism, multifocal neurologic defects, and an intracranial mass. N Engl J Med 1994;331:861-8.  Back to cited text no. 15
    
16.
Takasu M, Takeshita S, Tanitame N, Tamura A, Mori M, Fujihara M, et al. Primary hypothalamic third ventriclular Burkitt's lymphoma: A case report with emphasis on differential diagnosis. Br J Radiol 2010;83:e43-7.  Back to cited text no. 16
    
17.
Layden BT, Dubner S, Toft DJ, Kopp P, Grimm S, Molitch ME. Primary CNS lymphoma with bilateral symmetric hypothalamic lesions presenting with panhypopituitarism and diabetes insipidus. Pituitary 2011;14:194-7.  Back to cited text no. 17
    
18.
Kawai N, Zhen H, Miyake K, Yamamaoto Y, Nishiyama Y, Tamiya T. Prognostic value of pretreatment 18F-FDGPET in patients with primary central nervous system lymphoma: SUV-based assessment. J Neuro Oncol 2010;100:225-32.  Back to cited text no. 18
    
19.
Kawai N, Miyake K, Yamamoto Y, Nishiyama Y, Tamiya T. 18F-FDG PET in the diagnosis and treatment of primary central nervous system lymphoma. Biomed Res Int 2013;2013:247152.  Back to cited text no. 19
    
20.
Mohile NA, Deangelis LM, Abrey LE. The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol 2008;10:223-8.  Back to cited text no. 20
    


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