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|LETTERS TO EDITOR
|Year : 2019 | Volume
| Issue : 3 | Page : 911-915
Metastatic pituitary lymphoma: Report and literature review
Palak A Jaiswal1, Prakash Nair1, Priya M Jacob2, Mathew Abraham1, Jaypalsinh Gohil1
1 Department of Neurosurgery, Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, Kerala, India
2 Department of Pathology, Regional Cancer Centre, Trivandrum, Kerala, India
|Date of Web Publication||23-Jul-2019|
Dr. Prakash Nair
Department of Neurosurgery, Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum - 695 011, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jaiswal PA, Nair P, Jacob PM, Abraham M, Gohil J. Metastatic pituitary lymphoma: Report and literature review. Neurol India 2019;67:911-5
Systemic lymphoma with metastasis to the pituitary gland is very rare accounting for <0.5% of all reported pituitary metastasis. Their clinical presentation is not specific and may mimic pituitary adenomas. Radiological distinction of pituitary lymphoma is essential for staging the disease as well as to select an appropriate treatment plan. In this case of systemic lymphoma metastasizing to the pituitary, we report the clinical findings at presentation and the distinctive radiological features of secondary pituitary lymphoma [SPL]. We also present a detailed review of literature from PubMed till 2018 on SPL.
A 42-year female patient with no comorbidities presented with complaints of amenorrhea for 6 months and dull aching bifrontal headache with visual field diminution. On further enquiry, she also complained of easy fatiguability, weight loss, and left lower limb pain. The patient denied any history of fever, night sweats, or any family history of lymphomas or autoimmune diseases. The hormonal analysis revealed panhypopitutarism. Visual field charting done by Humphrey field analyzer (HFA) showed bitemporal hemianopia. Magnetic resonance imaging [MRI] of the brain showed a sellar mass extending into the suprasellar cisterns and into the cavernous sinus on both sides, infiltrating the Meckel's cave on the right side and superiorly extending up to the hypothalamus [Figure 1] and [Figure 2]. MRI screening of the spine showed a perineural infiltrative mass in the spinal canal of the lumbosacral region with extraspinal extension through the right S1 spinal foramina and an enlarged left adrenal gland. She was further evaluated with computed tomography of the abdomen and thorax, which showed a massive hepatomegaly with diffusely enlarged homogeneously enhancing left adrenal gland and a lymph node mass in the right presacral region [Figure 3]. Her 24-h urine metanephrine was 119.8 μg/24 h (normal range 25-312 μg/24 h), serum ESR- 30 mm/h, and serum creatine phosphokinase (total) was 101.00 U/L [normal range: 30-135 U/L]. She was negative for human immunodeficiency virus (HIV), hepatitis B surface antigen H(BsAg), and hepatitis C virus (HCV). She underwent laparoscopic biopsy of the presacral lymph node mass. The histopathological examination and immunohistochemistry revealed diffuse B-cell lymphoma [DBCL] [Figure 4]. The cerebrospinal fluid study showed atypical large lymphoid cells with irregular nuclei intermixed with lymphocytes consistent with the diagnosis of large cell lymphoma. The bone scan did not show any abnormal uptake in the skeleton. The bone marrow examination was unremarkable. She was diagnosed with systemic DBCL with secondary central nervous system (CNS) lymphoma [metastasis to pituitary] Stage IV. She was started on chemotherapy but died 2 months after the initiation of chemotherapy.
|Figure 1: T2 axial (a), sagittal (b), coronal (c) images show a well-defined homogeneously T2 hypointense lesion in the sellar–suprasellar region, left cavernous sinus, sphenoid sinus with widening and erosion of sella, and infiltration into the clivus, hypothalamus, and anterior perforator substance region (arrows in c). There is complete encasement of the left cavernous internal carotid artery and further extension across foramen rotundum to the infratemporal fossa. Axial T2 FLAIR image (d) shows the hyperintensity involving the bilateral hypothalami. There is homogenous diffusion restriction with a low apparent diffusion coefficient (e and f)|
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|Figure 2: In T1 coronal image (a), the mass is homogeneously (arrow) T1 iso-intense. In postcontrast T1 coronal (b), axial (c), and sagittal (d) images, the entire lesion shows homogenous contrast enhancement, with involvement [arrows in (b)] of the hypothalamus and extension across the foramen rotundum. There is infiltration of left Meckel's cave and cisternal segment of the left trigeminal nerve [arrow in (c)]. The enhancing lesion extends to the sphenoid sinus, clivus, prepontine cistern, and over the planum sphenoidale (d)|
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|Figure 3: Postcontrast axial and coronal images of the chest, abdomen, and pelvis show (a) the enlarged liver without any focal lesion. (b) A well-defined, peripherally enhancing mass in the right presacral region. (c) Diffusely enlarged left adrenal gland with homogeneous mild enhancing echotexture|
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|Figure 4: Hematoxylin and eosin ×400 (a) sections show fragments of neoplasm composed of tumor cells in diffuse sheets along with extensive crushing artefact. Tumor cells have moderate cytoplasm, large vesicular nuclei, small nucleoli. CD20 ×400 (b): Tumor cells are CD20 positive. ×400 (c): Tumor cells are BCL6 positive. ×400 (d): Tumor cells have a high MIB1 labeling index of 80%|
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The first described case of pituitary metastasis was by L. Benjamin in an autopsy of a patient with disseminated melanoma. Since then, multiple cases of pituitary metastasis have been described and reviewed in detail. Systemic lymphoma with pituitary involvement is very uncommon (comprising just 0.5% of 380 cases) compared with that of secondaries from solid tumours.
After doing a literature search on PubMed using key words “pituitary gland,” “pituitary adenoma,” “pituitary lymphoma,” and “lymphoma of pituitary.” We were able to identify a total of 21 cases [males-16, females-5] of SPL [Table 1]. The mean age of the reported patients with SPL was around 54 years; our patient was around 10 years younger than the average age of presentation. Histologically, B-cell non-Hodgkin's lymphoma was the most common to metastasise to the pituitary.
Most cases of pituitary metastasis and pituitary involvement in lymphoma appeared to be asymptomatic. Diabetes insipidus due to the involvement of the posterior pituitary is the commonest presentation in cases of SPL. The predilection for posterior pituitary involvement may be due to the latter deriving its vascular supply directly from the systemic circulation. Involvement of anterior pituitary may occur due to contiguous spread from the posterior pituitary, the hypothalamus or bony walls of the sella or sinuses. Headache and visual diminution are usually late in appearing and do not help in differentiating a primary pituitary adenoma from metastatic involvement. In the case presented, our patient had features of adenohypophysis dysfunction in form of secondary amenorrhea due to hyperprolactinemia and low levels of luteinizing hormone/follicle stimulating hormone, and features of mass effect causing headache and visual diminution. Hyperprolactinemia in our patient can be attributed to stalk effect; serum prolactin [in dilution] was 112.48 ng/mL [normal value: 3.34–26.72 ng/mL, premenopausal], which is below the level indicative of prolactinoma [>200 ng/mL].
Central nervous system lymphoma typically presents with lesions that are iso- to hypointense on both T1 and T2 weighted images. In immunocompetent patients, the contrast enhancement is intense and homogeneous. When involving the pituitary, MRI also helps in identifying infiltration of the diencephalon, which is never seen in adenomas. A pituitary adenoma may exhibit MR signal intensity similar to those of a lymphoma, but the pituitary tumor does not spread perineurally, as was noted in our case where the lesion was seen progressing along the course of the mandibular nerve [Figure 2]b and [Figure 2]c. Metastasis to the pituitary, unlike pituitary adenomas, does not expand the sella due to the rapid course of the disease and has a low T1WI signal and a high T2WI signal. A simultaneous search for other lesions in the viscera is essential for staging the disease and for obtaining a tissue diagnosis.
Overall, the prognosis of SPL appeared to be guarded, when comparing the reported cases. Hence, an early diagnosis and initiation of treatment are mandatory.
In conclusion, pituitary lymphoma should be considered as a differential diagnosis for sellar masses with suprasellar extension when infiltration of the diencephalon and perineural extension are seen, particularly when the patient is immunocompromised or old. An early diagnosis, disease staging, and histological diagnosis are mandatory, after which an early initiation of treatment may be started. Prognosis depends on the histology at presentation and the stage of the disease.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]