|Year : 2021 | Volume
| Issue : 2 | Page : 52-55
Multiple myeloma masquerading as sellar mass: A case report and review of literature
Jaspreet Singh1, Swayamsidha Mangaraj1, Priyanka Samal2, Pritish Chandra Patra2
1 Department of Endocrinology, IMS & SUM Medical College and Hospital, Bhubaneswar, Odisha, India
2 Department of Hematology, IMS & SUM Medical College and Hospital, Bhubaneswar, Odisha, India
|Date of Submission||02-May-2021|
|Date of Acceptance||16-Feb-2022|
|Date of Web Publication||20-Apr-2022|
Dr. Swayamsidha Mangaraj
Department of Endocrinology, IMS & SUM Medical College and Hospital, Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
Tumors involving sellar and parasellar regions can present with headache, visual disturbances, cranial nerve deficits, and other neurological symptoms. Patients with such tumors can also present with endocrine dysfunction due to a hormone excess or deficient state. As many lesions in this critical area are slow growing in nature, they may evade early clinical detection and may present after a long period of time. Pituitary adenomas represent an overwhelming majority of sellar masses, whereas various nonpituitary tumors or metastases can also present with similar findings. Differentiation between pituitary and nonpituitary pathology is critical, as management strategies for the disorders mentioned earlier differ significantly. In such cases, radiological and histopathological evaluations are pivotal for arriving at a correct diagnosis. We describe an interesting case of sellar mass that presented with cranial nerve deficits and endocrine dysfunction, which was subsequently attributed to the myelomatous involvement of sella due to a relapse of multiple myeloma.
Keywords: Multiple myeloma, pituitary adenoma, plasmacytoma, sellar mass, sellar plasmacytoma
|How to cite this article:|
Singh J, Mangaraj S, Samal P, Patra PC. Multiple myeloma masquerading as sellar mass: A case report and review of literature. Int J Neurooncol 2021;4:52-5
| Introduction|| |
Sellar masses can arise due to various pituitary and nonpituitary pathologies. Among these, pituitary adenomas are one of the most common causes of sellar masses encountered in routine clinical practice. Patients with these are brought to clinical attention due to either compressive symptoms (such as headache, visual disturbances etc.) and/or symptoms pertinent to hormonal dysfunction. However, in contrast to pituitary adenomas, sellar metastases presenting as sellar masses are relatively rarely seen. The incidence of sellar metastases from primary malignancy is low. Despite many similarities in clinical presentation akin to pituitary adenomas, subtle differences exist with regard to findings seen in cases of sellar metastasis.
Multiple myeloma is one of the most common hematological malignancies seen in routine clinical care. It is a plasma cell disorder characterized by clonal proliferation of malignant plasma cells in the marrow. The common manifestations include anemia, various degrees of cytopenias, lytic bone disease, hypercalcemia, and renal dysfunction. Despite skeletal involvement being a well-known facet of multiple myeloma, intracranial involvement is, indeed, very rare. Hence, sellar/parasellar plasmacytoma is a rare entity. We describe an interesting case of sellar mass attributed to a relapse of multiple myeloma with a review of literature.
| Case report|| |
A 61-year-old female presented with complaints of generalized fatigue, lethargy, and backache for the past four months. She also complained of headache, double vision, and drooping of the left eyelid for the past one month. The patient was diagnosed with multiple myeloma 10 years ago and had completed the prescribed course of chemotherapy at that time. On examination, she had an average build with a BMI of 23.7kg/m2. She had a pulse rate of 90 per minute and blood pressure of 130/80 mm Hg. She was anemic. There was no evidence of icterus, cyanosis, clubbing, or pedal edema. Examination of the chest and the cardiovascular system revealed no significant abnormality. Neurological assessment revealed the presence of left third cranial nerve palsy. Hematological and biochemical assessment revealed hemoglobin of 7.7gm% (normal:12–15), total leukocyte count of 5720 cells/cu.mm (normal:4000–10000), platelet count of 1.72 lac/cu.mm (normal:1.5–4), serum urea of 41 mg/dl (normal:17–45), serum creatinine of 0.22 mg/dl (normal: 0.5–1.3 mg/dl), serum sodium of 134meq/l (normal:135–145), serum potassium of 4.5meq/l (normal: 3.5–5.3), serum calcium of 10.2 mg/dl (normal: 8.8–10.3), serum phosphorus of 4.0 mg/dl (normal: 2.5–4.5), serum albumin of 3.3 g/dl (normal: 3.5–5), serum globulin of 5.7 g/dl (normal: 2.5–3.2), and alkaline phosphatase of 151 IU/L (normal: 30–120). Hormonal evaluation revealed serum follicle stimulating hormone of 2.40 mIU/ml (normal: 30–130), serum luteinizing hormone of 3.1mIU/ml (normal: 16–64), serum cortisol (8 AM) of 12.36mcg/dl (normal: 5–18), serum triiodothyronine of 58.13ng/dl (normal: 84–201), serum tetraiodothyronine of 4.35 mcg/dl (normal:5–14), serum thyroid stimulating hormone of 0.43mIU/ml (normal: 0.2–4.2), and serum prolactin of 47ng/ml (normal <30). Serum electrophoresis revealed M band. Her bone marrow biopsy showed a hypercellular marrow with moderate to focal marked plasma cell infiltrates with 36% plasma cells, suggestive of multiple myeloma. MRI brain showed a large expansile lytic lesion arising from the clivus with a suprasellar and a parasellar extension [Figure 1]A and B. MRI spine showed multiple lytic lesions in dorsocervical and lumbar vertebra [Figure 2]. Apart from this, multiple lytic lesions were also noted in the pelvis, skull, and ribs. These radiological findings were suggestive of diffuse skeletal myelomatous involvement. For a definite diagnosis of the sellar lesion, a transspenoidal biopsy and subsequent immunohistochemistry analysis was planned. However, the patient refused for the procedure to be performed and, hence, it could not be done. Based on the earlier findings, a probable diagnosis of multiple myeloma relapse with widespread skeletal involvement (including sella) was made. The patient was started on daratumumab, carfazamil, and dexamethasone-based chemotherapy for the management of multiple myeloma as per the advice of a consultant hematologist. The patient was started on oral levothyroxine replacement (50mcg/day). For management of the sellar lesion, a course of palliative radiotherapy was planned as per the advice of the radiation oncologist. However, the patient was lost to follow-up.
|Figure 1: (A) Magnetic resonance imaging (sagittal view) of brain showing a large expansile mass arising from the clivus with suprasellar and infrasellar extension. (B) Magnetic resonance imaging (coronal view) of brain showing expansile sellar mass arising from the clivus with suprasellar and parasellar extension|
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|Figure 2: Magnetic resonance imaging of spine showed multiple lytic lesions in dorso-lumbar vertebral bodies and posterior elements with vertebral collapse|
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| Discussion|| |
Pituitary adenomas represent an overwhelming majority of the cases of sellar masses. These adenomas are reported to account for 90% of sellar and parsellar lesions., In one of the largest described surgical series involving 1469 subjects from a single center, Elena Valassi et al. reported that among all the operated sellar lesions, around 8% were nonpituitary in origin. Similarly, in one of the largest radiological series describing sellar and parasellar lesions involving 2598 subjects who underwent pituitary MRI, it was reported that only 18% of cases were attributed to non-adenomatous sellar masses. In their series, metastases accounted for around 5% of nonpituitary lesions, including a single reported case of sellar plasmacytoma. The malignancies with frequently described pituitary metastasis include breast cancer, lung cancer, and adenocarcinoma of the prostrate gland. Apart from classical multiple myeloma, plasma cell neoplasms may also present with solitary plasmacytoma (without marrow involvement or with minimal marrow involvement). Despite widespread skeletal involvement in multiple myeloma, sellar involvement is rarely seen.
To the best of our knowledge, around 100 cases of sellar and clival plasmacytomas have been described in literature till date [Table 1].,,,,,,,,,,,, Parasellar plasmacytoma is believed to arise from surrounding mucosa of the sellar region and clivus., These are usually locally aggressive, resulting in osteolytic destruction of the sphenoid and clivus bone. Yaman et al. have reported three distinct presentations of parasellar plasmacytoma. These can present as solitary plasmacytomas with no evidence of systemic disease, may lead to the development of overt multiple myeloma over a period of time, and may appear in individuals already suffering from multiple myeloma. Clinical manifestations of nonpituitary sellar include headache, visual disturbances, cranial nerve palsies, and features of hormone deficiency. These sign/symptoms frequently overlap with those seen in pituitary macroadenomas (especially nonfunctional adenomas).
|Table 1: Summary of clinical and radiological characteristics of reported cases, including index case|
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The radiological differentials of sellar plasmacytoma are broad and complex. However, two distinct entities that deserve special mention are chordoma and pituitary adenoma. It has been reported that on MRI, chordoma have iso/hypointense T1-weighted and hyperintense T2-weighted images with heterogeneous enhancement on gadolinium administration. In contrast to this, parasellar plasmacytomas exhibit homogeneous enhancement (post-gadolinium). This feature may help in differentiating the two conditions. Clival invasion and sellar floor erosion have been reported in around 75% of cases in parasellar plasmacytomas. On the other hand, the radiological findings of parasellar plasmacytoma and pituitary adenomas may be indistinguishable. Hence, in the majority of cases, they are often mistaken for pituitary adenomas. Therefore, it is not unusual in clinical practice that eventual diagnosis is made after transspenoidal biopsy and histopathological examination. It is interesting to note that sometimes poorly differentiated plasma cells may mimic the pattern of atypical pituitary adenoma and this can result in pathological dilemma. The demonstration of monoclonal plasma cells with CD138 and/or CD79a expression on immunohistochemistry can confirm the diagnosis of a plasma cell neoplasm.
The management strategies for these rare and difficult lesions include surgery, radiation therapy, and systemic chemotherapy., Frequently, a combination of these modalities is employed. Radiotherapy is very often the preferred modality of treatment because of its efficacy in reducing local tumor growth and the prevention of progression to overt multiple myeloma in case of solitary parasellar plasmacytoma., However, chemotherapy is attempted for systemic and widespread disease.
| Conclusion|| |
As exemplified by our case, multiple myeloma can present with sellar mass in rare cases. A high degree of clinical suspicion aided with appropriate radiological, pathological, and endocrine evaluation is necessary to differentiate primary pituitary from nonpituitary causes. Due to a myriad of symptoms and varied presentation, these patients may present to a wide array of clinicians such as physicians, neurologists, endocrinologists, neurosurgeons, oncologists, and hematologists. Hence, knowledge about this rare entity among treating physicians should be quite helpful for appropriate diagnosis and management.
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Conflicts of interest
There are no conflicts of interest.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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[Figure 1], [Figure 2]