Sellar and clival plasmacytomas: case series of 5 patients with systematic review of 65 published cases

Abstract

Purpose Parasellar plasmacytomas are rare tumors localized to the sellar region arising from plasma cells. Knowledge of clinical, imaging, surgical, and pathological characteristics is limited to single case reports. Methods A retrospective analysis of 5 primary cases was conducted, followed by systematic review of English language articles using PubMed in accordance with PRISMA guidelines. Results Five primary case patients include four men and one woman, ages 60–77, followed up to three years. A systematic review identified 65 additional patients, of whom 65% presented with cranial nerve palsies and 15% with hypopituitarism. Sixteen percent had history of known multiple myeloma (MM) while 37% were diagnosed concurrently with MM on presentation of parasellar plasmacytoma. Imaging showed median tumor size of 38 mm (range, 4–70 mm), with MRI intensity similar to that of other sellar masses. Surgical biopsy with immunohistochemical studies confirmed plasmacytoma diagnosis. Eighty-one percent underwent parasellar radiotherapy, and chemotherapy initiated in 59% of the 69 patients with MM. Overall survival rate was 74% at follow-up (median 12 months), with 18% having parasellar recurrences and 38% progressing to systemic MM after presentation of a solitary plasmacytoma (median 3 months). Conclusions Parasellar plasmacytomas are rare tumors that should be considered in the differential diagnosis for lesions involving the sella and arising from the clivus, especially when cranial nerve paresis is apparent, even in the absence of known MM. Although recurrence rates for parasellar plasmacytoma is low, patients should be monitored for progression to MM. Treatment depends on the presence of systemic disease at diagnosis.

Sellar plasmacytoma presenting with symptoms of anterior pituitary dysfunction

“…Data regarding the anterior pituitary function in patients with sellar plasmacytomas are not always reported, but it is usually well preserved ( 3 , 5 , 6 , 7 , 8 , 9 ), unlike our case. Lee and coworkers ( 6 ) present 1 patient with hypogonadotrophic hypogonadism and 1 patient with hyperprolactinemia, without hypopituitarism. In their review, they mention 38% (of 26 patients with reported prolactin levels) with hyperprolactinemia and 15% (of 33 patients with reported endocrine evaluation) with hypopituitarism (including their own).…”

“…There are less than a hundred known cases. Lee and coworkers ( 6 ) performed a recent review of 70 cases of sellar and parasellar plasmacytomas (including 5 new cases reported by the authors). Another 11 different cases have also been included by Joukhadar and coworkers in their previous review ( 7 ).…”

“…In most of these cases, patients presented with headaches and visual disturbances (mainly diplopia, but also visual loss or blurred vision) ( 1 , 6 , 8 ). Cranial nerve involvement was frequent, mostly III, IV and VI nerves ( 1 , 2 , 8 ), but also II, V, VII and VIII ( 1 , 7 , 9 ).…”

“…Sellar plasmacytomas may be easily mistaken with other sellar masses in imaging studies, so the definite diagnosis is histological ( 1 , 8 ). Some features described in the literature that are commonly related to plasmacytomas include large size, extension to adjacent areas and bone destruction (sellar floor, clivus) ( 6 , 7 ). In their review, Lee and coworkers ( 6 ) report a tendency for homogeneous contrast enhancement, with iso or hypointense lesions on T1-weighted imaging and hyperintense on T2, although they recognize when discussing this issue that plasmacytomas are usually homogeneous masses, iso or hyperintense on T1-weighted imaging and hypointense on T2.…”

“…The 2009 working group of UKMF Guidelines ( 10 ) recommends that solitary extramedullary plasmacytomas should be treated with radiation therapy, which usually allows a good local control of the disease (unlike our patient). Most patients described in the literature with local disease were treated with radiotherapy, many after surgery (only a few with gross total resection), with good response rates, and chemotherapy was mainly used for patients with systemic disease ( 2 , 6 , 7 ). Only a few patients underwent a bone marrow transplant ( 2 , 6 ).…”