Primary sellar lymphoma: intravascular large B-cell lymphoma diagnosed as a double cancer and improved with chemotherapy, and literature review of primary parasellar lymphoma

Abstract

Lymphoma is one of the causative factors of hypothalamus-pituitary dysfunction, and intravascular large B-cell lymphoma (IVLBCL) is a subtype of primary extranodal neoplasm. A 69-year-old woman visited our hospital because of general fatigue. We diagnosed her with presumable non-functional primary pituitary adenoma and subsequent dysfunction. Eight months after, the patient revisited our hospital because of dyspnea. Though we conducted systemic investigations including chest and abdomen enhanced computer tomography, transbronchial lung biopsy, and bone marrow biopsy, the diagnosis was not confirmed. Inadvertently, a breast cancer was found, and the surgical specimen proved that the patient had double cancer-adenocarcinoma and IVLBCL. Rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone regimen was initiated, and complete remission was achieved. Notably, the sellar mass returned to normal size with improved function. We reviewed 32 patients with primary parasellar lymphoma. In affected sites, both sellar and pituitary stalk (6.7%), both hypothalamus and pituitary stalk (6.7%), only sellar (63.3%), only pituitary stalk (6.7%), only hypothalamus (13.3%), and only clivus (3.3%) were observed. In hypothalamus-pituitary dysfunction, both anterior and posterior dysfunction (20.7%), only anterior dysfunction (58.6%), only posterior dysfunction (3.4%), and no dysfunction (17.2%) were observed. It seemed that hypothalamic lesion is related to both anterior and posterior dysfunction, while sellar lesion is related to mainly anterior dysfunction. In cranial nerve dysfunction, 2nd nerve dysfunction (45.2%) and 6th nerve dysfunction (35.5%) were frequently observed. It seemed that sellar lesion is related to both 2nd and 6th nerve dysfunction, while hypothalamic lesion is related to mainly 2nd nerve dysfunction.

Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy

“…Moreover, many of the patients died, and the hormonal evaluation after treatment was not always mentioned even in the survived cases (Wick et al 1986;Smadja et al 1991;Prayson et al 1991;Demirer et al 1994;Kraus et al 1999;Mathiasen et al 2000;Schleinitz et al 2002;Price et al 2002;Svajdler et al 2006;Lee et al 2011;Anila et al 2012;Akhtar et al 2013). Thus far, the endocrinological course has been described in only two cases in the literature (Table 4) (Pekic et al 2008;Yasuda et al 2010). In both cases, hormone replacement therapy was necessary.…”

“…Lymphoma is one of the potential causes for pituitary dysfunction, whereas there have been less than 20 case reports of hypopituitarism induced by IVLBCL. Moreover, only two reports have described the course of hormone levels after treatment of IVLBCL (Pekic et al 2008;Yasuda et al 2010).…”

Primary pituitary lymphoma in an immunocompetent patient: a rare clinical entity

“…High-dose MTX is the only independent treatment-related factor that clearly improved the prognosis [29]. Patients treated with chemotherapy followed by radiotherapy have considerable improvement in survival [30][31][32]. Many patients achieved disease-free survival with some regimen alone [33].…”

Pituitary and adrenal involvement in diffuse large B-cell lymphoma, with recovery of their function after chemotherapy

“…Diffuse large B-cell lymphoma (DLBCL) sometimes involves the endocrine organs, but the involvement of both the adrenal and pituitary glands is extremely rare, both at the time of initial presentation and late in the disease course [1,2]. Similar to pituitary adenomas, pituitary lymphomas may present with symptoms of anterior pituitary hormone dysfunction.…”