Primary Bilateral Adrenal Intravascular Large B-cell Lymphoma Associated with Adrenal Failure

Abstract

Wereport a rare case of bilateral primary adrenal non-Hodgkin's lymphoma with adrenal failure. A 66-year-old womandeveloped symptoms of adrenal failure. The cause of adrenal failure was suspected to be malignant lymphomabased on the high levels of serum soluble interleukin-2 receptor and LDH. Bilateral adrenalectomy was performed and pathological examination showed intravascular large B-cell lymphoma (IVL). Although complete remission was achieved, recurrence occurred three months later with brain metastases. IVL should be suspected in patients with bilateral adrenal tumors who present with rapidly progressive adrenal failure. (Internal Medicine 42: 609-614, 2003)

Intravascular Lymphoma: The Oncologist's “Great Imitator”

“…Proof of this supposition, however, can only come from extensive evaluation of integrin expression in IVL cases from disparate organs. Beyond these major presentations, there are single case reports of IVL presenting primarily in almost every organ system; as interstitial lung disease [18], adrenal failure [19], pulmonary hypertension [20], nephrotic syndrome [21], myocardial infarction [22], and symmetric polyarthritis [23].…”

Primary Bilateral Adrenal Diffuse Large B-cell Lymphoma Demonstrating Adrenal Failure

“…In the current case, a CT-guided fine needle biopsy was performed. Percutaneous fine needle biopsy under CT or ultrasound guidance can only be recommended in cases of suspicious PAL (8,(11)(12)(13)(14)(15)(16). The tumor was comprised of large cells, which were immunohistologically positive for CD20 and CD79a.…”

“…Thus, the tumor was diagnosed as a DLBCL. In fact DLBCL is the most common histological diagnosis for bPAL (4,12). As a histological differential diagnosis, we should consider intravascular large B-cell lymphoma (IVL).…”

“…IVL is classified as DLBCL subtype, according to the new WHO classification (17). IVL is characterized by intravascular proliferation of malignant lymphomatous cells, which frequently invade the central nervous system (CNS), skin, lungs and kidneys (12,18,19). Both pituitary and adrenal involvement are frequent in IVL patients (12,20,21).…”

“…IVL is characterized by intravascular proliferation of malignant lymphomatous cells, which frequently invade the central nervous system (CNS), skin, lungs and kidneys (12,18,19). Both pituitary and adrenal involvement are frequent in IVL patients (12,20,21). Noteworthy is that CNS infiltration is described in 50% of Japanese patients with primary adrenal IVL (12).…”