Mantle cell lymphoma presenting as bilateral adrenal huge masses

Abstract

We present a middle-aged woman complaining of weakness, lethargy and weight loss for 6 months. Positron emission tomography (PET)/CT scan revealed huge bilateral adrenal masses with intense 18F-fluorodeoxyglucose avidity. Biopsy and immunohistochemical staining were compatible with mantle cell lymphoma (MCL). The patient received six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) followed by four cycles of intrathechal methotrexate chemotherapy. Bone marrow aspiration biopsy was performed for initial staging and also before bone marrow transplantation. Follow-up PET/CT scan after completion of chemotherapy and before bone marrow transplantation demonstrated complete metabolic response with no evidence of abnormal metabolic activity in either adrenal gland or bone marrow. MCL has an aggressive nature and is usually considered incurable; however, there have been a few reports indicating favourable treatment response when MCL is rarely arising from the adrenal glands. Dramatic response of a primary adrenal MCL to R-CHOP is documented in this case.

Primary adrenal mantle cell lymphoma mimicking a non‐functional retroperitoneal paraganglioma

“…Primary adrenal lymphoma (PAL) is a rare extranodal involvement, which is very rare with MCL 14 . In about 70% of cases, it is bilateral, 15 unlike our case, which was unilateral and it is often misdiagnosed as adrenal tumors.…”

“…PAL generally has a good prognosis if detected as soon as possible by a combination of medical history, laboratory workup, imaging findings, and histological studies. 14 , 15 , 16 …”

“…It may also lead to adrenal insufficiency, which was normal in our case. PAL generally has a good prognosis if detected as soon as possible by a combination of medical history, laboratory workup, imaging findings, and histological studies 14–16 …”

“…PAL generally has a good prognosis if detected as soon as possible by a combination of medical history, laboratory workup, imaging findings, and histological studies. [14][15][16] In conclusion, patients presenting with abdominal pain and retroperitoneal mass in radiographic images may be in the early stages of primary adrenal mantle cell lymphoma, which requires laboratory studies to evaluate and exclude adrenal disorders, and histological studies for a definite diagnosis.…”

Histopathological Experience of Primary Adrenal Lymphoma From Two Tertiary Hospitals

“…Most of the reported cases of PAL worldwide were DLBCL. In the literature, other histopathological types of PAL have also been described, including Burkitt-like large-cell lymphoma [ 19 ], lymphoblastic [ 20 ], Mantle cell [ 21 ], anaplastic [ 22 ], T-cell lymphoma [ 5 , 23 ], follicular lymphoma [ 24 ], Hodgkin’s lymphoma [ 25 ], and intravascular large B-cell lymphoma (IVLBCL) [ 26 , 27 ]. PAL generally has a dismal prognosis; however, early diagnosis and immediate therapy with a rituximab-containing regimen combined with prophylactic intrathecal chemotherapy can improve the prognosis [ 2 ].…”