Bulky centroblastic non-Hodgkin's lymphoma of the cranium vault mimicking brain involvement managed with chemotherapy. A case report

Abstract

Background. Patients with central nervous system (CNS) involvement by high grade non‐Hodgkin's lymphoma (NHL) have a poor prognosis. The roles of computed tomography, radiotherapy, and intrathecal and systemic chemotherapy still need to be defined. Methods. A patient with bulky cranial lymphoma mimicking brain involvement is reported. A 62‐year‐old man was admitted with a huge scalp lump, headache, fatigue, and focal and generalized neurologic symptoms. Computed tomography showed an abnormal mass in the frontoparietal region involving the subcutaneous scalp, osteolytic destruction of the cranial vault, and a bulky mass that was interpreted to be intracranial. A systemic survey also revealed bulky retroperitoneal involvement and focal involvement of the spleen. Biopsy revealed a B‐cell NHL of centroblastic type according to the Kiel classification. Results. The patient was treated with a modified combination of cyclophosphamide plus mitoxantrone plus vincristine plus prednisone (CNOP) and intrathecal methotrexate. The patient responded with complete remission, including partial bone restoration of the cranium. At the time of this writing, his relapse free survival lasted 5 years. Conclusions. The initial interpretation of this case indicated that systemic chemotherapy with modified CNOP plus intrathecal methotrexate would be useful in the management of NHL with CNS involvement. The clinical outcome with rapid neurologic repair and also bone restoration of the cranial vault within 5 years suggests that the lymphoma probably never penetrated the dura and a successful treatment was achieved with combination chemotherapy only.

Primary Diffuse Large B-Cell Lymphomas of the Temporoparietal Dura Mater and Scalp Without Intervening Skull Bone Invasion -Case Report-

“…[2][3][4][5][6]10,11,[13][14][15]18,19) In contrast, in our case, the dural and subcutaneous tumors existed independently without intervening skull invasion as confirmed by the preoperative images and surgical findings. Histological diagnosis confirmed these tumors as DLBCLs.…”

Primary diffuse large B-cell lymphoma of the dura mater and cranial vault

“…Primary extraaxial involvement of large cell type B lymphoma is infrequent, with the worldwide literature containing only a few cases. 9,10,12,23,[35][36][37]41 The precise anatomical location of the neoplasm reported here, without systemic manifestation, makes this case unique in its type.…”

“…Approximately 30% of cases originate in extranodal sites, particularly the gastrointestinal tract and the Waldeyer ring, but also in bone, skin, sinuses, eyes, ocular adnexa, gonads, the CNS, thyroid, and lungs. 2,8,10,17,23,27,35,37,40 In unselected series of cases the incidence of primary CNS lymphomas in brain tumors varies from 0.3 to 1%, and the entity constitutes approximately 1% of all lymphomas. 2,17,27,40 Within the CNS, these lymphomas have a predilection for the midline or paraventricular structures.…”

A 60-Year-Old Indian Male With Altered Sensorium and Extensive Lymphoma of the Scalp

“…The clinical presentation usually involves an enlarging scalp mass, localized pain and headache, fatigue, and various neurologic symptoms (eg, seizures, focal weakness). 38 For primary lymphomatous tumors of the scalp and cranial vault, the clinical signs and symptoms are quite similar. 14,25,39–44 Most patients will present with an enlarging subcutaneous scalp mass, along with symptoms of headache, localized scalp pain, fatigue, and neurological complaints if the underlying brain is involved, such as seizures, confusion, and focal weakness.…”