Diffuse Large B-Cell Lymphoma with Mass Lesions of Skull Vault and Ileocecum

Abstract

We report a rare case of non-Hodgkin lymphoma with mass lesions of skull vault and ileocecum. The patient was an 82-year-old Japanese woman who exhibited a painless subcutaneous scalp tumor in the right parietal region associated with no neurological abnormalities. Magnetic resonance imaging of the head demonstrated a mass in the skull vault with iso- to hypointense signals on both T1- and T2-weighted imaging. Biopsy of the mass revealed that the tumor comprised large cells that were immunoreactive for CD20 (L-26) and CD79a. Diffuse large B-cell lymphoma (DLBCL) was therefore diagnosed. Further investigation could not identify any other evidence of systemic lymphoma other than ileocecal lesions. She was treated by irradiation (45 Gy) of the mass on the parietal bone and with rituximab, pirarubicin, cyclophosphamide, and vincristine. The patient achieved complete remission after 3 cycles of systemic chemotherapy. As of 30 months after presentation, no signs of lymphoma have been found.

Characteristics of Bone Destruction in Cranial Vault Lymphoma Compared with Other Skull Tumors

“…Symptoms of cranial vault lymphoma have been reported to include a painless scalp mass and focal neurological deficits secondary to infiltration of the cerebral cortex [1,[4][5][6][7][8][9][10][11][12]. Primary cranial vault lymphoma is rarely different from typical metastatic bone lymphomas, and it is defined as solitary mass lesion without any evidence of disease at another site and no systemic dissemination within 6 months of tumor detection [13]. Intracranial symptoms, such as seizures and hemiplegia, have also been observed [14][15][16].…”

Primary diffuse large B-cell non-Hodgkin lymphoma of the cranial vault

“…[4] Only 19 cases have been reported in the literature out of which six cases had tumor localization in the frontal region. [11] The incidence of lymphomas in the central nervous system are increasing nowadays due to the increasing incidence of immunocompromised patients. Primary malignant lymphomas involving only the cranial vault and scalp without any systemic or cerebral parenchymal involvement is rare, and only a few cases have been reported in the literature.…”

“…[5][6][7][8][9] The initial symptoms and signs of cranial vault lymphoma include a painless lump in the scalp, headache because of bone destruction or tumor infiltration of meninges and focal neurological deficits secondary to tumor infiltration of the cerebral cortex. [11] Lymphoma cells may infiltrate the spaces within the diploe and along the emissary veins extend to infiltrate the soft tissues on either side of the bone. Malignant lymphomas of the skull bone may present with bony changes at first and by infiltration may lead to complete destruction of the skull.…”

“…The incidence of large cell lymphomas reported in the literature is approximately 4.68 cases/100,000 persons per year. [3,10,11] These tumors may extend into the brain or the scalp mimicking a meningioma. [4] Most of these reported cases were in the age group of 50s and 60s whereas age of our patient was only 37 years.…”

Role of Ultrasound in a Rare Case of Primary Lymphoma of the Cranial Vault

“…Non-Hodgkin lymphomas of the skull are infrequent and generally secondary. A primary bone localization is rare [1,2], especially in the skull [3]; only 37 cases have been reported in the literature [4,5].…”