Diffuse primary non-Hodgkin's lymphoma of the cranial vault

Abstract

Primary non-Hodgkin's lymphoma of the skull vault is a rare disease. We describe a case occurring in a 72-year-old woman presenting with generalized tonic clonic seizures on a background of a 1-year history of headaches and progressively enlarging scalp masses. Imaging showed diffuse infiltration of the skull vault with multifocal intra- and extracranial soft tissue masses, causing compression and probably infiltration of the cerebral cortex. Further investigation failed to identify any other evidence of systemic lymphoma. Biopsy of one of the scalp masses showed a small to intermediate cell B cell lymphoma. The other nine reported cases of primary skull vault lymphoma are reviewed. The diffuse vault infiltration as well as the multiple intracranial, scalp and temporalis muscle masses renders this case unique.

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

“…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. Therefore, based on the clinical features, the clinician cannot tell if the lymphomatous involvement of the scalp and cranium is from a metastatic process or a primary tumor.…”

“…14,25,39–44 This is a very rare location for primary lymphoma, having been described in less than 40 cases in the world literature. 14 Patients have ranged in age from 19 years to over 70 years, with a mean age of 60 years.…”

Primary skull lymphoma: A case report and review of similar cases

“…We found that sporadic cases have been reported in different journals. [1234567891011121314151617181920212223] The patients have variable clinical presentation and management. To our knowledge, this is the largest series of 25 reviewed cases of primary skull lymphoma ever reported.…”

“…Twenty cases presented as single-focus lesion whereas rest five cases presented as multiple foci or diffuse lesions. [14] The later five cases were included in our study as the disease process was confined to skull bone only without other sites of dissemination.…”

A case of primary skull lymphoma: Review of literature

“…Subgaleal with epidural component Moragas et al [8] 38/M IC Not available Mongia et al [9] 25/M NIC Scalp swelling without BE Kantarci et al [10] 65/M NIC IE+CE without BE. With intra parenchymal extension Madan et al [11] 70/F NIC HD+CE+BE Agrawal et al [12] 43/F NIC HD+CE+HO Andrew et al [13] 80/F NIC HD+CE+BE and intra parenchymal extension Aquilina et al [14] 72/F NIC HD/HI+CE+BE and intra parenchymal extension Szucs et al [15] 42/F IC HD+CE+BE. Subgaleal and intra parenchymal extension Christopher et al [16] 76/F NIC HD/HI+CE+BE.…”