Aggressive primary scalp lymphoma mimicking an acute epidural hematoma: Case report and review of the literature

Neurochirurgie. 2022 Oct;68(5):e34-e39. doi: 10.1016/j.neuchi.2022.03.005. Epub 2022 Apr 25.

Abstract

Background: Cutaneous Central Follicular Lymphoma (CCFL) is a type B cutaneous lymphoma with a usually indolent course. Scalp localization of CCFL is extremely rare, we report a new case mimicking an epidural hematoma, and showing a rapid progression with aggressive infiltration of skin, calvaria, dura and brain parenchyma.

Case report: A 58-year-old patient with an unlabeled polymalformative syndrome was admitted to the Emergency department following a head injury secondary to a self-resolving tonic-clonic epileptic seizure. The initial CT-scan was interpreted as a minor subcutaneous and epidural hematoma initially deemed for conservative management. Within 4 days, the patient showed a progressive neurological deterioration culminating into a stuporous status which prompted a constrast-enhanced brain MRI. The scan revealed a multilayered solid lesion, extending from the subgaleal compartment to the subdural space, threatening the integrity of overlying skin and causing infiltration of the brain parenchyma. Following emergency neurosurgical excision a definitive histology diagnosis of central follicular lymphoma was made. A focused chemotherapy with high-dose Methotrexate with R-CHOP protocol led to disease control until the latest follow up at 2 years.

Conclusion: To our knowledge, this case represents the first CCFL invading the brain parenchyma and the second extending to the dura. Although such tumor is usually indolent the aggressive behavior herein reported extend the differential diagnosis to high-grade meningiomas, sarcomas, and metastases. Prognostication and appropriate adjuvant treatment require prompt surgical excision and histological confirmation.

Keywords: Calvaria; Central nervous system; Chemotherapy; Lymphoma; Subgaleal lesion.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Hematoma, Epidural, Cranial* / diagnosis
  • Hematoma, Epidural, Cranial* / etiology
  • Hematoma, Epidural, Cranial* / surgery
  • Humans
  • Lymphoma, Follicular* / complications
  • Methotrexate
  • Middle Aged
  • Scalp
  • Tomography, X-Ray Computed / adverse effects

Substances

  • Methotrexate