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reported in only 4% of cases, which is typically seen Differential diagnosis of cranial vault NHL with intra and
in femur, tibia, pelvis, spine mandible, and scapula. [1,6] extracranial extensions include metastatic or primary
Skull vault lymphoma needs special attention from skull tumors and intraosseous meningioma. There is
other bony lymphomas because the cerebral structures widespread cortical destruction in these cases, while
may get involved by direct invasion. Usually these little cortical destruction is noted in lymphomas. Total
are intraaxial with pachy- and leptomeningeal surgical excision is rarely possible in lymphomas and
involvement being frequent. True primary malignant treatment of these tumors is surgical removal, followed
lymphoma of the bone is defined as a solitary mass by radiotherapy and chemotherapy. Treatment with
lesion without any evidence of disease at another irradiation and corticosteroids often produces a partial
site and no systemic dissemination within 6 months response. Therefore, tumor recurs in > 90% of patients.
of tumor detection. [7,8] The present case thus fulfills Median survival is 10-18 months in immunocompetent
the criteria for primary malignant lymphoma of bone. patients and less in immunocompromised patients. [13-16]
Primary cranial vault lymphomas have been reported In our case, there was no sign of recurrence after
in immunocompromised or trauma patients; [9,10] 12 months of follow-up.
however primary NHL of the cranial vault with
extra- and intra-cranial extension without systemic or The prognosis of primary cranial vault malignant
skeletal manifestation in a nonimmunocompromised lymphoma is unknown, and any involvement of
or nontraumatic is extremely uncommon. Only cerebral structures and systemic involvement suggests
[4]
19 cases have been reported in the literature out an unfavorable prognosis. Until date, no optimum
of which six cases had tumor localization in the treatment for cranial vault malignant lymphoma has
frontal region. [11] The incidence of lymphomas in the been established. Therefore, a thorough search is
central nervous system are increasing nowadays due necessary to improve the treatment of cranial vault
to the increasing incidence of immunocompromised lymphoma treatment and its prognosis.
patients. Primary malignant lymphomas involving
only the cranial vault and scalp without any systemic REFERENCES
or cerebral parenchymal involvement is rare, and only 1. Braunstein EM, White SJ. Non-Hodgkin lymphoma of bone.
a few cases have been reported in the literature. [5-9] The Radiology 1980;135:59-63.
initial symptoms and signs of cranial vault lymphoma 2. Holtås S, Monajati A, Utz R. Computed tomography of
include a painless lump in the scalp, headache because malignant lymphoma involving the skull. J Comput Assist Tomogr
of bone destruction or tumor infiltration of meninges 3. 1985;9:725-7.
Maiuri F, Corriero G, Giamundo A. Primary lymphoma of the cranial
and focal neurological deficits secondary to tumor vault. J Neurosurg Sci 1987;31:183-6.
infiltration of the cerebral cortex. [11] Lymphoma cells 4. Agbi CB, Bannister CM, Turnbull IW. Primary cranial vault
may infiltrate the spaces within the diploe and along lymphoma mimicking a meningioma. Neurochirurgia (Stuttg)
the emissary veins extend to infiltrate the soft tissues 5. 1983;26:130-2.
Rosenberg SA, Diamond HD, Jaslowitz B, Craver LF.
on either side of the bone. Malignant lymphomas Lymphosarcoma: a review of 1269 cases. Medicine (Baltimore)
of the skull bone may present with bony changes 1961;40:31-84.
at first and by infiltration may lead to complete 6. Pear BL. Skeletal manifestations of the lymphomas and leukemias.
destruction of the skull. In our case, there was 7. Semin Roentgenol 1974;9:229-40.
Coley BL, Higinbotham NL, Groesbeck HP. Primary reticulum-cell
destruction of frontal bone and tumor mass infiltrated sarcoma of bone; summary of 37 cases. Radiology 1950;55:641-58.
the underlying dura. Furthermore, there was no focal 8. Shoji H, Miller TR. Primary reticulum cell sarcoma of bone. Significance
neurological deficit, and postoperative cerebrospinal 9. of clinical features upon the prognosis. Cancer 1971;28:1234-44.
Kelleher AD, Brew BJ, Milliken ST. Intractable headache as the
fluid cytology was negative for lymphoma cells. presenting complaint of AIDS-related lymphoma confined to bone.
Involvement of the meninges has been speculated J Acquir Immune Defic Syndr 1994;7:629-30.
due to the extension through the diploic spaces along 10. Tagawa M, Momita S, Irie J. Primary B cell lymphoma of the
the emissary veins and nerves that pass through the skull following head trauma; a case report. Rinsho Ketsueki
1987;28:589-93.
dura to the leptomeninges. [12] Being rare only a high 11. Kosugi S, Kume M, Sato J, Sakuma I, Moroi J, Izumi K, Sato Y,
index of suspicion can diagnose these cases prior to Nakamura N, Takahashi M, Miura I. Diffuse large B-cell lymphoma
histopathology. with mass lesions of skull vault and ileocecum. J Clin Exp Hematop
2013;53:215-9.
12. Mongia S, Shukla D, Devi BI, Reddy TV. Primary cranial vault
The incidence of large cell lymphomas reported in non-Hodgkin’s lymphoma. Neurol India 2003;51:293-4.
the literature is approximately 4.68 cases/100,000 13. Rezaei-Kalantari K, Samimi K, Jafari M, Karimi MA, Ansari K,
persons per year. [3,10,11] These tumors may extend into Davoodi M, Nabi-Meybodi M, Gorjian M. Primary diffuse large B
cell lymphoma of the cranial vault. Iran J Radiol 2012;9:88-92.
the brain or the scalp mimicking a meningioma. Most 14. Iwamoto FM, DeAngelis LM, Abrey LE. Primary dural lymphomas:
[4]
of these reported cases were in the age group of 50s a clinicopathologic study of treatment and outcome in eight patients.
and 60s whereas age of our patient was only 37 years. Neurology 2006;66:1763-5.

100 Neuroimmunol Neuroinflammation | Volume 1 | Issue 2 | September 2014