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Primary lymphoma of the cranial vault: case report and a systematic review of the literature

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Abstract

Background

Bone involvement is a common finding in many types of lymphoma. Cranial vault involvement is extremely rare, and the majority of patients are found at staging to have concurrent disease in lymph nodes. Thirty-eight cases of primary lymphoma of the cranial vault have been reported to date.

Methods

This article presents a rare case of primary cranial vault lymphoma and conducts a systematic review of the current literature. A total of 36 articles comprising 38 cases were included for analysis. The relevant demographic, clinical, and imaging characteristics, as well as the treatment and outcomes of this unique disease presentation were studied.

Results

The average patient age was 60 years. There was no significant difference in patient gender. The predominant patient complaint was a subcutaneous scalp mass. Of the patients, 11.7% were immunocompromised. CT scans showed signs of osteolysis in 74% of lesions and hyperostosis in 5%, and the cranial vault was observed as normal in 18% of cases. MRI was performed in 23 cases. There was a wide range of histological subtypes, with a slight predominance of diffuse large B-cell lymphoma. Treatment consisted of surgery alone, surgery followed by radiotherapy, and surgery followed by radiotherapy and chemotherapy. The follow-up periods ranged from 5 months to 6 years, with a median value of 7 months. Sixteen patients were followed up to 12 months; 13 of them were alive at 1 year from diagnosis.

Conclusion

Primary cranial vault lymphoma is an extremely rare finding. It should be considered in the differential diagnosis of scalp masses. Although the analysis of outcome of the reported cases is difficult because of the small number of occurrences of this entity and the variability of follow-up, a combination of surgery, radiotherapy, and chemotherapy seems to offer better outcomes.

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Conflicts of interest

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Authors and Affiliations

  1. Department of Neurosurgery, Mohammed V Military Teaching Hospital, University of Mohammed V - Suissi, Rabat, Morocco

    Abad Cherif El Asri, Ali Akhaddar, Hassan Baallal, Omar Boulahroud, Cherkaoui Mandour, Miloudi Gazzaz, Brahim El Mostarchid & Mohamed Boucetta

  2. Department of Pathology, Mohammed V Military Teaching Hospital, University of Mohammed V - Suissi, Rabat, Morocco

    Hafsa Chahdi

  3. Department of Hematology, Mohammed V Military Teaching Hospital, University of Mohammed V - Suissi, Rabat, Morocco

    Mohammed Mikdame

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  1. Abad Cherif El Asri
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Correspondence to Abad Cherif El Asri.

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Comment

Medical case reports—detailed descriptions of the symptoms, signs, diagnosis, and follow-up of a single or a few identical cases—have had an essential role in medical publishing. Many firsts in symptoms, diseases or procedures have been presented, including phocomelia from thalidomide (1) or Dr. Christiaan Barnard's first heart transplant (2). Proponents tell us that case reports may present novel data that would be revealed later and with more difficulty from larger series and clinical trials, are inexpensive to produce, and are useful training in medical writing for medical students and residents (if painstakingly supervised).

At present, there are a few open access journals devoted to cases reports only. They include the Journal of Medical Case Reports, for which PubMed has listed 1,681 entries since 2007—a drop in the ocean of what could be reported from sharp-eyed conduct of clinical medicine. The reports should show one of the following:

unreported or unusual side effects or adverse interactions involving medications;

unexpected or unusual presentations of a disease;

new associations or variations in disease processes;

presentations, diagnoses and/or management of new and emerging diseases;

an unexpected association between diseases or symptoms;

an unexpected event in the course of observing or treating a patient;

findings that shed new light on the possible pathogenesis of a disease or an adverse effect.

I am not fond of supervising neurosurgical case reports simply because they are:

anecdotal evidence;

totally at the mercy of reviewers (including me);

less cited than scientific articles;

not accepted as peer-reviewed original articles (n = 3) in the theses of our PhD students;

useless in the grant applications for our neurosurgical research.

This leads to the case reports published in Acta Neurochirurgica, say, since the 1980s:

how many have been published all together?

how did they cover different subspecilities of clinical neurosurgery?

how many times were they cited?

which were cited most?

Juha E. Jääskeläinen

Kuopio, Finland

1. William McBride, Letter to the Editor, in The Lancet, 16 December 1961

2. Barnard CN. The operation. A human cardiac transplant: an interim report of a successful operation performed at Groote Schuur Hospital, Cape Town. S Afr Med J 1967; 41:1271-4

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El Asri, A.C., Akhaddar, A., Baallal, H. et al. Primary lymphoma of the cranial vault: case report and a systematic review of the literature. Acta Neurochir 154, 257–265 (2012). https://doi.org/10.1007/s00701-011-1124-0

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