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Inicio Acta Otorrinolaringológica Española Secondary Presentation of Mantle Cell Lymphoma in the Subgaleal Layer of the Sca...
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Vol. 67. Issue 2.
Pages e13-e15 (March - April 2016)
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1311
Vol. 67. Issue 2.
Pages e13-e15 (March - April 2016)
Case study
DOI: 10.1016/j.otoeng.2016.03.007
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Secondary Presentation of Mantle Cell Lymphoma in the Subgaleal Layer of the Scalp
Afectación subgaleal secundaria del linfoma de células del manto
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Montserrat Borràs Pereraa,
Corresponding author
monboper@gmail.com

Corresponding author.
, Javier Galindo Ortegoa, Eduard Bodet Agustía, Jordi Tarragona Foradadab
a Servicio de Otorrinolaringología, Hospital de Santa María de Lleida, Lleida, Spain
b Servicio de Anatomía Patológica, Hospital de Santa María de Lleida, Lleida, Spain
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Clinical Case

We present the case of a male aged 57, with a 1.5cm stage 11b right laterocervical tumour and a 3cm ipsilateral occipital tumour, of 5 year duration, which had progressively enlarged over the last few months. A cervical scan was performed which showed a right laterocervical adenopathy, 17mm in diameter, with no signs of normal lipid hilum, and a occipital tumour suggestive of fibrolipoma. Computed tomography (CT) of the spine showed other lymph nodes with clinical criteria of adenopathy at right sublingual level and an asymmetry of right occipital musculature (Fig. 1). Fine-needle aspiration was non-specific, and it was therefore decided to carry out an excisional biopsy of the lymph node and occipital tumour. The histopathological report of the 2 specimens revealed mantle cell lymphoma (MCL) which was confirmed by immunohistological and molecular testing, showing the translocation of the Bcl-1 gene using the FISH technique (Fig. 2). The haematological service staged the patient as IV-A as the tumour had spread to the spinal cord and there was submucosa of the colon. R-CHOP (rituximab, doxorubicin, cyclophosphamide, vincristine and prednisone) treatment was administered in addition to granulocyte colony-stimulating factor (G-CSF), with a complete remission outcome. An autologous haematopoiec stem cell transplant was subsequently performed. Four years later the patient remains without reoccurrence.

Figure 1.

TC axial section: asymmetry in the right occipital musculature.

(0.15MB).
Figure 2.

FISH: we observe translocation in chromosome 11 of the Bcl-1 gene using specific probes in the Bcl-1-breakpoint region. Separation of the 2 probes (arrows).

(0.05MB).
Discussion

Both the scalp and the cranial bone are locations where different types of benign or malignant tumours, of primary or metastatic origin, may establish themselves.1

The scalp has 5 layers, which from outside to inside would be the skin (epidermis/dermis), the subcutaneous connecting tissue, the galea aponeurotica (aponeurosis forming the union between the frontal muscles and the occipitals), the subgaleal layer (subaponeurotica layer of loose connective tissue) and the cranial periosteum.1

Hodgkin's lymphoma (HL) infrequently appears in this region and when it does it generally presents as a bone tumour of the cranial bone2,3 or as cutaneous lymphomas,1,4 with isolated location in the thickness of the scalp without concomitant spread to skin or bone being very rare. Our patient presented with a MCL, which is an uncommon subtype of HL that is characterised by slow but aggressive progression and which represents between 2.5% and 10% of all HL.5 In fact, we were unable to find any reported literature with extra lymph node MCL with isolated location in the subgaleal layer of the scalp. Lymphomatose cells could attach themselves to this layer, through the bridging veins which connect the intradural sinuses with the skin's superficial veins.3

MCLs appear more frequently in patients of advanced age, on average at 60 years of age and mainly in males.5 In an article which reviews 38 cases of primary cranial vault lymphomas it was observed that in 90% of cases the first clinical symptom was the appearance of a painless tumour in the scalp, followed by headaches in 30% of cases.3

The initial study of a tumour in the scalp with preservation of the skin can be performed through a scan, but a CT is necessary to establish involvement of the layers of the scalp or the cranial bone (to assess lytic bony lesions, hyperostosis…) and/or a MRI scan (HL has no specific signal intensity, although gadolinium contrast shows up hyperintensity).2,3

Differential diagnosis must be carried out with other tumours of the scalp such as lipoma, epidermic cysts, pilomaxtixomas, foreign body granulomas or trichilemmal cysts,6,7 and if there is cranial vault involvement, then in addition with metastatic carcinoma, osteomyelitis, multiple myeloma, histiocytosis and Ewing's sarcoma.2,3

A final diagnosis requires histologic confirmation of the tumour. MCL is characteristically formed by atypical lymphoid cells which are small or medium in size and test positive in immunohistochemical markers for cells B CD20, CD5, CD43, and negative for CD10 and CD23. It is characterised by the overexpression of cyclin D1 due to t(11,14)(q13;q32) translocation in the Bcl-1 gene (Fig. 2).5

There is currently no etiologic treatment for MCL. In general, if the disease is extranodal in a single location, treatment is local with surgery and/or radiotherapy and with adjuvant chemotherapy in some cases.3 If there is secondary involvement, with advanced staging (which occurs in 70% of patients), R-CHOP treatment is administered and depending on age, other therapies may be added,5,8 as occurred in our patient who was given a stem cell autologous transplant. Complete remission is the outcome achieved in 6%–35% of patients, with mean total survival rate at 3–5 years.5 The scientific community is currently developing new guidelines and treatments with promising outcomes.8

To conclude, we should include MCL in differential diagnosis of scalp or cranial vault tumours, despite its rare occurrence, in order to establish early diagnosis and maximise a survival outcome.

Conflict of Interest

The authors declare no conflict of interest.

References
[1]
H.M. Richmond, M. Duvic, D.F. MacFarlane.
Primary and metastatic malignant tumors of the scalp. An update.
Am J Clin Dermatol, 11 (2010), pp. 233-246
[2]
Y. Fukushima, H. Oka, S. Utsuki, K. Nakahara, K. Fujii.
Primary malignant lymphoma of the cranial vault.
Acta Neurochir (Wien), 149 (2007), pp. 601-604
[3]
A.C. El Asri, A. Akhaddar, H. Baallal, O. Boulahroud, C. Mandour, H. Chahdi, et al.
Primary lymphoma of the cranial vault: case report and a systematic review of the literature.
Acta Neurochir, 154 (2012), pp. 257-265
[4]
A.K. Singh, G. Dixit, S. Sharma, S. Kumar, R. Yadav, N. Agrawal, et al.
Skin manifestations associated with mantle cell lymphoma: a case report.
Mediterr J Hematol Infect Dis, 5 (2013), pp. e2013020
[5]
O. Balagué, L. Colomo, E. Campo.
Linfoma de células del manto.
Rev Esp Patol, 37 (2004), pp. 159-172
[6]
C. Whittle, M. Cortés, G. Baldassare, A. Castro, R. Cabrera.
Subgaleal lipomas: ultrasound findings.
Rev Med Chil, 136 (2008), pp. 334-337
[7]
J. Gossner, J. Larsen.
Incidental subcutaneous nodules on the scalp in patients undergoing CT of the brain; frequency, appearance, and differential diagnosis.
Clin Radiol, 65 (2010), pp. 427-429
[8]
M.E. Williams, S.H. Bernstein, P. Jares, B.S. Kahl, T.E. Witzig, L.I. Gordon.
Recent advances in mantle cell lymphoma: report of the 2012 Mantle Cell Lymphoma Consortium Workshop.
Leuk Lymphoma, 54 (2013), pp. 1882-1890

Please cite this article as: Borràs Perera M, Galindo Ortego J, Bodet Agustí E, Tarragona Foradada J. Afectación subgaleal secundaria del linfoma de células del manto. Acta Otorrinolaringol Esp. 2016;67:e13–e15.

Copyright © 2014. Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello
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