Hostname: page-component-78c5997874-s2hrs Total loading time: 0 Render date: 2024-11-03T08:42:54.406Z Has data issue: false hasContentIssue false
  • English
  • Français

Unusual clivus lesion demonstrating extramedullary haematopoiesis: case report

Published online by Cambridge University Press:  08 March 2017

K Kulendra ,
C Butler ,
W Grant ,
A Sandison ,
G Cho  and
M C Patel
K Kulendra*
Affiliation:
Department of ENT, Charing Cross Hospital, London, UK
C Butler
Affiliation:
Department of Histopathology, Charing Cross Hospital, London, UK
W Grant
Affiliation:
Department of Histopathology, Charing Cross Hospital, London, UK
A Sandison
Affiliation:
Department of Histopathology, Charing Cross Hospital, London, UK
G Cho
Affiliation:
Department of Neuroradiology, Charing Cross Hospital, London, UK
M C Patel
Affiliation:
Department of Haematology, Central Middlesex Hospital, London, UK
*
Address for correspondence: Mr Kevin Kulendra, 25 Buxton Drive, New Malden, Surrey KT3 3UX, UK. Fax: +44 (0)20 8395 0117 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction:

An unusual clivus mass is reported, following investigation of memory disturbance and headaches in a patient with β thalassaemia and sickle cell disease. This lesion proved to be a site of extramedullary haematopoiesis.

Radiological findings:

Computed tomography demonstrated a 2 cm mass at the base of the sphenoid. However, magnetic resonance imaging showed little bony expansion, and the differential diagnosis included chordoma, dermoid cyst and fibrodysplasia.

Histological findings:

Examination of the biopsy showed the presence of bone marrow demonstrating erythroid hyperplasia and small aggregates of B-cell lymphocytes. The features were considered compatible with erythroid hyperplasia associated with haemolytic anaemia.

Management:

The patient was reassured that she did not have a neoplastic lesion, and was referred back to the haematologists for further management of her sickling β thalassaemia.

Conclusions:

Extramedullary haematopoiesis occurs outside the reticuloendothelial system in response to haemolytic anaemia. Extramedullary haematopoiesis causing a clivus mass is an unusual ENT presentation. Such haematopoiesis is occasionally seen in the calvarial skull, but this is the first report of this process occurring in the anterior skull base, to our knowledge.

Keywords

Extramedullary HaematopoiesisSphenoid SinusClivusB ThalassaemiaSickle Cell Disease
Type
Online Only Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 5 , May 2009 , e15
Copyright
Copyright © JLO (1984) Limited 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1 Socher, JA, Cassano, M, Augustinho, F, Cassano, P, Felippu, A. Diagnosis and treatment of isolated sphenoid sinus disease: a review of 109 cases. Acta Otolaryngol 2008;128:1004–10CrossRefGoogle ScholarPubMed
2 Ittipunkul, N, Martin, T, Siriwanasan, R, Olanratanachai, K, Rootman, J. Extra-medullary hematopoiesis causing bilateral optic atrophy in beta thalassaemia/Hb E disease. J Med Assoc Thai 2007;90:809–12Google Scholar
3 Wang, Z, Kanoh, N, Dai, C, Kutler, DI, Xu, R, Chi, F et al. Isolated sphenoid sinus disease: an analysis of 122 cases. Ann Otol Rhinol Laryngol 2002;111:323–7Google ScholarPubMed
4 Selmani, Z, Aitasalo, K, Ashamnalchi, N. Fibrous dysplasia of the sphenoid sinus and skull base presents in an adult with localised temporal headache. J Craniofac Surg 2004;15:261–3CrossRefGoogle Scholar
5 Mueller, DP, Dolan, KD, Yur, WTC. Fibrous dysplasia and the sphenoid sinus. Ann Otol Rhinol Laryngol 1992;101:100–1CrossRefGoogle ScholarPubMed
6 Keel, SB, Bhan, AK, Liebsch, AJ, Rosenberg, AE. Chondromyxoid fibroma of the skull base: a tumour which may be confused with chordoma and chrondosarcoma. A report of three cases and review of the literature. Am J Surg Pathol 1997;21:577–82CrossRefGoogle Scholar
7 Orzincolo, C, Castaldi, G, Scutellari, PN, Franceschini, F. The “lamellated” skull in B-thalassaemia. Skel Radiol 1989;18:373–6CrossRefGoogle Scholar
8 Joseph, M, Rajshekkar, V, Chandy, MJ. Haematopoietic tissue presenting as sphenoid sinus mass: a case report. Neuroradiol 2000;42:153–4CrossRefGoogle Scholar
9 Lund, RE, Aldridge, NH. Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr 1984;8:788–90CrossRefGoogle ScholarPubMed
10 Baskurt, E, Raghavan, P, Trelka, DP. Extramedullary haematopoiesis involving the bilateral lacrimal fossae. Am J Neuroradiol 2006;27:934–5Google ScholarPubMed