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Image of the external auditory canal with epithelial lining and massive cell infiltration of the skin. Haematoxilin-eosine.
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We describe a case of temporal granulocytic sarcoma in a 26-year-old patient after apparent molecular remission of an acute myeloid leukaemia. He complained of otodynia with hearing loss and facial paralysis on the right side. He was treated with chemotherapy and self-transplant haematopoietic stem cells. He was cured clinically, molecular remissio...
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... examination, the patient temperature was normal and showed tumefaction and retroauricular pain, as well as, painful bulging in the upper and posterior walls of the external auditory canal. The eardrum could hardly be seen but it looked opaque and swollen. The patient also had a grade III House-Brackmann facial paralysis on the right side. Eardrum paracentesis was performed but only blood stained fluid was retrieved, and this procedure did not improved the patient's clinical condition. Audiometry showed transmission hearing loss on the right ear. Full Blood Count and erythrocyte sedimentation rate were normal. Computed tomography (CT) with and without I.V. contrast demonstrated occupation of the right mastoid cells and the external auditory canal by a soft tissue density mass. Attic was also filled with soft tissue density mass but no bone or ossicle destruction was evident on CT (Figure 1). Under the clinical impression that this patient might have been suffering from atypical mastoiditis and, after having obtained informed consent from him, mastoidec- tomy and antrectomy were performed opening the posterior attic and preserving untouched the ossicular chain. A soft and friable tissue infiltrated the subcutaneous tissue and filled the mastoid and posterior attic. Peroperative biopsy showed leukaemia infiltration of this tissue. Therefore, more aggressive surgery was not attempted. Final histopathological report showed the tumour to be a granulocytic sarcoma (Figures 2 and ...
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... Based on this, there is very little evidence to speculate about the association between FLT3 ITD mutations and occurrence of GS even why we were not able to demonstrate the presence of FLT3-ITD mutation in the mastoid sarcoma tissue. Following the general trend in the current literature, GS should always be suspected in young [23,24] as well as in young adult AML patients with symptoms of acute otomastoiditis associated with facial palsy [25]. In our case, the atypical involved sites and the confounding clinical presentation of mastoid localization have probably deferred the diagnosis by focusing the diagnostic process on an infective problem. ...
Granulocytic sarcoma (GS) is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML) as well as following treated AML. The incidence of GS in AML patients is 3-8% but it significantly rises in M2 FAB subtype AML. This variety of AML harbors t(8;21) in up to 20-25% of cases (especially in children and black ones of African origin) and, at a molecular level, it is characterized by the generation of a fusion gene known as RUNX1-RUNX1T1. Approximately 10% of M2 AML patients will develop GS, as a consequence, the t(8;21) and the relative transcript represent the most common cytogenetic and molecular abnormalities in GS. FLT3-ITD mutation was rarely described in AML patients presenting with GS. FLT3 ITD is generally strongly associated with poor prognosis in AML, and is rarely reported in patients with t(8;21). GS presentation is extremely variable depending on organs involved; in general, cranial bones and sinus are very rarely affected sites. We report a rare case of GS occurring as a recurrence of a previously treated t(8;21), FLT3-ITD positive AML, involving mastoid bones and paravertebral tissues.
Myeloid sarcoma is an uncommon neoplasm, comprising an extramedullary tumorous proliferation of immature myeloid cells. Its rarity and tendency for diverse clinical presentation contribute to difficulty in timely diagnosis. This report describes a case of a painful back lump in an elderly Chinese male, which was initially diagnosed as a carbuncle. Histology on the saucerization specimen was reported as inflamed granulation tissue. Failure of expected wound healing prompted surgical debridement; microscopic examination on the subsequent specimen revealed an immature myeloid population with an increased MIB-1 proliferative index, highlighted by myeloperoxidase, lysozyme, CD117, and CD43 immunoreactivity, in keeping with myeloid sarcoma. Despite aggressive management, the patient eventually died. This report draws attention to potential pitfalls in the pathological diagnosis of this uncommon tumor and briefly summarizes its salient features.
© The Author(s) 2015.
