Abstract
Myeloid sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells. This disorder may occur in concomitance with or precede development of acute or chronic myeloid leukemia. Sometimes, it is the initial manifestation of relapse in a previously treated acute myeloid leukemia. We report a case of 11 years old boy with acute myeloid leukemia in remission state, presented with short history of right otalgia associated with facial nerve palsy. Diagnosis of right acute mastoiditis with facial nerve palsy as complication of acute otitis media was made initially. Patient underwent simple cortical mastoidectomy but histopathology from soft tissue that was sent revealed diagnosis of myeloid sarcoma. A leukemic relapse was confirmed by paediatric oncologist through bone marrow biopsy. Chemotherapy was commenced but patient responded poorly to the treatment.
Keywords: Myeloid sarcoma, Acute mastoiditis, Acute myeloid leukemia, Facial nerve palsy
Introduction
Myeloid sarcoma, also known as granulocytic sarcoma, chloroma or myeloblastoma is a rare condition that is characterized by the occurrence of one or more tumour masses. It consists of immature myeloid cells and presents at an extramedullary site [1]. It may develop de novo or concurrently with acute myeloid leukemia, myeloproliferative disorder, or myelodysplastic syndrome [2]. It is found in 10.9 % of children and 10 % of adult cases of acute myeloid leukemia [3]. The commonest sites were skin (28.2 %), lymph node (16.3 %), testis (6.5 %), intestine (6.5 %) and bone (3.25 %) [2]. Myeloid sarcoma involving temporal bone is very rarely seen and there is no standard treatment. Prognosis of myeloid sarcoma however is unfavorable.
Case Report
An 11 years old boy presented with 1 week history of right sided otalgia, reduced hearing and subsequently facial asymmetry 2 days after initial presentation. He had history of acute myeloid leukemia in which he completed chemotherapy 7 months ago and was in remission state. The right otalgia was throbbing in nature and increased in severity for 1 week. At the same time, he was noted by caregiver to have reduced hearing over right side. There was no otorrhea, vertigo, tinnitus or fever. He did not have easy bruising, unresolved fever, and lethargy or anemia symptoms to suggest relapse of leukemia at that time. There was no history of recent trauma to the ears.
Two days after the onset of otalgia, he developed sudden right facial asymmetry. On further questioning, he had multiple neuropathies including left facial nerve palsy which lead to diagnosis of acute myeloid leukemia 1 year ago. There was no ear symptom at that time. He gained full recovery of facial asymmetry after chemotherapy. There was no family history of similar disease.
On examination, he had right lower motor neuron facial nerve palsy, House Brackman grade V with no exposure keratitis. There was mastoid tenderness but no post auricular swelling. Otoscopic examination revealed posterior sagging of right external auditory canal and unable to visualize tympanic membrane. Left external auditory canal was normal with dull looking tympanic membrane but no fluid or mass effect seen. Rinne was negative bilaterally with Weber test lateralized to diseased ear. There was no hepatosplenomegaly or enlargement of lymph node.
He was started on intravenous ceftriazone and further investigated for causes of right facial nerve palsy. High Resolution Temporal Bone scan showed opacities over right external auditory canal, middle ear and mastoid cavity with soft tissue density within (Fig. 1). There was no dehiscence of right facial canal. There was no bony erosion over tegmen tympani, scutum or sigmoid plate. The left middle ear and mastoid was noted to be filled up by soft tissue density. Full blood count revealed normal cell count with hemoglobin14.0 g/dL, white cell count 6.8 × 10/L, platelet 211 × 10/L.
Fig. 1.
a Coronal view and b Axial view of high resolution computed tomography of temporal showing soft tissue density in right middle ear and mastoid cavity with no destruction to tegmen tympani and scutum. There was soft tissue density over left mastoid and middle ear. On physical examination, tympanic membrane on left showed dullness but no fluid or mass
Examination under anesthesia and right cortical mastoidectomy was performed. Intraoperatively, right external auditory canal was edematous with posterior wall sagging obscuring the view of tympanic membrane. On cortical mastoidectomy, there was polypoidal tissue seen in the mastoid area and obstructing the antrum. Lateral semicircular canal was identified and it was of normal appearance. Incus was identified and noted mobile. Tympanic segment of facial nerve identified anatomically and confirmed physiologically.
He was treated as acute otitis media complicated with mastoiditis and facial nerve palsy. He had uneventful recovery and was discharged well five days post operation with oral prednisolone, oral cefuroxime and physiotherapy. Histopathology examination of right mastoid mucosa however revealed diagnosis of myeloid sarcoma. Immunohistochemical stainings of mastoid mucosa were positive toward LCA, MPO and CD 117 (Fig. 2).
Fig. 2.
Sheets of atypical cells infiltrated the fibro-connective tissue and display irregular medium sized nuclei with open chromatin, prominent nucleoli and scanty cytoplasm (a). Immunohistochemical study showed the atypical cells are immunoreactive towards LCA (b), CD117 (c) and myeloperoxidase (d)
Referral to primary paediatric oncology unit was made and bone marrow aspiration and trephine biopsy confirmed relapse of leukemia. He was re-induced with chemotherapy but did not respond well to treatment.
Discussion
Myeloid sarcoma of temporal bone is not easily diagnosed as the presenting symptoms may mimic otomastoiditis. Given the short history of otalgia with complication of facial nerve palsy in an 11 years old child, acute otitis media with mastoiditis is the more likely provision diagnosis.
Otologic manifestations have been reported in 15–40 % of leukemia patients [4]. Leukemic patients are highly susceptible to bacterial, fungal or viral infections and thus prone for ear infection. The common presenting symptom in patient with temporal bone involvement includes an aching post-auricular swelling, conductive hearing loss, otalgia, and tinnitus. Massive bony erosion, deafness and vertigo may develop at a later stage as the lesion progresses and untreated [5]. Perineural and/or meningeal leukemic infiltration are associated with hemorrhages or edema which can give rise to facial nerve paralysis [6].
Myeloid sarcoma involving temporal bone will have a greater tendency of mastoid involvement while involvement of petrous apex is more commonly seen in metastatic lesions [7]. The predominance of the tumor in the mastoid cavity can be explained by fact that mastoid cavity is more vulnerable to harboring a mass than the hard petrous bone. Thus the patients presented with symptoms related to mastoid involvement. In this case, patient had mastoid involvement rather than petrous apex involvement.
Imaging finding is non-specific for myeloid sarcoma. It appeared homogenously enhancing and well demarcated with adjacent bony erosion in CT or MRI imaging [8]. Myeloid sarcoma should be strongly considered whenever a mass is discovered in a patient with myelogenous leukemia. Biopsy is the most preferred diagnostic method to diagnose myeloid sarcoma.
A biopsy sample may be difficult to obtain especially when the mass is in an anatomically difficult sites, for example at petrous bone. Myeloid sarcoma can be misdiagnosed as lymphoblastic, Burkitt’s or diffuse large B cell lymphoma or even as a hematopoietic tumor [9]. On immunohistochemistry, CD 68KP1 is the most commonly expressed marker followed by MPO, CD 117, CD 34 and CD 99 [2]. All granulocytic cells, including myeloblasts and myelocytes, stain positive for lysozyme, whereas lymphocytes are negative.
The development of myeloid sarcoma in patients with leukemic population implies a poor prognosis. The knowledge of an underlying MPD, MDS or AML is commonly thought to be a negative prognostic factor [1]. Pileri et al. [2] reported only 11.5 % of overall survival rate in his study of 67 samples. In a case series of myeloid sarcoma of temporal bone with AML by Chang et al. [7], only one out of five patients achieved complete remission with the remaining died within 6 months of diagnosis. Complications from treatment or progression of disease attribute to death in these patients. High dose therapies as a front line approach are recommended to achieve complete remission and to cure the disease.
Surgical, radiotherapy and chemotherapy are choices of treatment but remain controversial. Surgical role of myeloid sarcoma is restricted to obtaining a tissue sample to identify the lesion, drain infection if present and reducing neoplastic infiltration before chemotherapy. Chemotherapy is the mainstay of treatment to induce complete remission both in systemic disease or myeloid sarcoma. Involved area radiation therapy is not a general accepted first line treatment modality in children [3]. However, additional radiotherapy is considered when the disease persists after chemotherapy. Periodic temporal bone MRI should be performed in patients with myeloid sarcoma of ear to differentiate between inflammatory changes and relapse of myeloid sarcoma as inflammatory changes might take some time to disappear.
In this case, patient had previous episode of neuropathies but resolved after chemotherapy. He might have undiagnosed myeloid sarcoma of temporal bone concurrent with acute myeloid leukemia during the first presentation as he did not have any ear symptoms. High-dose therapies as front line approach can better achieve complete remission and to possibly cure the disease. Radiotherapy for residual mass and bone marrow transplantation can better improve the outcome of this patient. Subjects treated with bone marrow transplantation corresponded to long survivors. Overall survival at 48 months was reported as 76 % in those with bone marrow transplantation compared with 0 % in those received conventional therapies [2]. Despite re-induce with chemotherapy, blast cells persists in bone marrow and thus not a candidate for bone marrow transplantation.
Conclusion
Myeloid sarcomas of temporal bone are rare tumors which present in association with myeloid leukemia. Their presentation mimics acute mastoiditis and may be misdiagnosed. Myeloid sarcoma should be suspected in patient with enhancing mass in the mastoid and or middle ear with previous history of myeloid leukemia. Therefore biopsy from mastoid and/or middle ear during mastoidectomy is crucial to make early and accurate diagnosis. Despite poor prognosis, early diagnosis and aggressive treatment might improve outcome.
Compliance with Ethical Standards
Conflict of interest
Authors declare that they have no conflict of interest.
Ethical approval
This article does not contain any studies with human participants or animals performed by any authors.
Informed consent
Informed consent was obtained from all individual participants included in this study.
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