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UNIVERSIDADE FEDERAL DO RIO DE JANEIRO 
FACULDADE DE ODONTOLOGIA 
MESTRADO PROFISSIONAL EM CLÍNICA ODONTOLÓGICA 
 
 
 
 
RENAN DE BARROS FARNEZE 
 
 
 
 
 
 
 
 
SARCOMA MIELÓIDE EM CAVIDADE ORAL: RELATO DE CASO 
E REVISAÕ DE 84 CASOS DA LITERATURA 
 
 
 
MYELOID SARCOMA OF THE ORAL CAVITY: A CASE REPORT 
AND REVIEW OF 84 CASES FROM THE LITERATURE 
 
 
 
 
 
 
Rio de Janeiro 
2016
 
 
RENAN DE BARROS FARNEZE 
 
 
 
SARCOMA MIELÓIDE EM CAVIDADE ORAL: RELATO DE CASO 
E REVISAÕ DE 84 CASOS DA LITERATURA 
 
 
 
MYELOID SARCOMA OF THE ORAL CAVITY: A CASE REPORT 
AND REVIEW OF 84 CASES FROM THE LITERATURE 
 
 
 
 
Dissertação apresentada ao corpo 
docente do Curso de Mestrado 
Profissional em Clínica Odontológica 
da Faculdade de Odontologia da 
UFRJ como parte dos requisitos para 
obtenção do título de Mestre em 
Clínica Odontológica 
 
 
 
 
Orientador: Prof. Dr. Mário José Romañach Gonzalez Sobrinho 
 
 
 Rio de Janeiro 
2016
http://lattes.cnpq.br/5084438190869898
 
 
FICHA CATALOGRÁFICA 
 
 
 
 
 
 Farneze, Renan de Barros 
 Sarcoma mieloide em cavidade oral: relato de caso e revisão de 84 
casos da literatura. / Renan de Barros Farneze. -- Rio de Janeiro: UFRJ / 
Faculdade de Odontologia, 2016. 
 21 f. : il. ; 31 cm. 
 Orientador: Mário José Romañach Gonzalez Sobrinho. 
 Dissertação (mestrado) – UFRJ, Faculdade de Odontologia, 
Programa de Pós-graduação em Odontologia, Clínica Odontológica, 
2016. 
 Referências bibliográficas: f. 16-21. 
 1. Sarcoma Mieloide - patologia. 2. Leucemia Mieloide Aguda - 
diagnóstico. 3. Leucemia Promielocítica Aguda - diagnóstico. 4. Neoplasias 
Bucais. 5. Clínica Odontológica - Tese. I. Sobrinho, Mário José Romñach 
Gonzalez. II. Universidade Federal do Rio de Janeiro, Faculdade de 
Odontologia, Programa de Pós-graduação em Odontologia, Clínica 
Odontológica. III. Título. 
ii 
 
RENAN DE BARROS FARNEZE 
 
 
 
SARCOMA MIELÓIDE EM CAVIDADE ORAL: RELATO DE CASO 
E REVISAÕ DE 84 CASOS DA LITERATURA 
 
MYELOID SARCOMA OF THE ORAL CAVITY: A CASE REPORT 
AND REVIEW OF 84 CASES FROM THE LITERATURE 
 
 
 
Dissertação apresentada ao corpo 
docente do Curso de Mestrado 
Profissional em Clínica Odontológica 
da Faculdade de Odontologia da 
UFRJ como parte dos requisitos para 
obtenção do título de Mestre em 
Clínica Odontológica 
 
Aprovado em:_______/______/_____ 
 
 
BANCA EXAMINADORA 
 
________________________________________________________________ 
Prof. Dra. SIMONE DE QUEIROZ CHAVES LOURENÇO 
 
________________________________________________________________ 
Prof. Dr. MARIO JOSÉ ROMAÑACH GONZALEZ SOBRINHO 
 
________________________________________________________________ 
Prof. Dra. MARCIA GRILLO CABRAL 
 
Rio de Janeiro 
 
 
iii 
 
DEDICO 
 
A minha noiva, Estela Valdetaro, e a minha avó, Ana de Macedo 
Barros, por sempre me apoiarem, incentivarem e acreditarem em mim, mesmo 
nos momentos mais difíceis. 
 
iv 
 
 
AGRADECIMENTOS 
 
 
 Agradeço a minha noiva, Estela Valdetaro, por ser minha companheira 
em todos os momentos, me ouvindo, me acalmando sempre apoiando 
incondicionalmente e me fazer buscar melhorar em todos os aspectos da minha 
vida. Obrigado por ser minha luz me guiando e me protegendo e por dividir o 
resto da sua vida comigo. 
 Aos meus pais, Cesar Farneze e Ana Maria Farneze pela vida que me 
proporcionaram e valores ensinados, fornecendo todos os recursos e incentivos 
que tornaram possível alcançar cada uma de minhas conquistas. Obrigado por 
ajudarem a formar o meu caráter. 
 Aos meus irmãos, Cesar Augusto Farneze e Gabriela Farneze, por toda 
preocupação, carinho e conselho durante esta etapa e por sempre protegerem o 
irmão caçula. 
 A todos os meus amigos por fazerem parte da minha vida de forma tão 
intensa e vibrarem com cada conquista. Por serem companheiros de aventuras 
e estarem presentes em cada momento feliz que renderam memórias que 
levarei pelo resto da minha vida. 
 Ao professor Mário Romañach e Bruno Augusto Benevenuto por me 
orientarem nessa jornada sempre com muita calma e paciência. 
Aos meus companheiros de turma por dividirem não só as aulas mas 
também os medos e incertezas. 
 
 
 
 
 
 
 
 
 
 
 
v 
 
Resumo 
 
 
Sarcoma mielóide é uma massa tumoral de céluas mielóides imaturas ou células 
granulocíticas que afeta locais anatómicos extramedulares, incluindo a cavidade 
oral com pouca frequência. Uma mulher de 24 anos de idade, foi encaminhada 
para avaliação de um núdulo gengival doloroso de rápido crescimento com 
duração de 2 semanas, associada a febre, fadiga e linfadenopatia cervical. O 
exame intra-oral mostrou um nódulo azulado em região posterior inferior da 
gengiva à direita exibindo superfície necrótica. A biópsia da lesão gengival 
mostrou infiltração difusa de células tumorais indiferenciadas com aparência 
granulocítica, fortemente imuno positivo para CD99, mieloperoxidase e Ki-67 
(60%), e negativo para CD20, CD3, CD34 e TdT. Os exames de sangue 
apresentaram uma pancitopenia grave, e análise genética confirmou o 
diagnóstico de leucemia promielocítica aguda. O diagnóstico final foi de sarcoma 
mielóide oral associado a leucemia promielocítica aguda com t (15; 17). A 
paciente foi submetida à quimioterapia, mas morreu da doença um mês depois. 
As características clinicopatológicas e imuno-histoquímico do presente caso são 
comparadas com os 84 casos de sarcoma mielóide oral, previamente relatados 
na literatura de língua Inglês. 
 
 
 
 
 
 
 
 
 
Palavras Chaves: Sarcoma Mielóide, cloroma, sarcoma granulocítico, gengiva, 
oral, leucemia promielocítica aguda, leucemia mieloide aguda. 
vi 
 
Abstract 
 
Myeloid sarcoma is a tumor mass of immature myeloid or granulocytic cells that 
affects extramedullary anatomic sites, including uncommonly the oral cavity. A 
24-year-old female was referred for evaluation of a fast growing painful gingival 
swelling lasting 2 weeks, associated with fever, fatigue, and cervical 
lymphadenopathy. Intraoral examination showed a bluish swelling on the right 
posterior lower gingiva exhibiting necrotic surface. Incisional biopsy of the 
gingival lesion displayed diffuse infiltration of undifferentiated tumor cells with 
granulocytic appearance, strongly immunopositive for CD99, myeloperoxidase 
and Ki-67 (60%), and negative for CD20, CD3, CD34 and TdT. Blood tests 
presented a severe pancytopenia, and genetic analysis confirmed the diagnosis 
of acute promyelocytic leukemia. The final diagnosis was of oral myeloid 
sarcoma associated with acute promyelocytic leukemia with t(15;17). The patient 
was submitted to chemotherapy but died of the disease one month later. The 
clinicopathologic and immunohistochemical features of the present case are 
compared with the 84 cases of oral myeloid sarcoma previously reported in the 
English-language literature. 
 
 
 
 
Key words: myeloid sarcoma, chloroma, granulocytic sarcoma, gingiva, oral, 
acute promyelocytic leukemia, acute myeloid leukemia. 
vii 
 
SUMÁRIO 
 
1 INTRODUÇÃO......................................................................................................... 1 
2 OBJETIVOS............................................................................................................. 3 
3 CAPÍTULO 1 – CASE REPORT.............................................................................. 4 
4 CONSIDERAÇÕES FINAIS..................................................................................... 15 
5 REFERÊNCIAS....................................................................................................... 16 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
1- INTRODUÇÃO 
 O sarcoma mieloide (SM), também conhecido como sarcoma 
granulocítico ou cloroma, representa uma massatumoral de células mielóides 
imaturas que normalmente ocorre em um sítio extramedular ou ósseo.1-4 O 
SM foi primeiramente descrito por Burns e Rei em 1811 sob termo “cloroma”, 
por causa da sua coloração esverdeada causada por uma reação da 
mieloperoxidade quando exposta ao ar.1-2 Como essa coloração nem sempre 
está presente e por ter sua origem granulocítica comprovada, os termos atuais 
mais apropriados são sarcoma granulocítico ou sarcoma mieloide.1-3 
 As características microscópicas do SM incluem a presença de 
mieloblastos imaturos misturados a infiltrado inflamatório misto, podendo ser 
confundido com infiltrado inflamatório reacional62. O diagnóstico final do SM 
requer avaliação microscópicas dos aspectos morfológicos e imuno-
histoquímicos13-20. Alguns marcadores são úteis para confirmar um fenótipo 
mielóide imaturo de células tumorais, tais como CD68, CD117, CD34, e CD99, 
mas principalmente o anticorpo mieloperoxidase.21-31 
 O primeiro caso de SM associado a leucemia mieloide aguda 
(LMA) foi descrito por Turk et al (1903)61 e desde então observou-se que o SM 
ocorre em cerca de 1% a 3% de todos os pacientes com LMA58. O SM pode 
se apresentar em (i) pacientes que não desenvolvem leucemia no início do 
SM, (ii) aqueles que já desenvolveram leucemia no início (secundária a 
leucemia), e (iii) aqueles com desordem mieloproliferativas ou síndrome 
mielodisplásica como uma doença subjacente65. O prognóstico do SM é 
considerado desfavorável, dependendo da situação clínica e hematológica 
apresentada13, uma vez que é considerado uma manifestação de uma doença 
hematológica subjacente, sendo portanto tratado de forma semelhante ao da 
doença principal. A quimioterapia de combinação para leucemia aguda pode 
induzir a remissão completa da lesão e radioterapia reservada para casos 
onde a doença persiste61. 
 O SM pode ocorrer em qualquer parte do corpo, sendo mais 
comumente observado na pele, tecidos moles, osso e gânglios linfáticos de 
pacientes adultos, com discreta predileção pelo gênero feminino5,51. Na região 
2 
 
de cabeça e pescoço o sarcoma mielóide é visto frequentemente em tecidos 
moles da órbita, cavidade nasal e seios paranasais, sendo o envolvimento da 
cavidade oral incomum66. Até o momento, 84 casos de SM na cavidade oral 
foram publicados na literatura de língua Inglesa, e apenas quatro deles foram 
associados com leucemia promielocítica aguda (LPA).1,69 A LPA está 
frequentemente associada com coagulação intravascular disseminada grave, 
condição que exerce um elevado risco de morte prematura devido à 
hemorragia cerebral ou fenômenos semelhantes. LPA é causada pelo gene 
de fusão PML-RARa, o qual é formado como o resultado de uma translocação 
genética t(15:17), suprimindo a transcrição de genes que é mediada pelo 
receptor de ácido retinóico (RARa), e prejudicando a diferenciação mielocítica. 
O tratamento da LPA vem apresentando melhoras, graças à quimioterapia de 
indução sistêmica com ATRA (ácido all-trans-retinóico), com taxas de 
remissão atualmente superior às taxas de sobrevivência de 90% e a longo 
prazo mais de 70%63. 
 
 
 
 
 
 
 
 
 
 
 
 
 
3 
 
2- OBJETIVO 
 
Este trabalho tem por objetivo relatar um caso de SM oral em uma mulher de 24 
anos de idade como manifestação inicial de leucemia promielocítica aguda, 
incluindo uma revisão da literatura. 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
4 
 
3- CAPÍTULO 1 – CASE REPORT 
 
 
MYELOID SARCOMA OF THE ORAL CAVITY: A CASE REPORT AND 
REVIEW OF 84 CASES FROM THE LITERATURE 
 
Running title: Oral myeloid sarcoma 
 
Renan de Barros Farneze, DDS1, Bruno Augusto Benevenuto de Andrade, DDS, 
PhD1, Aline Corrêa Abrahão, DDS, PhD1, Marcia Grillo Cabral, DDS, PhD1, 
Michelle Agostini, DDS, PhD1, Alicia Rumayor Piña, DDS, MSc2, Mário José 
Romañach, DDS, PhD1 
 
1Department of Oral Diagnosis and Pathology, Federal University of Rio de 
Janeiro (UFRJ) School of Dentistry, Rio de Janeiro, Brazil. 
2Oral Pathology, Department of Oral Diagnosis, Piracicaba Dental School, 
University of Campinas (FOP/UNICAMP), Piracicaba, Brazil. 
 
 
 
CONFLICT OF INTEREST 
The authors declare that they have no conflict of interest. 
 
 
 
 
Corresponding author: 
 
Mário José Romañach, DDS, PhD 
Department of Oral Diagnosis and Pathology, Federal University of Rio de 
Janeiro (UFRJ) School of Dentistry. Av. Carlos Chagas Filho 373, Prédio do 
CCS, Bloco K, 2° andar, Sala 56. Ilha da Cidade Universitária, Rio de 
Janeiro/RJ. 21.941-902. Phone: +55 21 39382071. 
E-mail: marioromanach@yahoo.com.br 
5 
 
INTRODUCTION 
 
 Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, 
is a tumor mass of immature myeloid cells that usually occurs in an 
extramedullary site or bone of male patients in the sixth decade of life [1-4]. MS 
has been associated with acute myeloid leukemias (AML) or other 
myeloproliferative disorders, including acute promyelocytic leukemia, a subtype 
of AML associated with the chromosomal translocation t(15;17) [5-12]. Treatment 
and prognosis of MS depends on the hematological status and clinical 
presentation [13]. 
 The microscopical features of MS include the presence of immature 
myeloblasts within a dense inflammatory background, which are better identified 
after careful histological and immunohistochemical evaluation [13-20]. Some 
markers are useful to confirm an immature myeloid phenotype of tumor cells, 
such as myeloperoxidase (MPO), CD68, CD117, CD34, and CD99 [21-31]. 
 Oral involvement by MS is uncommon. To the best of our knowledge, only 
84 cases of oral MS have been published in the English-language literature so 
far, and only four of them were associated with acute promyelocytic leukemia [1-
69] (Table 1). Herein, we report an additional case of oral MS in a 24-year-old 
female with acute promyelocytic leukemia, including a review of the literature. 
 
CASE REPORT 
 A 24-year-old female was referred by a general dentist for evaluation of a 
fast growing gingival swelling that had been present for 2 weeks. The patient 
reported a 3-weeks history of fever and fatigue. Physical examination revealed 
cervical lymphadenopathy, and intraoral examination showed discrete areas of 
clotted blood within the gingival sulcus of some teeth, and a 3 cm painful 
brownish swelling with necrotic and bleeding surface localized in the right 
posterior lower gingiva (Figure 1). Radiographic examination of the mandible 
showed no bone involvement (Figure 2). Under the presumptive clinical 
diagnosis of lymphoma/leukemia, a blood study was requested and the patient 
was submitted to an incisional biopsy. 
 The gingival specimen showed a diffuse connective tissue infiltration by 
poorly differentiated blast-like cells intermingled with chronic inflammatory 
6 
 
infiltrate. Tumor cells were large, round to oval, with mild to moderately 
basophilic cytoplasm containing granules, and round to folded nuclei with fine 
chromatin. Occasional mitotic figures were found. By immunohistochemistry, 
tumor cells were intensely positive for myeloperoxidase and CD99, and negative 
for CD20, CD3, CD34 and TdT. Ki-67 labeling was high, with 60% of tumor cells 
positive (Table 2 and Figure 3). Blood findings showed pancytopenia (0.7 
x109/L leucocytes, 31 x 109/L platelets, hemoglobin 6.3 g/dl, and hematocrit 
18.6%) and the specific chromosomal translocation t(15;17) revealed by genetic 
analysis confirmed the diagnosis of acute promyelocytic leukemia with recurrent 
genetic abnormality. The final diagnosis of the oral lesion was myeloid sarcoma 
associated with acute promyelocytic leukemia with t(15;17). The patient was then 
referred to a hematology-oncology service, and submitted to chemotherapy 
including all trans retinoic acid (ATRA), idarubicin, and cytarabine. Unfortunately, 
the patient died one month later after severe hemorrhagicepisodes. 
 
DISCUSSION 
 Myeloid sarcoma (MS) is an extramedullary solid tumor composed of 
myeloblasts or immature cells of granulocytic lineage and erythroid precursors 
[5-10]. First described by Burns and King in 1811, MS is also referred as 
“chloroma” due its green colored appearance when exposed to air, by the 
presence of myeloperoxidase in the tumor cells [1-2]. Since the greenish color is 
not a consistent clinical finding and considering a proved granulocytic origin, the 
terms granulocytic sarcoma or myeloid sarcoma are preferably adopted by most 
authors [1-3]. 
 MS is mainly found in the bone and soft tissues, but it may affect virtually 
any site of the body such as the skin, lymph nodes, orbit and eye, oral cavity, 
bronchi, pericardium, peritoneum, gastrointestinal tract, kidney, reproductive 
organs, breast, and bladder [1-5,51]. Considering the 84 cases of oral MS 
previously published in the English literature, the average age of the patients is 
45 years (ranging from 1 to 89 years), with slight predilection for females (1,2:1). 
The mandible is the most common affected site (25 cases), followed by maxilla 
(20 cases), gingiva (13 cases), and palate (seven cases) [1-69]. MS affected 
multiple intraoral anatomical sites in seven cases, five of them with concomitant 
involvement of maxilla and mandible [20,43,45,54,64,66]. The most common 
7 
 
clinical feature of oral MS is a painful swelling or nodule with reddish to brownish-
colored ulcerated surface. From all cases reviewed from the literature, only five 
exhibited evident greenish coloration [3,7,16,53,55]. The clinical differential 
diagnosis is wide, ranging from lymphoma, squamous cell carcinoma and 
sarcomas to benign reactive or inflammatory lesions. The diagnosis of oral MS is 
usually based on histopathological and immunohistochemical analysis, and a 
history of symptoms associated with hematological diseases that might be 
absent [42-45,52]. In the present case, the patient was a 24-years-old female 
patient, who was diagnosed concomitantly with a subtype of acute myeloid 
leukemia. The clinical appearance of the present case was consistent with a 
malignant tumor, showing an ulcerated and painful brownish-colored swelling in 
the lingual aspect of the lower posterior gingiva with no bone involvement. The 
identification of pale-appearing mucosae and areas of coagulated blood within 
the gingival sulcus led us to suspect of a leukemia, which was confirmed after 
blood test and genetic analyses. 
 Microscopically, oral MS exhibits variable numbers of primitive, poorly 
differentiated cells with granular cytoplasm, round to oval nuclei with well-defined 
membrane and prominent nucleoli, intermingled with reactive inflammatory 
infiltrate. The tumor cells show different stages of myeloid differentiation, 
including the eosinophilic myelocytes and blastic cells with minimal granulocytic 
differentiation [40,42,44,51-52]. The microscopical differential diagnosis of oral 
MS include diffuse large B-cell lymphoma, Burkitt lymphoma, lymphoblastic 
lymphoma, and poorly-differentiated squamous cell carcinoma [44]. The 
histopathological diagnosis can be difficult, especially in cases of prominent 
reactive inflammatory infiltrate background and limited correlation with clinical 
features. Immunohistochemical analysis is necessary to prove the granulocytic 
origin of tumor cells [34], which are usually positive for myeloperoxidase (MPO) 
and CD99, important markers for the diagnosis of myeloid sarcoma [38,47,52]. In 
the present case, the identification of the granulocytic appearance of the tumor 
cells was difficult on hematoxylin and eosin-stained slides, and the 
immunohistochemical positivity for myeloperoxidase in a cytoplasmic pattern and 
for CD99 in a membrane pattern were essential to achieve the final diagnosis. 
 MS may precede, coexist with, or follow a presentation of acute myeloid 
leukemia (AML), but might also result from an acute blastic transformation of 
8 
 
myelodysplastic syndromes or myeloproliferative neoplasms [38-40]. In fact, the 
diagnosis of MS is currently considered synonym of AML, and the same 
chemotherapeutic regimens are used [34]. Acute promyelocytic leukaemia (APL; 
also previously known as AML-M3) accounts for around 8% of all AML cases, 
occurring mainly in early adulthood. APL is characterized by the predominance of 
abnormal promyelocytes in the bone marrow and identification of a specific 
chromosomal translocation t(15;17)(q24.1;q21.1) resulting in a fusion transcript 
between the genes promyelocytic (PML on chromosome 15) and retinoic acid 
receptor alpha (RARA on chromosome 17). The most common diseases 
associated with oral MS are AML (42 cases), followed by myelodysplastic 
syndrome (10 cases), and chronic myeloid leukemia (seven cases), nevertheless 
11 patients with oral MS did not present nor developed associated leukemia or 
myelodysplastic neoplasms [1-69]. 
 Approximately 90% of patients with APL have shown complete remission 
of disease after the advent of ATRA and anthracycline-based therapeutic 
regimens [40-44]. However, the rate of early induction death is still high in 
patients with APL, and one of the most common causes is hemorrhage, as 
observed in the present case. Only four out of 84 cases of oral MS reported in 
the English literature were associated with APL [10,19,48,63], and one died of 
the disease, as the present case. From all oral MS cases reported in the 
literature, 53 patients died of disease, 13 had no evidence of disease, and 11 
were alive with disease [1-69]. 
 In summary, oral MS is uncommon, with clinical and microscopical 
features that may mimic inflammatory lesions or other malignant tumors. Careful 
morphological and immunohistochemical analyses, correlating with clinical data 
are necessary to establish the diagnosis of oral MS. Clinicians and oral 
pathologists should consider MS when evaluating gingival swellings and 
ulcerations in patients with clinical findings suggestive of hematological 
abnormalities. 
 
 
 
 
 
9 
 
FIGURES 
 
 
Figure 1. Clinical features of oral myeloid sarcoma. (A) Intraoral examination 
showing pale oral mucosa, blood accumulation within the gingival sulcus of 
various teeth, and a normal colored swelling on the buccal posterior lower 
gingiva of the right side. (B) Brownish swelling with ulceration on the lingual 
aspect of the right posterior lower gingiva exhibiting also necrotic and bleeding 
surface. 
 
 
 
 
Figure 2. Panoramic radiography exhibiting absence of bone involvement. 
 
 
10 
 
 
 
 
 
Figure 3. Histopathological and immunohistochemical features of oral myeloid 
sarcoma. (A) Diffuse infiltration of the gingival connective tissue by sheets of 
poorly differentiated hematopoietic cells, exhibiting dense nuclei, and basophilic 
cytoplasm within a background of capillary proliferation and abundant erythrocyte 
extravasation (HE, 100X). (B) The infiltrate is composed mostly of myelocytes 
promyelocytes, and myeloblasts. The cells are large in size and round to oval in 
shape, and the cytoplasm was mild to moderately basophilic (HE, 400X). Tumor 
cells showed a strong positivity for (C) myeloperoxidase, and (D) Ki-67 
(Immunoperoxidase, 400X). 
 
 
 
 
 
 
11 
 
 
TABLES 
 
Table 1. Clinical features of 84 cases of oral myeloid sarcoma reported in the English-language literature and the 
present case. 
Nº Authors Age/
Gnd 
Location Clinical features Associated 
disease 
Follow
-up 
1 Ross (1955) [1]
 
24/M Mand Grayish painful swelling AML DOD 
2 Oldham & John (1966) [2]
 
2/F Mand Painless swelling AML DOD 
3 Wiernik & Serpick (1970) [3] 35/F Cheek Greenish-brown nodule AML DOD 
4 Brooks et al. (1974) [4] 8/M Max Painful swelling AML DOD 
5 Neiman et al. (1981) [5] NS Soft palate NS NS NS 
6 Hansen et al. (1982) [6] 83/FMax Painful ulcerated lesion AML DOD 
7 Conran et al. (1982) [7] 23/F Mand Greenish lesion NS NED 
8 Takagi et al. (1983) [8]
 
25/F Mand Painful firm nodule AML DOD 
9 Castella et al. (1984) [9]
 
89/F Hard palate Gray-white painful lesion NS DOD 
10 Reichart et al. (1984) [10] 35/F Mand Brownish painful lesion APL DOD 
11 Timmis et al. (1986) [11] 52/M Mand Nodular and ulcerated 
lesion 
NS DOD 
12 Welch et al. (1986) [12] 3/F Max Nodular lesion NS DOD 
13 Dreizen et al. (1987) [13] NS Max Red nodular lesion AML DOD 
14 Ficarra et al. (1987) [14] 67/F Max Painful swelling AML DOD 
15 Saleh et al. (1987) [15] 62/F Mand Painful mass MDS DOD 
16 Alessi et al. (1988) [16] 6/F Max Green nodular lesion AML DOD 
17 Barker & Sloan (1988) [17] 4/F Max Bluish ulcerated lesion AML DOD 
18 Rodriguez et al. (1990) [18] 56/M Mand Red ulcerated lesion AML DOD 
19 Cho et al. (1990) [19] 3/M Mand NS APL AWD 
20 Eisenberg et al. (1991) [20] 33/M Max/Mand Multiple nodular lesions AML AWD 
21 Stack & Ridley (1994) [21] 70/M Mand Nodular lesion CML DOD 
22 Ritter et al. (1994) [22] NS Gingiva NS AML NS 
23 Roth et al. (1995) [23] 80/M Gingiva NS AML DOD 
24 Tuset et al. (1995) [24]
 
NS Mand NS MDS DOD 
25 Lynch et al. (1998) [25] 86/F Max Brownish firm lesion AML DOD 
26-27 Menasce et al. (1999) [26] 54/F Gingiva NS MDS AWD 
 63/F Gingiva NS AML AWD 
28 Tong & Lam (2000) [27] 76/F Max Diffuse ulcerated lesions AML DOD 
12 
 
29 Tomás Carmona et al. (2000) 
[28] 
60/F Mand Pain and teeth mobility CML DOD 
30 Lee et al. (2001) [29] 43/F Max Black-pigmented lesion Not 
developed 
NED 
31 Amin et al. (2002) [30] 58/M Hard palate Hard palatal mass AML DOD 
32 Jordan et al. (2002) [31] 62/F Mand Purulent discharge from 
tooth apex 
MML DOD 
33 Antmen et al. (2003) [32] 12/F Max Painful red soft lesion AML DOD 
34 Asna et al. (2003) [33] 72/F Tongue Ulcerated red lesion MDS NED 
35 Dikbas et al. (2004) [34] 32/M Mand NS AML NS 
36 Stoopler et al. (2004) [35] 50/M Palate Multiple ulcerated lesions AML AWD 
37 Colella et al. (2005) [36] 62/F Max NS Not 
developed 
DOD 
38 Goteri et al. (2006) [37] 84/F Hard palate Infiltrative mass Not 
developed 
NED 
39 Jinbu et al. (2006) [38]
 
65/M Gingiva Red swelling AML NED 
40 Koudstaal et al. (2006) [39]
 
NS Max Painful blue-grayish 
lesion 
AML AWD 
41 Puranen et al.(2006) [40] 59/M Max Diffuse radiolucency in 
the apical area of 
maxillary canine 
AML DOD 
42 Yinjun et al. (2006) [41] 44/F Max Painful ulcerated lesion AML NS 
43 Yoon et al. (2006) [42] 63/M Max/Mand Firm and diffuse lesion AML DOD 
44-45 Matsushita et al. (2007) [43] 50/M Max Diffuse, granular, 
ulcerated lesions 
AML NED 
 59/M Mand Diffuse, granular, 
ulcerated lesions 
CML DOD 
46 Pileri et al. (2007) [44]
 
NS Gingiva NS NS NS 
47 Xie et al. (2007) [45] 32/F Max/Mand Painful, firm lesion CML NED 
48 Cho et al. (2008) [46]
 
52/F Max Painful lesion Not 
developed 
DOD 
49 Srinivasan et al. (2008) [47] 77/M Lower lip Firm, ulcerated lesion MDS DOD 
50 Mohamedbhai et al. (2008) 
[48] 
45/M Tongue Ulcerated lesion APL NED 
51 Cheng et al. (2009) [49]
 
56/M Buccal 
mucosa 
Firm painful mass AML DOD 
13 
 
52 Kim et al. (2009) [50] 4/F Mand Expansive and 
destructive mass 
AML NS 
53 Osterne et al. (2009) [51] 23/F Mand Painful red tumor AML NS 
54 Pau et al. (2010) [52] NS Max Painful red firm lesion CML NED 
55 Fasanmade et al. (2010) [53] 75/F Mand Brown-greenish ulcerated 
granular mass 
Myelofibrosis NED 
56 Papamanthos et al. (2010) 
[54] 
70/F Palate and 
mandible 
Painless ulcerated mass AML DOD 
57 Qiu et al. (2010) [55] 16/F Condyle Green painful swelling Not 
developed 
NED 
58 da Silva Santos et al. (2010) 
[56] 
47/F Gingiva Bleeding gingival 
overgrowth 
CML DOD 
59 Seema et al. (2011) [57] 5/M Mand Painless swelling AML DOD 
60 Younis et al. (2013) [58] 73/M Mand Painful swelling AML DOD 
61 Kurdoğlu et al. (2013) [59] 29/F Gingiva Painful gingival swelling Not 
developed 
DOD 
62 Mei et al. (2013) [60]
 
56/M Cheek Soft and solid mass Not 
developed 
NED 
63 Niscola et al. (2013) [61] 63/M Gingiva Painful ulcer MDS DOD 
64-65 Roby et al. (2013) [62]
 
1/NS Mand Mass AML DOD 
 12/N
S 
Tongue NS AML DOD 
66 Yamachita et al. (2013) [63]
 
1/M Mand Tumor-like lump APL NED 
67-78 Zhou et al. (2013) [64] 77/F Tongue Tongue lesion with 
bleeding 
MDS NS 
 55/M Mand Jaw pain CML DOD 
 47/F Tonsil and 
tongue 
NS AML AWD 
 61/M Tonsil Throat pain AML DOD 
 65/F Lower lip Painless mass AML AWD 
 55/M Gingiva Nodule MML DOD 
 21/M Tonsil Large left tonsil MML DOD 
 48/M Max/Mand NS MDS DOD 
 77/M Max Mouth pain MDS DOD 
 56/M Mand NS AML AWD 
 85/F Upper lip NS AML DOD 
14 
 
 17/F Buccal 
mucosa 
NS Not 
developed 
DOD 
79 Dosho et al. (2014) [65]
 
68/M Soft palate Painful mass AML DOD 
80 Moshref et al. (2014) [66] 45/M Max/Mand Multiple masses Not 
developed 
DOD 
81 Ponnam et al. (2014) [67]
 
45/F Gingiva Red firm swelling Not 
developed 
DOD 
82 Sharma et al. (2014) [68] 9/M Max Diffuse swelling Not 
developed 
AWD 
83-84 Yap et al. (2014) [69] 73/M Mand Erythematous nodule MDS DOD 
 65/M Gingiva White, homogeneous and 
well-circumscribed lesion 
AML AWD 
85 Present case 24/F Gingiva Painful brownish swelling APL DOD 
Gnd, Gender; F, female; M, male; NS, not specified; DOD, dead of disease; NED, no evidence of disease; AML, acute 
myeloid leukemia; APL, acute promyelocytic leukemia; MDS, myelodysplastic syndrome; AWD, alive with disease; MML, 
myelomonocytic leukemia; CML, chronic myeloid leukemia. 
 
 
 
 
 
 
 
Table 2. Antibodies used for immunohistochemical evaluation of oral myeloid sarcoma. 
Antibody Clone, Source Dilution Results 
CD20 L26, Dako®* 1:1000 Neg 
CD3 Polyclonal, Dako®* 1:500 Neg 
CD34 QBEnd10, Dako®* 1:50 Neg 
TdT Polyclonal, Dako®* 1:50 Neg 
Myeloperoxidase Polyclonal, Dako®*, 1:5000 + 
CD99 12E7, Dako®* 1:100 + 
Ki-67 MIB-1, Dako®* 1:100 + (60%) 
*Dako®, Glostrup, Denmark; +, positive; Neg, negative. 
 
15 
 
4- CONSIDERAÇÕES FINAIS 
 
 O sarcoma mieloide oral é incomum, com características clínicas e 
microscópicas que podem simular lesões inflamatórias ou outros tumores 
malignos. O diagnóstico final do SM oral depende da avaliação das 
características morfológicas e imuno-histoquímicas, incluindo a positividade para 
mieloperoxidase. A correlação com achados clínicos é necessária para 
confirmar associação com doença hematológica subjacente. Estomatologistas e 
patologistas orais devem considerar a possibilidade de sarcoma mieloide 
durante a avaliação de nódulos ou úlceras localizados na gengiva de pacientes 
com suspeita de alterações hematológicas. 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
16 
 
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