CASE REPORT


https://doi.org/10.5005/jp-journals-10077-3308
Journal of South Asian Association of Pediatric Dentistry
Volume 7 | Issue 2 | Year 2024

Bilateral Myeloid Sarcoma of Mandible in a Child: A Case Report


Rajesh Ragulakollu1https://orcid.org/0000-0003-4620-233X, Atluri N Supraja2https://orcid.org/0000-0002-2298-0965, Alekhya Achanta3https://orcid.org/0009-0004-3663-8409, Sunkara P Vardhan4, Mounika Erlapally5, Divya Reddymosali6

1–3Department of Pediatric Dentistry, Malla Reddy Institute of Dental Sciences (MRIDS), Hyderabad, Telangana, India

4Department of Pediatric and Preventive Dentistry, GITAM Dental College and Hospital, Visakhapatnam, Andhra Pradesh, India

5,6Department of Pediatric and Preventive Dentistry, Mamata Dental College, Mamata General and Super Specialty Hospital, Khammam, Telangana, India

Corresponding Author: Rajesh Ragulakollu, Department of Pediatric Dentistry, Malla Reddy Institute of Dental Sciences (MRIDS), Hyderabad, Telangana, India, Phone: +91 9014485370, e-mail: ragrajeshr@gmail.com

Received: 03 June 2024; Accepted: 01 July 2024; Published on: 06 September 2024

ABSTRACT

The solid malignant tumor of undifferentiated myeloid cells with extramedullary infiltration is known as myeloid sarcoma (MS), which is uncommon in occurrence. Other names for MS include myeloblastoma, extramedullary myeloid cell tumor, and granulocytic sarcoma. Lymph nodes, skin, and bones are the most frequent sites, while the orbits and central nervous system are less frequently affected. Comprehensive research on this condition has been hindered by the rarity of MS in pediatric populations. The main objective of this paper was to provide a case report of bilateral mandibular MS diagnosed in an 8-year-old female child.

Keywords: Case report, Extramedullary, Malignant, Myeloblastoma, Myeloid sarcoma

How to cite this article: Ragulakollu R, Supraja AN, Achanta A, et al. Bilateral Myeloid Sarcoma of Mandible in a Child: A Case Report. J South Asian Assoc Pediatr Dent 2024;7(2):109–112.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.

INTRODUCTION

Cancer is a set of disorders that are distinguished by abnormal, uncontrolled cell growth. Compared to adults, it occurs less frequently in children. It is estimated that approximately 10,500 children may suffer from cancer, with mortality potentially reaching 1,190 in 2021, as it is the primary cause of childhood deaths in developed nations, after accidents. Cancer ranks as the ninth most prevalent cause of death for children in India.1

The majority of swellings in the head-and-neck region are inflammatory in origin, but the occurrence of malignant swellings in children is rare. Approximately, 5–10% of malignant swellings in the head and neck region contribute to overall childhood malignancies, and their incidence rate is increasing considerably. Chronic, painless, firm enlarged lymph nodes present within the posterior triangle or supraclavicular space of the neck for > 6 weeks may suggest malignancy.

Frequently reported pediatric malignancies in the head and neck region include Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, rhabdomyosarcoma, nasopharyngeal carcinoma, neuroblastomas, salivary gland malignancies, and thyroid malignancies. Other, less frequently reported malignancies are soft-tissue sarcoma, malignant teratomas, nonrhabdomyosarcoma, primitive neuroectodermal tumors, and skin cancers.2

Myeloid sarcoma (MS) represents an atypical solid malignant tumor that develops when immature myeloid cells infiltrate into the extramedullary space. Originally identified by King and Burns in 1811 and later termed ”chloroma” in 1853, this name refers to the green color the tumor assumes when exposed to air, attributed to myeloperoxidase (MPO) present in the tumor cells. Various other terminologies used to describe it are myeloblastoma, extramedullary myeloid cell tumor, and granulocytic sarcoma.2

Myeloid sarcoma cases are usually observed in the oral cavity, involving the tonsils, tongue, gingiva, lips, buccal mucosa, periapical areas, and both soft and hard palates. MS might appear as the first sign of recurrent acute myeloid leukemia (AML) or during the pathogenesis of acute or chronic myelogenous leukemia. The diagnosis of MS is based on immunohistochemistry (IHC) [Auer bodies are typical markers of myeloid cells, whereas immunostaining of MS is always positive for cluster of differentiation 43 (CD43) and MPO].

CASE DESCRIPTION

A female child, aged 8 years, presented to the Department of Pedodontics with the primary complaint of swelling on her right cheek that had been progressively growing over the past year. It has been associated with dull, poorly localized pain and recurrent low-grade fever. The child has a history of traumatic injury related to the medial malleolus of the left ankle 1 year ago and the right ankle 2 months ago, both associated with fever. On extraoral examination, gross asymmetrical swelling was noted (Fig. 1). The swelling on the right side extends superior-inferiorly from the infraorbital region to 3 cm from the lower border of the mandible and mediolaterally from the ala of the nose to 2 cm from the tragus of the right ear, with intact overlying skin and a scar noted below the lower border of the mandible. The swelling on the left side extends superior-inferiorly from the left lateral canthus to the corner of the mouth and mediolaterally 4 cm from the tragus of the left ear to 6 cm from the ala of the nose, with intact overlying skin and no scarring on the left side. On palpation, there was a local rise in temperature, and the swellings on either side were firm, nonfluctuant, nontender, with no auscultatory findings observed. Bilaterally, the submandibular and cervical lymph nodes were enlarged, palpable, nontender, and indurated. Intraorally, there was reduced mouth opening and a swelling on the right buccal mucosa and alveolar ridge in relation to teeth 85 and 46 that was firm and nontender. Another swelling was observed on the left alveolar region in relation to tooth 75, and was firm and nontender (Fig. 2). Laboratory studies, such as complete blood picture analysis, revealed no abnormalities, but a slight increase in T3 and T4 levels was noted. Orthopantomogram showed multiple isolated radiolucencies extending up to the condyles (Fig. 3). Cone beam computed tomography revealed inferomedial displacement of the mandibular canal, dislocation of teeth, destruction of the right maxillary sinus, and damage to cortical plates with bicortical expansion, all of which were indicative of osteolytic lesions. An intraoral approach was adopted to carry out the incisional biopsy (Fig. 4). Histopathological analysis of the biopsy tissue revealed diffuse infiltration of monomorphous immature blast-like cells under the intact oral epithelium. The tumor cells were round to oval in form, with low to medium basophilic cytoplasm and devoid of granules. There were two distinct cell types in the tumor—some had pale nuclei with distinct nucleoli, while others had large, angular, hyperchromatic nuclei. Loss of cohesion and an increased nuclear-to-cytoplasmic ratio were observed among the tumor cells (Fig. 5). Through IHC, the tumor cells showed a highly positive reaction to leukocyte common antigen, CD34, and MPO monoclonal antibodies, while showing a very mild positive reaction to CD3 and CD117. The cells were profoundly negative for κ, λ, α smooth muscle actin, desmin, S-100, Melan-A, HMB-45, pan-keratin, CD15, CD20, CD45, CD56, and CD68 monoclonal antibodies (Fig. 6).

Fig. 1: Figure showing gross asymmetrical swelling of mandible

Fig. 2: Intraoral photograph showing swelling on the right and left buccal mucosa and alveolar ridge

Fig. 3: Figure showing orthopantomogram

Fig. 4: Figure showing cone beam computed tomography

Fig. 5: Figure showing histopathology

Fig. 6: Immunohistochemistry analysis showing positive reactions to CD117 and MPO, respectively

DISCUSSION

Myeloid sarcoma constitutes an uncommon malignancy occurring at variable ages and is distinguished by the hallmark immature myeloid cells present in extramedullary sites such as bone, lymph nodes, the gastrointestinal tract, soft tissues, skin, lungs, orbit, and central nervous system.3 There are several clinical manifestations of MS in childhood. The World Health Organization (WHO) classifies MS according to its clinical presentation. These subtypes include de novo MS, MS with peripheral blood involvement and bone marrow involvement, and MS that progressed from a previous myelodysplastic syndrome or myeloproliferative neoplasm, or both.4

Myeloid sarcoma is very uncommon in children with oral manifestations. Studies evaluated between the years 1990 and 2022 have reported five cases of MS in children involving the mandible (Table 1). Previous case reports have presented the occurrence of MS in the mandible, maxillary sinus, maxillary alveolus, palate, mandibular gingiva, and parotid gland in children.9 Oral MS may manifest as a solitary painful swelling with an ulcerated surface. Due to its low incidence and the possibility of tumors from nearly any lineage developing in the head and neck region, MS in these areas might present diagnostic challenges and lead to a wide differential diagnosis.10

Table 1: Table showing the summary of reported cases of MS of mandible in children
Authors Year Patient age/gender Location
Cho et al.5 1990 3/M Right side of mandible
Kim et al.6 2009 4/F Left side of mandible
Seema et al.7 2011 6/M Right side of mandible
Yamashita et al.8 2013 1/M Mentum of the mandible
Sengupta et al.9 2016 2/M Left side of the body of the mandible

It is speculated that the migration of cancerous cells from the bone marrow via Haversian canals is the cause of MS. Once the cells are no longer restricted by bone, they may hematogenously expand to more distant area.11 The local widespread impact causes symptoms such as swelling, pain, otitis media related to Eustachian tube blockage, alterations in vision, epistaxis, and nasal obstruction. The tumors appear on computed tomography as a well-defined region of enhanced attenuation featuring a peripheral zone of enhancement, and they are radiographically ambiguous. Their signal strength on magnetic resonance imaging is comparable to that of bone marrow. Despite being nondiagnostic, imaging can determine the extent of disease involvement.

Oral findings of MS are usually nonspecific, with localized or generalized swelling mimicking a dentoalveolar abscess, pyogenic granuloma, or gingival hyperplasia.12 Diagnosis of MS based solely on clinical features is challenging, especially in children. In this case report, the presence of multiple carious teeth associated with swelling is misleading, and the presence of a scar on the right lower border of the mandible adds to the diagnostic difficulty.

Accurate and prompt diagnosis of isolated MS can be difficult. Furthermore, nonspecific imaging alterations may appear as soft tissue occupying lesions, with or without bone erosion. In a study conducted by Yamauchi and Yasuda,13 35 out of 74 nonleukemic MS patients were misdiagnosed, and the diagnosis remained unrectified until bone marrow involvement or peripheral blood findings were identified.

Myeloid sarcoma’s vague radiographic characteristics lead to a wide range of differential diagnoses. Diffusely margined radiolucent regions are usually observed. Radiolucent lesions in the maxillofacial region can be roughly classified as either odontogenic or nonodontogenic, with the latter including cysts and tumors, which can be further classified as benign or malignant. Examples of odontogenic lesions include ameloblastomas, dentigerous cysts, myxomas, odontogenic keratocysts, and aneurysmal bone cavities. The two most common nonodontogenic lesions in children are eosinophilic granuloma and giant cell lesions. Based on the rapid onset of symptoms, widespread ill-defined margins on radiographic imaging, and growth with bone degradation, it is more likely that the lesion is malignant, such as lymphoma, rhabdomyosarcoma, or Ewing’s sarcoma.14 MS was not taken into account preoperatively in our patient owing to its low occurrence.

When observed clinically, displacements and resorption of teeth and surrounding tissues suggest the aggressive nature of MS. MS can result in blindness, deafness, and paralysis, depending on where it develops in the body. Despite starting in the bone marrow, the tumor cells are believed to move to the periosteum before hematogenously spreading to other sites.

Systemic chemotherapy, hematopoietic stem cell transplantation, and local therapy, including surgery, radiation, or both, are among the methods used to treat MS. Systemic chemotherapy must be initiated promptly in all cases, including solitary MS, which might ultimately progress to AML. In isolated MS patients, chemotherapy can improve overall survival and delay or even halt the disease’s progression to AML. Chemotherapy can relieve symptoms related to extramedullary lesions, as MS is a systemic condition that responds to systemic treatment. Therefore, the long-term outcome of MS, especially isolated MS, depends on timely detection and therapy. It is not advisable to rely solely on local therapy, as it does not appear to enhance the prognosis or slow the transition from MS to AML.15

CONCLUSION

Myeloid sarcoma has a rare occurrence and can develop at any site, which may or may not involve the bone marrow. A high index of clinical suspicion and integrated radiologic and histopathologic evaluation, including IHC, are important for the diagnosis of MS. Recognizing MS early can lead to better prognosis and treatment options. IHC and cytogenetic studies play a critical role in differentiating MS from histologically similar tumors that are treated differently.

ORCID

Rajesh Ragulakollu https://orcid.org/0000-0003-4620-233X

Atluri N Supraja https://orcid.org/0000-0002-2298-0965

Alekhya Achanta https://orcid.org/0009-0004-3663-8409

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