Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2015) 16, 279–282

HO ST E D BYEgyptian Society of Ear, Nose, Throat and Allied Sciences

Egyptian Journal of Ear, Nose, Throat and Allied

Sciences

www.ejentas.com

CASE REPORT

Unilateral parotid gland swelling as the sole

presenting symptom of acute lymphoblastic

leukaemia in children

* Corresponding author at: Department of Otorhinolaryngology-

Head and Neck Surgery, School of Medical Sciences, Universiti Sains

Malaysia Health Campus, 16150 Kubang Kerian, Kelantan, Malaysia.

Tel.: +60 97673000x6428.

E-mail address: dryatiee@gmail.com (Y.S. Lahuri).

Peer review under responsibility of Egyptian Society of Ear, Nose,

Throat and Allied Sciences.

http://dx.doi.org/10.1016/j.ejenta.2015.07.0062090-0740 � 2015 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. All rights reserv

Yatiee Swany Lahuria,*, Irfan Mohamad

a, Mohd Iszuari Md Ismail

b,

Hasmah Hashim c, Hasleani Ibrahim c

aDepartment of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia HealthCampus, Kubang Kerian, Kota Bharu, Kelantan, MalaysiabDepartment of Otorhinolaryngology, Hospital Melaka, Melaka, MalaysiacDepartment of Pathology, Hospital Melaka, Melaka, Malaysia

Received 31 March 2015; accepted 22 July 2015

KEYWORDS

Parotid gland;

Acute lymphoblastic

leukaemia;

Acute myeloid leukaemia

Abstract Acute lymphoblastic leukaemia (ALL) in infants below 1 year of age contributed 2.5–5%

of childhood ALL incidence. Children with ALL commonly presented with fever, bruising, mucosal

bleeding, bone pain, pallor, hepatosplenomegaly, and lymphadenopathy. Common extra medullary

leukaemic infiltration has also been reported at diagnosis of ALL to sites such as the liver, spleen,

lymph nodes, brain, testes and even nephromegaly. However ALL presented with parotid

infiltration is exceedingly rare. We herein present a case of unilateral parotid enlargement in a child

with newly diagnosed ALL. This unusual presentation focuses on the importance of considering

ALL in the differential diagnosis of parotid enlargement especially when associated with abnormal

blood counts.� 2015 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V.

All rights reserved.

1. Introduction

Parotid gland enlargement in children is most commonly sec-ondary to infectious and inflammatory lesions.1 Acute lym-

phoblastic leukaemia (ALL) is the most common malignancy

in children and accounts for 25% of all childhood cancers.2

Leukaemia typically presents with non-specific symptomsand signs such as anorexia, fatigue and irritability. As the dis-ease progresses, other signs and symptoms such as pallor,

bleeding tendency, hepatosplenomegaly and lymphadenopathymay appear. Uncommonly, joint pain, proptosis, abdominalpain, malena, diarrhoea, dysphagia can also be noted as initial

manifestations, which may bewilder the clinician as well as thepathologists.3 Leukaemia may cause enlargement in the sali-vary glands with or without lacrimal gland involvement.

Primary neoplasms of the parotid gland are rarely seen inchildhood. Parotid swelling seen in ALL could be due to

ed.

280 Y.S. Lahuri et al.

infiltration of blasts into the parotid gland, which may mimicmumps.4 Parotid gland involvement in acute myeloid leukae-mia (AML) has been reported previously as an uncommon

presenting manifestation or relapse of disease. However paro-tid gland enlargement as a presenting manifestation of acutelymphoblastic leukaemia (ALL) is very rare.5

2. Case summary

A 2-year-old boy presented to Otorhinolaryngology (ORL)

Clinic with a complaint of painless right parotid swelling for1 week duration with no history of toothache or trauma. Nopallor, fever or other symptoms were documented.

Examination revealed right parotid mass, size 2 � 1 cm whichwas firm in consistency, non-tender and with no surroundingskin changes. Facial nerve examination showed no abnormal-

ity. Oral cavity, oropharynx and otoscopic examinationshowed normal findings. Full blood counts showed haemoglo-bin 12.3 g/L, TWBC 14.2/L and platelets 459/L. Erythrocytesedimentation rate is 10 mm/h. An ultrasonography of the

neck and parotid region showed an irregular multiseptated cys-tic intra parotid lesion size 2.4 cm � 2.0 cm � 1.6 cm. UponDoppler study no colour flow was seen. Multiple enlarged

bilateral cervical lymphadenopathy with variable sizes are alsoseen in ultrasound of neck and parotid.

Further investigation with Magnetic Resonance Imaging

(MRI) of head and neck showed enlargement of the right par-otid gland. There is a well-defined and homogenous lesionmeasuring 2.0 cm � 4.4 cm � 3.8 cm involving the superficialand the deep lobe of the right parotid gland, and it envelopes

the mandibular vessels. There is also an intraluminal thrombusmeasuring 0.4 cm in diameter and 2.0 cm in length within theright internal jugular vein with bilateral cervical lymphadeni-

tis. The lesion in MRI was most likely representing parotitis.Subsequent fine needle aspiration cytology (FNAC) of theright parotid mass reported atypical cells seen suspicious of

lymphoma. Following this, 2 weeks later, the child underwentright superficial parotidectomy and post-operative recoverywas good with no facial nerve palsy. Histopathologic analysis

Figure 1a Diffuse atypical lymphoid cells with occasional

residual salivary ducts (arrows).

from the right parotid mass specimen confirmed its diagnosisas acute lymphoblastic leukaemia (Fig. 1).

Immunohistochemical stains done to the neoplastic cells

showed positive for CD79 alpha, strongly positive for Pax 5,CD10, CD34 and Tdt, (Fig. 2). They are negative for CD20,CD3, CD5, CD117 and LCA. The proliferative index marker

(Ki67) is 50–60%. Upon confirmation of the diagnosis, patientwas then referred to paediatric haematology team. A bonemarrow trephine finding which was performed later revealed

thatmarrow cells are consistent with acute lymphoblasticleukaemia.

3. Discussion

In the paediatric population parotid neoplasms are very rare,with benign tumours accounting for 80% of the masses in

the parotid gland. Incidence of malignant parotid tumours inthe paediatric population is even rarer. Mucoepidermoid carci-noma is the most common malignant neoplasm found in pae-diatric patients. The parotid gland has been described as a site

of extramedullary relapse in patients with primary ALL andAML. It is also seen in newly diagnosed AML in childrenand adults with newly diagnosed T-cell leukaemia and newly

diagnosed lymphoma.1 Some literature also described the inci-dence of secondary neoplasms of the parotid gland (includingmucoepidermoid carcinoma, acinar cell carcinoma) in patients

previously treated for childhood ALL and AML.1,2 However,not much is known about the involvement of the parotid glandin newly diagnosed ALL.1

There are reports on relapse of disease or secondary malig-

nancy in the parotid/salivary glands after treatment of ALL orAML but ALL presenting with parotid mass, although it hasbeen mentioned previously is infrequent, and lacked supportive

radiologic evidence.1,3,6 Parotid gland has been reported to bea sanctuary site for the relapse of AML either in isolation, orin association with the central nervous system but the signifi-

cance of parotid infiltration by lymphoblasts is uncertain.5

Parotid gland involvement in ALL patients at initial presenta-tion is possibly a part of bulky extramedullary disease. It is

Figure 1b Diffuse atypical lymphoid cells consisting of small to

medium cells with irregular nuclei and a few residual salivary ducts

(arrows).

Figure 2a The atypical lymphoid cells are positive for Pax 5.

Figure 2b The atypical lymphoid cells are positive for Tdt.

Symptoms of acute lymphoblastic leukaemia in children 281

undetermined whether this rare occurrence of parotid glandinvolvement in ALL seen in our patient or in other reportswarrants aggressive local or systemic therapy or signifies a risk

of second malignancy or may later on act as a sanctuary site.5,6

Facial nerve involvement which co-exists with enlargementof the parotid gland in AML has been described in a previous

report.6 Nevertheless parotid gland involvement in ALLtogether with facial nerve palsy have not been reported.Hypothetically infiltration of lymphoblast from the parotid

mass could be the cause of facial nerve palsy.6 The involvementof the parotid gland along with mental neuropathy and facialand oral numbness restricted to the distribution of the mental

nerve called ‘‘numb chin syndrome” as initial symptoms ofALL has been described before by Hiraki et al.7 In the case ser-ies, 2 out of 3 patients aged 12–15 years presented with bilat-eral parotid and submandibular gland swelling first before

being diagnosed later as ALL.7

Parotid lesion in the paediatric population requiring paro-tid surgery is uncommon. Its pathology varies and differs from

that seen in adults, with congenital anomalies and infectionsbeing the commonest, thus poses a challenge.8,9 The decision

to proceed with surgery is difficult to make as a result of thepatients younger age, the perception of the anatomical field,possible consequences of facial nerve damage and the rarity

of clinical presentation.8,9 In our case, superficial parotidec-tomy was performed to obtain a definitive histopathologicdiagnosis for the patient as the FNAC of the right parotid

mass results shows atypical cells seen suspicious of lymphoma,comparing to MRI of the head and neck which shows featuresthat most likely represent parotitis. The surgery was performed

by a senior consultant otolaryngologist and the patient recov-ered well post operatively without facial nerve palsy or anyother complication. It is imperative to have a good anatomicalfield understanding along with a meticulous surgical technique

to minimise complications.8,9 A surgeon operating on parotidlesions in children faces particular challenges, and that experi-ence in adult parotid surgery may not be enough to ensure

optimal outcomes.8 Hence paediatric parotid lesions are bestmanaged by surgeons with specific skills and experience in thisfield.8,9

A different approach and modalities in investigating paro-tid lesions may also be required. FNAC has been reported tobe less useful in children than in adults. This is because, in chil-

dren, its accuracy decreased in the case of paediatric lymphoidtumours, embryonal tumours and vasoformative lesions.8,9 Italso may lead to skin breakdown and even sinus formationin cases of atypical mycobacterial infection. FNAC is also

poorly tolerated in young children without general anaesthesiathus making it hard to justify when the diagnostic yield is low.It is recommended that FNAC, mini-Trucut or core biopsy

should only be considered in older children in whom good tol-erance and compliance may be expected.8,9 Imaging modalitiessuch as ultrasonography is well tolerated in children and car-

ries no radiation risks. It has the advantages of not requiringsedation and could differentiate a solid from a cystic mass.On the other hand it may not be as accurate as MRI in defin-

ing the extent of the lesion. MRI gives superior soft tissuecharacterisation and less ionising radiation compared to com-puted tomograms (CT).8,9

4. Conclusion

Parotid gland enlargement is a very unique clinical presenta-tion of ALL. It is essential to include ALL in the differential

diagnoses of parotid gland enlargement in both children andadults especially in patients with abnormal blood counts, orga-nomegaly or even during the atypical course of any disease

during childhood. Patients are often misdiagnosed as mumpsand received symptomatic therapy, hence resulting in latereferrals and diagnostic delays. It emphasises the importance

of a detailed clinical examination with a high index of suspi-cion to look for non-infectious causes of parotid swelling, espe-cially if persistent, and that of subtle changes in blood countwhich could aid early diagnosis and referral by primary physi-

cian. Moreover, the differential diagnosis for parotid swellingin paediatrics is vast and differs from the one found in theadult population. Hence it is difficult to rule out the pathology

or rule out malignancy, based upon the clinical course, imag-ing or FNAC results alone. Surgical excision remains the stan-dard for management of these patients as it serves both

therapeutic and diagnostic purposes.

282 Y.S. Lahuri et al.

Conflict of interest

We have no conflict of interest to declare.

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