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Official Publication of OrofacialChronicle , India

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CASE REPORT

UNUSUALLY DELAYED RECURRENCE OF A LOW GRADE

MUCOEPIDERMOID CARCINOMA OF THE MAXILLARY

SINUS

Nitish Virmani 1, Jyoti P. Dabholkar

2

1-Medical officer, 2- Prof & HOD, Department of ENT and Head-Neck Surgery, Seth G.S. Medical

College and KEM Hospital, Mumbai, Maharashtra, India

ABSTRACT:

Mucoepidermoid carcinoma (MEC) is a malignant epithelial neoplasm composed of both mucus

secreting cells and epidermoid-type cells. Mucoepidermoid carcinoma arising from mucous

glands of maxillary sinus is extremely rare and accounts for 13% of all malignancies occurring in

maxillary sinus. While the high-grade MEC is a highly aggressive tumor, its low-grade

counterpart usually demonstrates a more benign nature. However, both local recurrence and an

aggressive clinical course have been reported to occur even with low-grade tumors.

We report a case of low grade mucoepidermoid carcinoma of the maxillary sinus who presented

to us with a recurrence 20 years after undergoing a total maxillectomy and post-operative

adjuvant radiotherapy. The patient was successfully managed with wide excision of the tumor.

KEYWORDS- Maxillary sinus, Low grade, Mucoepidermoid carcinoma

Cite this Article: Nitish Virmani , Jyoti P. Dabholkar , Unusually delayed recurrence of a low grade

mucoepidermoid carcinoma of the maxillary sinus , Journal of Head & Neck physicians and surgeons Vol 3 ,

Issue 3, 2015 :Pg 137- 144

INTRODUCTION:

Mucoepidermoid carcinoma (MEC) is a malignant epithelial neoplasm, believed to arise from

the reserve cells of excretory ducts. Mucoepidermoid carcinoma arising from mucous glands of

maxillary sinus is extremely rare and accounts for 13% of all malignancies occurring in

maxillary sinus.1Mucoepidermoid carcinoma displays a spectrum of biological behaviors; while

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the high-grade MEC is a highly aggressive tumor, its low-grade counterpart usually demonstrates

a more benign nature. However, both local recurrence and an aggressive clinical course have

been reported to occur even with low-grade tumors. The prognosis of patients with MEC

depends on the adequacy of treatment, the clinical stage, and the tumor grade and location.2-6

We report an unusual case of low grade muco-epidermoid carcinoma of the maxillary sinus who

presented with a delayed recurrence 20 years after a total maxillectomy and post-operative

adjuvant radiotherapy. The patient was successfully managed by a wide excision of the tumor.

CASE REPORT:

A 42 year old female patient presented to our outpatient department with a progressively

increasing left cheek and intra-oral swelling since 3 months. It was not associated with pain,

nasal obstruction, and epistaxis or reduced vision. 20 years back, this lady had developed same

sided cheek swelling in addition to a palatal swelling for which she had undergone total

maxillectomy at an outside centre. Records indicated that the post-operative histopathology was

suggestive of mucoepidermoid carcinoma subsequent to which she had received adjuvant

radiotherapy. Detailed pre-operative or intra-operative records were not available to us and

neither were the details of radiation dose or fields. Physical examination revealed a 5 cm X 4 cm

swelling over her left cheek extending between the zygomatic arch laterally, naso-maxillary

groove medially, oral commissure inferiorly and the lower lid superiorly. On palpation, the

swelling was non-tender with a variegate consistency with both firm and cystic areas. A healed

scar of Weber-Ferguson incision was evident along with ectropion of the medial part of lower

eyelid. Left ala was pulled inwards causing narrowing of the left nostril (Figure 1). Intra-oral

examination revealed a 4 cm X 3 cm mucosa-covered swelling in the region of maxillectomy

defect extending medially upto the midline and posteriorly upto the anterior margin of the soft

palate. There was a 2 cm X 0.5 cm fistula just adjacent to the medial margin of swelling (Figure

2).

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Figure 1: Maxillary swelling left side wih ectropion(black arrow)

Figure 2: Intraoral swelling left side with oro-nasal fistula ( black arrow)

A contrast-enhanced CT scan of the nose and paranasal sinuses revealed the post-maxillectomy

status. It showed a well-defined hypodense soft tissue lesion measuring 3 X 2.2 X 2.8 cm in the

operated left maxillary region, anterolateral to the intact pterygoid plate. It appeared to project

into the oral cavity and also showed a rim of peripheral post contrast enhancement. There was a

soft tissue lesion of similar characteristics in the left infraorbital region, measuring 3.2 X 5.2 X

2.5 cm. Overlying skin and subcutaneous tissue appeared to be uninvolved. (Figure 3-6)

Figure 3 Figure 4

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Figure 5 Figure 6

Figure 3-6 showing a hypodense soft tissue lesion in operated left maxillectomy cavity; anterolateral to the

intact pterygoid plate and projecting into oral cavity. The lesion shows a rim of peripheral enhancement. A

similar lesion in seen in the left infraorbital region. Left orbit is spared.

In view of the suspected recurrence, a PET-CT scan was done. It revealed a low-grade FDG

uptake of SUV Max 2.0 and 1.23 in the two lesions respectively. There was no evidence of

cervical adenopathy or distant metastasis (Figure 7).The patient was taken up for a revision

surgery by an external approach. A Weber-Ferguson incision over the previous scar was made

and the cheek flap elevated. En-bloc wide local excision of both the lesions was performed

including the overlying oral mucosa (Figure 8).The same incision was used to correct ectropion

of lower lid. Oro-nasal separation was achieved with an obturator.

Figure 7- PET-CT low grade FDG uptake.

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Figure 8: Resected specimen

Histopathological examination of the resected specimen revealed a multiloculated cyst filled

with inspissated mucus and lined by cells resembling squamous epithelial cells, cells with

moderated to abundant cytoplasm having irregular, eccentric nuclei resembling clear cells, and a

few intermediate cells. There was no evidence of atypia/necrosis/mitosis. A pathological

diagnosis of low grade mucoepidermoid carcinoma was made with all the resected margins free

of tumor. Post-operatively, the patient achieved a normal oral feeding and good voice. No

recurrence has been observed at 6 months and the patient has been advised a long term regular

follow-up.

DISCUSSION:

Mucoepidermoid carcinoma is a malignant epithelial neoplasm composed of both mucus

secreting cells and epidermoid-type cells in varying proportions. It was first studied and

described as a separate entity by Stewartet al. in 1945.3 After a systematic review of its histology

and degree of differentiation, the WHO classification in 1991 recommended that, the term

“mucoepidermoid tumor” be changed to “mucoepidermoid carcinoma”.4Mucoepidermoid

carcinoma is the most common malignant neoplasm observed in the major and minor salivary

glands among children and adults.5Spiro et al in his study of 367 mucoepidermoid carcinomas

found an incidence of 2 % for nasal cavity and 3% for maxillary antrum.6

Malignant tumors of the paranasal sinus are uncommon, constituting less than 1% of all

malignancies and 3% of all head and neck cancers. The majority of these tumors are in the

maxillary sinus, and squamous cell carcinoma is the commonest histological type.1As a subset of

maxillary sinus malignancies, non-squamous cell cancers of the maxillary sinus are rare entities.7

Kraus et al. in a study of 49 patients with nonsquamous tumors of maxillary sinus reported

sarcomas, adenoid cystic carcinomas, lymphoma, and adenocarcinoma accounting for most of

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their cases.8 In contrast Bhattacharya

7 in his series of 188 cases had adenoid cystic carcinoma

(34%) as the predominant histological type followed by sarcomas (24%).7Mucoepidermoid

carcinoma arising from mucous glands of maxillary sinus is extremely rare and accounts for 13%

of all malignancies occurring in maxillary sinus.1

MEC arising in maxillofacial region can have its origin from the maxillary sinus lining or central

MEC arising from within the bone or from the minor salivary gland.9In the absence of pre-

operative imaging from the first surgery, it is difficult to determine the origin of tumor in our

patient. The prognosis of patients with MEC depends on the adequacy of treatment, the clinical

stage, and the tumor grade and location.2-6

Maxillary sinus malignancies are traditionally considered to be difficult tumors to treat and

consequently have been associated with a poor prognosis. Close proximity of these tumors to

vital structures such as the skull base, brain, orbit, and carotid artery often makes complete

surgical resection a challenging task.7 Moreover, these tumors, including the MEC of maxillary

sinus tend to be asymptomatic at early stages, appearing more frequently at late stages once

extensive local invasion has occurred.This unfortunate combination of complex anatomy, vital

surrounding structures and advanced stage at presentation leads to the frequent local recurrence

and subsequent poor outcome associated with sinonasal malignancies.9

The 3-level grading approach to classifying tumors has found general acceptance among

pathologists, and differences in biologic behaviour can be demonstrated, even though clinical

stage became a better prognosticator.2, 10, 11

Suggested grading criteria for MEC have included,

either singly or in combination, the relative proportion of cell types, degree of invasion, pattern

of invasion, mitotic rate, proportion of tumor composed of cystic spaces relative to the solid

growth degree of maturation, and neural and vascular invasion.2, 12

Various histological grading

systems are in use among the pathologists. In our patient, the presence of macrocysts lined by

differentiated epidermoid and clear cells, few intermediate cells, and extravasated mucin with

absent mitoses and pleomorphism led to a diagnosis of low grade mucoepidermoid carcinoma

according to Batsakis and Luna’s modification of Healey’s system. Even with the Auclair and

Goode’s grading system, or by its Brandwein’s modification, the tumor in our patient would be

classified as low grade with a score of 0, considering the presence of > 20% cystic component,

absence of necrosis, mitoses, perineural spread and atypia. Survival is closely related to

histologic grade.13, 14

Although staging and grading are related, they seem to function independently of each other.

Low-grade lesions behave less aggressively than do high-grade lesions, regardless of stage;

conversely, stage I and II tumors have a better prognosis than do stage III or IV tumors,

regardless of grade.6Patients are more likely to experience a recurrence if the margins of

resection are positive, regardless of grade. Healey et al reported that 0 of 33 low-grade and

intermediate-grade lesions recurred when the margins were free of carcinoma, but 6 of 12 of the

same grade recurred when the margins were positive.15

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Ozawa et al experienced two patients with low-grade MEC who were diagnosed as stage IV and

had multiple neck metastases at first medical examination and died as a result of distant

metastasis.16

Moreover, when a discrepancy existed between the grade and stage of a tumor

(high-grade, stage I or low grade, stage III), the outcome was influenced more by the clinical

stage than the histologic grade.6

Although, mucoepidermoid carcinoma has been considered a radioresistant tumor, postoperative

radiation is thought to be effective.16

Postoperative radiotherapy for MEC patients with positive

surgical margin has been reported to decrease local failure.17

Although, most of the

mucoepidermoid carcinomas that recur do so within 1 year of therapy, delayed recurrences have

been reported.6, 15

Varghese reported a case of mucoepidermoid carcinoma of the base of tongue

which recurred loco-regionally after 20 years.18

In Chen’s study of recurrences in salivary gland

cancer, three recurrences occurred after a period of 15 years, the maximum being 23.1 years but

they did not correlate the time to recurrence with specific tumor type.19

To our knowledge, our

patient seems to be the first case reported in literature, of a low grade mucoepidermoid

carcinoma of the maxillary sinus to recur after an unusually long period of 20 years.

Thus, it is important to remember that patients with low grade MECs are neither “immune” to

local recurrences, nor to distant metastasis. The local recurrence in our patient occurred after a

long period of around 20 years and the patient had received adjuvant radiotherapy after her first

surgery. In the absence of a detailed histopathological report of the first surgery, we can only

speculate on the cause of recurrence. It could probably be the result of an inadequate resection

done at the first instance with the subsequent indolent growth of the low-grade tumor. Thus, it is

important to ensure a wide excision with negative margins even in cases of low grade

MECs.Thus, post-operative local recurrence is likely to occur when the resection is inadequate

(or margins are positive) irrespective of tumor grade. Adjuvant radiotherapy may not be able to

compensate for an incomplete excision, as demonstrated in our case. Considering that the final

histopathology was consistent with a low grade tumor and all the resected margins were free, we

decided not to re-irradiate the patient. She has been recurrence-free so far and has been advised a

long term follow-up.

In view of the delayed recurrence as seen in this case, we advise a long term follow-up of all

patients with low grade muco-epidermoid carcinomas.

CONCLUSION:

Mucoepidermoid carcinoma of the maxillary sinus is a rare entity. Through this case report, we

wish to emphasize that although, low grade MECs are biologically less aggressive tumors, they

are not immune to loco-regional recurrences. It is imperative to ensure an en-bloc excision of

these tumors with negative margins to avoid recurrences. Long term follow-up for all patients is

recommended as delayed recurrences may occur.

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CONFLICT OF INTEREST- NIL

ACKNOWLEDGEMENT- NIL

ETHICAL APPROVAL- TAKEN

CORRESPONDANCE ADDRESSES:

Dr. Nitish Virmani

H.No. 576, Sector – 37, Faridabad, Haryana – 121003

Mob no. 09702980431

Email: nitish_virmani@yahoo.com