Post on 29-May-2020
transcript
137
Official Publication of OrofacialChronicle , India
www.jhnps.weebly.com
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
138
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).
139
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
140
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.
141
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
142
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
143
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.
144
REFERENCES:
1. Dulguerov P, Jacobsen MS, Allal AS, Lehmann W, Calcaterra T. Nasal and paranasal sinus carcinoma:
Are we making progress? A series of 220 patients and a systematic review. Cancer. 2001;92:3012–29.
2. Luna MA. Salivary mucoepidermoid carcinoma: revisited. AdvAnatPathol. 2006 Nov;13(6):293-307.
3. Shafer WG, Hine MK and Levy BM Text book of Oral Pathology 4 th Ed Philadelphia W B Saunders
1983, pg 248-49.
4. Seifert G, Sobin LH. In International Classification of tumours. New York: Springer-Verlag; 1991.
Histological typing of salivary gland tumours.
5. Goode RK, El-Naggar AK . Lyon: IARC Press; 2005. Mucoepidermoid Carcinoma. WHO Organization
Classification of Tumours. Pathology and Genetics of Head and Neck Tumours; p. 219-20.
6. Spiro RH, Huvos AG, Berk R, et al. Mucoepidermoid carcinoma of salivary gland origin. A
clinicopathologic study of 367 cases. Am J Surg. 1978;136:461–468.
7. Bhattacharyya N. Survival and staging characteristics for nonsquamous cell malignancies of the maxillary
sinus. Arch Otolaryngol Head Neck Surg. 2003;129:334–7.
8. Kraus DH, Roberts JK, Medendorp SV, Levine HL, Wood BG, Tucker HM, et al. Nonsquamous cell
malignancies of the paranasal sinuses. Ann OtolRhinolLaryngol. 1990;99:5–11.
9. Daryani D, Gopakumar R, Nagaraja A. High-grade mucoepidermoid carcinoma of maxillary sinus. Journal
of Oral and Maxillofacial Pathology : JOMFP. 2012;16(1):137-140.
10. Jakobsson PA, Blanck C, Encroth CM. Mucoepidermoid carcinoma of the parotid gland. Cancer.
1968;22:111–124.
11. Fonseca I, Clode AL, Soares J. Mucoepidermoid carcinomas. A survey of 43 cases. Int J SurgPathol.
1993;1:3–12.
12. . Auclair PL, Ellis GL. Mucoepidermoid carcinoma. In: Ellis GL, Auclair PL, Gnepp DR, eds. Surgical
Pathology of the Salivary Glands. Philadelphia: WB Saunders; 1991;269–298.
13. Evans HL. Mucoepidermoid carcinoma of salivary glands: a study of 69 cases with special attention to
histologic grading. Am J ClinPathol. 1984;81:696–701.
14. Batsakis JG, Luna MA. Histopathologic grading of salivary gland neoplasms: I. Mucoepidermoid
carcinoma. Ann OtolRhinolLaryngol. 1990;99:835–838.
15. Healey WV, Perzin KH, Smith L. Mucoepidermoid carcinoma of salivary gland origin. Cancer.
1970;26:368–388.
16. Ozawa H, Tomita T, Sakamoto K, et al: Mucoepidermoid carcinoma of the head and neck: clinical analysis
of 43 patients. Jpn J ClinOncol 2008, 38:414–418
17. Hosokawa Y, Shirato H, Kagei K, Hashimoto S, Nishioka T, Tei K, et al. Role of radiotherapy for
mucoepidermoid carcinoma of salivary gland. Oral Oncol1999;35:105-11
18. Varghese BT, Jacob MM, Madhavan J, Nair MK. Late scar recurrence in mucoepidermoid carcinoma of
base of tongue. J Laryngol Otol. 2000 Apr;114(4):299-301.
19. Chen AM, Garcia J, Granchi PJ, Johnson J, Eisele DW. Late recurrence from salivary gland cancer: when
does "cure" mean cure? Cancer. 2008 Jan 15;112(2):340-4.
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