Page 1 of 4
Case study
CY Park, KE Lee, SJ Lim, HJ Kim*
Abstract
Adenoid cystic carcinoma is an epi­
thelial tumour of the major and mi­
nor salivary glands, accounting for
about 1% of all malignant tumours of
the oral and maxillofacial region. Sur­
gical excision with wide margins is
the treatment of choice for adenoid
cystic carcinoma, but the tendency of
this tumour to locally recur and to de­
velop distant metastasis is correlated
with an ultimately poor prognosis.
Spontaneous regression of cancer,
especially in the sinonasal tract, is a
rare biological event. Here, we pres­
ent a case of recurred adenoid cystic
carcinoma of the nasal cavity that
spontaneously regressed after an in­
tranasal biopsy. We also present a
brief literature review.
Introduction
Spontaneous regression (SR) of a ma­
lignant tumour is defined as partial or
complete disappearance of a tumour
in the absence of any treatment or
during therapy that does not exert a
significant effect on neoplastic dis­
ease1,2. SR is known to be very rare,
with an estimated incidence of >1 in
60,000–100,000 cases3. The mecha­
nisms underlying SR of cancer have
not yet been fully determined; how­
ever immunologic action, elimination
of carcinogens, hormones, trauma,
diet and medication have been re­
ported as possible causes4. Among
the reported cases of SR of cancer,
more than half are related to renal
cell cancer, neuroblastoma, malignant
melanoma and choriocarcinoma5.
* Corresponding author
Email: [email protected]
Department of Otolaryngology–Head and
Neck Surgery, Chung-Ang University College of
Medicine, Seoul, Korea
Adenoid cystic carcinoma (ACC) is
the most frequent malignant tumour
of the submandibular and minor sali­
vary glands, constituting 10% of sali­
vary neoplasms, but it is an
uncommon malignancy in the sino­
nasal tract6,7. ACC in the sinonasal
tract tends to be extensively involved
in surrounding structures, including
the brain, orbit or carotid artery, and
it has a moderate to high-risk of local
recurrences7. In addition, there is a
propensity for discreet regions of tu­
mour infiltration along the cranial
nerves. These findings make the clin­
ical course of ACC potentially morbid.
Because ACC in the sinonasal tract
is uncommon, it is difficult to define
the characteristics, but there are a
few reported cases of SR of sinonasal
tract ACC where the patient did
not receive any type of therapy8.
In this study, we present a case of
recurred ACC that spontaneously re­
gressed after a biopsy was performed
to confirm the pathologic diagnosis
without treatment. We also present a
literature review on SR of ACC in the
nasal cavity.
Case presentation
In October 2011, a 74-year-old woman
was admitted to our department due
to complaints of frequent nasal bleed­
ing over a three-month period. The
patient had been diagnosed with ACC
(pT4aN0M0) of the hard palate in
2006 and had undergone total maxil­
lectomy. She also received adjuvant
radiotherapy (7000 cGy) to prevent
recurrence and she received regular
follow-up for five years without any
signs of recurrence. Upon physical ex­
amination, there was a 1 × 2 cm-sized
haemorrhagic mass on the medial
portion of the right middle turbinate,
which extended from the sphenoid
sinus antrum and tended to bleed
easily (Figure 1a). Enhanced perineu­
ral spread magnetic resonance imag­
ing (PNS MRI) was performed, which
revealed a well-defined heteroge­
neous enhancing mass between the
right middle turbinate and nasal sep­
tum (Figure 1b). Therefore, we sus­
pected the recurrence of ACC in the
nasal cavity and recommended an in­
tranasal biopsy for pathologic diag­
nosis and further treatment.
Intranasal biopsy using straight
cup forceps was performed (Figure 2)
under local anaesthesia, which estab­
lished the histological diagnosis of
recurred ACC (rT1N0M0) of the right
nasal cavity (Figures 3a and 3b).
Consid­ering the patient’s general
weakness, booster radiotherapy was
planned and it was scheduled to start
after one month.
The following month, physical
examination of the patient’s nasal
cavity was remarkable. The size of
the mass was clearly reduced with­
out the administration of any kind of
treatment and nasal bleeding was
not noted. Thus, radiotherapy was
delayed and we decided to closely
observe the changes in tumour size
at our outpatient clinic. Follow-up
MRI was performed at two months
after biopsy, which indicated that
the mass had completely regressed
(Figure 4a). At six months after bi­
opsy, an intranasal endoscopy showed
a barely visible mass lesion in the pa­
tient’s nasal cavity (Figure 4b). Since
then, the patient has remained in
good health and currently shows no
signs of recurrence.
Discussion and conclusion
ACC usually originates in the major
or the minor salivary gland of the
head and neck6. Although uncommon,
Copyright © 2012 OA Publishing London
For citation purposes: Park CY, Lee KE, Lim SJ, Kim HJ. Spontaneous regression of recurred adenoid cystic carcinoma in
the nasal cavity. Head Neck Oncol. 2012 Sep 9;4(2):48.
Competing interests: none declared. Conflict of interests: none declared.
All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
Spontaneous regression of recurred adenoid cystic
carcinoma in the nasal cavity
Page 2 of 4
(a)
(b)
Figure 1: (a) Pre-treated endoscopic finding in the present case. Intranasal
endoscope showed a 1 × 2 cm-sized haemorrhagic mass (white arrow) on the
medial portion of the right middle turbinate extending to the sphenoid sinus
antrum. (b) Pre-treated perineural spread magnetic resonance imaging (PNS
MRI) findings in the present case. The axial view of enhanced T2-weighted PNS
MRI shows a well-defined heterogeneous enhancing mass lesion (white arrow)
between the upper part of the middle turbinate and the nasal septum. *, middle
turbinate; #, nasal septum.
Figure 2: Endoscopy showing an intra­
nasal biopsy using straight cup forceps.
A histological diagnosis of ACC was
established from tissue obtained by
intranasal biopsy. *, middle turbinate;
#, nasal septum.
(a)
(b)
Figure 3: Pathologic results in the present case. (a) Low-power view of the mass
showing a solid lesion with variably sized, compact glandular and acinar struc­
tures (H & E, ×40). (b) High-power view of the mass showing numerous small
monotonous cells with gland formation, characterised by a cribriform pattern
(H & E, ×100).
it has also been reported to develop
in the breast, lacrimal gland of the
eye, lung, brain, trachea and parana­
sal sinuses. ACC is very slow-growing,
but has aggressive char­acteristics.
It spreads to the surrounding tissue,
and tends to keep recurring even
with repeated and wide resections6.
Typical perineural invasion causes
pain and invasion along the cranial
nerve. Regional lymph node invasion
is frequent and distant metastases
can occur early6,7. Reports show a
survival rate of 89% at five years, but
this is dramatically reduced to only
40% at 15 years. Radiotherapy may
induce temporary regression, but
does not cure the disease and chemo­
therapy is not known to be effective
either7,8. In the present case, a newly
developed ACC around the antrum of
the sphenoid sinus occurred five
years later, although ACC on the hard
palate was completely cured through
sur­gical resection and post-operative
radiotherapy.
SR is defined as the complete disap­
pearance of malignant disease with­
out medical treatment1,2. Everson
and Cole reviewed 176 cases show­
ing SR of malignant tumours and
determined that the incidence of SR
was only 1 in 60,000–100,000 cases3.
Of 176 cases of SR of cancer, renal
cell carcinoma (RCC) was the most
common malignancy and the major­
ity involved regression of pulmonary
metastases3. The regression of me­
tastasis can occur after the ­removal
of the primary tumour, such as
nephrectomy in the case of RCC
or hysterectomy in the case of
­choriocarcinomas. Few studies have
reported SR of primary malignan­
cies, and only five cases of SR of pri­
mary thoracic lesion have been
reviewed8. SR of primary malignan­
cies in the sinonasal tract is rare and
few cases of SR of ACC have been re­
ported. To the best of our knowledge,
our patient is the first case of SR
of ACC.
The reason for SR of cancer re­
mains unclear. One hypothesis is that
Copyright © 2012 OA Publishing London
For citation purposes: Park CY, Lee KE, Lim SJ, Kim HJ. Spontaneous regression of recurred adenoid cystic carcinoma in
the nasal cavity. Head Neck Oncol. 2012 Sep 9;4(2):48.
Competing interests: none declared. Conflict of interests: none declared.
All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
Case study
Page 3 of 4
Case study
(a)
(b)
ACC is known to be a highly inva­
sive malignancy with a typically fatal
prognosis. It is also difficult to achieve
adequate locoregional control with
ACC. In this report, we presented a
case of ACC with SR in the sinonasal
tract and suggested that surgical
trauma may have been the cause of
improvement in the clinical progno­
sis of ACC in our patient.
Figure 4: (a) Perineural spread magnetic resonance imaging (PNS MRI) find­
ings after an intranasal biopsy. PNS MRI was performed at two months after the
intranasal biopsy. The enhanced T2-weighted axial view showed that the previ­
ous mass lesion on the medial portion of the middle turbinate had disappeared
and no suspected malignant lesion was observed. (b) Endoscopic finding after
an intranasal biopsy. Endoscopic examination was performed at six months af­
ter the intranasal biopsy and healthy nasal mucosa was observed around the
middle turbinate and the nasal septum. No tumour-like lesion was detected.
*, middle turbinate; #, nasal septum.
tumours may sometimes grow more
rapidly than their blood supply,
which may cause direct necrosis and
regression of the tumours. However,
the definite causes for such a rapid
growth of tumour cells have not yet
been verified9.
Paraneoplastic sensory neuronop­
athy (PSN), which is a rare syndrome
causing sensory ataxia in the distal
extremities, has been suggested as a
cause of SR of small-cell lung cancer
in some patients10. It has been re­
ported that specific autoantibodies
such as anti-Hu, anti-Yo and anti-Ri
antibodies react with tumour tissues,
resulting in the activation of the T
lymphocyte immune response11.
Although neurologic toxicity is a con­
cern, patients with these autoanti­
bodies tend to have a better cancer
prognosis, with the tumour being
smaller, less metastatic and more
slow growing11.
Recent reports have focused on al­
teration of the immune system such
as T-cell and/or B-cell dysfunction as
an explanation for SR, which has been
observed in a variety of clinical set­
tings such as in immune suppressive
therapy and immune deficiency syn­
dromes, through the use of biological
modulators, or during infection or
hormonal changes. In particular,
surgical trauma may also induce im­
mune dysfunction of tumour cells,
thereby resulting in direct necrosis
and SR in patients9,12.
In the case of thoracic malignan­
cies, surgical trauma had also been
reported to result in SR13–16.
Interestingly, 43% of thoracic malig­
nancies that regressed spontane­
ously were observed in patients who
had received surgical trauma3,15. In
those cases, surgical trauma included
procedures such as bronchoscopy
and incomplete tumour removal. As
for the patient in our case, an intrana­
sal biopsy was performed for a patho­
logic diagnosis and the recurred
ACC regressed without additional
treatment one month after the bi­
opsy. We did not find any clinical or
physical signs of neurologic toxicity
to confirm PSN but estimated that
surgical trauma to recurred ACC
may have potentially acted as a trig­
ger for an immune response, result­
ing in SR.
ACC, adenoid cystic carcinoma; MRI,
magnetic resonance imaging; PNS,
perineural spread; PSN, paraneoplas­
tic sensory neuronopathy; RCC, renal
cell carcinoma; SR, spontaneous
regression.
References
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man. Tex Rep Biol Med. 1952;10(1):
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2. Everson TC, Cole WH. Spontaneous
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6. Fordice J, Kershaw C, El-Naggar A,
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Copyright © 2012 OA Publishing London
For citation purposes: Park CY, Lee KE, Lim SJ, Kim HJ. Spontaneous regression of recurred adenoid cystic carcinoma in
the nasal cavity. Head Neck Oncol. 2012 Sep 9;4(2):48.
Competing interests: none declared. Conflict of interests: none declared.
All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
Abbreviations list
Page 4 of 4
Case study
with paraneoplastic neuronal antibodies.
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et al. [Spontaneous regression of small
cell lung cancer]. Nihon Kyobu Shikkan
Gakkai Zasshi. 1994 Apr;32(4):324–7.
Copyright © 2012 OA Publishing London
For citation purposes: Park CY, Lee KE, Lim SJ, Kim HJ. Spontaneous regression of recurred adenoid cystic carcinoma in
the nasal cavity. Head Neck Oncol. 2012 Sep 9;4(2):48.
Competing interests: none declared. Conflict of interests: none declared.
All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
9. Montie JE, Straffon RA, Deodhar SD,
Barna B. In vitro assessment of cell-­
mediated immunity in patients with renal
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