[Rare Tumors 2011; 3:e13][page 39]
NY; 2Albert Einstein College of Medicine,
Center, Department of Pediatrics, New
follow-up and remission
Linda Granowetter,4Deepa Manwani1,2
New York, NY; 3University of Alabama at
York, NY, USA
early infancy. It was originally considered a
benign neoplasm, however a number of reported
cases have documented locoregional recurrence
and distant metastases. Currently, there is no
consensus on the appropriate treatment for this
neoplasm. We report on long term follow-up of a
patient with metastatic sialoblastoma, and a
brief discussion of the possible treatment
modalities currently being considered.
for a rare salivary malignancy
1Montefiore Medical Center, New York,
Birmingham, AL; 4NYU, Langone Medical
Kanwal M. Farooqi,1,2Rachel Kessel,1,2
Sialoblastoma is a rare salivary neoplasm
which presents either congenitally or during
all patients with salivary gland tumors.1In a
review of the Salivary Gland Register of the
University of Hamburg, only 2.5% of all epithe-
lial neoplasia occurred in patients younger
than 20 years.2Sialoblastoma is a very rare
salivary tumor initially reported in 1966 by
Vawter and Tefft under the nosology of embry-
oma. This tumor has also been referred to as
congenital basal cell adenoma, basal cell ade-
noma, and basaloid adenocarcinoma. It may
present congenitally or perinatally.3Most com-
monly, it originates in the parotid but may also
arise in the submandibular gland.4,5
Sialoblastomas were initially classified as
benign. However, increasing experience,
including reports of distant metastasis, has
lead to the reclassification of sialoblastomas as
malignant.6,7Although the mainstay of treat-
ment is surgical resection with adequate mar-
gins,8there are reported patients who have
received alternative treatments such as neoad-
juvant chemotherapy, radiation therapy, and
even brachytherapy. We have previously
reported a young child who presented with a
parotid sialoblastoma at the age of 3.5
months.9This report extends the follow-up on
The pediatric population, specifically under
15 years of age, accounts for only about 4% of
this patient who is now 17 years old and in
remission for 7 years. This represents the
longest published follow up for a patient with
metastatic sialoblastoma, and the first report
of prolonged remission.
months of age.9The tumor was locally invasive
and recurred multiple times. Increasing tumor
mitotic index and cytological pleomorphism
was noted with recurrences (Figure 1). She
was treated with incomplete surgical resec-
tion, chemotherapy (vincristine, adriamycin,
cytoxan, etoposide and cisplatinum) and high-
dose radiation therapy at 3.5 years of age.
Orbital recurrence developed 1.5 years later,
which was completely resected with negative
margins; adjuvant radiation therapy was
administered. At 6.5 years of age, she devel-
oped metastasis to the right lung. There was
complete radiologic response after six cycles of
Ifosfamide, Carboplatinum and Etoposide;
however the patient developed metastasis to
the left lung two years later, which responded
to two cycles of Cytoxan and Topotecan (Figure
2). The left lung metastases were resected
with negative margins, and she received six
more courses of adjuvant chemotherapy with
the same agents. Unfortunately 6 months later,
she developed a third pulmonary recurrence in
the right upper lobe, this metastasis was treat-
ed by a wedge resection.
Presently, this patient is 17 years old; she
remains in disease-remission seven years
from the last intervention. This remission cer-
tainly seems indicative of a durable response,
as all recurrences had occurred at most 24
months from the end of previous treatment.
We originally reported a patient with a
sialoblastoma of the left parotid diagnosed at 3
for sialoblastomas.5,8,10,11In cases complicated
by metastases, recurrences, or difficulties in
surgical access or resectability, additional
chemotherapy and/or radiation may be neces-
sary.12,13Neoadjuvant chemotherapy and/or
radiation may result in good response.
Saribeyoglu et al. reported on a patient with
locally recurrent sialoblastoma who was treat-
ed with chemotherapy only, obviating the need
for surgical intervention.13Optimistically, that
patient was in remission for 4 years at the time
of publication. However, this is an isolated
report of a durable response; most of the pub-
lished reports lack long term follow-up. Scott et
Primary surgical resection with negative
margins is considered the mainstay therapy
Rare Tumors 2011; volume 3:e13
Correspondence: Deepa Manwani, Children’s
Hospital at Montefiore, 3415 Bainbridge avenue,
Rosenthal 3, Bronx NY 10467. Tel. 718-741-2342.
Key words: sialoblastoma, parotid gland neo-
Conflict of interest: the authors report no con-
flicts of interest.
Received for publication: 6 February 2011.
Accepted for publication: 10 March 2011.
This work is licensed under a Creative Commons
Attribution 3.0 License (by-nc 3.0).
©Copyright K.M. Farooqi et al., 2011
Licensee PAGEPress, Italy
Rare Tumors 2011; 3:e13
Figure 1. Recurrent sialoblastoma, which
forms nests of pleomorphic basaloid cells
with brisk mitotic activity.
Figure 2 Upper panel: low power photomi-
crograph of pulmonary metastasis. Lower
panel: high power photomicrograph demon-
strating primitive gland formation (G).
[page 40][Rare Tumors 2011; 3:e13] Download full-text
al. reported a young girl with a sialoblastoma
which had also metastasized to the lungs. The
tumor was resected after a good response to
metastases were treated by partial lobectomy.14
Only six months of follow up was reported for
this patient. Ersoz et al. describe a patient still
receiving chemotherapy for pulmonary relapse
at the time of the report.6Likewise, Prigent et
al. described a patient with sialoblastoma and
pulmonary metastases who was in remission
one year following neoadjuvant chemotherapy
and resection.12A novel approach to tumor
recurrence was recently reported by Shan et al.
In an effort to preserve facial nerve function
and avoid a second extensive resection,
brachytherapy with a 125I seed implant was
utilized, which lead to a complete clinical
response after two months. The patient was
disease-free 21 months after this intervention;
however the durability of this response cannot
In our case, the increased tumor mitotic
index and cytological pleomorphism preceded
development of distant metastases. Despite the
initial response to Ifosfamide, Carboplati num,
and Etoposide, and Cytoxan and Topo tecan,
chemotherapy and radiation, these modalities
did not prevent ultimate disease-progression.
Importantly, surgical resection of pulmonary
metastasis has lead to a durable remission.
While treatment planning for children with
sialoblastomas should be individualized for
each patient, surgical resection of recurrent
and metastatic disease provided our patient
with a long-term remission.
cases. Cancer 1972;30:459-69.
2. Seifert G, Okabe H, Caselitz J. Epithelial
salivary gland tumours in children and
adolescents. Analysis of 80 cases. (Salivary
Gland Register 1965-1984). ORL J
Otorhinolaryngol Relat Spec 1986;48:137-
3. Vawter GF, Tefft M. Congenital tumors of
the parotid gland. Arch Pathol 1966;82:242-
4. Batsakis JG, Mackay B, Ryka AF, Seifert
RW. Perinatal salivary gland tumours
(embryomas). J Laryngol Otol 1988;102:
5. Williams SB, Ellis GL, Warnock GR.
Sialoblastoma: a clinicopathologic and
immunohistochemical study of 7 cases.
Ann Diagn Pathol 2006;10:320-6.
6. Ersoz S, Turgutalp H, Cobanoglu U, et al.
Sialoblastoma in the parotid gland: a case
report. Pediatrics International. Pediatr
7. Ellis GL. What’s new in the AFIP fascicle on
1. Krolls SO, Trodahl JN, Boyers RC. Salivary
gland lesions in children. A survey of 430
salivary gland tumors: a few highlights
from the 4th series atlas. Head Neck
8. Alvarez-Mendoza A, Calderon-Elvir C,
Carrasco-Daza D. Diagnostic and thera-
peutic approach to Sialobastoma: report of
a case. J Ped Surg 1999;34:1875-7.
9. Brandwein M, Al-Naeif NS, Manwani D, et
al. Sialoblastoma: clinicopathological/
immunohistochemical study. Am J Surg
10. Vidyadhar M, Amanda C, Thuan Q, et al. J
Ped Surg 2008;43:e11-13.
11. Garrido A, Humphrey R, Squire RS,
Nishikawa H. Sialoblastoma. Br J Plast
12. Prigent M, Teissier N, Peuchmaur M, et al.
Sialoblastoma of salivary glands in chil-
dren: chemotherapy should be discussed
as an alternative to mutilating surgery. Int
J Pediatr Otorhinolaryngol 2010;74:942-5.
13. Saribeyoglu ET, Devecioglu O, Karakas Z,
et al. How to manage an unresectable or
recurrent sialoblastoma. Pediatr Blood
14. Scott JX, Krishnan S, Bourne AJ, et al.
Treatment of metastatic sialoblastoma
with chemotherapy and surgery. Pediatr
Blood Cancer 2008;50:134-7.
15. Shan XF, Cai ZG, Zhang JG, et al.
Management of sialoblastoma with sur-
gery and brachytherapy. Pediatr Blood