Noussios et al.

Journal of Medical Case Reports 2010, 4:130

http://www.jmedicalcasereports.com/content/4/1/130
JOURNAL OF MEDICAL
CASE REPORTS
CASE REPORT Open Access

Alveolar soft part sarcoma of the tongue in a

Case report

3-year-old boy: a case report

George Noussios*1,2, Pantelis Chouridis1, Ioannis Petropoulos1, Kostas Karagiannidis1 and George Kontzoglou1

Abstract
Introduction: Alveolar soft tissue sarcoma of the tongue is a very rare and aggressive tumor which occurs in infancy
with a relatively controversial histogenesis. It may mimic benign vascular neoplasms and may metastasize to the lungs,
brain or bones.
Case presentation: We present the case of a three-year-old Caucasian boy who was admitted to our department with
a history of dysphagia and two episodes of oral bleeding which had lasted for a period of six months. A thorough
histological examination together with imaging techniques form the basis of a reliable diagnosis.
Conclusion: Alveolar soft tissue sarcoma of the tongue is a rare tumor which occurs in infancy and which is often
misdiagnosed pre-operatively. It should therefore be included in the differential diagnosis of oral pediatric lesions.

Introduction his pharynx and was initially thought to be a heman-

Alveolar soft part sarcoma of the tongue (ASPS) is a rare gioma (Figures 1 and 2). A biopsy was performed under
and aggressive malignancy which comprises 0.4% to 1.0% general anesthesia and histological examination showed a
of all soft tissue sarcomas. It accounts for up to 5% of all hemangioma of the tongue. He was treated conserva-
pediatric soft tissue sarcomas, apart from rhabdomyosar- tively with corticosteroids and interferon (IFN) 2A. A
comas [1]. In infants and children, the most common site biopsy specimen was also sent to a hospital laboratory
of origin is the head and neck region, especially the orbit which specialized in vascular anomalies. Microscopic
and tongue. It occurs extremely rarely in children under 5 examination also showed a benign capillary hemangioma.
years of age: only a dozen such cases have been reported Despite the treatment, the mass had accreted exces-
in the last 50 years. We present a case of ASPS occurring sively and six months later it was causing partial airway
in the dorsum of the tongue. obstruction. Our patient was put in contact with another
specialized center in pediatric tumors and he underwent
Case report a tracheostomy and wide excision of the mass at the same
A 3-year-old Caucasian boy originating from Northern time. A second diagnosis of hemangioma was also made
Greece was admitted to our department in Hippokratio by this center. The tracheostomy was closed successfully
General Hospital of Thessaloniki with a history of dys- 21 days later.
phagia and repeated episodes of oral bleeding which had Carefully controlled biopsies were then performed and
lasted for a period of six months. A physical examination the subsequent histological examination showed ASPS of
revealed a reddish-blue, soft, painful and immobilized the tongue. Microscopically the tumor consisted of struc-
mass in the mid-dorsum area of the tongue extending to tured, irregular and rough clusters of cells (Figure 3).
its base. There was no cervical lymph node enlargement. Most of the vascular channels were enclosed by thin con-
Computed tomography (CT) and magnetic resonance nective tissue septa. The tumor cells were large and
imaging (MRI) demonstrated a 3.3×1.9×2.0 cm mass with polygonal with small and rounded nuclei. The cytoplasm
new vessel generation. It was obstructing the entrance to of the tumor cells contained periodic acid-Schiff (PAS)-
positive diastase-resistant material that was partly rod-
* Correspondence: [email protected] shaped. This aspect has been observed in 80% of cases in
1ENT Department, (Street Konstantinoupoleos 49) Hippokratio General other studies, and is helpful in differentiating this tumor
Hospital, Thessaloniki, Greece
Full list of author information is available at the end of the article from a rhabdomyoma. Rhabdomyomas also present PAS-
© 2010 Noussios et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
BioMed Central Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
Noussios et al. Journal of Medical Case Reports 2010, 4:130 Page 2 of 4
http://www.jmedicalcasereports.com/content/4/1/130

Figure 1 Baccillary view of the mass (red arrow).

positive cells, but the intracytoplasmatic glycogen gran-

ules disappear after pre-treatment with diastase [2].
According to a series of cases reported, adjuvant ther-
apy may not be necessary if the small primary lingual
ASPS can be completely resected and the patient does Figure 3 Microscopic view showing nests of large granular cells
not experience clinical recurrence or metastasis [3]. separated by fibrovascular stroma (hematoxylin-eosin stain,
magnification ×100).
Therefore our patient was not treated with any post-
operative chemotherapy as the removal of the tumor was
considered to be an R0 ectomy (curative intent resection) Discussion
and MRI, CT and scintigraphy of bones showed no local Ôhe first case of ASPS was described by Christopherson
or distal metastasis. At three and a half years follow-up, et al. in 1952 [4,5]. Its clinical characteristics are slow
our patient remained disease-free and asymptomatic growth and a high risk of mortality. The tumor occurs
(Figure 4). most commonly in the soft tissues of the extremities

Figure 4 One year after a complete excision of the sarcoma (red

Figure 2 Transversal view of the tumor (red arrow). circle: post-operative area).
Noussios et al. Journal of Medical Case Reports 2010, 4:130 Page 3 of 4
http://www.jmedicalcasereports.com/content/4/1/130

(27%) and rarely arises in the oral cavity, especially on the The treatment of choice for ASPS of the tongue is gen-
tongue (25%). Although there are a few reports of tumors erally surgical excision with sufficient margins. Surgery
occurring in the oral cavity, only 12 cases are reported in with chemotherapy or radiotherapy is useful in some
the English literature of ASPS presenting on the tongue in patients. Treatment has been difficult to evaluate because
the first decade of life. Local recurrence of the tumor is of the small number of cases seen, but it seems that
rare, but metastasis to the lung, bones and brain may although the ectomy of the primary tumor is often suc-
occur. The tumor occurs predominantly in females, with cessful, treatment of metastatic tumors is problematic
a female:male ratio of 2:1. [11]. For surgical excision, Marker et al. [12] recommend
The pathogenesis of ASPS is not known and there is that a tumor-free zone of 1 to 1.5 cm should be main-
some controversy over this. One view is that it is of myo- tained around the tumor.
genic or neuroendocrine derivation. Although no theory Another approach to treatment is the use of IFN alpha-
has been specifically proven, the work of Mukai and col- 2b as a monotherapy. There is some evidence that high
leagues [6] provides substantial support for the theory doses of IFN may induce an impressive tumor response in
that it is of a myogenic origin. In some cases, a structural ASPS with pulmonary metastases that have no response
rearrangement of chromosome 17 involving band q25 has to chemotherapy. However, this needs further research
been reported [7]. [13]. The prognosis for ASPS is poor overall, with five-
The translocation (X; 17), producing an ASPL-TFE3 year and 20-year survival rates of only 59% and 15%,
transcript fusion which is detected on tumoral cell walls, respectively, being reported. Local recurrence and distant
is specific to ASPS [8]. Patients with ASPS of the tongue metastasis are primarily responsible for the poor progno-
experience dysphagia, dysphonia, or mild discomfort. sis of this neoplasm [14]. By contrast, distant metastases
Clinical examination often reveals pulsation with a thrill. from oral cavity carcinomas vary over a broad interval (8-
Although there is usually no overlying ulceration or 17%) and also depend on the stage of the disease [15]. Pri-
bleeding, our patient had significant hemorrhaging as the mary and metastatic tumors are generally treated by sur-
tumor extended through the overlying mucosa [9]. gery, chemo-hormone therapy and immunotherapy [16].
The typical histological characteristics of ASPS include The outcomes also depend on prognostic factors such as
a variable-size that is irregularly circumscribed, and divi- a patient's age, tumor size and the presence of metastasis
sion by thin fibrous connective tissue bands with delicate at the time of diagnosis. In contrast to ASPS in other
vessels delineating small pockets of variably-sized polyg- parts of the body, lingual ASPS have a rather good prog-
onal cells. The cells are of a uniform size and shape and nosis [7], particularly in young children.
contain granular, eosinophilic cytoplasm surrounding a
vesicular nucleus that hosts a prominent nucleolus. The Conclusion
pseudoalveolar pattern characteristic of this tumor is ASPS of the tongue is a rare tumor which occurs in
seen when the alveolar cells undergo degeneration, lose infants and children, usually in the head and neck region.
their cohesiveness and become necrotic. The orbital and lingual sites are predominantly affected.
ASPS has a close clinical and imaging resemblance to Treatment is primarily surgical, with limited roles for
common benign vascular tumors such as hemangiomas, adjuvant chemotherapy, radiotherapy and perhaps IFN.
which may lead to misdiagnosis and inadequate or CT and MRI can be used to make the correct diagnosis of
delayed treatment. Vascular malformations may present ASPS and to help the surgeon to perform a wide surgical
at birth, whereas ASPS does not, but if it did it grows rap- resection to reduce the risk of local recurrence. Addi-
idly and occurs in an older age group. In addition to tional study of the histological types of ASPS that occur
hemangiomas and vascular malformations, other less in children, specifically in the head and neck region, is
likely differential diagnoses include a hyperplastic lingual suggested to determine whether differences in patient
thyroid and dermoid cysts. These neoplasms are easily outcome and treatment exist between the subtypes.
differentiated from ASPS in CT and MRI findings. ASPS
can also be differentiated from other histologically simi- Consent
lar malignant neoplasms in its cytological uniformity, Written informed consent was obtained from the
lack of nuclear atypia, and paucity of mitotic figures. patient's next-of-kin for publication of this case report
Most ASPSs also contain PAS-positive, diastase-resistant and any accompanying images. A copy of the written con-
cytoplasmic inclusions that are thought to consist of sent is available for review by the Editor-in-Chief of this
actin. The differential diagnosis of ASPS includes granu- journal.
lar cell tumor, paraganglioma, metastatic renal cell carci-
Competing interests
noma, malignant melanoma, alveolar The authors declare that they have no competing interests.
rhabdomyosarcoma and ectopic lingual thyroid [10].
Noussios et al. Journal of Medical Case Reports 2010, 4:130 Page 4 of 4
http://www.jmedicalcasereports.com/content/4/1/130

Authors' contributions
GN analyzed and interpreted the patient data regarding the disease. PC per-
formed the data analysis and was a major contributor in writing the manu-
script. IP, KK and GK performed the operations. All authors read and approved
the final manuscript.

Author Details
1ENT Department, (Street Konstantinoupoleos 49) Hippokratio General

Hospital, Thessaloniki, Greece and 2Department of Physical Education (Serres)

of 'Aristotelian' University, (Agios Ioannis-Serres), Thessaloniki, Greece

Received: 21 October 2009 Accepted: 8 May 2010

Published: 8 May 2010
©
This
Journal
2010
is
article
an
Noussios
ofOpen
Medical
is available
Access
etCase
al; licensee
from:
article
Reports
http://www.jmedicalcasereports.com/content/4/1/130
distributed
BioMed
2010, 4:130
Central
underLtd.
the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

References
1. King VV, Fee WE Jr: Alveolar soft part sarcoma of the tongue. Am J
Otolaryngol 1983, 4(5):363-366.
2. de Fátima Correia-Silva J, Barroso Duarte EC, César Tanos Lacerda J,
Cantanhede Orsini Machado de Sousa S, Alves Mesquita R, Santiago
Gomez R: Alveolar soft part sarcoma of the tongue. Oral Oncol Extra
2006, 42(6):241-243.
3. Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers EL: Lingual
alveolar soft part sarcoma; 14 cases: novel clinical and morphological
observations. Histopathology 2004, 45(5):526-537.
4. Aulmann S, Longerich T, Schirmacher P, Mechtersheimer G, Penzel R:
Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded
alveolar soft part sarcomas. Histopathology 2007, 50(7):881-886.
5. Christopherson MW, Foote FW Jr, Stewart FW: Alveolar soft part
sarcomas: structurally characteristic tumors of uncertain histogenesis.
Cancer 1952, 5:100-111.
6. Mukai M, Torikata C, Iri H, Mikata A, Hanaoka H, Kato K: Histogenesis of
alveolara soft part sarcoma. An immunohistochemical and
biochemical study. Am J Surg Pathol 1986, 10:212-218.
7. Rodríguez A, Ferman F, Cerecedo F: Rare tumor of the tongue in a child:
alveolar soft part sarcoma. Case report. Pediatr Dev Pathol 2008, 16:1.
8. Marchac A, Picard A, Landman-Parker J, Larroquet M, Vazquez MP, Franchi
G: A pediatric case of alveolar soft part sarcoma. Rev Stomatol Chir
Maxillofac 2007, 108(6):547-550.
9. Kanhere HA, Pai PS, Neeli SI, Kantharia R, Saoji RR, D'cruz AK: Alveolar soft
part sarcoma of the head and neck. Int J Oral Maxillofac Surg 2005,
34(3):268-272.
10. Koch CA, Picken C, Clement SC, Aumi N, Sarlis NJ: Ectopic lingual thyroid:
an otolaryngologic emergency beyond childhood. Thyroid 2000,
10(6):511-514.
11. Anderson ME, Hornicek FJ, Gebhardt MC, Raskin KA, Mankin HJ: Alveolar
soft part sarcoma: a rare and enigmatic entity. Clin Orthop Relat Res
2005, 438:144-148.
12. Marker P, Jensen ML, Siemssen SJ: Alveolar soft-part sarcoma of the oral
cavity: report of a case and review of the literature. J Oral Maxillofac
Surg 1995, 53:1203-1208.
13. Roozendaal KJ, De Valk B, A ten Velden JJ, Woude HJ Van der, Kroon BBR:
Alveolar soft-part sarcoma responding to interferon alpha-2b. Br J
Cancer 2003, 89:243-245.
14. Ashley HA, Stone AJ: Alveolar soft-part sarcoma of the tongue. Am J
Neuroradiol 2003, 24:1156-1158.
15. Betka J: Distant metastases from lip and oral cavity cancer. ORL J
Otorhinolaryngol Relat Spec 2001, 63:217-221.
16. Misawa H, Yanagita N, Iwagaki T, Asahi Y, Yokoi H, Kato K, Qian B, Shamoto
M: Primary malignant melanoma arising from the base of the tongue.
ORL J Otorhinolaryngol Relat Spec 2000, 62:134-139.

doi: 10.1186/1752-1947-4-130
Cite this article as: Noussios et al., Alveolar soft part sarcoma of the tongue
in a 3-year-old boy: a case report Journal of Medical Case Reports 2010, 4:130