Int. J. Oral Maxillofac. Surg. 2000; 29: 370–372 Copyright C Munksgaard 2000Printed in Denmark . All rights reserved

ISSN 0901-5027

Kenji Yoshida 1, Jun Kurauchi 2,Haruyuki Shirasawa 3, Isao Kosugi 3Alveolar soft part sarcoma of1First Department of Oral & MaxillofacialSurgery, School of Dentistry, Aichi-GakuinUniversity, Nagoya; 2Department of Oralthe tongue Surgery, Gamagori Municipal Hospital,Gamagori, 3Department of Pathology,Hamamatsu University School of Medicine,Report of a case Hamamatsu, Japan

K. Yoshida, J. Kurauchi, H. Shirasawa, I. Kosugi: Alveolar soft part sarcoma ofthe tongue. Report of a case. Int. J. Oral Maxillofac. Surg. 2000; 29: 370–372.C Munksgaard, 2000

Abstract. The clinicopathologic, immunohistological, and ultrastructural featuresof an alveolar soft part sarcoma of the tongue occurring in a 2-year-old girl are

Key words: alveolar soft part sarcoma;described. A primary alveolar soft part sarcoma arising in the dorsum part of tongue.the tongue is quite rare. There has been no recurrence or metastasis as of 7 yearspostoperatively. Accepted for publication 31 May 2000

Alveolar soft part sarcoma (ASPS) is anextremely rare malignant tumor of thehead and neck. The tumor occurs pri-marily in the soft tissues of the extremi-ties and rarely arises in the oral cavity,especially the tongue. Most ASPS ofthe tongue are found within the base ofthe tongue5,6. We describe here a caseof ASPS occurring in the left part ofthe dorsum of the tongue.

Case report

A 2-year-old girl in good general health wasreferred in December 1992 because of a pain-less mass in the tongue. There was no pre-vious history of eating or speech disorders.Clinical examination showed that the tonguewas normal in color, but contained a 20¿20mm elastic, soft mass in the left side of thedorsum. There was no cervical lymph nodeenlargement. Since the lesion was thought tobe benign, an excisional biopsy was per-formed under general anesthesia. The tumordid not adhere to the surrounding tissues.The histopathological diagnosis was ASPS.To minimize the risk of residual tumor, wideexcision was performed. The surgical mar-gins were tumor-free. Postoperative chemo-therapy consisted of vincristine (total 4 mg),actinomycin D (total 3.6 mg), cyclophos-phamid (total 1.08 g), adriamycin (total 54mg), ifosfamid (total 4.0 g), and cisplatin

(total 120 mg). There has been no evidenceof recurrence or metastasis as of 7 years 2months postoperatively.

Histological and immunohistochemicalfindings

The resected specimen was fixed in 10% neu-tral phosphate-buffered formalin and em-bedded in paraffin. Sections were stainedwith hematoxylin-eosin (HE), periodic acid-Schiff (PAS), before and after diastase diges-tion.

Microscopically, the tumor consisted ofstructured, irregular, and rough clusters ofcells. Most vascular channels were enclosedby thin connective tissue septa. Tumor cellswere large and polygonal. Nuclei were smalland rather round (Fig. 1A). The cytoplasm

Table 1. Immunohistochemical findings

Antibody (clone number) Antigen Results

MyoD1 (5.8A) Human muscle MyoD1 protein ππDesmin (D33) Human muscle desmin πMyoglobin (polyclonal) Human muscle myoglobin ππa-SMA (1A4) Human smooth muscle actin πVimentin (V9) Swine vimentin πNSE (BBS/NC/VI-H14) Human neuron specific enolase ππFactor VIII (polyclonal) von Willebrand factor ªKeratin (polyclonal) Bovine keratin ªEMA (E29) Human epithelial membrane antigen ª

ππ diffusely positive, π focally positive, ª negative.All antibodies were purchased from DACO, Japan.

of the tumor cells contained PAS-positive di-astase-resistant material that was occasion-ally rod-shaped (Fig. 1B). Immunohisto-chemically, the tumor cells were diffuselypositive for MyoD1, Myoglobin and NSE.Desmin, a-SMA and Vimentin were focallypositive (Fig. 1C, D, Table. 1).

Electron microscopic findings

For electron microscopy, the paraffin-embed-ded block was post-fixed in 4% glutaralde-hyde. Tissues were post-fixed in osmium te-troxide, dehydrated in acetone, and embed-ded in epoxy resin.

Some tumor cells showed numerous crys-talloids with periodicity and membrane-bound intracytoplasmic granules (Fig. 2).

Alveolar soft part sarcoma of tongue 371

Fig. 1. A. The tumor shows structured, irregular, and rough clusters of cells. Most vascularchannels are enclosed by thin connective tissue septa (Hematoxylin and eosin stain). B. Thecytoplasm of the tumor cells contains diastase-resistant PAS-positive material that was oc-casionally rod-shaped (Periodic acid-Schiff stain). C. The tumor cells were focally positive forDesmin. D. The tumor cells were diffusely positive for MyoD1. In Figs. 1A–D, scale is 50 mm(shown in Fig. 1A).

Fig. 2. A biopsy specimen shows numerous crystalloids and membrane-bound granules. Ir-regularly shaped or rhomboidal membrane-bound granules can be observed.

Discussion

ASPS was first described in 1952 byC et al.2. Clinical charac-teristics include slow growth and a highrisk of mortality. Local recurrence israre, but metastasis to the lung, bone,and brain may occur. In the oral region,ASPS arises most frequently in thetongue. To our knowledge, only three

cases involving the dorsum of the tonguehave been documented previously3,7,8.Our case would be the fourth reportedcase to arise in the dorsum of the tongue.

C et al.1 reviewed cases ofASPS arising in the tongue and re-ported that most tumors occurred inchildren. Previously reported cases havearisen in patients aged between 4months and 74 years. Despite the wide

range in age, few reported cases have in-volved children 4 months to 3 years ofage4. Our patient was 2 years old.

ASPS is characterized by PAS-posi-tive diastase-resistant crystals in thecytoplasm of tumor cells. The diagnosisis confirmed by the presence of rod-shaped crystalline, membrane-boundstructures with a regular network of 60-Å periodicity on electron microscopicexamination.

Immunohistochemical analyses oftenlead to equivocal results, and there isno consensus on antigen reactivity.H4 reviewed the histogenesis ofASPS and the myogenic theory of originis the most popular at the present time.In our case, reactions were negative forFactor VIII, keratin and EMA. Tumorcells were diffusely positive for MyoD1,Myoglobin, and NSE. Desmin, a-SMAand Vimentin were focally positive.

ASPS rarely arises in the oral cavity,and previously reported cases have beenfollowed for only short periods. Thetreatment of choice is generally surgicalexcision with sufficient margins. Surgerywith chemotherapy or radiotherapy isuseful in some patients. For surgical ex-cision of ASPS, M et al.6 rec-ommended that a tumor-free zone of 1 to1.5 cm be maintained around the tumor.We followed this recommendation andhistopathologically confirmed that theresection margins were negative for tu-mor cells. To prevent metastasis, chemo-therapy was given postoperatively. Therehas been no evidence of local recurrenceor metastasis as of 7 years 2 months afteroperation. We attribute this good out-come to surgical excision with adequatetumor-free margins and to a good re-sponse to postoperative chemotherapy.We believe that our patient is the longestsurvivor among all patients with ASPSof the tongue described in the literature.At the time of this writing, there is noevidence of tumor recurrence or meta-stasis.

Acknowledgment. The authors thank Dr.Gernot Jundt (Institute of Pathology, Uni-versity of Basel) for his encouragement topublish the case.

References

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372 Yoshida et al.

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5. K A, T Y, K T. Al-veolar soft-part sarcoma of the tongue.Report of a case with electron micro-scopic study. Oral Surg Oral Med OralPathol 1984: 57: 532–9.

6. M P, J ML, S SJ. Al-veolar soft-part sarcoma of the oral cav-ity. Report of a case and review of theliterature. J Oral Maxillofac Surg 1995:53: 1203–8.

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Address:Kenji YoshidaFirst Department of Oral & Maxillofacial

SurgerySchool of DentistryAichi-Gakuin University2-11 Suemori-dori, Chikusa-kuNagoya 464-8651JapanTel: π81 52 751 7181Fax: π81 52 752 5990