Lingual alveolar soft part sarcoma, ASPS

Definition

Alveolar soft part sarcoma most commonly occurs in the thigh/buttock of young adults. Lingual ASPS, the equivalent tumour occurring within the tongue is rare.

Epidemiology

The literature contains only about 40 cases. Lingual ASPS represents perhaps 5% of all ASPS.. This is a tumour of childhood (as opposed to young adults for the generality of ASPS), with a median age of 5-8 years¹.

Clinical features

Patients present with a mass, pain, dysphasia or dysphagia. The bluish colour of the lesion often gives rise to a clinical diagnosis of haemangioma.

Macroscopic appearances

Tumours are solitary but multinodular,with fibrous septa separating the nodules.

Histopathology

In most cases, the predominant pattern is solid rather than alveolar. Vascular invasion is common and there may be perineural growth. Tumour cells are large and polygonal. The cytoplasm is generally eosinophilic, focally clear in some cases. In most cases, the cytoplasm contain rhomboid PAS-positive crystals. Nuclei are uniform (rarely pleomorphic) and vesicular with prominent nucleoli. The nuclei show a characteristic "apple core" concave indentation. The mitotic rate is from 0 to 3 / 10HPF.

Immunohistochemistry

	Desmin	5/10 (at least focal positivity)1, 1/13, 1/1 (focal)4, 0/15, 1/16, 1/17
	SMA	10/10 (at least focal positivity in all cases)1, 1/13, 0/14, 0/15, 1/17
	Myo-D1	10/10, (focal: in all cases, positivity was only focal and weak and was scored "negative")1, 1/13
	Myogenin	0/101
	Myoglobin	0/101, 1/13, 0/15, 0/16
	Caldesmon	0/101
	Muscle-specific actin	0/15
	S-100	0/101, 1/1 (focal)4, 0/15, 0/16
	HMB-45	0/101
	Lysozyme	0/101, 0/15
	Synaptophysin	0/101, 0/15
	Chromogranin	0/101, 0/15
	NSE	1/13
	b-enolase	1/16
	CD 34	0/101
	CD 68	0/101, 0/15
	Cytokeratin	0/101, 0/15, 0/16, 0/18
	Vimentin	0/101, 1/13, 1/14, 0/16, 0/17, 1/18
	CEA	0/18
	EMA	0/18
	Factor VIIIRA	0/18

Differential diagnosis

• Granular cell tumour/myoblastoma

• paraganglioma

• salivary gland tumours, various

• rhabdomyoma

• rhabdomyosarcoma

• leiomyoma

• melanoma

• hibernoma

• metastatic ASPS from a distant site²

Management

Surgical excision.

Prognosis

The rate of metastasis and the prognosis appear to be better than for the generality of ASPS.

References

¹ Fanburg-Smith, J. C., M. Miettinen, et al. (2004). "Lingual alveolar soft part sarcoma; 14 cases: novel clinical and morphological observations." Histopathology 45(5): 526-37.

² Porter, K. M., S. R. Porter, et al. (1988). "Lingual metastasis of alveolar soft-part sarcoma." Oral Surg Oral Med Oral Pathol 65(6): 742-4.

³ Yoshida, K., J. Kurauchi, et al. (2000). "Alveolar soft part sarcoma of the tongue. Report of a case." Int J Oral Maxillofac Surg 29(5): 370-2.

⁴ Bentley, R. P., M. J. Wake, et al. (1999). "Alveolar soft part sarcoma of the tongue." Br J Oral Maxillofac Surg 37(6): 451-4.

⁵ Castle, J. T. and R. K. Goode (1999). "Alveolar soft part sarcoma of the tongue: report of an unusual pattern in a child." Ann Diagn Pathol 3(5): 315-7.

⁶ Matsuno, Y., K. Mukai, et al. (1990). "Alveolar soft part sarcoma. A clinicopathologic and immunohistochemical study of 12 cases." Acta Pathol Jpn 40(3): 199-205.

⁷ Takita, M. A., M. Morishita, et al. (1990). "Alveolar soft-part sarcoma of the tongue. Report of a case." Int J Oral Maxillofac Surg 19(2): 110-2.

⁸ Donald, P. J. (1987). "Alveolar soft part sarcoma of the tongue." Head Neck Surg 9(3): 172-8.

This page last revised 12.1.2005.

©SMUHT/PW Bishop