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Year : 2010  |  Volume : 47  |  Issue : 2  |  Page : 234-235

Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy

1 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, U.P, India
2 Department of Pediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, U.P, India

Date of Web Publication5-May-2010

Correspondence Address:
M Kumar
Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, U.P
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.63023

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How to cite this article:
Kumar M, Patne S, Vishwanath A, Hasan Z. Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy. Indian J Cancer 2010;47:234-5

How to cite this URL:
Kumar M, Patne S, Vishwanath A, Hasan Z. Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy. Indian J Cancer [serial online] 2010 [cited 2019 Aug 18];47:234-5. Available from:


Alveolar soft part sarcoma (ASPS) is a rare malignant tumor of uncertain histogenesis, primarily affecting the lower extremities of adolescents and adults aged between 15 and 35 years. [1] Lingual ASPS is an extremely rare neoplasm that comprises about 5% of all the ASPS. [2] We herein report a 7-year-old boy who presented with a slow-growing painless swelling of the tongue for 2.5 years and difficulty in deglutition for the past 6 months. Intraoral examination showed a firm nontender nodule of 2 cm diameter on the right dorsolateral aspect of the tongue. Detailed clinical examination and preoperative work-up was within the normal limits. Past medical and family histories of the patient were unremarkable. With a clinical impression of neurofibroma, the swelling was excised under general anesthesia. In order to salvage the tongue, the entire tumor could not be removed [Figure 1]. Grossly, the excised tumor measured 2.5 cm in diameter. The cut surface was grayish white and lobulated. Hematoxylin and eosin-stained sections showed a nested pattern of the tumor cells arranged in the lobules separated by fibrovascular septa [Figure 2]A. The tumor cells were arranged in an alveolar pattern. The individual cells were uniform oval to polygonal with ample amounts of granular eosinophilic cytoplasm [Figure 2]B and C. The nuclei were eccentrically placed with minimal pleomorphism and 1-2 prominent nucleoli. No necrosis, hemorrhage, appreciable mitotic figures and vascular or perineural invasion were noted. The alveolar pattern was reinforced on reticulin staining [Figure 2]D. The tumor cells showed intracytoplasmic periodic acid Schiff (PAS)-positive granular material. Ki-67 (MIB-1) immunostaining was negative in the tumor cells. The histopathological and histochemical findings were consistent with primary ASPS of the tongue. In view of partial resection of the tumor, the patient received four cycles of postoperative chemotherapy. Each cycle comprised of ifosfamide (1.8 g/m2/day) for 5 days and doxorubicin (30 mg/m2/day) for 2 days. He responded well to the chemotherapy, resulting in complete regression of the residual lesion. No evidence of local recurrence or distant metastasis was present in an 11-month postoperative follow-up. Subsequently, the patient was lost to follow-up and did not return for further examination.

Lingual ASPS is a very rare tumor as only about 30 cases have been described in the English literature. A majority of these tumors are seen in the first two decades of life, with a clear female preponderance. [2],[3] In contrast to the other pediatric sarcomatous lesions, lingual ASPS is essentially a slow-growing painless lesion with an indolent behavior, closely mimicking a benign neoplasm. In comparison to ASPS of the other anatomical locations, lingual ASPS arises at a much younger age (median age, 5 years) and enjoys a relatively favorable outcome. [2] Lingual ASPS metastasize or recur in about 30% of the cases. [3] Complete excision is the primary therapy for both oral and extraoral ASPS. [4],[5] Adjuvant therapeutic alternatives such as radiation and chemotherapy may prevent recurrence or metastasis if there is a high risk of residual disease after the surgical resection. [4] This was the problem in our patient, where complete excision of the tumor was not possible in order to salvage the growing tongue. The patient responded successfully to the chemotherapy regimen, with complete regression of the residual tongue lesion.

  References Top

1.Enzinger FM, Weiss SW. Soft tissue tumors. St Louis: CV Mosby; 1995.  Back to cited text no. 1      
2.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;45:526-37.  Back to cited text no. 2  [PUBMED]  [FULLTEXT] Nascimento Souza KC, Faria PR, Costa IM, Duriguetto AF Jr, Loyola AM. Oral alveolar soft-part sarcoma: Review of literature and case report with immunohistochemistry study for prognostic markers. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:64-70.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Ryu J, Kwon Y, Park BK, Jung YS. Lingual alveolar soft part sarcoma treated only by conservative resection. Int J Pediatr Otorhinolaryngol Extra 2006;1:243-8.  Back to cited text no. 4      
5.Kayton ML, Meyers P, Wexler LH, Gerald WL, LaQuaglia MP. Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg 2006;41:187-93.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  


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